Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>MPV17-related mitochondrial DNA depletion syndrome has a poor prognosis; nonetheless, depending on the mutated gene, there have been some cases of survival after liver transplantation with few neurological complications. However, there have been reports of a poor prognosis after liver transplantation in patients with neurological disorders prior to the procedure. Therefore, the indications for liver transplantation remain controversial. The patient was a 14-year-old girl. She was diagnosed as having MPV17-related mitochondrial DNA depletion syndrome at the age of 3 years on the basis of the liver biopsy finding of decreased mitochondrial DNA levels and the skin biopsy findings of c.293C>T and c376-1G>A gene mutations. Living donor liver transplantation from her father was performed for decompensated cirrhosis at 14 years of age. Hepatocellular carcinoma was incidentally detected in the explanted liver. Two years after liver transplantation, there was no evidence of rejection, liver dysfunction, or recurrence of hepatocellular carcinoma. However, her neurological symptoms gradually worsened. Careful consideration of the indications for liver transplantation in each case and further investigation of life and neurological prognoses after liver transplantation are needed.</p>

DOI： 10.11164/jjsps.60.6_949

CiNii Research

Language：English   Publisher：World Journal of Gastrointestinal Oncology  

BACKGROUND Pediatric pancreatic tumors are rare and account for < 0.1% of all childhood cancers. The primary treatment for pancreatic tumors is surgical resection. However, because of the lack of knowledge regarding pediatric pancreatic tumors, no comprehensive treatment plans for pediatric pancreatic tumors have been developed. AIM To compared the clinical features, treatment methods, and prognosis of pediatric pancreatic tumors in Japan with those in other countries. METHODS Questionnaires were sent to 213 pediatric surgical units in Japan. Pancreatic tumors that were not surgically treated were excluded from the survey. The primary survey investigated the number of patients aged 0–18 years who underwent pancreatic tumor surgery and the type of tumors managed during the 22-year study period (from January 1, 2000 to December 31, 2021) by post card. The secondary survey assessed the clinical images, treatment methods, and tumor outcomes via email. RESULTS The primary survey enrolled 228 patients. In the secondary survey, 213 patients were eventually enrolled. The most common type of pancreatic tumor was solid pseudopapillary neoplasm (SPN) [n = 164 (77.0%)], followed by pancreatoblastoma [n = 16 (7.5%)], pancreatic endocrine tumor [n = 14 (6.6%)], non-epithelial tumor [n = 9 (4.2%)], pancreatic tumor [n = 7 (3.3%)], and metastatic pancreatic tumor [n = 3 (1.4%)]. Overall, 123 (57.7%) patients underwent distal pancreatectomy, of whom 49 underwent laparoscopic surgery. Forty-four (20.7%) patients underwent enucleation, of whom eight underwent laparoscopic surgery. Thirty-two (15.0%) patients underwent pancreaticoduodenectomy, of whom one underwent laparoscopic surgery. All patients with SPN, including those with distant metastases and recurrent disease, survived. CONCLUSION SPN was more common in Japan than in other countries. Regardless of the histological type, resection is the most effective treatment for pediatric pancreatic tumors.

DOI： 10.4251/wjgo.v16.i10.4166

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Language：English   Publisher：Surgery Today  

The purpose of our narrative review is to summarize the utilization of social media (SoMe) platforms for research communication within the field of surgery. We searched the PubMed database for articles in the last decade that discuss the utilization of SoMe in surgery and then categorized the diverse purposes of SoMe. SoMe proved to be a powerful tool for disseminating articles. Employing strategic methods like visual abstracts enhances article citation rates, the impact factor, h-index, and Altmetric score (an emerging alternative metric that comprehensively and instantly quantifies the social impact of scientific papers). SoMe also proved valuable for surgical education, with online videos shared widely for surgical training. However, it is essential to acknowledge the associated risk of inconsistency in quality. Moreover, SoMe facilitates discussion on specific topics through hashtags or closed groups and is instrumental in recruiting surgeons, with over half of general surgery residency programs in the US efficiently leveraging these platforms to attract the attention of potential candidates. Thus, there is a wealth of evidence supporting the effective use of SoMe for surgeons. In the contemporary era where SoMe is widely utilized, surgeons should be well-versed in this evidence.

DOI： 10.1007/s00595-024-02891-1

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Language：English   Publisher：Journal of Gastroenterology and Hepatology (Australia)  

Background and Aim: Even with advancement of medical technologies, liver transplantation still faces several major challenges. Hence, other treatment modalities are urgently needed for patients with end-stage liver disease. Stem cells from human exfoliated deciduous teeth (SHED) was discovered to have highly proliferative and pluripotent properties; including differentiation into hepatocyte-like cells. This study aims to investigate the capability of intrasplenic transplanted SHED and SHED-Hep cells in inducing proliferation of stem cells and native hepatocytes in order to accelerate liver regeneration in liver fibrosis mice models. Methods: Three carbon tetrachloride (CCl4)-injured male mice groups were used in this study. Two of those groups were transplanted with either SHED or SHED-Hep, while the other did not undergo transplantation. One age- and sex- matched healthy mice group was used as control. All specimens were immunohistochemically stained with anti-Ki-67 antibodies and anti-proliferating cell nuclear antigen (PCNA) antibodies before counter stained with hematoxylin–eosin. Results: Anti-Ki-67 antibodies staining: at both 8 and 12 weeks, proliferating activity was predominantly seen on both SHED- and SHED-Hep-transplanted CCl4-injured mice groups, while control and non-transplanted CCl4-injured mice group showed little to no sign of proliferation activity. Anti-PCNA staining: at both 8 and 12 weeks, significant proliferating activity was detected by PCNA staining, mainly on stem cells population area on SHED- and SHED-Hep-treated group. Conclusions: In conclusion, this study has provided the evidence that transplantation of SHED or SHED-Hep on liver-injured mice induced proliferation of both transplanted stem cells and native liver cells in order to accelerate liver regeneration.

DOI： 10.1111/jgh.16651

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Publisher：Journal of Applied Physics  

The B20 type chiral magnet FeGe exhibits the formation of skyrmion-lattice (SkL) phases in the vicinity of the magnetic ordering temperature. The SkL is a magnetic superlattice composed of vortex-type topological spin objects, and it has experimentally been known that its formation requires the existence of an intermediate (IM) phase between SkL and the paramagnetic (PM) phases. We take interest in how the crystal lattice experiences the formation of these topological spin texture. In this study, we observed the so-called spin-orbit coupling induced magnetostriction related to these topological spin texture formation, in addition to the ac magnetization anomalies. The temperature and magnetic field dependences of the lattice parameter reflected the transformation of phases, such as helimagnetic (HM), SkL, IM, conical (CM), and PM phases. In the PM region, a phase characterized as gaseous skyrmions was detected similarly to the case of the same B20 type MnSi. Furthermore, the HM, CM, and IM phases were also divided into two regions. Thus, the precise phase diagram near T c was reconstructed from the prospect of the magnetostriction such that we demonstrated that the stabilization of skyrmions needs a finite magnetic field.

DOI： 10.1063/5.0227382

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000948

CiNii Research

Publisher：診断と治療社  

DOI： 10.34433/pp.0000001202

CiNii Research

Language：English   Publisher：Pediatric Surgery International  

Purpose: In this study, we attempted to create skeletal muscle sheets made of directly converted myoblasts (dMBs) with a nanogel scaffold on a biosheet using a mouse gastroschisis model. Methods: dMBs were prepared by the co-transfection of MYOD1 and MYCL into human fibroblasts. Silicon tubes were implanted under the skin of NOG/SCID mice, and biosheets were formed. The nanogel was a nanoscale hydrogel based on cholesterol-modified pullulan, and a NanoClip-FD gel was prepared by freeze-drying the nanogel. 7 mm in length was created in the abdominal wall of NOG/SCID mice as a mouse gastroschisis model. Matrigel or NanoCliP-FD gel seeded with dMBs was placed on the biosheet and implanted on the model mice. Results: Fourteen days after surgery, dMBs with Matrigel showed a small amount of coarse aggregations of muscle-like cells. In contrast, dMBs with NanoCliP-FD gel showed multinucleated muscle-like cells, which were expressed as desmin and myogenin by fluorescent immunostaining. Conclusion: Nanogels have a porous structure and are useful as scaffolds for tissue regeneration by supplying oxygen and nutrients supply to the cells. Combining dMBs and nanogels on the biosheets resulted in the differentiation and engraftment of skeletal muscle, suggesting the possibility of developing skeletal muscle sheets derived from autologous cells and tissues.

DOI： 10.1007/s00383-024-05811-z

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Language：English   Publisher：Pediatric Surgery International  

Purpose: The study aimed to explore and describe the lives of patients with persistent cloaca (PC) from childhood to adulthood. Methods: Semistructured interviews were conducted with nine adult patients with PC. Their experiences and thoughts regarding this disease were analyzed qualitatively and inductively. Results: After classifying the experiences and thoughts of patients with PC, 13 categories were extracted. The following five themes emerged from these categories. (1) Difficulties with excretion and vaginal management because of the disease. (2) The degree of understanding of those around them and society has a huge effect on their way of life. (3) The inferiority of a woman who is not a “normal woman.” (4) A “never-ending disease” in which problems continue even after the transition period. (5) Differences in the central point of the narrative depending on the age group. Conclusions: In this study, qualitative and inductive analyses of data from semistructured interviews with patients with PC revealed their experiences and thoughts. The results will provide a guide for young patients and the medical professionals who treat them. Accordingly, monitoring their lives until adulthood is necessary.

DOI： 10.1007/s00383-024-05807-9

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Language：English   Publisher：International Journal of Computer Assisted Radiology and Surgery  

Purpose: The development of innovative solutions, such as simulator training and artificial intelligence (AI)-powered tutoring systems, has significantly changed surgical trainees’ environments to receive the intraoperative instruction necessary for skill acquisition. In this study, we developed a new objective assessment system using AI for forceps manipulation in a surgical training simulator. Methods: Laparoscopic exercises were recorded using an iPad®, which provided top and side views. Top-view movies were used for AI learning of forceps trajectory. Side-view movies were used as supplementary information to assess the situation. We used an AI-based posture estimation method, DeepLabCut (DLC), to recognize and positionally measure the forceps in the operating field. Tracking accuracy was quantitatively evaluated by calculating the pixel differences between the annotation points and the points predicted by the AI model. Tracking stability at specified key points was verified to assess the AI model. Results: We selected a random sample to evaluate tracking accuracy quantitatively. This sample comprised 5% of the frames not used for AI training from the complete set of video frames. We compared the AI detection positions and correct positions and found an average pixel discrepancy of 9.2. The qualitative evaluation of the tracking stability was good at the forceps hinge; however, forceps tip tracking was unstable during rotation. Conclusion: The AI-based forceps tracking system can visualize and evaluate laparoscopic surgical skills. Improvements in the proposed system and AI self-learning are expected to enable it to distinguish the techniques of expert and novice surgeons accurately. This system is a useful tool for surgeon training and assessment.

DOI： 10.1007/s11548-024-03253-5

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Language：English   Publisher：Pediatric Surgery International  

Background/purpose: Living donor liver transplantation (LDLT) is vital for pediatric end-stage liver disease due to organ shortages. The graft-to-recipient weight ratio (GRWR) preoperatively measured predicts the outcomes of LDLT. We typically target between 0.8 and 3.0–4.0%, but the ideal GRWR remains controversial. We compared the outcomes of LDLT according to the GRWR to examine whether the criteria could be expanded while ensuring safety. Methods: We retrospectively reviewed 99 patients who underwent LDLT in our department by dividing them into three groups according to their GRWR: Group S, with GRWR values lower than the normal range (GRWR < 0.8%); Group M, with GRWR values in the normal range (GRWR ≥ 0.8 to < 3.5%); and Group L, with GRWR values above the normal range (GRWR ≥ 3.5%). Results: In Groups S and L, 46.2 and 44.4% of patients underwent splenectomy and delayed abdominal wall closure, respectively. After these intraoperative adjustments, there were no significant differences between the groups in 5-year patient survival, 5-year graft survival, or the occurrence of post-transplantation thrombosis. Conclusion: When the GRWR is beyond the normal threshold, the risk of complications associated with graft size might be reduced by adjustments to provide appropriate portal blood flow and by delayed abdominal wall closure.

DOI： 10.1007/s00383-024-05814-w

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Language：English   Publisher：Pediatric Surgery International  

Purpose: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is defined as a congenital visceral myopathy with genetic mutations. However, the etiology and pathophysiology are not fully understood. We aimed to generate a gene leiomodin-1a (lmod1a) modification technique to establish a zebrafish model of MMIHS. Methods: We targeted lmod1a in zebrafish using CRISPR/Cas9. After confirming the genotype, we measured the expression levels of the target gene and protein associated with MMIHS. A gut transit assay and spatiotemporal mapping were conducted to analyze the intestinal function. Results: Genetic confirmation showed a 5-base-pair deletion in exon 1 of lmod1a, which caused a premature stop codon. We observed significant mRNA downregulation of lmod1a, myh11, myod1, and acta2 and the protein expression of Lmod1 and Acta2 in the mutant group. A functional analysis of the lmod1a mutant zebrafish showed that its intestinal peristalsis was fewer, slower, and shorter in comparison to the wild type. Conclusion: This study showed that targeted deletion of lmod1a in zebrafish resulted in depletion of MMIHS-related genes and proteins, resulting in intestinal hypoperistalsis. This model may have the potential to be utilized in future therapeutic approaches, such as drug discovery screening and gene repair therapy for MMIHS.

DOI： 10.1007/s00383-024-05809-7

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Language：English   Publisher：Pediatric Surgery International  

Purpose: We aimed to identify factors predicting the need for future liver transplantation (LT) at 18 years of age in patients with biliary atresia (BA). Methods: BA patients with native liver survival at > 18 years of age were retrospectively reviewed. The clinical characteristics, outcomes, hepatobiliary function, and liver fibrosis markers of native liver survivors (NLS group) were compared with patients who subsequently underwent LT (LT group). Results: The study population included 48 patients (NLS, n = 34; LT, n = 14). The male-to-female ratio, age at Kasai procedure, and type of BA in the two groups did not differ to a statistically significant extent. There was no significant difference in the MELD scores between the groups at 18 years of age. The aspartate aminotransferase-to-platelet ratio index (APRI), albumin-bilirubin (ALBI), and BA liver fibrosis (BALF) scores at 18 years of age were significantly higher in the LT group. The AUCs for APRI, ALBI, and BALF were 0.91, 0.79, and 0.85, respectively. Conclusion: Adult BA patients have limited options for LT owing to the lack of donor candidates and the low prevalence of deceased donors. The elucidation of prognostic factors for LT in adulthood is important. APRI was the most useful marker in this study.

DOI： 10.1007/s00383-024-05805-x

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Language：English   Publisher：Laboratory Investigation  

Epithelioid sarcoma (ES) is a rare aggressive sarcoma that, unlike most soft-tissue sarcomas, shows a tendency toward local recurrence and lymph node metastasis. Novel antitumor agents are needed for ES patients. Forkhead box transcription factor 1 (FOXM1) is a member of the Forkhead transcription factor family and is associated with multiple oncogenic functions; FOXM1 is known to be overexpressed and correlated with pathogenesis in various malignancies. In this study, we immunohistochemically analyzed FOXM1 expression levels and their clinical, clinicopathologic, and prognostic significance in 38 ES specimens. In addition, to investigate potential correlations between FOXM1 downregulation and oncologic characteristics, we treated ES cell lines with thiostrepton, a naturally occurring antibiotic that inhibits both small interfering RNA (siRNA) and FOXM1. In the analyses using ES samples, all 38 specimens were diagnosed as positive for FOXM1 by immunohistochemistry. We separated specimens into high (n = 19) and low (n = 19) FOXM1–protein expression groups by staining index score, and into large (n = 12), small (n = 25), and unknown (n = 1) tumor-size groups using a cutoff of 5 cm maximum diameter. Although there were significantly more samples with high FOXM1 expression in the large tumor group (P = .013), there were no significant differences with respect to age (P = 1.00), gender (P = .51), primary site of origin (P = .74), histologic subtypes (P = 1.00), depth (P = .74), or survival rate (P = .288) between the high and low FOXM1–protein expression groups. In the in vitro experiments using ES cell lines, FOXM1 siRNA and thiostrepton successfully downregulated FOXM1 mRNA and protein expression. Furthermore, downregulation of FOXM1 inhibited cell proliferation, drug resistance against chemotherapeutic agents, migration, and invasion and caused cell cycle arrest in the ES cell lines. Finally, cDNA microarray analysis data showed that FOXM1 regulated cIAP2, which is one of the apoptosis inhibitors activated by the TNFα-mediated NF-κB pathway. In conclusion, the FOXM1 gene may be a promising therapeutic target for ES.

DOI： 10.1016/j.labinv.2024.102093

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000873

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Language：English   Publisher：Pediatric Surgery International  

Background: We previously showed that total tumor resection enhances metastatic growth in a syngeneic metastatic mouse model of neuroblastoma. In this study, we further investigated which surgical factors contributed most to metastatic growth. Methods: Tumor cells derived from MYCN transgenic mice were subcutaneously injected into wild-type mice. Mice were randomly assigned to receive partial resection (PR group), subcutaneous implantation of a sponge (Sp group), or observation (Obs group). The lymph node metastasis volume and the frequency of lung metastasis were compared 14 days after assignment by measuring C-reactive protein (CRP) and interleukin-6 (IL-6) levels. Results: The lymph node metastasis volume in the Sp group was larger than in the Obs group (148.4 [standard deviation {SD}: 209.5] vs. 10.2 [SD 12.8] mm3). The frequency of lung metastasis was greater in the Sp group than in the PR group (11.9 [SD 12.2] vs. 6.6 [SD 4.0] counts/slide). The CRP level in the Sp group was higher than in the PR group (2.3 [SD 0.5] vs. 1.5 [SD 0.4] μg/mL), and the IL-6 level in the Sp group was higher than in the PR or Obs groups (28.4 [SD 34.5] vs. 12.4 [SD 19.0] vs. 5.4 [SD 8.1] pg/mL). Conclusion: Metastatic growth may be enhanced by systemic inflammation.

DOI： 10.1007/s00383-024-05788-9

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Language：English   Publisher：American Journal of Surgical Pathology  

Isolated hypoganglionosis (IHG) is histologically characterized by small numbers of myenteric ganglion cells and small myenteric ganglia; however, no numerical diagnostic criteria for IHG have been established. Therefore, this study aimed to develop quantitative pathologic criteria for IHG. We evaluated 160 resected intestinal tissue specimens from 29 pediatric autopsies and 10 IHG cases. These specimens were obtained from the jejunum, ileum, ascending colon, transverse colon, and rectum. Morphologic features of the myenteric ganglion cells and myenteric ganglia were quantified and analyzed in digitized HuC/HuD-immunostained and CD56-immunostained sections, respectively. Quantitative criteria were developed with a scoring system that used parameters with the area under the receiver operating characteristic curve (AUC) values > 0.7 and sensitivity and specificity exceeding 70%. The selected parameters were the number of myenteric ganglion cells per cm and the number of myenteric ganglia with an area > 2500 µm2per cm. The score for each parameter ranged from −1 to 2, and the total score of the scoring system ranged from −2 to 4. With a cutoff value of ≥ 2 (AUC, 0.98; 95% CI: 0.96-1.00), the scoring system had a sensitivity of 96% (95% CI: 0.82-1.00) and a specificity of 99% (95% CI: 0.95-1.00). We devised a novel pathologic criterion based on the quantification of the number of myenteric ganglion cells and ganglia. Furthermore, this criterion showed high diagnostic accuracy and could lead to a definitive diagnosis of IHG in clinical practice.

DOI： 10.1097/PAS.0000000000002243

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000855

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Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>A female neonate presenting with vesicoumbilical fistula with bladder prolapse was delivered by cesarean section at 32 weeks and 5 days of gestation owing to threatened preterm labor. Prenatal ultrasonography at 12 weeks and 4 days of gestation revealed a gourd-shaped umbilical cord cyst, but the cyst disappeared at 24 weeks and 2 days of gestation. Her ruptured bladder was prolapsed through a defect at the lower basement of the umbilical cord, which required emergent surgery. During the surgery, a vesical capacity of 8 ml/kg was preserved when the resection line of the bladder was determined, because excessive bladder resection may cause urological complications. Histopathological analysis revealed no urachus tissue, but bladder tissue was involved in the resected specimen. The voiding cystourethrogram at POD25 revealed no urinary complications, vesicourethral reflux, or lower urinary tract obstruction, and she was discharged at POD36. With the progress in fetal ultrasonography, umbilical cysts have been reported to be diagnosed as the urachal remnant or urachal cyst after birth. When the fetal umbilical cyst disappears during the prenatal period, a rupture of the umbilical cyst is suspected. At radical surgery, it is important to measure the vesical capacity to avoid urological complications after surgery.</p>

DOI： 10.11164/jjsps.60.4_719

CiNii Research

Language：English   Publisher：Pathology Research and Practice  

Hepatoblastoma (HB) is the most common malignant liver tumor in childhood. Although pre-operative cisplatin (CDDP)-based chemotherapy is often used in cases of HB, about 20％ of HB patients exhibit resistance to CDDP. Forkhead box protein M1 (FOXM1) and chromo-domain-helicase-DNA-binding protein 4 (CHD4) have been associated with CDDP resistance in various tumors. We here analyzed the immunohistochemical expression of FOXM1 and CHD4 in HB specimens of 33 patients (mean age: 20 months) post-chemotherapy. The differentiation of specimens was assessed using the digital pathology software QuPath®, and then the relation between the FOXM1 or CHD4 expression and the differentiation and various other clinicopathological parameters was investigated. The histological type was epithelial in 19 cases (57.6%) and mixed epithelial and mesenchymal in 14 cases (42.4%). Nine cases had only a fetal component, 1 case had only an embryonal component, 22 cases had both fetal and embryonal components, and 1 case had no viable tumor. Both the FOXM1 and CHD4 immunoexpressions were found significantly more frequently in the embryonal than fetal components (p<0.0001 and p<0.0001, respectively). Regarding chemotherapy efficacy, the alpha-fetoprotein (AFP) level after chemotherapy was correlated with both the imaging shrinkage rate (R=-0.52) and histological residual rate (the percentage of the viable tumors of HB after chemotherapy)(R=0.62). High FOXM1 score was correlated with a high-postoperative AFP value (p<0.01) and a low AFP attenuation rate (p<0.05), but the FOXM1 score was not correlated with the imaging shrinkage rate (p=0.4418) or histological residual rate (p=0.4418). High CHD4 score showed a nonsignificant trend toward correlation with high postoperative AFP value (p=0.0849) and was not significantly correlated with the other parameters. Collectively, our results showed that FOXM1 expression may be useful in evaluating the response to CDDP-based chemotherapeutic regimens. Accurate measurement of FOXM1 expression by our scoring system using QuPath® is important in cases with mixed HB components of various differentiation levels.

