Language：English   Publisher：Nucleic Acids Research  

CS1-LS4 and CS2-LS12 are ultra-high affinity and orthogonal RNA-protein pairs that were identified by PD-SELEX (Phage Display coupled with Systematic Evolution of Ligands by EXponential enrichment). To investigate the molecular basis of the lab-coevolved RNA-RBP pairs, we determined the structures of the CS1-LS4 and CS2-LS12 complexes and the LS12 homodimer in an RNA-free state by X-ray crystallography. The structural analyses revealed that the lab-coevolved RNA-RBPs have acquired unique molecular recognition mechanisms, whereas the overall structures of the RNP complexes were similar to the typical kink-turn RNA-L7Ae complex. The orthogonal RNA-RBP pairs were applied to construct high-performance cell-free riboswitches that regulate translation in response to LS4 or LS12. In addition, by using the orthogonal protein-responsive switches, we generated an AND logic gate that outputs staphylococcal γ-hemolysin in cell-free system and carried out hemolysis assay and calcein leakage assay using rabbit red blood cells and artificial cells, respectively.

DOI： 10.1093/nar/gkaf212

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

＜文献概要＞はじめに　重症心身障害児や医療的ケア児に対する胃瘻造設術は,嚥下や経口摂取が困難な場合の経