DOI： 10.1016/j.prp.2024.155348

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Language：Japanese   Publisher：(一社)日本小児外科学会  

症例は日齢0の女児.在胎12週4日に胎児超音波検査でひょうたん型臍帯嚢胞を指摘されたが,在胎24週2日には臍帯嚢胞は消失していた.在胎32週5日に母体の切迫早産のため緊急帝王切開で出生した.臍帯基部尾側が裂け,膀胱粘膜が外反して露出していた.膀胱臍瘻の診断で緊急手術を施行した.膀胱切離ラインの決定の際に,術中膀胱容量測定を用いることで過度な膀胱壁の切除を回避した.病理検査では摘出標本内に明らかな尿膜管組織は含まれなかった.術後25日目の排尿時膀胱尿道造影で,膀胱尿管逆流や下部尿路狭窄のないことを確認し,術後36日目に退院した.近年の出生前診断の進歩に伴い,胎児期から臍帯嚢胞を指摘され,出生後に尿膜管遺残,膀胱臍瘻と診断される症例報告が散見される.胎児期に指摘された臍帯嚢胞が消失した場合,膀胱臍瘻の可能性があり,出生直後の手術介入の際には術後の泌尿器合併症を回避するための術式の工夫が必要である.(著者抄録)

Publisher：東京医学社  

DOI： 10.24479/ps.0000000821

CiNii Research

Language：English   Publisher：Frontiers in Pediatrics  

Background: Gastroesophageal reflux (GER) disease (GERD) is a condition wherein GER causes troublesome symptoms that can affect daily functioning and/or clinical complications within the esophagus or other systems. To avoid this, patients with GERD often require treatment; hence, it is important to distinguish GER from GERD. Patients with GERD exhibiting alarm signs should be examined early to differentiate it from GER and treated accordingly. Herein, we present a case of GERD caused by a hiatal hernia that required surgical intervention for esophagial cicatrical stenosis despite oral treatment. We also discussed how to choose the appropriate acid suppressants for GERD. Case presentation: A 1-year-old boy was referred to our hospital for repeated vomiting and poor weight gain. He received histamine 2 receptor antagonists (H2RAs) that contributed slightly to the decreased frequency of vomiting and aided weight gain; however, he soon stopped gaining weight and had bloody vomit. His upper gastrointestinal series revealed hiatal hernia, a 24 h impedance pH monitoring test indicated abnormal values for acid reflux, and esophagogastroduodenoscopy (EGD) revealed esophagitis. He was subsequently diagnosed with GERD associated with hiatal hernia. A proton pump inhibitor (PPI) was intravenously administered to him, following which his medication was changed to a potassium-competitive acid blocker (P-CAB). Thereafter, his vomiting episodes significantly decreased and his weight increased. However, 6 months after starting P-CAB, his vomiting episodes suddenly increased in frequency. EGD revealed the presence esophageal stricture due to scarring from GERD. He was then treated via laparoscopic fundoplication, gastrostomy, and esophageal balloon dilation. Thereafter, his vomiting episodes stopped and food intake improved, leading to weight gain. Conclusion: It is essential to identify the cause of GERD early and take an appropriate treatment approach depending on the cause of GERD with alarm signs. Further, as a drug therapy for GERD as a clear acid mediated disease or in children with alarm signs, PPIs or P-CAB should be used from the beginning instead of H2RAs.

DOI： 10.3389/fped.2024.1305585

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Language：English   Publisher：John Wiley & Sons Australia, Ltd  

神経芽細胞腫の診断と術前リスク評価に用いられるスコアリングシステムを策定し、その有用性について検討した。神経芽細胞腫患者68例228検体、対照群227例227検体を用いて、液体クロマトグラフィー-質量分析法によって尿中カテコラミン代謝産物バニル乳酸(VLA)と3-メトキシチラミン硫酸塩(MTS)を測定しスコアリングシステムを作成、さらに患者群を低リスク群10例、中リスク群9例、高リスク群22例に分類した。治療前後における患者群のVLA、MTS、ホモバニリン酸(HVA)、バニリルマンデル酸(VMA)は対照群と比較して有意に高く、VLAとMTSのみ低リスク群、中リスク群、高リスク群の間に有意差が認められた。新規の尿中バイオマーカー(VLA、MTS)と従来の尿中バイオマーカー(HVA、VMA)との比較では、診断および治療前リスク評価における曲線下面積(AUC)は新規マーカーの方が大きく、予後予測因子の評価に際しても新規マーカーの方が良好であった。VLAとMTSを組み合わせたスコアリングシステムは高リスク神経芽細胞腫の検出能に優れ、スクリーニング時のリスク評価にも有用であることが示された。

Language：English   Publisher：Cancer Science  

The urinary catecholamine metabolites, homovanillic acid (HVA) and vanillylmandelic acid (VMA), are used for the adjunctive diagnosis of neuroblastomas. We aimed to develop a scoring system for the diagnosis and pretreatment risk assessment of neuroblastoma, incorporating age and other urinary catecholamine metabolite combinations. Urine samples from 227 controls (227 samples) and 68 patients with neuroblastoma (228 samples) were evaluated. First, the catecholamine metabolites vanillactic acid (VLA) and 3-methoxytyramine sulfate (MTS) were identified as urinary marker candidates through comprehensive analysis using liquid chromatography–mass spectrometry. The concentrations of these marker candidates and conventional markers were then compared among controls, patients, and numerous risk groups to develop a scoring system. Participants were classified into four groups: control, low risk, intermediate risk, and high risk, and the proportional odds model was fitted using the L2-penalized maximum likelihood method, incorporating age on a monthly scale for adjustment. This scoring model using the novel urine catecholamine metabolite combinations, VLA and MTS, had greater area under the curve values than the model using HVA and VMA for diagnosis (0.978 vs. 0.964), pretreatment risk assessment (low and intermediate risk vs. high risk: 0.866 vs. 0.724; low risk vs. intermediate and high risk: 0.871 vs. 0.680), and prognostic factors (MYCN status: 0.741 vs. 0.369, histology: 0.932 vs. 0.747). The new system also had greater accuracy in detecting missing high-risk neuroblastomas, and in predicting the pretreatment risk at the time of screening. The new scoring system employing VLA and MTS has the potential to replace the conventional adjunctive diagnostic method using HVA and VMA.

DOI： 10.1111/cas.16116

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Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>An 11-year-old girl was admitted to our hospital with severe abdominal pain after suffering from enteritis. Abdominal ultrasonography revealed a target sign, and contrast-enhanced CT revealed an ileocolic intussusception. She underwent laparoscopic surgery at first, but we transitioned to open surgery owing to difficulty in reduction, and the ileum with a tumor was resected. The postoperative course was uneventful and she was discharged on postoperative day 9. The pathological diagnosis was lymphangioma. Generally, the age of onset of intussusception ranges from 6 months to less than 3 years. If the intussusception occurs at an older age, in many cases, the intussusception is caused by an organic lesion such as Meckel’s diverticula and neoplastic lesions. There are few reports on lymphangioma as the cause of intussusception. Since our present case of lymphangioma arose from the lumen of the small intestine discovered by ileocolic intussusception, we report this case on the basis of previous cases.</p>

DOI： 10.11164/jjsps.60.2_181

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Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p><i>Purpose</i>: Acute epididymitis is a disease with a good prognosis for the testis, but recently, there have been reports of testicular atrophy after epididymitis, and we encountered a similar case in our hospital.</p><p><i>Methods</i>: We retrospectively reviewed 77 cases of acute epididymitis (excluding recurrent cases) diagnosed and treated at our hospital from April 2008 to April 2022 on the basis of medical records.</p><p><i>Results</i>: The age at onset was in the range from 1 month to 14 years (median 8 years). Acute epididymitis occurred on the right side in 36 patients, on the left side in 40, and bilaterally in one, and the median time from its onset to the consultation was 8 h (IQR, 5–14.5 h). Symptoms were scrotal pain in 75 patients (97%), scrotal swelling in 44 patients (57%), redness in 28 patients (36%), abdominal pain in eight patients (10%), and fever in five patients (7%). Post-treatment follow-up was as follows: 65 (84%) patients were reexamined and followed up, and 12 (16%) patients were reexamined only on occasion, with a median observation period of 40 days (IQR, 7–249 days). The prognosis of the testes was as follows: 33 patients (43%) were followed up for more than one month after treatment, and one patient developed testicular atrophy.</p><p><i>Conclusions</i>: Acute epididymitis is a disease with a good prognosis for testicular disease, but in rare cases, it may lead to epididymitis and cause testicular atrophy, and long-term observation should be considered for patients with severe inflammation. Therefore, certain criteria for treatment and a follow-up period are required.</p>

DOI： 10.11164/jjsps.60.2_147

CiNii Research

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>We report the case of a 10-year-old boy who presented with secondary GnRH-dependent precocious puberty caused by a testicular Leydig cell tumor. He was initially followed up by the pediatric endocrinology department with the diagnosis of mental retardation and growth hormone deficiency. At 9 years and 10 months of age, a rapid increase in height, skeletal growth acceleration, and penile growth was seen. He was hospitalized for examination at the previous hospital. He was diagnosed with GnRH-dependent precocious puberty and referred to our department with the suspicion of a testicular tumor. Testicular ultrasonography showed a hypoechoic left testicular mass with a diameter of 10 mm. Positron emission tomography-computed tomography revealed an accumulation of fluorodeoxyglucose in the left testicle, and the undeniable presence of malignancy. Therefore, a left orchiectomy was performed. The pathological diagnosis was a Leydig cell tumor; however, there were no histopathological features of malignancy. There are few pediatric case reports of Leydig cell tumors in Japan; this is the 24th reported case. Furthermore, this is the first case in which GnRH-dependent precocious puberty was diagnosed before surgery. We reviewed the clinical characteristics of previous Japanese pediatric case reports and concluded that pediatric Leydig cell tumor should be differentiated when prepubertal overgrowth symptoms are observed. Early diagnosis could be expected by performing testicular ultrasonography.</p>

DOI： 10.11164/jjsps.60.2_172

CiNii Research

Publisher：金原出版  

DOI： 10.18888/sh.0000002991

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Pediatric Blood and Cancer  

PURPOSE: Survival rates of patients with high-risk neuroblastoma are unacceptable. A time-intensified treatment strategy with delayed local treatment to control systemic diseases has been developed in Japan. We conducted a nationwide, prospective, single-arm clinical trial with delayed local treatment. This study evaluated the safety and efficacy of delayed surgery to increase treatment intensity. PATIENTS AND METHODS: Seventy-five patients with high-risk neuroblastoma were enrolled in this study between May 2011 and September 2015. Delayed local treatment consisted of five courses of induction chemotherapy (cisplatin, pirarubicin, vincristine, and cyclophosphamide) and myeloablative high-dose chemotherapy (melphalan, etoposide, and carboplatin), followed by local tumor extirpation with surgery and irradiation. The primary endpoint was progression-free survival (PFS). The secondary endpoints were overall survival (OS), response rate, adverse events, and surgical complications. RESULTS: Seventy-five patients were enrolled, and 64 were evaluable (stage 3, n = 8; stage 4, n = 56). The estimated 3-year PFS and OS rates (95% confidence interval [CI]) were 44.4% [31.8%-56.3%] and 80.7% [68.5%-88.5%], resspectively. The response rate of INRC after completion of the treatment protocol was 66% (42/64; 95% CI: 53%-77%; 23 CR [complete response], 10 VGPR [very good partial response], and nine PR [partial response]). None of the patients died during the protocol treatment or within 30 days of completion. Grade 4 adverse effects, excluding hematological adverse effects, occurred in 48% of patients [31/64; 95% CI: 36%-61%]. Major Surgical complications were observed in 25% of patients [13/51; 95% CI: 14%-40%]. CONCLUSION: This study indicates that delayed local treatment is feasible and shows promising efficacy, suggesting that this treatment should be considered further in a comparative study of high-risk neuroblastoma.

DOI： 10.1002/pbc.30976

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Language：Japanese   Publisher：(一社)日本小児外科学会  

【目的】急性精巣上体炎は急性陰嚢症と総称される疾患の一つで,比較的頻度の高い疾患である.精巣予後が良好な疾患であるが,近年発症後に精巣萎縮を来した報告があり,当施設でも同様の症例を経験した.小児急性精巣上体炎の治療方針について改めて検討すべく,当施設で経験した症例を後方視的に検討したので報告する.【方法】2008年4月～2022年4月の期間中に急性精巣上体炎と診断され治療を行った77例を対象とし(再発例を除く),年齢,患側,発症から来院までの時間,臨床症状,身体所見,検査所見,治療内容,転帰について診療録を元に後方視的に検討した.【結果】発症時年齢は1ヵ月～14歳(中央値8歳),患側は右側36例,左側40例,両側1例,発症から受診までの時間は中央値8時間(IQR,5～14.5時間)であった.症状は,陰嚢痛75例(97%),陰嚢の腫脹44例(57%),発赤28例(36%),腹痛8例(10%),発熱5例(7%)であった.治療後の経過観察は,再診し経過観察をした症例が65例(84%)で,有事再診のみが12例(16%),観察期間の中央値は40日(IQR,7～249日)であった.精巣予後は,治療後1ヵ月以上経過が追えた症例が33例(43%),そのうち精巣萎縮を来した症例を1例認めた.萎縮を来した症例は,経過より,精巣上体炎の波及から精巣炎を来したものと考えられた.【結論】急性精巣上体炎は精巣予後良好な疾患だが,稀に精巣炎への波及を来し精巣萎縮の原因となる可能性があり,炎症が強い症例は長期的な観察も考慮するべきである.そのために,治療方針や経過観察期間の一定の基準が求められる.(著者抄録)

Language：Japanese   Publisher：(一社)日本周産期・新生児医学会  

胎便関連性腸閉塞(MRI)に対する保存的治療としてガストログラフィン注入療法(注入療法)が行われているが、その手技についてコンセンサスが得られたものはない。今回、当科における注入療法の適応と手技について紹介した。適応は、グリセリン浣腸で反応の得られない胎便排泄遅延例で、全身状態が安定し、かつ腹部X線検査で壊死性腸炎や消化管穿孔が否定された症例としている。手技は、6倍希釈ガストログラフィン2mL/kgを初回投与の原則とし、直腸内に挿入したカテーテルから注入している。ガストログラフィンが結腸から排泄されることを確認した後に6倍希釈ガストログラフィン2mL/kgの胃内注入を開始し、胃内注入した造影剤の排泄を確認することでMRIが解除されたと判断する。当科で2015～2022年に注入療法を行った20例を対象とし、MRIが解除された群(12例)と、腸閉塞症状の改善なく外科的介入を要した群(8例)に分け、「在胎週数」「出生体重」「アプガースコア」「注入療法の開始日齢」等について群間比較したところ、いずれの比較項目にも有意な群間差は認められなかった。

Language：Japanese   Publisher：(一社)日本小児外科学会  

症例は11歳女児,腸炎の先行感染を認め,激しい腹痛を主訴に受診となった.腹部超音波検査で下腹部正中にtarget sign,造影CTを撮像すると小腸-結腸型の腸重積を認め,腸重積症の診断となった.腹腔鏡下で手術を行ったが,整復が困難であったため開腹移行し,回腸内に隆起性の腫瘤性病変を認めたため小腸切除を行った.術後経過は良好であり,術後9日目に退院となった.最終病理結果では小腸リンパ管腫の診断となった.腸重積症の好発年齢は6ヵ月から3歳未満であり,好発年齢外の年齢発症の多くはMeckel憩室,ポリープ,腫瘍性病変などの器質的疾患が起点となり腸重積が起こると言われており,リンパ管腫を原因とする報告は小児においては極めて少ない.今回小腸-結腸型腸重積症により発見された小腸内腔発生のリンパ管腫の症例を経験したため,過去の症例を踏まえて報告する.(著者抄録)

Language：English   Publisher：Journal of Pediatric Surgery  

Purpose: To validate the effectiveness of Deflux® treatment for vesicoureteral reflux (VUR) following pediatric renal transplantation (RT), based on our single-institution experience. Method: A retrospective study was conducted using the medical records of pediatric patients who underwent Deflux® treatment for VUR after RT from April 2008 to March 2022. Results: Sixty-eight pediatric patients underwent RT. VUR was subsequently detected in 22 (32 %) of these patients. Seven of the 22 patients (32 %) underwent Deflux® treatment to avoid renal dysfunction due to urinary infection (UTI). The median age at the time of RT was 4 years (range:2–12). All 7 patients had urinary UTIs before Deflux® treatment. The median estimated glomerular filtration rate (eGFR) before Deflux® treatment was 67 ml/min/1.73 m2 (range:42–138 ml/min/1.73 m2). After Deflux® treatment, VUR was downgraded in three cases (43 %). Four patients (57 %) experienced postoperative UTI, two of who underwent a second Deflux® treatment, one underwent submuscular tunnel reconstruction, and the other one experienced UTI without VUR after 1st Deflux® treatment but did not reoccur. All seven patients continued prophylactic medication after Deflux® treatment, without any history of recurrent UTIs during the observation period after treatment (median 37 months [range 7–86 months]). Furthermore, the eGFRs did not significantly decrease after Deflux® treatment (median eGFR 58 ml/min/1.73 m2 [range:33–99 ml/min/1.73 m2], p > 0.1). Conclusion: Deflux® treatment for VUR after RT is technically challenging because the new ureteral orifice is ventrally anastomosed at the bladder. We believe our results indicate the possibility of reducing the frequency of UTIs and contributing to preservation of the renal function after RT. Type of study: Retrospective Study. Level of evidence: Level III.

DOI： 10.1016/j.jpedsurg.2023.12.005

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Language：English   Publisher：Molecular Genetics and Genomic Medicine  

Gaucher disease (GD) is a lysosomal storage disorder caused by a deficiency in the GBA1-encoded enzyme, β-glucocerebrosidase. Enzyme replacement therapy is ineffective for neuronopathic Gaucher disease (nGD). High-dose ambroxol has been administered as an alternative treatment for a group of patients with nGD. However, little is known about the clinical indication and the long-term outcome of patients after ambroxol therapy. We herein report a case of a female patient who presented with a progressive disease of GD type 2 from 11 months of age and had the pathogenic variants of p.L483P (formerly defined as p.L444P) and p.R502H (p.R463H) in GBA1. A combined treatment of imiglucerase with ambroxol started improving the patient's motor activity in 1 week, while it kept the long-lasting effect of preventing the deteriorating phenotype for 30 months. A literature review identified 40 patients with nGD, who had received high-dose ambroxol therapy. More than 65% of these patients favorably responded to the molecular chaperone therapy, irrespective of p.L483P homozygous, heterozygous or the other genotypes. These results highlight the long-lasting effect of ambroxol-based chaperone therapy for patients with an expanding spectrum of mutations in GBA1.

DOI： 10.1002/mgg3.2427

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Publisher：Journal of Materials Science  

We explored superconductors with high superconducting transition temperatures (Tc) tuning the stability of Magnéli phase through high-pressure torsion (HPT). This study has started from exploring superconducting states in the Al–Mg–O ternary system along with the prediction using machine learning. We successfully found superconducting states with Tc = 4.0 and 7.3 K for a composition of Al:Ti = 1:2 in the mixture of Al and surface-oxidized Ti powders. Another magnetic anomaly was also observed at ~ 93 K, being supported by the Tc prediction using the machine learning for the Al–Ti–O system. In this study, the HPT processing was also performed on a Magnéli material Ti4O7 and such mixtures of stable materials as Al + TiO2, Al2O3 + Ti, Al2O3 + TiO2, and Al + Ti4O7. In HPT-processed Ti4O7, the metal–insulator transition was maintained even after the HPT processing. HPT experiments using stable oxides indicate the difficulty of newly stabilizing the Magnéli phase starting from thermodynamically stable materials under severe plastic deformation. A series of attempts reveals that the superconducting state in the mixture with ratio Al:Ti = 1:2 is attributed to both strained Ti-oxide created on the surface of the Ti powder and its reaction with Al under HPT processing, resulting in the stabilization of a Magnéli phase. Graphical Abstract: (Figure presented.).

DOI： 10.1007/s10853-024-09406-w

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Language：Japanese   Publisher：(一社)日本小児外科学会  

症例は10歳1ヵ月の男児.軽度の精神発達遅滞と成長ホルモン分泌不全性低身長症のため前医小児科にて経過観察されていた.9歳10ヵ月時に急激な二次性徴進行と成長率増加,骨年齢促進を認めたため,前医に精査入院したところ,GnRH依存性思春期早発症の診断に加えて,精巣腫瘍が疑われ当施設へ紹介された.左精巣内に超音波検査で10mm大の低エコー域およびPET-CTでの集積を認め,悪性を否定できず左高位精巣摘出術を施行した.病理診断はLeydig細胞腫(以下本症と称す)で,悪性所見は認めなかった.本症の本邦小児報告は少なく,本症例を含め24例であり,術前よりGnRH依存性思春期早発症の診断がなされたのは本症例のみであった.本邦小児報告例の臨床的特徴から,思春期前に過成長(年齢に不相応な成長率増加)を認めた場合,本症を鑑別し,精巣超音波検査を含めた精査を行うことで早期診断が得られる可能性が示唆された.(著者抄録)

Publisher：株式会社医学書院  

DOI： 10.11477/mf.1403203469

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000717

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Language：English   Publisher：Frontiers in Medicine  

Introduction: Intractable lymphatic anomalies (LAs) include cystic lymphatic malformation (LM; macrocystic, microcystic, or mixed), generalized lymphatic anomaly, and Gorham–Stout disease. LAs can present with severe symptoms and poor prognosis. Thus, prospective studies for treatments are warranted. We conducted a prospective clinical trial of sirolimus for intractable LAs. Methods: This was an open-label, single-arm, multicenter, prospective trial involving five institutions in Japan. All patients with LAs received oral sirolimus once daily, and the dose was adjusted to ensure that the trough concentration remained within 5–15 ng/mL. We prospectively assessed the drug response (response rate for radiological volumetric change in target lesion), performance state, change in respiratory function, visceral impairment (pleural effusion, ascites, bleeding, pain), laboratory examination data, quality of life (QOL), and safety at 12, 24, and 52 weeks of administration. Results: Eleven patients with LAs (9 generalized lymphatic anomaly, 1 cystic LM, 1 Gorham–Stout disease) were treated with sirolimus, of whom 6 (54.5%; 95% confidence interval: 23.4–83.3%) demonstrated a partial response on radiological examination at 52 weeks of administration. No patients achieved a complete response. At 12 and 24 weeks of administration, 8 patients (72.7%) already showed a partial response. However, patients with stable disease showed minor or no reduction after 12 weeks. Adverse events, such as stomatitis, acneiform dermatitis, diarrhea, and fever, were common with sirolimus. Sirolimus was safe and tolerable. Conclusion: Sirolimus can reduce the lymphatic tissue volume in LAs and may lead to improvements in clinical symptoms and QOL.

DOI： 10.3389/fmed.2024.1335469

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Language：English   Publisher：International Journal of Molecular Sciences  

Severe obesity in young children prompts for a differential diagnosis that includes syndromic conditions. Rapid-Onset Obesity with Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) syndrome is a potentially fatal disorder characterized by rapid-onset obesity associated with hypoventilation, neural crest tumors, and endocrine and behavioral abnormalities. The etiology of ROHHAD syndrome remains to be established, but recent research has been focusing on autoimmunity. We report on a 2-year-old girl with rapid-onset obesity during the first year of life who progressed to hypoventilation and encephalitis in less than four months since the start of accelerated weight gain. The patient had a high titer of anti-ZSCAN1 antibodies (348; reference range < 40), and the increased values did not decline after acute phase treatment. Other encephalitis-related antibodies, such as the anti-NDMA antibody, were not detected. The rapid progression from obesity onset to central hypoventilation with encephalitis warns about the severe consequences of early-onset ROHHAD syndrome. These data indicate that serial measurements of anti-ZSCAN1 antibodies might be useful for the diagnosis and estimation of disease severity. Further research is needed to determine whether it can predict the clinical course of ROHHAD syndrome and whether there is any difference in antibody production between patients with and without tumors.

DOI： 10.3390/ijms25052820

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Language：Japanese   Publisher：(株)医学書院  

＜文献概要＞過去23年間に当科および関連施設にて診断された大腸の孤立性若年性ポリープ(JP)101例,孤立性PJP(Peutz-Jeghers type polyp)29例,IMGP(inflammatory myoglandular polyp)18例を対象とし,その臨床像,内視鏡像,病理組織像を遡及的に検討した.その結果,JPは平均年齢が低かった.内視鏡所見では,JPはびらん,白苔,白斑,NBI拡大観察下の開大した腺管開口部や疎に分布した腺窩辺縁上皮,細かい血管の増生,色素拡大観察下の星芒状,疎に分布したpitの,PJPは分葉状,NBIや色素拡大観察下の樹枝状構造の,IMGPは白斑,NBI拡大観察下の開大した腺管開口部,細かい血管の増生,色素拡大観察下の星芒状pitの頻度がそれぞれ高かった.以上よりJP,IMGPとPJPは異なる内視鏡像を呈していたが,JPとIMGPの内視鏡像は比較的類似していた.しかしながら,JPはIMGPに比べ,白苔,NBIや色素拡大観察下の疎に分布する腺窩辺縁上皮やpitの頻度が高いことを加味すると鑑別できる可能性があるものと推察する.

Language：English   Publisher：Cancers  

Retinoids are vitamin A derivatives and include trans-retinoic acid, isotretinoin, tamibarotene, and bexarotene, all of which are currently available for clinical use. The clinical development of retinoid therapy for neuroblastoma has a history spanning more than four decades. The most promising agent is isotretinoin, which can contribute to improving event-free survival in patients with high-risk neuroblastoma by approximately 10% when administered over six months as maintenance therapy. Although isotretinoin is regarded as an essential component in the standard clinical management of high-risk neuroblastoma, its use for this purpose in the US and EU is off-label. To promote isotretinoin use in Japan as a treatment for neuroblastoma, our clinical research team is planning to launch an investigator-initiated, registration-directed clinical trial. The present review article discusses the basic science behind retinoid therapy, pre-clinical/clinical evidence on neuroblastoma, the concept of the proposed clinical trial, and prospects for this therapy.

DOI： 10.3390/cancers16030544

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Language：English   Publisher：Frontiers in Pediatrics  

Introduction: Biliary atresia (BA) is a cholestatic hepatopathy caused by fibrosing destruction of intrahepatic and extrahepatic bile ducts, and its etiology has not been clearly revealed. In BA, liver fibrosis progression is often observed even after Kasai portoenterostomy (KPE), and more than half of cases require liver transplantation in their lifetime in Japan. Macrophages play an important role in liver fibrosis progression and are classically divided into proinflammatory (M1) and fibrotic macrophages (M2), whose phenotypic transformation is called “macrophage polarity.” The polarity has been reported to reflect the tissue microenvironment. In this study, we examined the relationship between macrophage polarity and the post-KPE clinical course. Materials and methods: Thirty BA patients who underwent KPE in our institution from 2000 to 2020 were recruited. Multiple immunostainings for CD68, CD163, CK19, and α-SMA were carried out on liver biopsy specimens obtained at KPE. ROC curves were calculated based on each clinical event, and the correlation with the clinical data was analyzed. Results and discussion: The M2 ratio, defined as the proportion of M2 macrophages (CD163-positive cells), was correlated inversely with the occurrence of postoperative cholangitis (AUC: 0.7602). The patients were classified into M2 high (n = 19) and non-high (n = 11) groups based on an M2 ratio value obtained from the Youden index (= 0.918). As a result, pathological evaluations (Metavir score, αSMA area fraction, and CK19 area fraction) were not significantly different between these groups. In mild liver fibrosis cases (Metavir score = 0–2), the M2 non-high group had a significantly lower native liver survival rate than the high group (p = 0.02). Moreover, 4 out of 8 cases in the M2 non-high group underwent early liver transplantation within 2 years after KPE. Conclusions: Non-M2 macrophages, including M1 macrophages, may be correlated with postoperative cholangitis, and the M2 non-high group in mild liver fibrosis cases had a significantly lower native liver survival rate than the high group, requiring early liver transplantation in this study. Preventing advanced liver fibrosis is a key factor in improving native liver survival for BA patients, and liver macrophages may play important roles in liver homeostasis and the promotion of inflammation and fibrosis.

DOI： 10.3389/fped.2024.1338131

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Language：English   Publisher：Pediatrics International  

Background: Recently, reports of endoscopic approaches for neuroblastoma, ganglioneuroblastoma, and ganglioneuroma (peripheral neuroblastic tumor; PNTs) have been increasing. This study aimed to clarify the indications for endoscopic surgery for PNTs. Methods: Pediatric patients who underwent endoscopic surgery for PNTs at our institution were included in this study. Image-defined risk factors (IDRFs) were analyzed using preoperative computed tomography (CT). Results: Twenty-four patients underwent endoscopic surgery for PNTs. The diagnoses included neuroblastoma (n = 11), ganglioneuroma (n = 10), and ganglioneuroblastoma (n = 3). Regarding the tumor site, there were 18 cases of adrenal tumors, five cases of mediastinal tumors, and one case of retroperitoneal tumors. Image-defined risk factors were positive in eight cases (contacted with a renal vessel, n = 6; compression of principal bronchi, n = 2). Complete resection was accomplished in 21 cases (14 of 16 IDRF-negative cases and seven of eight IDRF-positive cases). All patients survived without recurrence during the follow-up period. Conclusions: The CT findings of contact with renal vessels and compression of principal bronchi do not seem to be indicators of incomplete resection. An endoscopic approach to PNTs in pediatric patients is feasible with a good prognosis if patients are selected strictly.

DOI： 10.1111/ped.15754

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Language：English   Publisher：Journal of Pediatric Surgery  

Background: The optimal balance between the graft volume (GV) and portal venous flow (PVF) in living donor liver transplantation (LDLT) is unclear. As lactate is mainly metabolized in the liver, perioperative lactate levels are reportedly a useful biomarker for early graft dysfunction (EGD). The present study analyzed perioperative lactate levels according to the PVF. Methods: The PVF/GV (mL/min per 100 g GV) of 97 recipients from 1996 to 2022 was retrospectively classified as low (LPVF; PVF/GV ≤ 100, N = 29), moderate (MPVF; PVF/GV 100–250, N = 40), or high (HPVF; PVF/GV > 250, N = 28). Lactate levels were obtained preoperatively (L0), immediately after graft reperfusion (L1), 4 h after reperfusion (L2), and on postoperative day 3 (L3). The lactate clearances were then calculated. Results: The lower the PVF/GV ratio, the younger the age at LDLT and the higher the graft-to-recipient weight ratio. The median L2 and L3 in the HPVF group were significantly higher than those in the other groups (p = 0.019 and p = 0.003, respectively). The median ΔL1 in the HPVF group was lower than that in the LPVF and MPVF groups (0.23 vs. 0.50, p < 0.0001 and 0.23 vs. 0.41, p = 0.011, respectively). ΔL1 was negatively correlated with the PVF/GV. Although no patient had EGD, three patients with HPVF with low ΔL1 developed small-for-size syndrome. Conclusions: Graft hyperperfusion may delay the recovery of the graft function and result in poor lactate clearance. The combination of the PVF/GV and lactate clearance may be useful as a prognostic marker for optimal graft perfusion in LDLT. Level of evidence: IV.

DOI： 10.1016/j.jpedsurg.2024.07.032

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Language：Japanese   Publisher：Japanese Study Group on Pancreatico biliary Maljunction  

DOI： 10.34410/jspbm.47.0_36

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Language：English   Publisher：John Wiley & Sons Australia, Ltd  

Language：Japanese   Publisher：The Japanese Society of Pediatric Hematology / Oncology  

<p>In April 2008, liver transplantation (LT) for hepatoblastoma became covered under the Japanese medical insurance. Unresectable hepatoblastoma is considered an indication for LT; however, identifying “unresectable” hepatoblastoma is often difficult. Generally, unresectable tumors are those in cases categorized into post-treatment extent of disease grouping system POST-TEXT IV or III P+, V+. However, there are cases in which it is difficult to determine the resectability by preoperative imaging; although the tumor is considered resectable, a final surgical decision should be made at a facility capable of LT. Previously, it was safe to prioritize LT in cases of doubtful resectability owing to reports of extremely poor post-transplantation outcomes in salvage LT for reasons, such as recurrence after hepatectomy. Recently, there have been reports, including those from Japan, that the outcomes of salvage LT are good, and if the risk of long-term complications after LT is considered, challenging liver resection is reevaluated in this LT era. This article outlines the potential of atypical extreme hepatectomy using techniques used in transplant surgery, such as vascular reconstruction and organ preservation, and the surgical approach in cases with insufficient residual liver volume.</p>

DOI： 10.11412/jspho.61.144

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of Pediatric Surgery  

BACKGROUND: Tumor recurrence, anorectal and urinary dysfunction, and lower limb dysfunction after surgery are observed in infantile sacrococcygeal teratoma (SCT). In this paper, a multi-institutional retrospective observational study was conducted to clarify the long-term functional prognosis in Japan. METHODS: This study was conducted using a paper-based questionnaire distributed to 192 facilities accredited by the Japanese Society of Pediatric Surgeons, covering patients who underwent radical surgery at less than 1 year old and who survived for at least 180 days after birth from 2000 to 2019. RESULTS: A total of 355 patients were included in this analysis. Altman type was I-II in 248 and type III-IV in 107, and the median maximum tumor diameter was 6.1 (range: 0.6-36.0) cm. There were 269 mature teratomas, 69 immature teratomas, and 10 malignant tumors. Total resection was performed in 325, subtotal or partial resection in 27, and surgical complications were noted in 54. The median postoperative follow-up was 6.6 (0.5-21.7) years. Eighty-three patients (23.4 %) had functional sequelae, including 62 (17.5 %) with anorectal dysfunction, 56 (13.0 %) with urinary dysfunction, and 15 (4.2 %) with lower limb motor dysfunction. Recurrence occurred in 42 (11.8 %) at a median age of 16.8 (1.7-145.1) months old. Risk factors for dysfunction included preterm delivery, a large tumor diameter, Altman type III-IV, incomplete resection, and surgical complications. Risk factors for recurrence included immature teratoma or malignancy, incomplete resection, and surgical complications. CONCLUSIONS: Postoperative dysfunction was not low at 23.4 %, and 11.8 % of the patients experienced recurrence occurring more than 10 years after surgery, suggesting the need for periodic imaging and tumor markers evaluations in patients with risk factors. It is necessary to establish treatment guidelines for best practice monitoring of the long-term quality of life. LEVEL OF EVIDENCE: Level II Retrospective Study.

DOI： 10.1016/j.jpedsurg.2023.11.016

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000637

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Publisher：東京医学社  

DOI： 10.24479/peri.0000001159

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Language：English   Publisher：Pediatric Surgery International  

Background/Purpose: Whether Roux-en-Y hepatic jejunectomy (HJ) or duct-to-duct biliary reconstruction (DD) is more useful in pediatric living donor liver transplantation has not yet been fully investigated. Therefore, to assess the feasibility and safety of DD, we compared the surgical outcomes of DD to HJ. Methods: We divided 45 patients, excluding those with biliary atresia, into the DD group (n = 20) and the HJ group (n = 25), according to the type of biliary reconstruction they received. Results: The 5-year survival rates (DD vs. HJ = 79.7% vs. 83.6%, p = 0.70) and the incidence of biliary complications, including bile leakage and stricture (DD vs. HJ = 1 [5.0%] vs. 1 [4.0%], p = 0.87) were not significantly different between the groups. However, intestinal complications, including bowel perforation or ileus, were significantly common in the HJ group (9/25 [36.0%]) than in the DD group (1/20 [5.0%]; p = 0.01). The three patients in the HJ group with intestinal perforation all suffered perforation at the anastomosed site in the Roux-en-Y procedure. The subgroup analysis showed the non-inferiority of DD to HJ for biliary or intestinal complications in patients weighting < 10 kg. Conclusion: With a proper selection of cases, DD should be a safe method for biliary reconstruction in pediatric recipients with little risk of biliary complications equivalent to HJ and a reduced risk of intestinal complications.

DOI： 10.1007/s00383-023-05568-x

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Biology  

High-risk neuroblastoma (HR-NB) patients remain far from obtaining optimal outcomes, with more than 50% relapse/regrowth rate despite current intensive multimodal therapy. This originated from the activation/proliferation of chemoresistant minimal residual disease (MRD). MRD with a significant prognostic was reported by several quantitative PCR (qPCR) or droplet digital PCR (ddPCR) assays quantitating different sets of NB-associated mRNAs (NB-mRNAs). The 7NB-mRNAs ddPCR assay quantitating CRMP1, DBH, DDC, GAP43, ISL1, PHOX2B, and TH mRNAs was reported to outperform other qPCR assays by a retrospective in-house observational study. In the present study, the Japan Children's Cancer Group (JCCG) Neuroblastoma Committee conducted a prospective multicenter observational study aimed at evaluating a prognostic value of MRD in bone marrow (BM-MRD) and peripheral blood (PB-MRD) detected by 7NB-mRNAs ddPCR assay. Between August 2018 and August 2022, 7 HR-NB patients who registered for JCCG clinical trials (JN-H-11 and JN-H-15) were enrolled. A total of 19 BM and 19 PB samples were collected, and 4/15 BM and 4/15 PB samples were classified as progressive disease (PD)/non-PD samples. BM-MRD and PB-MRD estimated area under curve (AUC) of 0.767 and 0.800 with a significant accuracy (AUC > 0.7). The present study validated a prognostic value of BM-MRD obtained by a previous study (AUC 0.723) and revealed the significant accuracy of PB-MRD as well as BM-MRD.

DOI： 10.3390/biology12101350

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Publisher：Journal of Pediatric Surgery Case Reports  

Introduction: Nephroblastoma is the most common type of malignant renal tumor in children, but hemorrhage in ruptured nephroblastoma is rare. Case presentation: A three-month-old boy was admitted to our hospital with a large right renal tumor and hemorrhagic shock. Laparotomy showed that the tumor had invaded the right lobe of the liver; therefore, surgeons decided to perform only hemostasis and a tumor biopsy. The following day, intratumoral bleeding persisted, necessitating continuous blood infusion, resulting in abdominal compartment syndrome. Therefore, tumor resection was performed. Intra-tumoral hemorrhage continued during the operation, resulting in bradycardia and chest compressions. Histopathological examination confirmed a diagnosis of nephroblastoma. The postoperative course was uneventful. Conclusion: This report underlines the need to choose treatment based on both extratumoral and intratumoral bleeding, keeping in mind the risks of each treatment option.

DOI： 10.1016/j.epsc.2023.102711

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000581

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Surgery Today  

Hirschsprung disease (HSCR) and its associated disorders (AD-HSCR) often result in severe hypoperistalsis caused by enteric neuropathy, mesenchymopathy, and myopathy. Notably, HSCR involving the small intestine, isolated hypoganglionosis, chronic idiopathic intestinal pseudo-obstruction, and megacystis-microcolon-intestinal hypoperistalsis syndrome carry a poor prognosis. Ultimately, small-bowel transplantation (SBTx) is necessary for refractory cases, but it is highly invasive and outcomes are less than optimal, despite advances in surgical techniques and management. Thus, regenerative therapy has come to light as a potential form of treatment involving regeneration of the enteric nervous system, mesenchyme, and smooth muscle in affected areas. We review the cutting-edge regenerative therapeutic approaches for managing HSCR and AD-HSCR, including the use of enteric nervous system progenitor cells, embryonic stem cells, induced pluripotent stem cells, and mesenchymal stem cells as cell sources, the recipient intestine's microenvironment, and transplantation methods. Perspectives on the future of these treatments are also discussed.

DOI： 10.1007/s00595-023-02741-6

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Early Human Development  

INTRODUCTION: To establish actionable neonatal screening during the first month of life, we investigated critical diseases in seemingly healthy newborns discharged from birth hospitals. METHODS: This retrospective study enrolled previously healthy full-term infants who visited our hospital, a tertiary hospital in Japan, from home between 5 and 28 days after birth from 2009 to 2018. Infants with known perinatal or congenital diseases, positive newborn screening results, or accidental injuries were excluded. Data were collected from electronic medical records, including principal diagnosis, clinical details, and prognosis at 18 months of age. RESULTS: Ninety-seven (58 %) of 168 eligible neonates were admitted to the hospital, and 71 (42 %) were not. The median admission rate in patients with disease onset at ≤14 days after birth (80 %) was significantly higher than that in patients with disease onset at ≥15 days (42 %). Among 45 patients who received intensive medical care, 5 died and 10 developed neurodevelopmental sequelae. Four of 5 patients died by 100 days. Among 25 diseases treated in intensive care unit, 17 (68 %) diseases had a prevalence of <1 per 2000 live births. The commonly used diagnostic methods were imaging (n = 58, 35 %) and physical examination (n = 34, 20 %). CONCLUSION: Critical diseases due to rare and heterogeneous causes in ostensibly healthy newborns occurred predominantly in the first two weeks of life. Optimal newborn screening and health check-up protocols may benefit from the wide spectrum of life-threatening diseases occurring in home after birth.

DOI： 10.1016/j.earlhumdev.2023.105869

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000523

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Language：Japanese  

Language：Japanese   Publisher：(公社)日本小児科学会  

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DOI： 10.24479/ps.0000000480

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Language：Japanese  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Reproductive Biology and Endocrinology  

BACKGROUND: It has been hypothesized that the origin of early-onset endometriosis could be from endometrial mesenchymal stem cells (eMSCs) in neonatal uterine blood (NUB). There is no information on the possible mechanistic basis linking an association between NUB/neonatal endometrium and development of early-onset endometriosis. In this study we performed a series of experiments to clarify the mechanistic link between NUB and/or neonatal endometrium and development of early-onset endometriosis. METHODS: We retrospectively collected postmortem neonatal endometria (n = 15) and prospectively collected NUB (n = 18) of female babies for the analysis of different biological markers including eMSCs. Immunohistochemical analysis of neonatal endometria was performed to examine the expression patterns of ovarian steroid receptors (ER/PGR), decidualization (prolactin, IGFBP1), pre-decidualization (Glycodelin A, α-SMA), proliferation (Ki-67 index), vascularity (CD31 + cells), immunocompetent CD68+, CD45+, CD56 + cells and some putative markers of eMSCs. Cell transfer method and immunocytochemistry were used to investigate the eMSCs and/or endometrial cells in NUB. RESULTS: Immunohistochemical analysis of postmortem neonatal endometria revealed variable staining response to ER/PGR, decidual markers, and substantial proliferative and angiogenic activity. A moderate to strong immunoexpression of Glycodelin-A was found in both neonatal and adult endometria. The tissue infiltration of CD56+, CD45 + and CD68 + immunocompetent cells was significantly low in neonatal endometria than that in adult endometria (p = 0.0003, p < 0.0001, p = 0.034, respectively). No eMSCs or even endometrial cells were detected in NUB. However, a variable expression of some phenotypes of eMSCs (CD90/CD105) was found in neonatal endometria. CONCLUSIONS: Based on our serial experiments we did not find any supporting evidence for the role of NUB in early-onset endometriosis. Neonatal endometria showed variable expression of ovarian steroid receptors, decidualization, and a substantial amount of proliferative and angiogenic activity. As an alternative mechanism, a significantly less tissue accumulation of immunocompetent cells in neonatal endometria may explain the survival of ER + and PGR + cells should they make entry into the pelvis and consequent development of early endometriosis with the onset of ovarian function. Future study with large sample size and application of modified technological tools is warranted to test the NUB hypothesis and to clarify their biological or clinical significance. TRIAL REGISTRATION: not applicable.

DOI： 10.1186/s12958-023-01099-1

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Language：Japanese   Publisher：(一社)日本周産期・新生児医学会  

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Language：English   Publisher：Translational Pediatrics  

Background: Hypoganglionosis resembles Hirschsprung’s disease as in both diseases, patients may present with severe constipation or pseudo-obstruction. To date, diagnosis of hypoganglionosis is still difficult to be established due to lack of international consensus regarding diagnostic criteria. This study aims to evaluate the use of immunohistochemistry to provide objective support for our initial subjective impression of hypoganglionosis as well as to describe the morphological features of this study. Methods: This is a cross-sectional study. Three resected intestinal samples from patients with hypoganglionosis at Kyushu University Hospital, Fukuoka, Japan were included in this study. One healthy intestinal sample was used as control. All specimens were immunohistochemically stained with anti-S-100 protein, anti-α-smooth muscle actin (α-SMA), and anti-c-kit protein antibodies. Results: (I) S-100 immunostaining: hypoplasia of the myenteric ganglia and marked reduction of intramuscular nerve fibers were observed in several segments of the intestine. (II) α-SMA immunostaining: the pattern of the muscular layers was almost normal in all segments; however, some areas showed hypotrophy of the circular muscle (CM) layers and hypertrophy of the longitudinal muscle (LM) layers. (III) C-kit immunostaining: a decreased in the number of interstitial cells of Cajal (ICCs) was observed in almost all segments of the resected intestine, even around the myenteric plexus. Conclusions: Each segment of intestine in hypoganglionosis had different numbers of ICCs, sizes, and distributions of ganglions, as well as patterns of musculature, which may range from severely abnormal to nearly normal. Further investigations regarding the definition, etiology, diagnosis, and treatment of this disease should be performed to improve the prognosis of this disease.

DOI： 10.21037/tp-22-592

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Language：Japanese   Publisher：(一社)日本小児外科学会  

症例は生後2ヵ月の男児で,胎児期から心奇形と右横隔膜上腫瘤を指摘され,出生後に両大血管右室起始症,肺動脈閉鎖症,動脈管開存症,心房中隔欠損症と診断された.横隔膜上腫瘤は検査所見から悪性腫瘍の可能性は低く,当初は待機的加療の方針であった.しかし生後1ヵ月時の造影CTで,腫瘤は肺分画症病変が疑われた上,右先天性横隔膜ヘルニアも新たに指摘された.本症例は心奇形の治療を進める過程で,肺血流を増やし肺血管床を育てる必要があり,上記のような胸腔内占拠性病変は可及的早期の治療が望ましいと判断された.生後2ヵ月時に胸腔鏡下に右分画肺切除術及び,横隔膜縫縮術を施行した.術中,術後に特記すべき合併症は認めなかった.肺分画症及び遅発性先天性横隔膜ヘルニアに対する手術は,有意な臨床症状がなければ,待機的に施行されることが多いが,本症例のように重症心奇形を伴う場合は,病態に応じた早期手術の検討が重要と考えられた.(著者抄録)

Language：Japanese  

Language：Others   Publishing type：Research paper (scientific journal)   Publisher：Anticancer Research USA Inc.  

Background/Aim: Using the tyrosine hydroxylase (TH)-MYCN mouse neuroblastoma (NB) model, we have previously reported the accumulation of mouse mesenchymal stem cells (mMSCs) on tumors in vivo and the antitumor effect of mMSCs transfected with a small molecule (IFN-β) expression gene. In this study, we have developed novel MSCs secreting anti-disialoganglioside GD2 antibody (anti-GD2-MSCs) and evaluated their antitumor effects in vitro. Materials and Methods: We generated an anti-GD2 antibody construct (14.G2a-Fcx2-GFP) incorporating FLAG-tagged single-chain fragment variable against GD2 fused to a linker sequence, a fragment of the constant portion of human IgG1, and GFP protein. The construct was lentivirally transduced into mMSCs and the transduction efficiency was assessed by GFP expression. The secretion of FLAG-tagged anti-GD2 antibody was detected by Western blotting using anti-FLAG antibody. Antibody binding capacity was confirmed by flow cytometry. Antibody-dependent cellular cytotoxicity (ADCC) was evaluated using human NB cells and human natural killer (NK) cells to assess whether the antitumor activity was enhanced in the presence of the produced antibodies. Results: The transduction efficiency of anti-GD2-MSCs was more than 90%. anti-GD2-MSCs secreted antibodies extracellularly and these antibodies had high affinity to GD2-expressing human NB cells. ADCC assays showed that the addition of antibodies secreted from anti-GD2-MSCs significantly increased the cytotoxic activity of NK cells against NB cells. Conclusion: Newly developed antiGD2-MSCs produced functional antibodies that have affinity to the GD2 antigen on NB cells and can induce ADCC-mediated cytotoxicity. Anti-GD2-MSCs based cellular immunotherapy has the potential to be a novel therapeutic option for intractable NB.

DOI： 10.21873/anticanres.16409

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>A 12-year-old girl had been suffering from abdominal pain, constipation, and vomiting for 3 months, and eventually presented failure to thrive. These symptoms worsened suddenly, and she visited a previous hospital and was suspected of having bowel obstruction. She was referred to our department. Abdominal CT showed thickening and narrowing of the jejunal wall, dilation of the proximal intestinal tract, and a closed loop. She was diagnosed as having acute abdomen due to small bowel obstruction, and an emergency laparotomy was performed. During surgery, no ischemic change in the intestine was observed. Massive edema, wall thickening, and dilation of the jejunum were noted, and a mass formation involving the omentum was also observed. The affected jejunum was resected, and the specimen showed a cobblestone appearance and longitudinal ulcers on the mucosa. The histopathological findings revealed a noncaseating granuloma, and her final diagnosis was Crohn’s disease. Her postoperative course was uneventful, infliximab was started from the 14th postoperative day, and she was discharged on the 32nd day. Surgeons should be aware of the possibility of future multiple surgeries and postoperative enteral during emergency surgery for undiagnosed pediatric Crohn’s disease.</p>

DOI： 10.11164/jjsps.59.2_191

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Publisher：Japanese Journal of Applied Physics  

Electron spins in gate-defined quantum dots (QDs) formed in semiconductor quantum wells (QWs) are promising stationary qubits for implementing large-scale quantum networks in a scalable manner. One key ingredient for such a network is an efficient photon-spin interface that converts any polarization state of a flying photonic qubit to the corresponding spins state of the electron in gate-defined QDs. A bull’s-eye cavity is an optical cavity structure that can enhance the photon absorption of an embedded gate-defined QD without polarization dependence. In this paper, we report the successful fabrication of air-bridge bull’s-eye cavities with metal electrodes and demonstrate the nearly polarization-independent optical absorption of a GaAs QW embedded in the cavities. This work marks an important step toward realizing an efficient photon-spin interface using gate-defined QDs.

DOI： 10.35848/1347-4065/acac3a

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Language：Japanese   Publisher：(一社)日本外科学会  

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症例は12歳女児.3ヵ月前から腹痛,便秘,嘔吐を繰り返すようになり近医小児科にて経過観察されていたが,腹痛・嘔吐症状が急激に増悪し腸閉塞が疑われたため当科へ紹介受診となった.CTにて空腸の壁肥厚と狭小化,口側腸管の拡張,closed loopを認め,急性腹症と判断し緊急開腹術を施行した.虚血性変化はなく,空腸の広範囲に浮腫,壁肥厚,拡張及び大網を巻き込んだ腫瘤形成を認めた.同部を切除し,敷石状病変・縦走潰瘍を認めCrohn病による消化管狭窄と術中診断した.合併症を回避するために,機械吻合ではなく正常な粘膜部で手縫での端々吻合を行い,吻合部口側に減圧チューブを留置し手術を終了した.病理学検査にて非乾酪性肉芽腫を認めCrohn病と最終診断した.術後経過は良好で,14日目にインフリキシマブを開始し,32日目に退院した.小児の本症の未診断例に対し緊急手術を行う際は,再手術や手術に起因する瘻孔形成を念頭に術式や合併症対策を検討すべきである.(著者抄録)

Language：Japanese   Publisher：(一社)日本外科学会  

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Language：English   Publisher：John Wiley & Sons Australia, Ltd  

梨状窩嚢胞(PSC)および梨状窩瘻孔(PSF)に対して、電気凝固療法を施行した症例を後方視的に検討した。内部開口部は全身麻酔下で内視鏡により同定し、DualKnifeJにより焼灼した。3例のPSCおよび3例のPSFに対して加療を行い、処置後の経過は全例良好であった。頸部痛がみられた症例はなく、反回神経麻痺を呈した症例も認めなかった。6例中5例(83%)で初回焼灼により開口部が閉鎖され、1例(16.6%)で複数回の処置が必要であった。経過観察期間(中央値1年)中に、再発は認められなかった。新生児PSCに対しても電気凝固は有効であった。大規模臨床試験が必要と考えられた。

Language：Japanese   Publisher：(一社)日本小児外科学会  

食道アカラシアは小児期での発症はごく稀な疾患である.故に治療法や適切な治療時期については未だコンセンサスが得られていない.今回,高解像度食道内圧検査(HRM)でシカゴ分類type IIと診断し,治療には内視鏡的バルーン拡張術を選択し,段階的に30mmまで拡張することによって十分な治療効果を得た小児の症例を経験した.症例は8歳女児.繰り返す就寝時の咳嗽と嘔吐を契機に食道アカラシアと診断された.シカゴ分類type IIと診断し,最も治療反応性の良い群と考え,治療は最も低侵襲な内視鏡的バルーン拡張術を第一選択とした.複数回に分けて段階的に30mmまで拡張を施行した.治療後は症状は消失し,食道造影検査でも食道径の減少が確認できた.Eckardt scoreは治療前4点から治療後0点と改善を認めている.現在,術後約2年が経過しているが再発を認めず,追加治療は不要な状態である.(著者抄録)

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Language：English   Publisher：John Wiley & Sons Australia, Ltd  

症例は1歳4ヵ月男児で、A型インフルエンザに罹患し、ラニナミビルオクタン酸を投与された。吸入後、下痢、食欲低下、黒色便が出現し、翌日には下血が認められ、出血性ショックに陥った。造影CT検査で、十二指腸下行脚からの造影剤の血管外漏出が認められた。上部消化管内視鏡検査で、十二指腸の主乳頭近位のデュラフォイ病変からの噴出性出血が確認され、クリッピングによる止血を行った。十二指腸球部から下行脚にかけて、粘膜は萎縮性で、輪状襞上に点状発赤が認められ、多発する不整形糜爛を伴っていた。第8病日にはほぼすべての粘膜病変が治癒した。その後1年以上外来で経過観察したが、再出血は認められなかった。

Publisher：Journal of Physical Chemistry C  

We synthesized spinel manganese oxide Mn3O4 nanoparticles with sizes ranging from 7 to 25 nm and investigated their crystal structure and magnetic properties through X-ray diffraction, magnetization, and electron spin resonance (ESR) measurements. As the particle size decreased, the lattice constant a increased monotonically, while the lattice constant c and tetragonal distortion of the unit cell reached a minimum at 11 nm and then increased rapidly below 9 nm. The ESR spectra of the nanoparticles differed from those of the bulk crystal, and the nanoparticles exhibited a hyperfine structure of Mn2+ ions at the nanoparticle surface. The size dependence of the line width of the ESR spectrum of the nanoparticles was similar to that of the tetragonal distortion. The magnetic transition temperatures and coercive field of the nanoparticles decreased rapidly below 10 nm. The Mn3O4 nanoparticles maintained three magnetic transition temperatures similar to those of bulk crystals above 9 nm. However, the nanoparticles exhibited only two distinct transition temperatures below 9 nm. Distortion of the crystal structure leads to a change in the magnetic interactions and magnetic state. It is suggested that the change of a phase boundary appears to exist at approximately 9 nm in the phase diagram of the magnetic properties as a function of the particle size.

DOI： 10.1021/acs.jpcc.2c08680

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Publisher：東京医学社  

DOI： 10.24479/pm.0000000812

CiNii Research

Publisher：東京医学社  

DOI： 10.24479/pm.0000000805

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Gastrointestinal symptoms are one of the most common presentations of Coronavirus disease-19 (COVID-19), even in children. Higher rates of complicated appendicitis have been demonstrated in the era of the COVID-19 outbreak, and it has been recently suggested that acute appendicitis may occur as a complication of COVID-19. However, the relationship between appendicitis and COVID-19 remains unclear. CASE PRESENTATION: A 7-year-old male presented to the pediatric emergency department with 2 days' history of lower abdominal discomfort and tenderness. On examination, his abdomen was distended with diffuse mild tenderness at the lower abdomen, which was aggravated by movement. He was also tested and was found to be positive for SARS-CoV-2. Computed tomography showed perforated appendicitis with a fecalith. The patient was admitted and laparoscopic appendectomy was successfully performed. Postoperatively, a minor intra-abdominal abscess was present, which successfully treated with antibiotics. Histopathology showed a markedly inflamed appendix with mucosal ulceration and transmural neutrophilic inflammation, which was consistent with phlegmonous appendicitis. Reverse transcription quantitative polymerase chain reaction using a surgically extracted appendix specimen revealed the presence of SARS-CoV-2 virus, which indicated a pathophysiological relationship between appendicitis and COVID-19. CONCLUSION: The present case will provide further understanding of pediatric patients with concomitant COVID-19 and acute appendicitis.

DOI： 10.1186/s40792-023-01618-7

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Language：English   Publisher：Springer Berlin Heidelberg  

症例は7歳男児。父親に新型コロナウイルス感染症(SARS-CoV-2)の既往があった。2日前からの下腹部痛のため救急医療部へ搬送された。搬送時の体温は39.1度で、血圧は102/61mmHg、心拍数は101bpmであった。受診時に腹部膨満を認め、下腹部にびまん性の圧痛を認めた。臨床検査で白血球数は9900/μL、C反応性蛋白は12.3mg/dLであった。造影CTで腫大した虫垂を認めた。虫垂根部に異常はなく、虫垂内部に糞石と穿孔を認めた。穿孔性虫垂炎と診断し、腹腔鏡下虫垂切除術を施行した。サージカルマスク、N95マスク、フェイスシールド等の個人防護具を装着し、緊急手術を施行した。手術時間は93分であった。術後の培養検査で大腸菌、Streptococcus constellatus、Bacteroides fragilisが検出された。抗菌剤をタゾバクタム/ピペラシリンからメロペネムとメトロニダゾールに変更した。切除標本を用いたリアルタイムPCR法でSARS-CoV-2に陽性を認めた。術後経過は良好で、呼吸器症状もなく、17日後に退院となった。

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Publisher：Physical Review B  

A prototype of the monoaxial chiral magnet CrNb3S6 exhibited two magnetostriction (MS) effects: (1) spontaneous MS due to the exchange interaction and spin-orbit coupling (SOC) at zero dc magnetic field (H) [Phys. Rev. B 102, 014446 (2020)2469-995010.1103/PhysRevB.102.014446] and (2) paramagnetic MS due to SOC and Zeeman energy at room temperature [Phys. Rev. B 105, 104412 (2022)2469-995010.1103/PhysRevB.105.104412]. CrNb3S6 has various magnetic structures, such as helimagnetic, first chiral soliton lattice (CSL-1), second CSL (CSL-2), CSL-2 with irreversibility, and forced ferromagnetic phases, resulting from the Dzyaloshinskii-Moriya interaction as a function of H below the magnetic ordering temperature (Tc). In this study, we conducted powder x-ray diffraction analyses to investigate the effects of H and temperature (T) on the MS at the atomic level. The T dependence of the lattice constants reveals that below Tc, the MS depends on the magnetic structure. The MS below Tc is discussed in this study in terms of both the hybridization between the z2 orbital of Nb(4f) and delocalized a1 orbital of Cr and the structural symmetry of the CrS6 octahedron.

DOI： 10.1103/PhysRevB.107.054427

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Language：Others   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

Background: There is no standard timing for switching to surgical management for children with adhesive small bowel obstruction (ASBO) who initially receive conservative treatment. We hypothesized that an increased gastrointestinal drainage volume may indicate the need for surgical intervention. Methods: The study population included 150 episodes in the patients less than 20 years of age who received treatment for ASBO in our department from January 2008 to August 2019. Patients were divided into two groups: the successful conservative treatment group (CT) and the eventual surgical treatment group (ST). Following the analysis of all episodes (Study 1), we limited our analysis to only first ASBO episodes (Study 2). We retrospectively reviewed their medical records. Results: There were statistically significant differences in the volume on the 2nd day in both Study 1 (9.1 ml/kg vs. 18.7 ml/kg; p < 0.01) and study 2 (8.1 ml/kg vs. 19.7 ml/kg; p < 0.01). The cut-off value was the same for both Study 1 and Study 2 (11.7 ml/kg). Conclusions: The gastrointestinal drainage volume on the 2nd day in ST was significantly larger than that in CT. Accordingly, we considered that the drainage volume may predict eventual surgical intervention for children with ASBO who initially receive conservative treatment. Level of evidence: Level IV.

DOI： 10.1016/j.jpedsurg.2023.01.052

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Language：Others   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

Introduction: Food protein-induced enterocolitis syndrome (FPIES) is a T-cell-mediated allergy that can occur in newborns and infants who are introduced to milk protein. Some of the serious complications of FPIES include necrotizing enterocolitis (NEC), massive bloody stools, and disseminated intravascular coagulation. Here we report a case of NEC caused by FPIES. Presentation of case: A 28-day-old girl born at full term suddenly developed marked abdominal distention and shock a few hours after being fed highly regulated milk protein. Emergency laparotomy was performed, and extensive small-intestinal necrosis was found. The histological examination showed chronic inflammation with typical ghost crypts, hemorrhage, and extensive pneumatosis intestinalis, a presentation consistent with NEC. Discussion: In this case, the fragile intestinal mucosa associated with FPIES was stimulated by milk protein, leading to NEC. The greatest diagnostic difficulty is the lack of a definitive method for distinguishing between NEC and FPIES. The allergen-specific lymphocyte stimulation test with lactotransferrin was positive, indicating that the primary condition was FPIES. However, no eosinophilic infiltrate was found in the histological examination, but there was chronic inflammation with typical ghost crypts, hemorrhage, and extensive pneumatosis intestinalis. Consequently, the final histological diagnosis in our case was NEC rather than FPIES. Conclusion: FPIES has a variable clinical course, and severe FPIES may become exacerbated even after ingestion of highly regulated milk protein. Taking appropriate actions after correct diagnosis can prevent progression to surgical emergency and secondary NEC.

DOI： 10.1016/j.ijscr.2023.107885

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Language：Others   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of Pediatric Surgery  

PURPOSE: The aim of this study was to clarify the appropriate management after birth for congenital biliary dilatation (CBD, choledochal cyst) patients with a prenatal diagnosis. METHOD: Thirteen patients with a prenatal diagnosis of CBD who underwent liver biopsy during excision surgery were divided into two groups and retrospectively analyzed: group A, with liver fibrosis above F1 and group B, without liver fibrosis. RESULTS: Excision surgery was performed earlier in group A (F1-F2), at a median of 106 days old (p = 0.04). There were significant differences between the two groups in the presence symptoms and sludge, the cyst size, and the level of serum bilirubin and gamma glutamyl transpeptidase (GGT) before excision surgery (p < 0.05). Especially, in group A, prolonged serum GGT elevation and larger cysts were consistently observed from birth. The cut-off values of predictions for the presence of liver fibrosis in serum GGT and cyst size were 319 U/l and 45 mm. No significant differences were observed in the postoperative liver function or complications during the follow-up period. CONCLUSION: In patients with prenatally diagnosed CBD, the postnatal serial changes of serum GGT values and cyst size, in addition to symptoms, could help to prevent progressive liver fibrosis. LEVEL OF EVIDENCE: Ⅲ. TYPE OF STUDY: Treatment Study.

DOI： 10.1016/j.jpedsurg.2023.01.050

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Language：Japanese   Publisher：(一社)日本小児外科学会  

肝芽腫破裂は,出血性ショックにより,ときに致命的になり得る.今回,肝芽腫破裂に対して,経カテーテル動脈塞栓術(TAE)を施行し,全身化学療法後に肝切除を施行しえた3例について報告する.症例1は10ヵ月女児,顔色不良を契機に診断され,ヘリコプターにより転院搬送となり,緊急TAEを施行した.CITA療法4コース後に肝右葉切除を行った.症例2は6歳男児,肝腫瘍精査中に突然ショックとなって発症し,TAEを行い,CITA療法6コース後に肝左葉切除を行った.1.5年後に局所再発に対して切除を施行した.症例3は2歳男児,転院搬送中にショック状態となり,緊急TAEにて止血をした.C5VD療法を4コース施行後に肝右葉切除を施行した.肝芽腫破裂においては迅速なTAEへのアクセスとその完遂が救命につながる.自験例より,乳幼児例においてもTAEは十分施行可能であり,治療の第一選択になり得ると考えられた.また,止血後は化学療法を優先し腫瘍の縮小を図った後に腫瘍切除を行うべきである.(著者抄録)

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Publisher：Journal of Applied Physics  

Using the nanosized porous medium SBA-15, we construct the condensed spin-1 network composed of oxygen molecules (O2) with tube and rod structures. The condensed network can be controlled by changing the amount of O2 molecules and substituting nonmagnetic nitrogen molecules (N2) at the O2 site. The characteristics of the two-dimensional spin model were observed in the monolayer and double layer physisorption systems. In a typical bulk system, the liquidizing temperature T liq - gas is 90 K, while in SBA-15 with a pore size of 8.5 nm, the monolayer network of O2 has a T liq - gas value of 60 K. However, in the monolayer system, when a half of O2 site is diluted with N2, T liq - gas increases to 94 K. Even after the N2 molecules are vaporized, they affect the physisorption potential of the inner wall of SBA-15. The resultant electric cloud of N2 hinders the evaporation of O2, resulting in an increase in T liq - gas. The effect is weakened with increasing number of physisorption layers. Thus, this study presents the unique condensation phenomenon of two molecules with different T liq - gas under a strong physisorption potential.

DOI： 10.1063/5.0123318

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Pediatrics International  

BACKGROUND: The aim of this study was to clarify the characteristics and outcomes of pediatric patients with solid pseudopapillary neoplasms (SPNs) who underwent pancreatectomy. METHODS: Pediatric patients with SPNs who underwent pancreatectomy at our institution between 1995 and 2020 were included in the study. RESULTS: During the period under review, 12 patients underwent pancreatectomy for SPNs (median age: 10 years; range: 6-15 years). The surgical procedures included pancreatoduodenectomy (n = 2; 16.6%), distal pancreatectomy (n = 3; 25%), and enucleation (n = 7; 58.3%). The most common postoperative complication was postoperative pancreatic fistula (n = 6; 50%). Patients who underwent enucleation tended to have higher postoperative complication rates compared with those who underwent other procedures. All patients were alive without recurrence at the end of the study period. CONCLUSIONS: SPN is associated with a good prognosis, regardless of the surgical procedure. If surgeons select enucleation for pediatric SPNs, they should bear in mind that it is associated with a higher complication rate.

DOI： 10.1111/ped.15666

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Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>【緒言】Hypoganglionosis(以下Hypo)に対し、当科では乳児期に鬱滞性腸炎予防のため残存腸管切除を検討する一方、Bishop-Koop型腸瘻(以下BK)やSantulli型腸瘻(以下St.)を用いて肛門側未使用腸管の活用を試みる。【方法】2022年12月までに当科の定期的な受診歴のあるHypo症例5例を後方視的に検討し、上記治療戦略の成果を検討した。【結果・考察】5例中3例がBK(症例1:2歳7ヶ月、症例2:3歳、症例3:3歳2ヶ月に施行)を、1例がSt.(2歳6ヶ月施行)を、1例がチューブ腸瘻(以下Tube;1歳3ヶ月施行)を造設された。機能性腸瘻の症例では術前後でAST、ALTの改善が見られ、特にBK症例でより改善していた(症例1:AST86.6→53.3mg/dL,ALT121.7→54.7mg/dL, 症例2:AST77.3→61.8mg/dL,ALT43.7→40.3mg/dL, 症例3:AST106.4→51.6mg/dL,ALT76.3→32.8mg/dL, St.:AST77.5→68.2mg/dL,ALT57.4→52.7mg/dL, Tube:AST57.3→56.9mg/dL,ALT81.3→78.3mg/dL)。肛門側腸管をより積極的に使用するBKで腸管蠕動が刺激され、胆汁鬱滞が改善したと考えられた。示唆に富む結果と考え、ここに報告する。</p>

DOI： 10.11386/jst.58.supplement_s162_1

CiNii Research

Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>【背景】本邦における脳死肝小腸同時移植は肝臓希望者選択基準を考慮すると実現性が低く、異時性移植が多く行われてきたが、どちらを先行するにしても憂慮すべき周術期の課題は多い。今回当科における異時性肝小腸移植の経験から、解決すべき課題について考察したい。【症例】9歳女児。日齢6から下痢が持続し、小腸粘膜の病理所見から微絨毛封入体病(MVID)と診断された。中心静脈栄養(TPN)を併用しても体重-3SD、身長-5SDで推移し、7歳から肝障害に伴う出血傾向や病的骨折を認めた。8歳9か月時にMELDスコア19点、小腸はstatus 2で脳死肝小腸同時移植へ登録したが、急激に肝不全へ進行したため父親をドナーとする血液型不適合の生体肝移植を先行した。胆道は小腸移植に備えてduct-to-ductで再建した。肝移植後も腸管不全に対するTPNが必要なため徐々に脂肪肝が増悪して再度肝不全の状態となったが、肝移植後4ヶ月で脳死小腸移植に至った。小腸移植後20日でTPNから離脱し、肝不全も軽快した。現在小腸移植後10ヶ月で肝機能増悪や拒絶反応はない。【考察】本症例の肝不全進行が原疾患によるものか、TPNに伴う肝障害かは不明だが、肝移植単独での長期生存は困難であり、小腸移植までの間隔に課題が残る。MVIDのように肝・小腸共に病変を呈する疾患では同時移植や多臓器移植の必要性が高い。また、腸管不全関連肝不全は急速に増悪することが多く、より移植実現性の高いシステム構築が求められる。</p>

DOI： 10.11386/jst.58.supplement_s205_2

CiNii Research

Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>【目的】肝移植後の生存期間延長に伴い、晩期合併症として二次発癌が増加し問題となっている。当科では胆道閉鎖症（BA）に対する肝移植後、比較的若年成人でde novo悪性腫瘍を発症した2例を経験したため、文献的考察を加えて報告する。【方法】胆道閉鎖症に対する肝移植後、de novo悪性腫瘍を発症した2例を検討し、その対策やスクリーニングに関して考察する。【結果】（症例１）BAによる胆汁うっ滞性肝硬変のため、33歳時に脳死肝移植術を施行。40歳時に移植肝評価のため撮影したCT検査で偶発的に左腎に径1cmの腫瘍を認め、ロボット支援下左腎部分切除術を施行した。病理検査でclear cell renal cell carcinoma,pT1aN0M0の診断であった。（症例２）BAによる胆汁うっ滞性肝硬変のため、27歳時に生体肝移植術を施行。40歳時に持続性の下腹部痛を認め、腹部CT検査でS状結腸に腫瘍性閉塞を認めた。造影剤の腸管外漏出所見も認めたため、緊急で横行結腸人工肛門造設術を行った。生検でAdenocarcinomaの診断となり、18日後にS状結腸切除＋D3郭清を施行した。病理検査でwell to moderately differentiated adenocarcinoma,pT3N1bM1c1,Stage4であった。【考察】肝移植後は一般の２、３倍の頻度でde novo悪性腫瘍がみられる。BA患者は成人後も小児外科医が中心となって診療を継続している場合も多く,de novo悪性腫瘍対策としてスクリーニングプログラムの作成が必要と考えられた。</p>

DOI： 10.11386/jst.58.supplement_s320_2

CiNii Research

Language：Japanese   Publisher：Japanese Study Group on Pancreatico biliary Maljunction  

DOI： 10.34410/jspbm.46.0_50

CiNii Research

Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>　小腸移植は、長期にわたる重度栄養障害を背景とする症例が移植適応となる。さらに、移植後においてもグラフト臓器の状態がレシピエントの栄養状態に直結する臓器移植領域でもある。当然、小腸移植の最終的ゴールは、静脈栄養（PN）からの完全離脱と栄養状態の改善にある。わが国における小腸移植後患者のQOLについて、移植後1年以上経過したグラフト生着患者では、全員が少なくとも部分的にPNから離脱し、約9割が完全離脱可能となっている。しかしながら、ひとたび拒絶反応が生じると、小腸粘膜の脱落を反映して著明にシトルリン値が低下して吸収不全を生じ、その制御は困難な場合が多い。当科では、これまでに5例の腸管不全患者に脳死小腸移植を実施した。内訳は短腸症1例、腸管吸収不全1例、腸管運動障害3例であった。いずれも移植後経口摂取が進み、2-3か月以内でPNからの離脱が得られている。しかし、うち2例では制御困難な拒絶反応からPN管理を再導入し、最終的にはグラフト摘出に至った。残りの3例は、PN不要な状態が維持されており、大幅な栄養状態の改善からQOL向上が得られている。今後の課題として、本邦では、胃や十二指腸を含めた多臓器移植が施行できず、単独小腸移植（結腸含む場合あり）となるため、術後にも残存した自己消化管機能を考慮した管理を継続していく必要性があり、思うように経口摂取が進まない可能性が懸念される点などにある。</p>

DOI： 10.11386/jst.58.supplement_s131_1

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Language：English   Publisher：John Wiley & Sons Australia, Ltd  

1995～2020年に膵切除術が施行されたsolid-pseudopapillary neoplasm(SPN)児12例(年齢6～15歳)の特徴とアウトカムについて検討した。術式は膵頭十二指腸切除術(2例;16.6%)、膵体尾部切除術(3例;25%)、膵腫瘍核出術(7例;58.3%)であった。術後合併症としては術後膵液瘻が最も多かった(6例;50%)。膵腫瘍核出術を施行した患児では他の術式の患児に比べ、術後30日までの合併症発生率が高い傾向を示し(85% vs 20%、p=0.04)、CT画像でトレースした膵切除断端外縁長が有意に長かった(34±10mm vs 10±2mm、p<0.01)。研究期間終了時点で全例が再発をみることなく生存している。

Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>【はじめに】糖原病Ia型はグルコース-6-ホスファターゼ欠損により引き起こされる。低血糖発作や乳酸アシドーシスの管理に難渋する症例や、高頻度に出現する肝細胞腺腫が増大傾向にあり悪性化が懸念される症例では肝移植が適応とされるが、本邦での本疾患への肝移植施行例は数例のみである。</p><p>【症例】8歳女児。生後10か月時に発達の遅れと肝腫大を指摘され当院小児科紹介となった。精査にて糖原病Ia型と診断され、服薬・食事療法で加療されていた。次第に内科的コントロールが不良になり、また肝細胞腺腫が多発し一部に増大傾向を認めるため肝移植の適応と診断し、血液型適合の父から左葉グラフト（グラフト重量290ｇ、GRWR 1.17%、GV/SLV 48.8%）を移植した。肝移植時の身長108.5cm(-3.3SD)、体重23.5kg(-0.5SD)、標準肝容量（SLV）582.7mlであったが、摘出肝は黄色調で2012gと著明な腫大を認めた。 摘出肝の病理診断で両葉に多数の結節病変を認め、炎症性肝細胞腺腫と診断されたが、悪性所見は認めず、肝細胞癌は否定された。グラフト再灌流直後から血糖が安定化し、術後も低血糖発作を認めることなく食事制限は解除することができ、移植後35日目に自宅退院した。現在移植後3か月経過し拒絶反応を認めず、現在外来にて管理継続中である。</p><p>【まとめ】肝移植後の経過は良好であり、内科的コントロールが不良な症例や多発増大傾向のある肝細胞腺腫を認める症例には肝移植を検討すべきである。</p>

DOI： 10.11386/jst.58.supplement_s321_3

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Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>A six-year-old boy was admitted to the pediatric department of our hospital because of abdominal and chest pains. Blood test findings on admission showed elevated CRP and LDH levels. The patient was referred to our department because of worsening abdominal pain after admission. Abdominal computed tomography showed left-sided deviation of the small intestine with wall thickening and poor fixation of the colon. Because of the rapid progression of the abdominal pain, torsion of the small intestine or internal hernia was suspected, and emergency laparotomy was performed. The surgical findings included venous congestion in the jejunum and a 13 × 16 × 65 mm Landzert fossa, and we diagnosed the patient as having a left paraduodenal hernia. There was no evidence of intestinal necrosis, and the operation was completed with the closure of the hernia gate. Postoperatively, the patient developed paralytic ileus and required conservative treatment. The patient was discharged from the hospital 24 days after the operation without symptom recurrence. Although it has been recently reported that the finding of a sac-like appearance on CT images is useful for the diagnosis of paraduodenal hernia, it was difficult to detect in our patient preoperatively. It was suggested that it was necessary to recognize left paraduodenal hernia as one of the differential indicators of acute abdomen.</p>

DOI： 10.11164/jjsps.58.7_978

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Language：Japanese   Publisher：東京医学社  

DOI： 10.24479/ps.0000000319

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Pediatric Surgery International  

Development of minimally invasive cancer immunotherapy using anti-disialoganglioside GD2 antibody-producing mesenchymal stem cells for a neuroblastoma mouse model.
PURPOSE: Mouse IgG anti-disialoganglioside GD2 antibody-secreting mouse mesenchymal stem cells (anti-GD2-MSCs) were developed, and their anti-tumor effects were validated in an in vivo neuroblastoma mouse model. METHODS: Anti-GD2 antibody constructs were generated, incorporating FLAG-tagged single-chain fragment variables against GD2 fused to a linker sequence, and a fragment of a stationary portion was changed from human IgG to mouse IgG and GFP protein. The construct was lentivirally introduced into mouse MSCs. A syngeneic mouse model was established through the subcutaneous transplantation of a tumor tissue fragment from a TH-MYCN transgenic mouse, and the homing effects of anti-GD2-MSCs were validated by In vivo imaging system imaging. The syngeneic model was divided into three groups according to topical injection materials: anti-GD2-MSCs with IL-2, IL-2, and PBS. The tumors were removed, and natural killer (NK) cells were counted. RESULTS: Anti-GD2-MSCs showed homing effects in syngeneic models. The growth rate of subcutaneous tumors was significantly suppressed by anti-GD2-MSCs with IL-2 (p < 0.05). Subcutaneous tumor immunostaining showed an increased NK cell infiltration in the same group (p < 0.01). CONCLUSION: Anti-GD2-MSCs using mouse IgG showed a homing effect and significant tumor growth suppression in syngeneic models. Anti-GD2-MSC-based cellular immunotherapy could be a novel therapeutic strategy for intractable neuroblastoma.

DOI： 10.1007/s00383-022-05310-z

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Language：English   Publisher：Pediatric Blood and Cancer  

DOI： 10.1002/pbc.30155

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Language：Japanese  

Language：English   Publisher：Molecular Metabolism  

Objective: Chronic liver diseases often involve metabolic damage to the skeletal system. The underlying mechanism of bone loss in chronic liver diseases remains unclear, and appropriate therapeutic options, except for orthotopic liver transplantation, have proved insufficient for these patients. This study aimed to investigate the efficacy and mechanism of transplantation of immature hepatocyte-like cells converted from stem cells from human exfoliated deciduous teeth (SHED-Heps) in bone loss of chronic liver fibrosis. Methods: Mice that were chronically treated with CCl4 received SHED-Heps, and trabecular bone density, reactive oxygen species (ROS), and osteoclast activity were subsequently analyzed in vivo and in vitro. The effects of stanniocalcin 1 (STC1) knockdown in SHED-Heps were also evaluated in chronically CCl4 treated mice. Results: SHED-Hep transplantation (SHED-HepTx) improved trabecular bone loss and liver fibrosis in chronic CCl4-treated mice. SHED-HepTx reduced hepatic ROS production and interleukin 17 (Il-17) expression under chronic CCl4 damage. SHED-HepTx reduced the expression of both Il-17 and tumor necrosis factor receptor superfamily 11A (Tnfrsf11a) and ameliorated the imbalance of osteoclast and osteoblast activities in the bone marrow of CCl4-treated mice. Functional knockdown of STC1 in SHED-Heps attenuated the benefit of SHED-HepTx including anti-bone loss effect by suppressing osteoclast differentiation through TNFSF11–TNFRSF11A signaling and enhancing osteoblast differentiation in the bone marrow, as well as anti-fibrotic and anti-ROS effects in the CCl4-injured livers. Conclusions: These findings suggest that targeting hepatic ROS provides a novel approach to treat bone loss resulting from chronic liver diseases.

DOI： 10.1016/j.molmet.2022.101599

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

症例は6歳男児.胸腹部痛と発熱を主訴に当院小児科入院.入院時血液検査所見では,CRPおよびLDHの上昇を認めた.入院後に腹痛増悪を認めたため当科紹介となった.腹部CTでは壁肥厚を伴った小腸の左側偏位および結腸の固定不良が示唆された.急激な腹痛の進行から小腸軸捻転や内ヘルニアの可能性を考え緊急手術を行った.手術所見では,空腸の静脈鬱滞所見および13×16×65mmのLandzert窩を認め左傍十二指腸ヘルニアと診断した.腸管血流は保たれておりヘルニア解除およびヘルニア門の閉鎖を行った.左傍十二指腸ヘルニアはTreitz靱帯周囲の腹膜窩に腸管が嵌入して生じる内ヘルニアであり,診断に有用なCT所見としてのsac-like appearanceを術後の再評価で認めた.今回,我々は術前診断困難であった左傍十二指腸ヘルニアの1例を経験したので,若干の文献的考察を加えて報告する.(著者抄録)

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000288

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Language：Japanese  

Language：Japanese   Publisher：(株)東京医学社  

1歳男児。出生前より陰嚢腫大を指摘され、生後11ヵ月時のMRIにて隔壁を有する嚢胞性病変が認められ、当院へ紹介となった。初診時、左鼠径部～陰嚢は著明に腫脹し、超音波検査で精系水瘤よりリンパ管腫が疑われた。漢方治療が行われたが、病変は縮小せず、1歳4ヵ月時にMRI検査を行ったところ、陰嚢内～左鼠径管～後腹膜腔に連続する嚢胞様腫瘤が認められた。以後、診断と治療目的で腹腔鏡手術が行われ、手術所見では大網嚢腫の陰嚢内への陥入が確認され、大網嚢腫の開窓、陰嚢底を結紮した。その結果、病理組織学的に大網嚢腫はリンパ管奇形と診断された。目下、術後3年経過で再発はみられていない。

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000246

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Publisher：東京医学社  

DOI： 10.24479/peri.0000000357

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Language：Japanese   Publisher：日本小児PD・HD研究会  

腹膜透析(PD)カテーテル留置における腹腔鏡手術は近年増加傾向にある。腹腔鏡補助下PDカテーテル留置術は開腹術に比べて術後に透析液リークを起こす危険性が高いのではないかとの仮説を立て、挿入法によるPDカテーテル関連合併症を後方視的に評価した。2004～2021年の18年間に当院で維持PDを導入した末期腎不全患者34例(32名)を対象とした。腹腔鏡未使用群19例、使用群15例であり、腸管損傷、出血についてはいずれの群でも認めなかった。PDカテーテル閉塞/位置異常については使用群が0例であったのに対し、未使用群が6例(31.5%)と有意に多かった。鼠径ヘルニア/陰嚢水腫/Nuck管水腫についても使用群2例(13.3%)に対し未使用群9例(47.3%)と有意に多かった。透析液リークは5例で発症し、うち4例が臍部を中心とした皮下への漏出であった。全例が腹腔鏡術後に発生しており、さらに5例中4例(80%)が単孔式ポートを使用していた。PDカテーテル関連合併症において、術式間(腹腔鏡未使用群と使用群)でのPDカテーテル留置術後の合併症に差がみられた。

Publisher：東京医学社  

DOI： 10.24479/ps.0000000230

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Language：Japanese  

Language：Others   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

Purpose: This study aims to clarify the relationship between changes in skeletal muscle mass during treatment and prognosis of pediatric malignant solid tumors. Methods: Patients with pediatric malignant solid tumors who were treated at Kyushu University Hospital from 2007 to 2017 were divided into two groups: the progression-free survival (PFS) group and the relapse/death (R/D) group; the psoas major muscle volume (PMV) was then compared. We also measured the PMV and psoas muscle area (PMA) of pediatric patients with no complications who underwent surgery for acute appendicitis (control) and compared the values with those of patients with malignant tumors. Results: No significant differences were observed in the PMV and PMA between patients with appendicitis and those with malignant tumors. Significant differences were found in the rate of change in PMV between the PFS (1.424) and R/D groups (1.071) (P = 0.0024). When the cut-off value of the rate of change in the PMV was 1.20, patients whose rate of change in PMV was ≥ 1.20 had longer PFS (P = 0.0231) and overall survival (P = 0.0229) than those whose rate of change was < 1.20. Conclusion: Pediatric patients with malignant solid tumors and increased skeletal muscle mass during treatment have a good prognosis.

DOI： 10.1007/s00383-022-05225-9

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Language：Others   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

DOI： 10.1007/s00383-022-05238-4

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Language：Others   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

Immaturity of ganglia (IG), is a rare entity of an allied disorder of Hirschsprung’s disease. We reviewed our IG cases to provide further pathological insight into IG. The clinical data and pathological findings of IG cases in our department from 2011 to 2020 were examined. Hematoxylin and eosin (HE) staining and immunostaining for HuC/D, BCL-2, SOX10, and CD56 were performed on full-thickness specimens. Sufficient clinical data and pathological specimens were available in five cases during the study period. The patient profiles were as follows: four term infants and one preterm infant with initial symptoms of abdominal distension or vomiting; all cases underwent ileostomy at a median age of 2 days and stoma closure at a median age of 5 months. Although the interpretation of HE staining was difficult, immunostaining for HuC/D and SOX10 clearly distinguished ganglion cells from glial cells. The number of ganglion cells in the IG group was significantly greater than that in the control group (p < 0.05), while the number of enteric glial cells and total area of the myenteric nerve plexus did not differ. The finding of the increased number of ganglion cells in a normal-size myenteric plexus is a novel feature of IG that contributes to its accurate diagnosis.

DOI： 10.1007/s00580-022-03390-w

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

PURPOSE: The prenatal diagnosis of the stomach position in congenital diaphragmatic hernia (CDH) has been a reliable prognostic factor, but few studies have focused on the postnatal position. We therefore evaluated the significance of the nasogastric (NG) tube position just after birth. METHODS: The Japanese CDH Study Group database enrolled 1037 CDH neonates over 15 years. In our multicenter retrospective study, 464 cases of left-sided isolated CDH with prenatal diagnoses were divided into two groups: NG tube below the diaphragm (BD; n = 190) or above the diaphragm (AD; n = 274). The primary outcome was the 90-day survival rate, and the secondary outcomes were mechanical ventilation duration, hospitalization duration, and recurrence rate. RESULTS: The BD group had a significantly higher 90-day survival rate (98.4 vs. 89.4%, p < 0.001), shorter mechanical ventilation (11 vs. 19 days, p < 0.001), shorter hospitalization (38 vs. 59 days, p < 0.001), and lower recurrence rate (p = 0.002) than the AD group. A multivariate analysis showed that BD (adjusted odds ratio, 3.68; 95% confidence interval 1.02-13.30) was a favorable prognostic factor for the 90-day survival. CONCLUSION: The assessment of the NG tube position revealed it to be a reliable prognostic factor of left-sided isolated CDH. Therefore, it should be included as a routine assessment.

DOI： 10.1007/s00383-022-05226-8

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Purpose: The number of accessible central veins (CVs) affects the prognosis of patients with intestinal failure (IF). The loss of residual CVs should be avoided. We, therefore, evaluated the efficacy of a new CV catheter-exchange technique using a subcutaneous fibrous sheath (FS) in pediatric IF patients. Methods: We retrospectively collected the CV catheter (CVC) data of pediatric IF patients managed from January 2009 to December 2019. The data were divided into two groups; Groups 1 (CVCs placed with the FS method) and Group 2 (CVCs placed by the primary or another insertion). The main outcome was the CVC indwelling time. Results: Eighty-five CVCs were analyzed. The FS method was attempted in 47 cases and succeeded in 40 (85%). No significant difference was observed between the groups regarding characteristics. A log-rank test revealed an equivalent CVC indwelling time between the two groups (Group 1: 268 [126–588] days vs. Group 2: 229 [126–387] days, p = 0.256). Conclusions: The FS method is highly recommended for pediatric IF patients, as its attempt showed a high success rate with an indwelling time equivalent to primary insertion. The FS method leads to the prolonged use of a single CV and thereby contributes to improving the outcomes of pediatric IF patients.

DOI： 10.1007/s00383-022-05233-9

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Language：English   Publisher：(一社)日本癌学会  

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Language：Japanese   Publisher：(一社)日本小児栄養消化器肝臓学会  

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Language：Japanese  

Language：Japanese   Publisher：金原出版(株)  

症例は3歳女児で、1ヵ月前から咳嗽があり、近医にて内服薬処方で経過観察されていた。3日前から発熱を呈し、炎症反応の高値も認めたため入院となった。胸部X線および胸部CT所見から重症細菌性肺炎と判断し、スルバクタム/アンピシリン+アジスロマイシンで加療を開始した。入院3日目に静脈血、鼻咽頭培養からHaemophilus influenzaeが検出されたため、抗菌薬をセフォタキシムに変更したが、入院8日目には胸部X線で右下肺野の浸潤影の増悪あり、胸部CTでも右胸膜の肥厚と胸水の貯留を認めた。胸腔穿刺によるドレナージを施行するも、胸水は粘性が高くほとんどドレナージができず、入院10日目から3日間ウロキナーゼの胸腔内注入療法を施行した。3回目の投与後には解熱と呼吸状態の改善を認め、画像検査でも肺炎・胸水の改善を認めた。その後、第23病日に抗菌薬投与を終了し、第30病日に退院となった。

Language：Japanese   Publisher：(一社)日本小児外科学会  

腸閉塞で発症し、虫垂炎による炎症の波及が原因であった新生児症例を経験した。症例は日齢18の男児で嘔吐と腹部膨満を主訴に前医を受診し、腸閉塞疑いで同日当院へ搬送された。腹部造影CTで回腸での腸閉塞と診断し、同日緊急開腹術を施行した。回腸末端から30cm口側に索状物による絞扼を認めた。また膿苔に覆われ根部と先端以外が広範囲に穿孔した虫垂を認め、虫垂炎の炎症の波及により索状物が形成されたと考えられた。虫垂は根部で切離した。絞扼部位の回腸は狭窄の程度が強く、切除して端々吻合した。術後経過は良好で術後16日に退院した。新生児虫垂炎は非常に稀である。新生児症例は虫垂炎の典型的症状に乏しく、本症例でも腸閉塞を契機に発見されたがそれ以前に虫垂炎を示唆する症状は全く認められなかったが、病理所見から新生児虫垂炎と診断した。開腹歴のない新生児の腸閉塞の原因として虫垂炎が背景にある可能性を考慮する必要があると思われた。(著者抄録)

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DOI： 10.24479/ps.0000000176

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Language：Others   Publishing type：Research paper (scientific journal)   Publisher：Wiley  

Gastrointestinal bleeding or perforation following influenza infection is rare. We encountered a pediatric case of hemorrhagic duodenal ulcer following influenza A infection. The patient was a 1-year and 4-month-old boy who was diagnosed with influenza A infection and treated with laninamivir octanoate. After inhalation, he had diarrhea, poor appetite, and melena. The next day, he had hematochezia and developed hemorrhagic shock. Contrast-enhanced computed tomography showed extravasation in the descending part of the duodenum. Esophagogastroduodenoscopy revealed spurting bleeding from a Dieulafoy's lesion on the oral side of the major papilla, and he underwent hemostasis by clipping. From the bulb to the descending part of the duodenum, the mucosa appeared atrophic with spotty redness on the circular folds and multiple and irregularly shaped erosions. Almost all mucosal lesions had healed by the eighth day, and he was monitored as an outpatient for more than one year without re-bleeding. Intestinal ischemia, viral invasion, and drug reaction of laninamivir octanoate may be involved in duodenal mucosal injury. Acute duodenal ulcers may occur in children with influenza infection, especially young children.

DOI： 10.1002/deo2.155

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Introduction: Infants with congenital diaphragmatic hernia (CDH) are at risk of neurodevelopmental disabilities. This study aimed to investigate the association between lung to thorax transverse area ratio (LTR) and neurodevelopmental outcomes at 3 years of age in fetuses with CDH. Methods: We performed a retrospective study of infants with prenatally diagnosed isolated left-sided CDH born in Kyushu University Hospital between 2008 and 2016. We examined the association between prenatal ultrasound findings including LTR and development quotient (DQ) at 36 to 42 months of chronological age. Results: We identified 34 live-born fetuses with isolated left-sided CDH, of which 30 survived and four died before discharge. The median LTR in the survivors was higher than in the non-survivors (p < 0.01). Among the survivors, 26 had available data on LTR (median 0.12, range 0.08–0.18) and overall DQ at 3 years of age (93, 61–112). Their median gestational age and birth weight were 37.6 (range 34.4–39.1) weeks and 2716 (2.256–3494) grams, respectively. There was no significant difference in overall DQ scores between the two groups divided according to the median LTR values (p = 0.62). LTR values were not associated with overall DQ scores after adjusting for gestational age (p = 0.39). In addition, no association was observed between LTR values and any subscale DQ scores. Conclusion: In fetuses with isolated left-sided CDH, prenatal LTR predicts the mortality but not neurodevelopmental outcomes at 3 years of age.

DOI： 10.1016/j.earlhumdev.2022.105598

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BACKGROUND: Immaturity of ganglia (IG), an allied disorder of Hirschsprung disease (AD-HSCR), develops as neonatal ileus, but the dysmotility spontaneously resolves after several months. The diagnosis of IG using HE staining is often difficult. We herein report a new pathological finding of IG called the 'palisading-like pattern', which may be helpful for improving the diagnostic accuracy. METHODS: Cases of IG that were managed over the past 28 years were retrospectively reviewed. We investigated the clinical course and pathological findings for Hematoxylin-Eosin (HE) staining. The conventional diagnostic criteria for IG were (1) a normal or slightly increased number of ganglion cells and (2) ganglion cells with small nuclei. RESULTS: Among the 155 cases, 28 were diagnosed with IG, and 10 were retrospectively confirmed by HE staining. A palisading-like pattern was confirmed at the time of the initial ileostomy (median age, 2.5 days), and the palisading-like pattern had completely disappeared by the time of stoma closure (median age, 215 days) in all 10 cases. A palisading-like pattern is not present in other diseases. CONCLUSIONS: Even if immunostaining data are not available for a further analysis, the detection of a palisading-like pattern on HE staining makes an accurate diagnosis possible. LEVEL OF EVIDENCE: LEVEL IV.

DOI： 10.1016/j.jpedsurg.2022.02.035

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Language：Japanese   Publisher：(株)東京医学社  

2008年～2019年に縦走連合筋および外肛門括約筋をすべて縦切開するPSARP原法にて根治術をおこなった中間位鎖肛17例(男児13例、女児4例)を対象に、術後1年および4年の排便機能を直腸肛門奇形研究会評価法にて評価し、注腸造影検査における客観的評価点について比較検討した。病型は、直腸腟前庭部瘻3例、直腸球部尿道瘻10例、無瘻孔4例であり、手術時年齢は8.4ヵ月であった。臨床的評価の合計は、術後1年で平均5.6点、術後4年で平均5.5点であり、いずれも90%以上の症例がgoodまたはfairであった。個々の項目では便秘、失禁は経時的に改善した症例が多かったが、汚染は増悪した症例を認めた。客観的評価として直腸開大率は全例が60%未満であった。直腸会陰曲は3例が良好、9例が不良であった。直腸検査時の造影剤の漏れは11例で確認され、直腸粘膜脱を3例で認めた。

Language：Japanese   Publisher：The Japanese Association of Rehabilitation Medicine  

<p>腸管不全は，長期の静脈栄養に伴うカテーテル感染症，血栓閉塞，腸管不全関連肝障害などにより予後は不良である．こうした患者の治療にあたり，残存腸管機能を最大限に活用し，合併症を極力抑えつつ，静脈栄養依存度の軽減ないしは離脱をめざすことになるが，この実現には，関連診療科および多職種による，移植医療を含んだ包括的・計画的な治療体制が必要であり，これを腸管リハビリテーションプログラム（intestinal rehabilitation program：IRP）と呼んでいる．IRPは欧米の主に小腸移植実施施設を中心に開始され，その有用性から広まってきた治療体制であるが，わが国では，まだ十分なIRPの体制確立には至っていないのが現状であり，今後の大きな課題である．</p>

DOI： 10.2490/jjrmc.59.603

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese  

Language：Others   Publishing type：Research paper (scientific journal)   Publisher：Wiley  

Abstract

Objectives

Pyriform sinus cyst (PSC) and pyriform sinus fistula (PSF) is a rare congenital malformation that arises from the third or fourth branchial structure. In our study, we describe the safety and the utility of endoscopic electrocauterization against PSC/PSF.

Methods

We retrospectively reviewed the records of patients who underwent endoscopic electrocauterization for PSC/PSF at our hospital. The internal opening of the fistula was identified under general anesthesia using a flexible endoscope (XQ‐260 or H‐290; Olympus, Tokyo, Japan), and the DualKnifeJ (KD‐655L; Olympus) was used to ablate the internal opening.

Results

We experienced three PSF and three PSC patients. The postoperative course was uneventful in all cases. The patients declared no pain in the neck, and there were no cases showing recurrent nerve paralysis. Five in six cases (83%), the closure of fistula was archived in the first cauterization. One case (16.6%) required repeated cauterization. No recurrence was found during the follow‐up period ( median: 1 year) in any cases.

Conclusions

Owing to its rarity in neonates, the diagnosis and treatment of PSC remains complicated and not clearly described. Complete removal of the fistula and the cyst with or without affected thyroid tissue was previously the most commonly used treatment. From our experience, we believe that endoscopic electrocauterization can be the first choice not only for PSF but also for neonatal PSC. In conclusion, endoscopic electrocauterization is feasible even for neonatal PSC. Further investigations including multicenter analyses are needed.

DOI： 10.1002/deo2.128

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Japanese Journal of Clinical Oncology  

BACKGROUND: In Japan, allogeneic hematopoietic stem cell transplantation is widely performed for recurrent neuroblastomas. This retrospective study aimed to investigate the prognosis of recurrent neuroblastoma in Japan and explore the effectiveness of allogeneic hematopoietic stem cell transplantation. METHODS: Clinical characteristics and data on the treatment of patients with high-risk neuroblastoma who experienced first progression between 2003 and 2010 after attaining complete remission or partial remission were collected from hospitals participating in the Japanese Neuroblastoma Research Group. RESULTS: Data from 61 patients who fulfilled these criteria were collected. The median interval from disease onset to first progression was 19 months (range, 7-65 months), whereas the median observation time of the surviving patients was 18 months (range, 1-69 months). All patients were treated with chemotherapy, where 22 and 3 patients received allogeneic and autologous hematopoietic stem cell transplantation, respectively. Seven patients were alive in second complete remission, and 39 died, including two in complete remission. The 3-year progression-free survival and overall survival rates were 15.3% (SE: 6.1%) and 16.9% (SE: 6.5%), respectively. For patients with allogeneic hematopoietic stem cell transplantation, the 3-year progression-free survival and overall survival were 28.3% (standard error, 12.0%) and 24.3% (standard error, 11.5%), respectively, and for patients without allogeneic hematopoietic stem cell transplantation, the 3-year progression-free survival and overall survival were 6.0% (standard error 5.5%) and 12.0% (standard error 7.6%), respectively. The duration of initial remission (≥ 18 months) and implementation of allogeneic hematopoietic stem cell transplantation were independently predictive of progression-free survival (P = 0.002 and P = 0.017), whereas for overall survival, only allogeneic hematopoietic stem cell transplantation was predictive (P = 0.012). CONCLUSION: Although allogeneic hematopoietic stem cell transplantation contributed to some improvement in prognosis, it was insufficient.

DOI： 10.1093/jjco/hyac007

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Language：English   Publisher：Oxford University Press  

日本国内での再発高リスク神経芽腫患児の臨床転帰と同種造血幹細胞移植(HSCT)の有効性および予後予測に関与する因子について検討した。2003年4月～2010年12月までの期間内に、日本神経芽腫研究グループ(JNBSG)の参加施設で治療を受けた再発高リスク神経芽腫患者のうち、選定基準を満たした小児患者61例(男児35例、女児26例、発症月齢13～81ヵ月)を調査対象とした。その結果、発症から初回進行をきたすまでの期間中央値は19ヵ月で、うち生存例の観察期間中央値は18ヵ月であった。なお、全例とも化学療法を受けており、22例には同種HSCT、3例には自家HSCTが施行され、うち7例では再寛解により生存転帰が得られたが、完全寛解が認められた2例を含む39例は死亡例であった。また、3年無増悪生存率(PFS)と全生存率(OS)は15.3%および16.9%で、同種HSCT施行患者と未施行患者の3年PFSおよびOSは、28.3%と24.3%、6.0%と12.0%で、多変量解析により、同種HSCTはPFSとOSの改善に寄与することが示唆された。これらの治療成績から、同種HSCTは再発高リスク神経芽腫患児の予後を改善させるものの、十分な効果は得られないことが示唆された。

Language：Japanese   Publisher：(一社)日本移植学会  

慢性特発性偽性腸閉塞症の30歳代男性に対する脳死単独小腸移植後の栄養管理について報告した。本例は既に初診時に中心静脈ルートがなく周術期管理が困難であった。鬱滞した自己胃・十二指腸内容はGJチューブを用いて排液・減圧し、経腸栄養はチューブ腸瘻からグラフト腸管への直接注入を中心として行った。チューブ腸瘻を介した経腸栄養は比較的早期に安定し、単独小腸移植後60日に静脈栄養から離脱可能となった。また、自己腸管からの排液であるGJチューブ排液自体をチューブ腸瘻を介してグラフト腸管へ注入し、補液から離脱できた。

Publisher：東京医学社  

DOI： 10.24479/ps.0000000098

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Publisher：Journal of Applied Physics  

The intermetallic compound Fe 1 - xCo xSi has a helical magnetic order for 0.05 < x < 0.8, whereas its orbital angular momentum contributing to the occurrence of a Dzyaloshinskii-Moriya interaction has not yet been verified. We applied soft x-ray magnetic circular dichroism spectroscopy on the Fe L 2, 3 and Co L 2, 3 edges below T c for Fe 0.75Co 0.25Si such that their orbital magnetic moments m orb were evaluated independently. The dichroic signals provide direct experimental evidence that m orb for both Fe and Co is coupled in a parallel manner with each spin counterpart m spin. The ratio of m orb to m spin is independently estimated as m orb/ m spin ∼ 3% for Fe and 9% for Co. By comparing the average of the two m spin values with the saturated magnetization using a commercial superconducting quantum interference device (SQUID) magnetometer, the hole number in the d bands for both Fe and Co is roughly estimated as approximately 1.5. This suggests that Fe and Co should be arranged closely for stabilizing the ferromagnetic moments.

DOI： 10.1063/5.0086493

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Publisher：Physical Review B  

We report that the magnetostriction (MS) effects occur in a paramagnetic state of a chiral magnet CrNb3S6. Through a series of experimental tests at room temperature, structural changes were observed at the level of a unit cell. The structural parameters are dependent of the strength and direction of magnetic field (H) even at temperature excessively higher than the magnetic ordering temperature Tc of 127 K. The present paramagnetic MS prominently appeared under H∥ the ab plane (easy plane) as opposed to under H∥ the c axis. Features observed in the paramagnetic MS effect significantly differ from those of the spontaneous MS in the vicinity of Tc [Phys. Rev. B 102, 014446 (2020)2469-995010.1103/PhysRevB.102.014446]. In this material, the orbital angular momentum L of Cr originates from the hybridization between Cr and Nb, and L is strongly coupled with the crystal structure [Phys. Rev. B 99, 174439 (2019)2469-995010.1103/PhysRevB.99.174439]. The present study clarified that the symmetry of the CrS6 octahedron is sensitive to H even at room temperature. The paramagnetic spin-orbit coupling should induce the distortion of CrS6 octahedron, resulting in the changes in Cr-Nb(4f) distance via the change in the hybridization between Cr-a1g and Nb-4dz2 orbitals.

DOI： 10.1103/PhysRevB.105.104412

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Language：Others   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1016/j.epsc.2022.102195

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Language：Japanese   Publisher：(一社)日本小児外科学会  

症例は7歳男児。先天性横隔膜ヘルニア術後にて外来経過観察中の患児。1歳6ヵ月時、スクリーニングの腹部エコーで肝下面に接する異所性脾を右季肋部に確認した。腹部症状、脾臓の遊走、脾機能亢進、脾梗塞などを認めないため経過観察の方針とした。その後も定期的に画像検索を継続していたが、7歳時のMRIにて骨盤内への脾臓の移動を認め、脾臓が原因と思われる膀胱圧迫症状も出現したため手術加療の方針となった。腹腔鏡下に手術を開始したが、癒着により脾動静脈根部の観察が困難であったため、上腹部に小開腹を追加し、腹腔鏡補助下に脾固定術を施行した。固定部位は左背側部とし、固定方法はretroperitoneal pouch法を選択した。術後は造影CTにて脾腫や血流障害を示唆する所見もなく経過良好である。我々が検索しえた範囲では、先天性横隔膜ヘルニア術後の遊走脾に対し腹腔鏡補助下脾固定術を施行した報告は本邦では初であり、文献的考察を加えて報告する。(著者抄録)

Language：Japanese   Publisher：(一社)日本小児血液・がん学会  

背景:神経芽腫の新しい治療戦略として,NK細胞の養子免疫治療が報告されているが,高活性化かつ高用量のNK細胞製剤の報告は未だにない.共同研究施設で開発された,ヒト末梢血から増幅製造されるoff-the-shelf型のNK細胞様製剤に関して報告する.方法:高活性化NK様細胞(GAIA-102)は,CD3陽性細胞を除去した末梢血単核球を接着培養の条件下で14日間培養して作成した.3D培養を行なったIMR-32(神経芽腫細胞株)を蛍光標識GAIA-102と共培養し,GAIA-102の腫瘍塊への浸潤を観察し,その細胞傷害活性をフローサイトメトリーを用いて観察した.結果:スフェロイドを用いた3Dアッセイでは,GAIA-102は,primary NK細胞,抗GD2抗体単独,または両方の組み合わせと比較して,IMR32の腫瘍塊に対する高い細胞傷害活性を示した.ライブイメージングにより,GAIA-102はスフェロイドに対して効率的に浸潤し,スフェロイドの腫瘍細胞が死滅することを確認した.結論:GAIA-102は,3D培養を行なった神経芽腫スフェロイドに対して高い浸潤能と細胞傷害活性を示した.GAIA-102は,再発難治神経芽腫の新規治療に有望な選択肢であると考え,現在,再発性または難治性の神経芽細胞腫の臨床試験を準備している.(著者抄録)

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Wiley  

BACKGROUND: In November 2011, rotavirus (RV) vaccine was launched in Japan as a voluntary vaccination to prevent RV-associated gastroenterocolitis. We examined the characteristics of intussusception following RV vaccination in our two centers. METHODS: We investigated intussusception patients <16 years old from January 2006 to September 2020. Patients were categorized according to the period (before [Group A] or after the introduction of arbitrary RV vaccination [Group B]). The patient characteristics and treatment of intussusception were retrospectively investigated. RESULTS: During the study period, 560 patients (group A, n = 233; group B, n = 327) were identified. The distribution of patients who were 0-6 months old was not significantly different between the groups (group A, n = 12, 5.2%; group B, n = 18, 5.5%). Among these 18 patients in Group B, 7 were vaccinated against RV, and 10 were not. One patient was excluded due to incomplete data. On comparing patients with and without RV vaccination, the mean age at the onset of intussusception was 3.3 ± 0.4 versus 4.0 ± 0.3 months (P = 0.19), the mean interval from the onset to treatment was 7.5 ± 2.4 versus 16.0 ± 2.2 h (P = 0.03), the time of the contrast enema for treatment was 9.1 ± 3.3 versus 7.7 ± 2.8 min (P = 0.76), and the final pressure of the contrast enema was 92.5 ± 4.4 versus 92.2 ± 4.4 cmH2 O (P = 0.97). CONCLUSIONS: Arbitrary RV vaccination did not influence the age distribution of intussusception, and the interval from the onset to treatment was significantly shorter in the patients with RV vaccination than in those without it. Recognizing the presence of intussusception following RV vaccination enables accurate treatment.

DOI： 10.1111/ped.15332

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Background: Core-needle biopsy (CNB) is used less frequently for the diagnosis of tumors in pediatric patients. In this report, the utility and safety of CNB for pediatric patients are described. Methods: The medical records of patients who underwent CNB at the Department of Pediatric Surgery, Kyushu University Hospital from April 2020 to November 2021 were retrospectively reviewed. A 14 G or 16 G BARDMISSION Disposable Needle Instrument was used. For the diagnosis of neuroblastoma, a 14 G needle was selected; for the diagnosis of other tumors a 16 G needle was selected. Results: During the above period 17 CNBs were performed in 17 patients, and the median patient age was 8 years (range, 15 days–19 years). The pathological diagnoses of the tumors were as follows: neuroblastoma, n = 6; lymphoma, n = 3; hepatoblastoma, n = 2; and others, n = 6. The quantity and quality of all tumor samples obtained by CNB was sufficient to make a diagnosis. The postoperative course after CNB was uneventful in most cases, with the exception of one case of hepatoblastoma (pseudoaneurysm). Conclusions: Core-needle biopsy is useful for pediatric patients. Sufficient tumor specimens were able to be obtained in all cases, irrespective of the type of tumor, and an accurate diagnosis could be made.

DOI： 10.1111/ped.15228

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DOI： 10.1111/ped.15026

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Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>【背景】腸管不全患者の予後を規定する合併症の一つに肝障害があり、重症例では肝小腸移植が必要となる。しかし、わが国における脳死肝小腸同時移植はallocation systemからも現実的には困難であり、また、composite graftとして移植することも不可能であるため手技的優位性も感じにくい。わが国におけるこれまでの肝小腸移植はほぼ異時性移植であり、当科の経験症例からその課題について検討する。</p><p>【症例】症例は8歳10か月、体重11kgの女児。1歳時に微絨毛封入体病と確定診断され、TPN管理下においても体重は-3SD、身長は—5SDで推移した。7歳頃から肝障害の進行とともに出血傾向や病的骨折が出現。8歳9か月時にMELDスコア19点、小腸はstatus 2で脳死肝小腸同時移植の登録をしたが、急激な肝不全の進行により父親をドナーとする生体肝移植を施行、現在、TPN管理継続下に異時性脳死小腸移植待機中である。先行した肝移植において、のちの小腸グラフトを門脈系へ吻合することを念頭にduct-to-ductでの胆道再建を行った。現在、グラフト肝機能は保たれているが、著明な脂肪肝を呈している。</p><p>【結語】脳死肝小腸同時移植推進の議論と並行して、異時性肝小腸移植の治療戦略について、特に、先行移植すべき臓器選択とドナー選択、想定される手術手技と容認でき得る移植間隔など検討しておくべき課題は多い。</p>

DOI： 10.11386/jst.57.supplement_s215_2

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Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>Intestinal failure is a complicated medical condition resulting in the loss of absorptive area or in severe gastrointestinal bacterial overgrowth. Prolonged use of parenteral nutrition (PN) puts patients at risk of multiple complications, including catheter-related bloodstream infection (CRBSI), intestinal failure-associated liver disease (IFALD), and renal impairment. Intestinal rehabilitation programs (IRPs) have recently been recognized as playing a significant role in outcome improvement, care coordination, and complication prevention. We herein report an adult case of chronic idiopathic intestinal pseudo-obstruction (CIIP) that underwent isolated intestinal transplantation (ITx) from a deceased donor. Because the loss of central vascular access was his indication for ITx, a catheter for perioperative management was inserted in the azygous vein via intercostal veins punctured under ultrasound guidance. A total of 255 cm of small intestine with a 10-cm ascending colon graft was transplanted, and the proximal side was anastomosed to the native duodenum. Because the native gastro-duodenum dysmotility remained even after isolated ITx for CIIP, decompression of the native gastroduodenum with a gastro-jejunum tube via gastrostomy and feeding via tube enterostomy at the graft intestine were needed during the first four to five months after ITx. However, such management was effective in achieving full enteral feeding at an early stage with stable immunosuppressive therapy. Intervention with an IRP for management before ITx helped prevent CRBSIs and facilitated his continuing parenteral nutrition until ITx. Multidisciplinary collaboration on an IRP team is mandatory for the perioperative management of ITx patients.</p>

DOI： 10.11386/jst.57.1_125

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Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>本邦での小腸移植患者の原疾患の約4割が腸管運動機能障害であり、海外に比して多い。当科ではこれまでにHirschsprung病類縁疾患に対する脳死単独小腸移植を3例実施した。症例１：慢性特発性偽性腸閉塞(CIIPs)の29歳男性。長期静脈栄養の結果、カテーテル関連血流感染(CRBSI)を繰り返し、中心静脈ルートが枯渇したため、脳死小腸移植を施行した。術後native十二指腸の滞留により急性膵炎、経口摂取困難が続き、十二指腸グラフト小腸吻合術を追加した。移植後147日目に退院したが、5日後に急性拒絶を発症し、CMV感染も併発した。最終的にグラフト摘出まで施行したが移植後1年2か月で死亡した。症例２：CIIPsの36歳男性。頻回のCRBSIと中心静脈ルート枯渇のため、脳死小腸移植を施行した。術後、graft腸管に留置していた栄養チューブが胃に迷入し、栄養内服管理が困難となったため、腸瘻再造設を要した。移植後141日に退院。移植後2年が経過し、電解質補正は要するが、これまで拒絶反応はなく、stoma, device-freeとなっている。症例３：Hypoganglionosisの19歳女性。新生児期から腸管運動機能障害を認め、頻回のCRBSIと中心静脈ルート枯渇のため、脳死小腸移植を施行した。栄養摂取は全量経口で可能で術後76日目に退院。術後4ヶ月経過し、1度も拒絶所見は認めていない。各例native腸管の機能的差異から、消化管再建や栄養・薬剤投与ルートの確保に工夫が必要であった。比較検討して報告する。</p>

DOI： 10.11386/jst.57.supplement_s331_2

CiNii Research

Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>【背景】小児肝移植ではレシピエント門脈径が細く,しばしば門脈再建に苦慮する.当科の小児生体肝移植における門脈再建について検討し報告する.</p><p>【対象と方法】2008年～2021年に当施設の小児生体肝移植94例について診療録を基に後方視的に検討した.結果は中央値を示す.</p><p>【結果】94例の移植時年齢1.0歳,体重8.2kg,胆道閉鎖症は54例(57.4%).門脈再建法は,Ⅰ:本幹と直接吻合:23例,Ⅱ:臍静脈まで伸ばして吻合：3例,Ⅲ:間置graft:4例,Ⅳ:前壁graft patch:24例,Ⅴ:branch patch:40例に分けられた.Ⅲ,Ⅳ群で使用したvein graftは1例を除き27例はドナーIMVを使用した.Ⅰ群は年齢8.2歳,体重21.8kgと年長児が多く,Ⅱ群は新生児,Ⅲ,Ⅳ,Ⅴ群は順に年齢:0.7歳;0.8歳;1.0歳,体重：5.6kg;6.4kg;8.3kgと順に低年齢,低体重でいずれもⅠ群と有意差を認めた.レシピエントのnative門脈径はⅠ:10mm,Ⅱ:9mm,Ⅲ,Ⅳ:4mm,Ⅴ:6.0mmとⅢ,Ⅳ,Ⅴのplastyを要した症例は優位に細かった.また,Ⅲ,Ⅳ群ではそれぞれ2例,5例の門脈逆流症例を認めた.Ⅲ,Ⅳ,Ⅴ群はplasty後レシピエント門脈径は1.75倍,2.5倍,2.14倍となった.グラフト肝とレシピエント門脈の吻合径の差はⅠ:4mm,Ⅱ:1.5mm,Ⅲ:7mm,Ⅳ:3mm,Ⅴ:0mmでⅢ群のみ形成後も有意に差が大きかった.術後門脈合併症はⅠ,Ⅱ群になく,Ⅲ:2例(50%),Ⅳ:7例(29.2%),Ⅴ:3例(7.5%)に認めた.形成法による統計学的有意差は見られなかったが,最終的なレシピエント門脈の吻合径が細いほど合併症が多い傾向にあった.</p><p>【結語】前壁graft patchおよびbranch patchは十分な吻合径が得られていたが,IMVを用いた間置graftは細く,使用するvein graftや形成法を慎重に選択する必要がある.</p>

DOI： 10.11386/jst.57.supplement_s327_3

CiNii Research

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

2020年4月～2021年11月に小児外科でコアニードル生検が実施された患者の診療記録を後向きに評価し、小児におけるコアニードル生検の有用性および安全性について検討した。神経芽腫の診断には14G針が選択され、その他の腫瘍の診断には16G針が選択された。患者17例(中央値8歳)に計17回の生検が実施された。腫瘍の病理診断、神経芽細胞腫6例、リンパ腫3例、肝芽腫2例、その他6例であった。その結果、コアニードル腫瘍生検より得られたすべての腫瘍サンプルの量および質は、診断を下すのに十分であることが示された。コアニードル生検後の術後経過は、肝芽腫1例(偽動脈瘤)を除き、大部分の症例で良好であった。以上から、コアニードル生検は小児患者に有用であることが示された。

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

ロタウイルス(RV)ワクチン接種と腸重積症の関連を評価するため、RVワクチン任意接種導入後の腸重積症の発生率と特徴について検討した。2006年1月～2020年9月に2施設において腸重積症と診断された16歳未満の患児560例を、診断時期によりRVワクチン任意接種導入前(233例、グループA)と導入後(327例、グループB)へ分類した。早期発症型(生後6ヵ月未満)の患児はグループA 12例(5.2%)、グループB 18例(5.5%)と有意差はなく、RVワクチン任意接種は腸重積症の年齢分布に影響を及ぼさないことが示された。グループBの早期発症型患児18例のうち、データが不完全であった1例を除外し、RVワクチン接種を行った7例と行わなかった10例を比較した。その結果、腸重積症発症時の平均月齢はそれぞれ3.3±0.4ヵ月、4.0±0.3ヵ月(P=0.19)、発症から治療までの平均時間はそれぞれ7.5±2.4時間、16.0±2.2時間とワクチン接種例で有意に短く(P=0.03)、注腸整復の所要時間はそれぞれ9.1±3.3分、7.7±2.8分(P=0.76)、整復圧はそれぞれ92.5±4.4cmH2O、92.2±4.4cmH2O(P=0.97)であった。

Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>＜はじめに＞生体肝移植(LDLT)では通常、0.8%≦グラフト対体重比（GRWR）<4.0%を目標とするが、最低要件に関して一定の見解がない。GRWR基準値外症例のリスクを当科の経験から検討した。</p><p>＜対象＞1996年〜2022年の初回LDLT症例を5群(extra-small群(XS;GRWR≦0.6%;4例）、small群(S;0.6% ＜結果＞各群のGRWRの平均値は各々0.51%,0.74%,1.58%,3.09%,4.15%(XS:S:M:L:XL)であり、年長児ほどGRWRが低かった。(XS:S:M:L:XL=18.5歳:18.7歳:6.5歳:0.7歳:0.7歳)。グラフト生存率は75%,90%,84%,84%,100%(XS:S:M:L:XL)であり、グラフト血管イベントの発生率(XS:S:M:L:XL=0%:0%:13.8%:15.6%:25%)に有意差はなかった。L群の1例、XL群の2例が一期的腹壁閉鎖困難であった。</p><p>＜結語＞GRWRの高い症例では腹壁閉鎖困難例があるものの、基準値外でもグラフトロスに直結する変化は見られなかった。</p>

DOI： 10.11386/jst.57.supplement_s315_2

CiNii Research

Language：Japanese   Publisher：Japanese Study Group on Pancreatico biliary Maljunction  

DOI： 10.34410/jspbm.45.0_91

CiNii Research

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

食道閉鎖(EA)修復術の最も頻度の高い合併症である胃食道逆流症(GERD)の発症を予測することできれば、患者の経過観察に役立ち、GERDによる合併症の予防にも役立つ可能性がある。本研究では、1995～2018年に当院でEA修復術を施行し、1年以上の経過観察を行った患児65例のうち、Gross C型EA患者45例を後ろ向きに解析した。この45例を、胃底部噴門形成術を施行した噴門形成術群13例(男児30.8%)と胃底部噴門形成術を施行しなかった非噴門形成術群32例(男児65.6%)に分け、臨床的特徴を比較した。単変量解析の結果、妊娠週数、体重、出生前診断、羊水過多、再手術、食道のgap長が両群間で有意に異なっていた(P<0.05)。

Language：Others   Publishing type：Research paper (scientific journal)  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Biomolecules  

Neuroblastomas (NBs) exhibit broad and divergent clinical behaviors and tumor risk classification at diagnosis is crucial for the selection of an appropriate therapeutic strategy for each patient. The present study aimed to validate the clinical relevance of International Neuroblastoma Risk Group (INRG) prognostic and genomic markers in a Japanese NB cohort using a retrospective analysis. Follow-up data based on 30 common INRG queries in 605 NB cases diagnosed in Japan between 1990 and 2014 were collected and the genome signature of each tumor sample was integrated. As previously indicated, age, tumor stage, MYCN, DNA ploidy, the adrenals as the primary tumor site, serum ferritin and lactate dehydrogenase (LDH) levels, segmental chromosome aberrations, and the number of chromosome breakpoints (BP) correlated with lower survival rates, while the thorax as the primary tumor site and numerical chromosome aberrations correlated with a favorable prognosis. In the patient group with stage 4, MYCN non-amplified tumors (n = 225), one of the challenging subsets for risk stratification, age ≥ 18 months, LDH ≥ 1400 U/L, and BP ≥ 7 correlated with lower overall and event-free survival rates (p < 0.05). The genome subgroup GG-P2s (partial chromosome gain/loss type with 1p/11q losses and 17q gain, n = 30) was strongly associated with a lower overall survival rate (5-year survival rate: 34&#37;, p < 0.05). Therefore, the combination of the tumor genomic pattern (GG-P2s and BP ≥ 7) with age at diagnosis and LDH will be a promising predictor for MYCN-non-amplified high-risk NBs in patient subsets, in accordance with previous findings from the INRG project.

DOI： 10.3390/biom12010018

Web of Science

Scopus

PubMed

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BACKGROUND: Minimally invasive surgery (MIS) is appropriate for the treatment of some neuroblastomas (NBs); however, the indications and technical issues are unclear. This study aimed to clarify the current status of MIS for mediastinal NB in Japan. METHODS: Preliminary questionnaires requesting the numbers of neuroblastoma cases in which MIS was performed from 2004 to 2016 were sent to 159 Japanese institutes of pediatric surgery. Secondary questionnaires were sent to institutions with MIS cases to collect detailed data. RESULTS: One hundred thirty-four (84.2&#37;) institutions returned the preliminary questionnaire and 83 institutions (52.2&#37;) reported a total of 1496 operative cases. MIS was performed for 175 (11.6&#37;) cases. Among the 175 cases, completed forms of 140 patients were returned and 40 (male, n = 28; female, n = 12) cases had mediastinal NB. Fourteen patients received thoracoscopic biopsy, none were converted to thoracotomy. Twenty-eight patients received MIS for radical resection, none were converted to thoracotomy. Perioperative complications (Horner's syndrome) were recognized after radical resection in one (2.5&#37;) case. CONCLUSIONS: MIS was performed in a limited number of mediastinal NB cases. A thoracoscopic approach would be feasible for mediastinal NB.

DOI： 10.1007/s00383-021-04998-9

Language：Japanese  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Surgical Endoscopy  

BACKGROUND: Some neuroblastoma (NB) cases are suitable for minimally invasive surgery (MIS), but indication and technical issue are unclear. We assessed the current status of MIS for abdominal NB after mass screening period in Japan. METHODS: Preliminary questionnaires requesting the numbers of NB cases that underwent MIS from 2004 to 2016 were sent to 159 Japanese institutes of pediatric surgery. The secondary questionnaires were then sent to the institutions that reported MIS cases of NB in order to collect detailed data. RESULTS: One hundred and thirty-four (84.2&#37;) institutions responded to the preliminary questionnaires, and 83 (52.2&#37;) reported managing operative cases. The total number of operative cases was 1496. MIS was performed for 175 (11.6&#37;) cases, of which the completed forms of 140 patients were returned, including 100 abdominal NB cases. The male/female ratio was 51/49. Forty-seven cases underwent a laparoscopic biopsy, and 2 (4.3&#37;) cases were converted to laparotomy due to bleeding. Sixty-five cases underwent MIS for radical resection, and 7 (10.8&#37;) were converted to laparotomy. The reasons for open conversion were bleeding and severe adhesion. Regarding open conversion, there were no significant relationships between conversion and neo-adjuvant chemotherapy, biopsies, stage, size, or MYCN amplification. We found no relationship between resectability and vascular encasement in this study. There was relationship between the resected tumor size and the patients' height, which was expressed using the following formula: [Formula: see text] (x, patients height, y, tumor size; p = 0.004219, SE: 1.55566). Postoperative complications after radical resection were recognized in 7 (10.8&#37;) cases. CONCLUSIONS: MIS was performed in limited cases of abdominal NB. A laparoscopic biopsy with careful attention to bleeding is feasible. The resected tumor size was shown to correlate with the patients' height. Tumor size within 6 cm of maximum diameter can be resected safely.

DOI： 10.1007/s00464-021-08599-4

Web of Science

Scopus

PubMed

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Language：Others   Publishing type：Research paper (scientific journal)  

DOI： 10.1007/s00383-020-04806-w

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Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND/AIM: Malignant rhabdoid tumor (MRT) is a rare, aggressive neoplasm found in young children, caused by inactivation of a single gene, SNF5 (INI1, SMARCB1). MRT cases with multifocal tumors at diagnosis are categorized as synchronous MRT, often with a germline mutation of SNF5. The aim of this study was to establish new models useful in clarifying the biological basis of synchronous MRT. MATERIALS AND METHODS: We established two novel MRT cell lines, designated as KP-MRT-KS and KP-MRT-KSa, derived from different lesions and at a different time from a synchronous multifocal 7-month-old female MRT patient. RESULTS: Both cells showed typical morphology of MRT, with a compound genomic mutation in exons 2 and 5 of the SNF5 gene. The exon 2 mutation was found in the germline. CONCLUSION: These cell lines could serve as powerful tools for unveiling the molecular mechanism of refractory synchronous MRT.

DOI： 10.21873/anticanres.14636

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In the October 2018 issue of International Journal of Clinical Oncology, in the article titled “Results of a phase II trial for high-risk neuroblastoma treatment protocol JN-H-07: a report from the Japan Childhood Cancer Group Neuroblastoma Committee (JNBSG)” (volume 23, pages 965–973; doi: https ://doi.org/10.1007/s1014 7-018-1281-8.), by Hishiki T et al., there was a printing error in Figure 1. In the description for the schedule of the induction regime 05A1 and 05A3, the arrow that shows the day of pirarubicin administration was intended to indicate day 3, but in error had been published with the arrows indicating purarubicin administration on day 2. We sincerely apologize for this error in the schedule of the regime.

DOI： 10.1007/s10147-020-01752-4

Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Pulmonary hypoplasia is an important cause of morbidity and mortality in infants with congenital diaphragmatic hernia (CDH). This study aimed to verify our hypothesis that the abnormal development of bronchial cartilage as well as alveolar immaturity, might play a central role in hypoplasia of the lung in human CDH. METHOD: We retrospectively analyzed autopsied lungs from 10 CDH cases and compared with nine age-matched controls to assess the bronchial cartilage and alveolar maturity using morphological techniques. RESULT: Ki-67 and thyroid transcription factor-1 (TTF-1) expression in the alveoli significantly increased in bilateral lungs with CDH. The shortest distance from the bronchial cartilage to the pleura was significantly shorter in ipsilateral (left) lungs with CDH, showing a positive correlation with the radial alveolar count (RAC). Regarding the small bronchial cartilages less than 20 000 μm2 , the average cartilage area significantly decreased in left lungs with CDH, and tended to decrease in right lungs with CDH. In addition, cartilage around the bronchi less than 200 μm in diameter tended to be smaller in left lungs with CDH. In contrast, regarding the cartilage around the bronchi 200 to 400 μm in diameter, the ratio of the total cartilage area relative to the bronchial diameter tended to be higher in left lungs with CDH, although there was a large variation. CONCLUSIONS: These opposite directional cartilage abnormalities around the distal and more proximal bronchi support our hypothesis that abnormal development of bronchial cartilage might play an important role in the hypoplastic lung in CDH.

DOI： 10.1002/ppul.24799

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Abstract

Background

The fundamental treatment for patients with pediatric malignant mediastinal tumors is chemotherapy. Therefore, accurate diagnosis is essential for selecting the appropriate chemotherapeutic regimen. However, malignant mediastinal tumors occasionally cause respiratory distress, and biopsies under general anesthesia are dangerous for such patients as invasive mechanical ventilation can aggravate airway obstruction caused by mass effect. In this study, we reviewed our 10-year diagnostic experience to evaluate the efficacy of our practices and confirm a safe diagnostic protocol for future patients.

Methods

We retrospectively reviewed medical records of children with malignant mediastinal tumors diagnosed at Nagoya University Hospital from 2007 to 2018 who demonstrated respiratory distress. Respiratory distress included dyspnea, massive pleural effusion, wheezing, and hypoxemia owing to tumors. Data on sex, age at onset, primary symptoms, location of tumor, management strategy (especially the method of diagnosis and definitive diagnosis), clinical course, prognosis during the acute phase (within 3 months from the onset of respiratory symptoms), and long-term outcome were collected.

Results

Twelve pediatric patients met the review criteria. There were seven anterior mediastinal tumors and five posterior mediastinal tumors. All anterior mediastinal tumors were diagnosed via bone marrow smear, thoracentesis, or core needle biopsy while maintaining spontaneous breathing. Regarding posterior tumors, two patients were diagnosed via a core needle biopsy and lymph node excisional biopsy under spontaneous breathing. Two cases were initially diagnosed solely using tumor markers. One patient with severe tracheal compression underwent tumor resection with extracorporeal membrane oxygenation stand-by. No patient died of diagnostic procedure-related complications.

Conclusions

In 11 of the 12 cases reviewed, safe and accurate tumor diagnosis was accomplished without general anesthesia. A diagnostic strategy without general anesthesia considering the tumor location proved to be useful.

DOI： 10.1186/s12887-020-02183-w

Language：English   Publishing type：Research paper (scientific journal)  

PURPOSE: We report here the outcomes and late effects of the Japanese Study Group for Pediatric Liver Tumors (JPLT)-2 protocol, on the basis of cisplatin-tetrahydropyranyl-adriamycin (CITA) with risk stratification according to the pretreatment extent of disease (PRETEXT) classification for hepatoblastoma (HB). PATIENTS AND METHODS: From 1999 to 2012, 361 patients with untreated HB were enrolled. PRETEXT I/II patients were treated with up-front resection, followed by low-dose CITA (stratum 1) or received low-dose CITA, followed by surgery and postoperative chemotherapy (stratum 2). In the remaining patients, after 2 cycles of CITA, responders received the CITA regimen before resection (stratum 3), and nonresponders were switched to ifosfamide, pirarubicin, etoposide, and carboplatin (ITEC; stratum 4). Intensified chemotherapeutic regimens with autologous hematopoietic stem-cell transplantation (SCT) after resection were an optional treatment for patients with refractory/metastatic disease. RESULTS: The 5-year event-free and overall survival rates of HB patients were 74.2&#37; and 89.9&#37;, respectively, for stratum 1, 84.8&#37; and 90.8&#37;&#37;, respectively, for stratum 2, 71.6&#37; and 85.9&#37;&#37;, respectively, for stratum 3, and 59.1&#37; and 67.3&#37;&#37;, respectively, for stratum 4. The outcomes for CITA responders were significantly better than those for nonresponders, whose outcomes remained poor despite salvage therapy with a second-line ITEC regimen or SCT. The late effects, ototoxicity, cardiotoxicity, and delayed growth, occurred in 61, 18, and 47 patients, respectively. Thirteen secondary malignant neoplasms (SMNs), including 10 leukemia, occurred, correlating with higher exposure to pirarubicin and younger age at diagnosis. CONCLUSION: The JPLT-2 protocol achieved up-front resectability in PRETEXT I/II patients with no annotation factors, and satisfactory survival in patients who were CITA responders in the remaining patients. However, outcomes for CITA nonresponders were unsatisfactory, despite therapy intensification with ITEC regimens and SCT. JPLT-2 had a relatively low incidence of cardiotoxicity but high rates of SMNs.

DOI： 10.1200/JCO.19.01067

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BACKGROUND: Few clinical trials have been reported for patients with intermediate-risk neuroblastoma because of the scarcity of the disease and the variety of clinical and biological characteristics. A multidisciplinary treatment that consists of multidrug chemotherapy and surgery is expected to lead to a good prognosis with few complications. Therefore, a clinical trial for patients with intermediate-risk tumors was designed to establish a standard treatment that reduces complications and achieves good outcomes. METHODS: We planned a prospective phase 2, single-arm study of the efficacy of image-defined risk factors (IDRF)-based surgical decision and stepwise treatment intensification for patients with intermediate-risk neuroblastomas. For the localized tumor group, IDRF evaluations will be performed after each three-course chemotherapy, and surgery will be performed when appropriate. For patients with metastatic tumors, a total of five chemotherapy courses will be performed, and primary lesions will be removed when the IDRF becomes negative. The primary endpoint is 3-year progression-free survival rate, and the secondary endpoints include 3-year progression-free survival rates and overall survival rates of the localized group and the metastasis group and the incidence of adverse events. From international results, 75&#37; is considered an appropriate 3-year progression-free survival rate. If this trial's expected 3-year progression-free survival rate of 85&#37; is statistically greater than 75&#37; in the lower limit of the 95.3&#37; confidence interval, with an accuracy 10&#37; (85 ± 10&#37;), both groups require more than 65 patients. DISCUSSION: This study is the first clinical trial on the efficacy of IDRF-based surgical decision and stepwise treatment intensification for patients with intermediate-risk neuroblastomas. We expect that this study will contribute to the establishment of a standard treatment for patients with intermediate-risk neuroblastoma. TRIAL REGISTRATION: UMIN000004700, jRCTs051180203; Registered on December 9, 2010.

DOI： 10.1186/s12887-020-02061-5

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総排泄腔外反症は緊急手術が必要な希少疾患であり,小児外科医育機関の専門医であっても一生の間に術者を経験する機会は決して多くない.京都府立医科大学小児外科では1978年から2019年に7例の本症を経験したが現在の当科の標準治療は,新生児期の一期的根治術を基本としており,その詳細としては(1)膀胱腸裂切離(2)後腸を利用した単孔式結腸瘻造設(3)膀胱閉鎖(4)恥骨結合離開縫合(5)腹壁閉鎖(臍帯ヘルニア根治術)(6)膀胱瘻造設を初回手術時に施行することにしている.これは過去の症例の治療経験から得られた貴重な教訓から選択されてきた術式である.本症の如く希少疾患の治療成績の向上には症例毎の検討は必要なのは当然であるが,緊急手術となることが多い本症は一例一例積み重ねてきた治療の変遷の記録保持しておくことも非常に重要である.また本症術後患児の長い経過では多くの診療科との連携で行われてきた管理状況の把握も重要である.そしてその状況では新生児期からの患児の経過と本症の特徴を熟知した小児外科医が臓器別専門診療科との連携調節を行わなければならないと考えられる.(著者抄録)

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Biliary atresia (BA) is characterized by the inflammation and obstruction of the extrahepatic bile ducts (EHBDs) in newborn infants. SOX17 is a master regulator of the fetal EHBDs formation. In mouse Sox17+/- BA models, SOX17 reduction causes cell-autonomous epithelial shedding together with the ectopic appearance of SOX9-positive cystic duct-like epithelia in the gallbladder walls, resulting in the BA-like symptoms during the perinatal period. However, the similarities with the human BA gallbladders are still unclear. In the present study, we conducted the phenotypic analysis with the Sox17+/- BA neonate mice, in order to compare with the gallbladder wall phenotype of human BA infants. The most characteristic phenotype of the Sox17+/- BA gallbladders is the ectopic appearance of SOX9-positive peribiliary glands (PBGs), so-called pseudopyloric glands (PPGs). Next we examined SOX17/SOX9 expression profiles of human gallbladders in thirteen BA infants. Among them, five BA cases showed a loss or drastic reduction of SOX17-positive signals throughout the whole region of gallbladder epithelia (SOX17-low group). Even in the remaining eight gallbladders (SOX17-high group), the epithelial cells near the decidual sites were frequently reduced in the SOX17-positive signal intensity. Most interestingly, the most characteristic phenotype of human BA gallbladders is the increased density of PBG/PPG-like glands in the gallbladder body, especially near the epithelial decidual site, indicating the PBG/PPG formation as a common phenotype between human BA and mouse Sox17+/- BA gallbladders. These findings provide the first evidence of the potential contribution of SOX17 reduction and PBG/PPG formation to the early pathogenesis in human BA gallbladders.

DOI： 10.1242/dmm.042119

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PURPOSE: Mesenchymal stem cells (MSCs) are reported to migrate toward damaged tissues or tumors. We previously reported the in vivo short-term (1 day) tumor-homing effect of xenogeneic human MSCs (hMSCs) using the TH-MYCN mouse neuroblastoma model (MYCN-TgM). In this study, we analyzed the long-term tumor-homing effect of allogeneic mouse MSCs (mMSCs) and explored the antitumor effect and drug delivery function of mMSCs. METHODS: mMSCs were administered intraperitoneally (i.p.) to MYCN-TgM and traced by an in vivo imaging system (IVIS). We administered green fluorescent protein (GFP)-transduced mMSCs into MYCN-TgM i.p. and examined the cell survival by immunohistochemistry. We also administered interferon beta-transduced mMSCs (mMSCs-IFN-β) to MYCN-TgM i.p. and measured the concentration of IFN-β in the tumor and organs by an enzyme-linked immunosorbent assay (ELISA). The survival curves of MYCN-TgM administered every week was analyzed. RESULTS: The IVIS revealed the accumulation of fluorescence was observed in the tumor both in vivo and after excision. Immunohistochemistry using anti-GFP antibody revealed that the mMSCs existed within the tumor until 14 days but not in the organs. The ELISA showed increased concentrations of IFN-β only in the tumors, with the values gradually diminishing over 14 days. The mMSCs-IFN-β group survived significantly longer than the control group (p < 0.03), while the mMSCs-alone group did not show a survival advantage. CONCLUSIONS: Allogeneic mMSCs showed a homing ability for mouse neuroblastoma and existed within the tumor for as long as two weeks. This may be a candidate drug delivery vehicle for antitumor agents against neuroblastoma.

DOI： 10.1016/j.jpedsurg.2019.08.023

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Purpose We aimed to evaluate the effect of human mesenchymal stem cells (hMSCs) on congenital diaphragmatic hernia (CDH) by intra-amniotic injection in a rat CDH model. Methods Nitrofen (100 mg) was administered to pregnant rats at E9.5. hMSCs (1.0 x 10(6)) or PBS was injected into each amniotic cavity at E18, and fetuses were harvested at E21. The fetal lungs were classified into normal, CDH, and CDH-hMSCs groups. To determine the lung maturity, we assessed the alveolar histological structure by H&E and Weigert staining and the alveolar arteries by Elastica Van Gieson (EVG) staining. TTF-1, a marker of type II alveolar epithelial cells, was also evaluated by immunohistochemical staining and real-time reverse transcription polymerase chain reaction. Results The survival rate after intra-amniotic injection was 72.1&#37;. The CDH-hMSCs group had significantly more alveoli and secondary septa than the CDH group (p < 0.05). The CDH-hMSCs group had larger air spaces and thinner alveolar walls than the CDH group (p < 0.05). The medial and adventitial thickness of the pulmonary artery in the CDH-hMSCs group were significantly better (p < 0.001), and there were significantly fewer TTF-1-positive cells than in the CDH group (p < 0.001). Conclusion These results suggest that intra-amniotic injection of hMSCs has therapeutic potential for CDH.

DOI： 10.1007/s00383-019-04561-7

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Human teratoma is a germ cell tumor that contains normal tissues (e.g., hair, skin or cartilage) differentiated from embryonal germ layers. Because of the feature of this tumor, we hypothesized that human teratomas contain multipotent stem cells that can develop into various non-cancerous normal tissues. In this study, we cultured neurospheres originally derived from a human infantile teratoma tissue, and the sphere cells were found to possess the characteristics of neural stem cells. Tumor tissues were obtained from an infantile immature teratoma at the time of surgical resection. In the primary cell culture, colonies were formed in two weeks and were individually cultured in serum-free conditioned neural stem cell medium (NSC medium). Colonies changed into spheres and grew in smooth round forms, or attached to the bottom of the dishes and extended processes and filaments around. Sphere cells were dissociated into single cells, and new spheres (secondary spheres) were formed in NSC medium. Cell differentiation was induced by culturing cells in serum-containing medium (differentiation medium), as cells spread and attached to the bottom of dishes and changed form. The expression of Nestin, Sox2, CXCR4, and (stem cell markers), β3-tubulin (a neural marker) GFAP (a glial marker) CNPase, SOX10 (oligodendrocyte markers) and NF-L in cells was analyzed by immunofluorescence and a Q-PCR. Nestin, SOX2, CXCR4 were abundant in both primary and secondary spheres. Neural and glial markers (β3-tubulin and GFAP, respectively) were increased in cells cultured in differentiation medium while stem cell markers were diminished. The oligodendrocyte markers SOX10 and CNPase were also found in both spheres and differentiated cells. In conclusion, spheres with the characteristics of neural stem cells were obtained from the primary culture of a human infantile teratoma. These spheres are considered to have the potential to undergo a natural course of neural development in humans.

DOI： 10.1016/j.scr.2019.101633

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Purpose We investigated how local tumor resection affects metastatic lesions in neuroblastoma. Methods MYCN Tg tumor-derived cells were injected subcutaneously into 129(+Ter)/SvJcl wild-type mice. First, the frequency of metastasis-bearing mice was investigated immunohistochemically (metastatic ratio) at endpoint or post-injection day (PID) 90. Second, the threshold volume of local tumor in mice bearing microscopic lymph node metastasis (mLNM) was investigated at PID 30. Finally, local tumors were resected after exceeding the threshold. Mice were divided into local tumor resection (Resection) and observation (Observation) groups, and the metastatic ratio and volume of LNM were compared between the groups at endpoint or PID 74. Results The metastatic ratio without local resection was 88&#37; at PID 78-90. The threshold local tumor volume in the mice with mLNM was 745 mm(3) at PID 30, so local tumors were resected after exceeding 700 mm(3). The metastatic ratio and LNM volume were significantly greater in the Resection group (n = 16) than in the Observation group (n = 16) (94&#37; vs. 38&#37;, p < 0.001; 2092 +/- 2310 vs. 275 +/- 218 mm(3), p < 0.01; respectively) at PID 50-74. Conclusion Local tumor resection might augment the growth of synchronous microscopic metastases. Our results provide insights into the appropriate timing of local resection for high-risk neuroblastoma.

DOI： 10.1007/s00383-019-04568-0

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Sirolimus Treatment for Intractable Vascular Anomalies: An Interim Analysis of an Open-Label, Single-Arm, Multicenter, Prospective Study

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BACKGROUND: The present study sought to reduce the incidence of treatment complications of low-risk neuroblastoma by using image-defined risk factors (IDRFs) to inform the timing of surgical resection. PROCEDURES: Eligible patients included children (<18 years of age) with stage 1 or 2 disease, children (<365 days of age) with stage 3 disease, and infants with stage 4S disease. In IDRF-negative cases, treatment was completed with surgical resection alone. In IDRF-positive cases, the timing of surgery was determined based on the IDRFs after low-dose chemotherapy with 2-3 of the following four drugs: vincristine, cyclophosphamide, pirarubicin, and carboplatin. The outcome measures were overall survival, progression-free survival, and adverse events. This study was registered with the UMIN Clinical Trials Registry (number 000004355). RESULTS: Of the 60 patients screened between 2010 and 2013, 58 eligible patients were enrolled; 32 were identified as IDRF negative at diagnosis while 26 were identified as IDRF positive and underwent induction chemotherapy. The 3-year overall and progression-free survival rates of the 58 patients were 100&#37; and 82.8&#37; (95&#37; confidence interval: 70.3-90.3), respectively. Neutropenia was the most frequently reported grade 3 or 4 chemotherapy-related form of toxicity (41.7&#37;). With regard to surgical complications, 2.5&#37; of all patients developed pleural effusion and ascites as early complications, while only 2.5&#37; developed renal atrophy as a long-term complication. No fatal toxicities were observed. CONCLUSION: Using IDRFs to inform surgical decision making for the treatment of low-risk neuroblastoma improved prognosis and reduced the incidence of long-term complications.

DOI： 10.1002/pbc.27914

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Emerging data have suggested that sirolimus may be a treatment option for complicated vascular anomalies (VAs). The present study aimed to investigate the immunologic effects of sirolimus treatment for 6 months in patients with VAs. Blood samples obtained from the patients enrolled in 2 multicenter studies to investigate the efficacy of sirolimus for VAs before and after sirolimus treatment for 6 months were used. Data for total white blood cell count, absolute lymphocyte count, serum immunoglobulins (Igs) levels (IgG, IgA, IgM), lymphocyte proliferation assays with mitogens including phytohemagglutinin and concanavalin A, and flow cytometric analysis of lymphocyte subsets were evaluated. A total of 18 patients with VAs receiving sirolimus treatment were included in the study. Comparisons of white blood cell, absolute lymphocyte count, IgG, IgA, IgM, and reaction rates of phytohemagglutinin and concanavalin A revealed no significant differences before and after treatment. No significant differences were observed in the absolute counts of lymphocyte subtypes before and after treatment, except for regulatory T-cell counts, which were significantly decreased after treatment. Severe infections were not observed during sirolimus treatment. The immunologic parameters assessed in the present study were hardly affected by sirolimus treatment for 6 months in patients with VAs.

DOI： 10.1097/MPH.0000000000001650

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骨盤内リンパ管腫はリンパ管腫の中でもまれである。また一般的にリンパ管腫は不完全切除では再発することがある一方で、合併症の面から完全切除困難な場合がある。症例は7歳男児で発熱と排尿時腹痛を呈した。超音波、造影CTで下腹部～骨盤腔右側に10×7×11cmの巨大な多嚢胞性病変および拡張した右腎盂・尿管を認めた。尿道造影で前立腺球部尿道に狭小部を認めた。嚢胞穿刺液の白血球はリンパ球優位であり、リンパ管腫と診断し腹腔鏡下嚢胞全摘術を試みたが、嚢胞背側は前立腺周囲と強固に癒着しており、術後排尿障害を懸念し、開窓術に留めた。嚢胞壁を可及的に切除し、再発予防として嚢胞内腔面にPGAシート貼付とフィブリン糊撒布を行った。術後経過は良好で、術後14日目に退院した。術後3年経過するが、再発や再燃なく経過している。(著者抄録)

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腸重積症初発から2年後の再発にて発見された、盲腸原発の若年性ポリープに悪性所見を認めた1例を報告する。症例は7歳男児。5歳時に腸重積症の非観血的整復の既往がある。今回、間欠的腹痛にて前医を受診し、腹部超音波にて腸重積症の診断で、高圧浣腸にて整復されたが再発し、再整復後に精査目的に当科へ転院となった。下部消化管内視鏡にて盲腸にIp型ポリープを認めたため、ポリペクトミーを施行したところ、若年性ポリープの先端部の一部にadenocarcinoma in situが認められた。全摘された単発病変であり、転移所見もないため、追加治療せず経過観察としている。単発性の若年性ポリープに癌化を認めることは非常に稀であるが、経過が長い症例ではその可能性を念頭に置く必要があると考えられた。(著者抄録)

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BACKGROUND: Sacrococcygeal teratoma (SCT) is the most common extragonadal germ cell tumor in neonates and infants. Although most cases of infantile SCT are benign tumors by nature, some develop into extremely large lesions, leading to massive bleeding, high-output heart failure, disseminated intravascular coagulation, and even fatal outcomes during the neonatal period. In addition, some patients may present with tumor recurrence, malignant transformation, long-term sequelae (including bladder and bowel dysfunction) and lower leg palsy during the long-term follow up. SCT, however, is very rare, and there are few opportunities to encounter this disease, therefore general physicians without expert credentials currently lack information relevant to clinical practice. For this reason, the research project committee has compiled guidelines concerning SCT. METHODS: The purpose of these guidelines was to share information concerning the treatment and follow up of infantile SCT. The guidelines were developed using the methodologies in the Medical Information Network Distribution System. A comprehensive search of the English- and Japanese-language articles in PubMed and Ichu-Shi Web identified only case reports or case series, and the recommendations were developed through a process of informal consensus. RESULTS: The clinical questions addressed the risk factors, the efficacy of cesarean section, the initial devascularization of tumor feeding vessels, interventional radiology, recommended clinical studies for follow up and possible long-term complications. CONCLUSIONS: These are the first guidelines for SCT to be established in Japan, and they may have huge clinical value and significance in terms of developing therapeutic strategies and follow up, potentially contributing to the improvement of the prognosis and quality of life of SCT patients.

DOI： 10.1111/ped.13844

Language：Japanese  

当科での低位鎖肛の治療成績について検討した。2004～2018年までの過去15年間に当科で外科治療を行った低位鎖肛症例で、術後2年以上経過している42例(男児7例、女児35例)を対象とした。転帰として42例中4例を染色体異常のために失い、4例がlost followとなった。残る34例を排便状況に応じて、内服薬や浣腸が一切不要である排便良好群20例、排便に対して緩下薬や浣腸を定期的または不定期に必要とする排便中等度群9例、治療にかかわらず高度便秘や便失禁をきたしている排便不良群5例の3群に分類した。排便良好群では完全に投薬を終了できたのは中央値2.5歳の時点であった。排便中等度群では2例が学童期に高度便秘のため再受診となり緩下薬の投薬が再開された。排便不良群では脊髄異常が2例に認められた。結果として、42例中20例(47.6&#37;)は最長12年にわたる外来治療により排便機能が正常化したが、22例(52.4&#37;)は2～9歳になってもいまだ治療を要する状態である。

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Introduction: Lymphatic anomalies (LAs) refer to a group of diseases involving systemic dysplasia of lymphatic vessels. These lesions are classified as cystic lymphatic malformation (macrocystic, microcystic or mixed), generalized lymphatic anomaly, and Gorham-Stout disease. LAs occur mainly in childhood, and present with various symptoms including chronic airway problems, recurrent infection, and organ disorders. Individuals with LAs often experience progressively worsening symptoms with a deteriorating quality of life. Although limited treatment options are available, their efficacy has not been validated in prospective clinical trials, and are usually based on case reports. Thus, there are no validated standards of care for these patients because of the lack of prospective clinical trials.Methods: This open-label, single-arm, multicenter, prospective study will assess the efficacy and safety of a mammalian target of the rapamycin inhibitor sirolimus in the treatment of intractable LAs. Participants will receive oral sirolimus once a day for 52 weeks. The dose is adjusted so that the nadir concentration remains within 5-15 ng/ml. The primary endpoint is the response rate of radiological volumetric change of the target lesion confirmed by central review at 52 weeks after treatment. The secondary endpoints are the response rates at 12 and 24 weeks, respiratory function, pleural effusion, ascites, blood coagulation parameters, bleeding, pain, quality of life, activities of daily living, adverse events, side effects, laboratory examinations, vital signs, and pharmacokinetic data.Results: This is among the first multicenter studies to evaluate sirolimus treatment for intractable LAs, and few studies to date have focused on the standard assessment of the efficacy for LAs treatment. Our protocol uses novel, uncomplicated methods for radiological assessment, with reference to the results of our previous retrospective survey and historical control data from the literature.Conclusions: We propose a multicenter study to investigate the efficacy and safety of sirolimus for intractable LAs (SILA study; trial registration UMIN000028905). Our results will provide pivotal data to support the approval of sirolimus for the treatment of intractable LAs. (C) 2019, The Japanese Society for Regenerative Medicine. Production and hosting by Elsevier B.V.

DOI： 10.1016/j.reth.2018.12.001

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Precision medicine strategies for treating rhabdomyosarcoma (RMS), a childhood malignancy, have not been developed. We examined the effect of CH5126766, a potent selective dual RAF/MEK inhibitor, on RMS cell lines. Among the eleven cell lines studied, one NRAS and two HRAS mutated cell lines were detected. CH5126766 inhibited the proliferation and growth in all of the RAS-mutated RMS cell lines, while it induced G1 cell cycle arrest in two of them. G1 cell cycle arrest was accompanied by p21 up-regulation and RB dephosphorylation. CH5126766 also suppressed the in vivo growth of RAS-mutated RMS tumor, and the mice showed improved survival. Thus, our results demonstrate that CH5126766 is an effective RAF/MEK inhibitor in RAS-mutated RMS. This study not only shows that in RMS, mutations in the RAS pathway can be a target for precision medicine, but also demonstrates that the evaluation of the gene mutation status is important in childhood malignancies.

DOI： 10.1016/j.bbrc.2019.03.038

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DOI： 10.1136/archdischild-2018-314885

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症例は13歳女児。左下腹部痛と頻回の嘔吐で発症翌日に前医を受診した。CTで骨盤内に嚢胞性病変を指摘され、卵巣嚢腫茎捻転を疑われ同日当院へ救急搬送となった。エコー・CT検査より左下腹部から膀胱頭側にかけて径10cmの充実成分のない多房性嚢胞性病変を認め、嚢胞成分はecho freeで鏡面形成は認められなかった。腹水を少量認め、卵巣嚢腫茎捻転を疑い同日緊急手術を施行した。術中所見で左卵管より突出した嚢腫と360°反時計方向の左卵管捻転を認めた。左卵巣の血流障害は認められず、左傍卵管嚢腫に伴う卵管捻転と診断し嚢腫核出術を施行した。術後経過は良好であり特に合併症を認めず術後7日目に退院となった。今回我々は卵巣嚢腫茎捻転疑いで緊急手術を行い、術中所見から傍卵管嚢腫に伴う卵管捻転と診断した1例を経験した。(著者抄録)

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Thymolipoma is a rare disease among benign tumors. We herein report the case of a child who underwent thoracoscopic resection of a large thymolipoma. A 3-year-old boy was diagnosed with an anterior mediastinal solid tumor. Thoracic imaging revealed a fat density mass that was 8.0 × 5.0 × 3.5 cm in size. Given the MRI findings of the tumor, we suspected that the lesion was a teratoma. We decided to perform thoracoscopic exploration and, if possible, resection of the solid tumor sequentially. We successfully resected the tumor thoracoscopically. A histological examination revealed thymolipoma. For large mediastinal tumors in the intrathoracic space in children, the thoracoscopic approach is recommended when the tumor is preoperatively considered to be benign and resectable.

DOI： 10.1111/ases.12627

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小児肝胆道手術において、副肝管の存在は術前診断が困難であり、術中損傷による術後胆汁漏や胆管炎により、病悩期間を長期化させ、患者のQOLを著しく低下させる可能性がある。さらに小児では胆管が細径であることが多く、壁の脆弱性もあり、胆管空腸吻合での再建が困難であることが少なくない。今回われわれは、術中の副肝管損傷に対して肝門部空腸吻合で再建した1例を経験した。症例は6歳、男児。肝芽腫、PRETEXTIIに対して肝左葉切除術を施行中に胆嚢管から分岐する副肝管(B5分枝)を損傷した。胆管が細く脆弱で、胆管空腸吻合が困難であったため、ステントレスの右肝門部空腸吻合で再建した。術後胆汁流出は良好であり、肝内胆管拡張などは認めていない。小児肝切除における術中胆管損傷に対して本例のように胆管が細く脆弱である場合や、一穴化不能な複数の胆管がある場合、肝門部空腸吻合は有用なサルベージ再建法であると考えられた。(著者抄録)

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Mesenchymal hamartoma of the chest wall in a 10-year-old girl mimicking malignancy: a case report.
We herein report a rare case of mesenchymal hamartoma of the chest wall in a 10-year-old girl. She complained of chest pain and was diagnosed with a large chest wall tumor originating from the left fourth rib. Malignancy such as osteosarcoma or chondrosarcoma could not be ruled out with imaging studies. Therefore, we performed a core needle biopsy assisted by thoracoscopy, which revealed no malignancy. Therefore, extended resection with chest wall reconstruction was unnecessary, and thoracoscopy-assisted tumor excision with only the removal of the involved fourth rib was performed without chest wall reconstruction. The postoperative course was satisfactory with no thoracic deformity and no recurrence.

DOI： 10.1007/s00256-018-3106-2

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BACKGROUND: The Japanese Children's Cancer Group (JCCG) Neuroblastoma Committee (JNBSG) conducted a phase II clinical trial for high-risk neuroblastoma treatment. We report the result of the protocol treatment and associated genomic aberration studies. METHODS: JN-H-07 was a single-arm, late phase II trial for high-risk neuroblastoma treatment with open enrollment from June 2007 to February 2009. Eligible patients underwent five courses of induction chemotherapy followed by high-dose chemotherapy with hematopoietic stem cell rescue. Surgery for the primary tumor was scheduled after three or four courses of induction chemotherapy. Radiotherapy was administered to the primary tumor site and to any bone metastases present at the end of induction chemotherapy. RESULTS: The estimated 3-year progression-free and overall survival rates of the 50 patients enrolled were 36.5 ± 7.0 and 69.5 ± 6.6&#37;, respectively. High-dose chemotherapy caused severe toxicity including three treatment-related deaths. In response to this, the high-dose chemotherapy regimen was modified during the trial by infusing melphalan before administering carboplatin and etoposide. The modified high-dose chemotherapy regimen was less toxic. Univariate analysis revealed that patients younger than 547 days and patients whose tumor showed a whole chromosomal gains / losses pattern had a significantly poor prognosis. Notably, the progression-free survival of cases with MYCN amplification were not inferior to those without MYCN amplification. CONCLUSIONS: The outcome of patients treated with the JN-H-07 protocol showed improvement over the results reported by previous studies conducted in Japan. Molecular and genetic profiling may enable a more precise stratification of the high-risk cohort.

DOI： 10.1007/s10147-018-1281-8

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気道異物は、小児診療において緊急を要する代表的な疾患である。当院で経験した気道異物症例30例を対象とし臨床的に検討した。年齢は生後7ヵ月から6歳で1歳が最も多く、中央値は1歳8ヵ月であった。異物内容は食物が24例で最も多かった。症状は咳嗽が21例、喘鳴が10例で、3例にチアノーゼを認めた。誤嚥後から当院紹介までの期間は0日から最長81日であり、0日が17例で最も多かったが全体の57&#37;であった。当院初診時の聴診所見で呼吸音に左右差を認めた症例は25例(83&#37;)であった。胸部X線では19例(63.3&#37;)に異常所見を認めるのみであった。今回の検討では気道異物の多くの症例で聴診所見に左右差を認めており気道異物を強く疑うべき重要な所見であると考える。画像のみでは気道異物を否定することはできず、臨床的に気道異物が疑わしい場合は早急に硬性気管支鏡による検索が必要であることを啓蒙する必要があると考えられる。(著者抄録)

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サブスペシャル領域を見据えた新専門医制度のあり方 小児外科からみた新専門医制度のあり方 日本小児外科学会の取り組み

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In vivo effects of short- and long-term MAPK pathway inhibition against neuroblastoma.

DOI： 10.1016/j.jpedsurg.2018.08.026

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Natural antibody against neuroblastoma of TH-MYCN transgenic mice does not correlate with spontaneous regression.
The mechanism underlying the spontaneous regression of neuroblastoma is unclear. Although it was hypothesized that this regression occurs via an immunological mechanism, there is no clinical evidence, and no animal models have been developed to investigate the involvement of immune systems, especially natural antibodies, against neuroblastoma. We performed an immunological analysis of homo- and heterozygous TH-MYCN transgenic mice as a model of aggressive neuroblastoma. Mice with no or small (<5 mm) tumors showed higher antibody titers in plasma than mice with large (>5 mm) tumors. A significant negative correlation was observed between the tumor diameter and the titer of antitumor antibody. This antibody had complement-dependent cytotoxicity but not antibody-dependent cellular cytotoxicity against neuroblastoma cells. Moreover, B-cell depletion had no effect on the tumor incidence in vivo. We revealed that TH-MYCN transgenic mice have a natural antibody against neuroblastoma that correlate with tumor size. However, this antibody does not correlate with the spontaneous regression of neuroblastoma. Thus, the function of the natural antibody is limited.

DOI： 10.1016/j.bbrc.2018.07.097

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胎児・新生児期診断された先天性胆道拡張症の外科治療の注意点

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Sequential actions of immune effector cells induced by viral activation of dendritic cells to eliminate murine neuroblastoma.
PURPOSE: In preclinical trails, we reported the antitumor effect of dendritic cells activated with Sendai virus (rSeV/DC) combined with γ-irradiation against neuroblastoma. However, what kind of effector cells for the combined therapy were used to show the antitumor effect was unclear. In this study, we performed radiation and rSeV/DC therapy in vivo and examined the effector cells involved. METHODS: Dendritic cells were cultured from bone marrow cells, activated with SeV and administered intratumorally at 106 weekly for 3weeks. Radiation was administered at 4Gy/time × 3 times. During the treatment, CD4+ and CD8+ cells and natural killer (NK) cells were removed by antibodies. RESULTS: Complete remission of neuroblastoma was observed in 62.5&#37; of individuals in the combined therapy group. By depleting the effector cells using antibodies, the tumor increased in size from an early stage of treatment in the CD4+ and NK cell-depleted group. In contrast, the tumor increased in size in the late stage of treatment in the CD8+ cell-depleted group. CONCLUSION: The combination of radiation and rSeV/DC therapy induces different effector cells, depending on the time point during treatment. LEVEL OF EVIDENCE: V.

DOI： 10.1016/j.jpedsurg.2017.08.019

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成人重症心身障害者に発症した褥瘡を小児病棟管理で改善しえた1例

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乳児巨大後腹膜奇形腫に対して、3D-CTによる側副血行路評価に基づいて術式を選択し、腎を温存しながら腫瘍全摘しえた2例を経験したので報告する。症例1は3ヵ月の女児。巨大腹部腫瘤で発見され、術前3D-CTで左腎静脈が圧排、途絶していたが、卵巣静脈への側副血行路の発達が想定された。これらの温存のため腎門部を愛護的に操作し、全摘し得た。症例2は4ヵ月女児。3D-CTで下大静脈および両側腎静脈が途絶しており椎骨静脈叢への側副血行路を認めた。下大静脈および右腎静脈を術中切離して腫瘍を全摘しえた。両症例とも重大な術後合併症は認めず、腎機能も温存できている。乳児後腹膜奇形腫では、臓器や重要血管が予想し難いほどの偏位を呈し、手術における致命的なリスクとなり得る。腫瘍と臓器の位置関係や、重要血管の走行を確認するうえで術前3D-CTは必須であり、側副血行路の発達に基づいた術式の選択が重要と考えられた。(著者抄録)

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骨盤内病変を有する仙尾部奇形腫に対する低侵襲手術

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Paternal uniparental disomyタイプのBeckwith-Wiedemann症候群に合併した肝芽腫の1例

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神経芽腫に対する内視鏡外科手術の適応基準の作成と標準化へ向けた挑戦 全国調査からみた現状と集約化および臨床試験へ向けた可能性

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膵芽腫の一例

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術前3D-CTが有用であった乳児巨大後腹膜奇形腫の一摘除例

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乳児期以降に診断・治療が開始された低位型直腸肛門奇形(以下、本症)の当科での経験を調査した。2008年1月から2017年4月に当科で経験した本症22例のうち該当症例は8例(女児7例男児1例)あった。初診時症状は便秘・肛門狭窄・肛囲びらん・肛門位置異常であった。7例で手術を要し、1例は排便機能が保たれており経過観察中である。乳児期以降に本症が診断される例は必ずしも少なくないことが明らかになった。この調査結果から、乳児期以降に診断される本症の特長や日常診療での留意点を考察し報告する。(著者抄録)

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DOI： 10.1016/j.pedneo.2017.08.002

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【「内視鏡外科手術の最前線」】 小児外科における内視鏡手術Up-to-Date

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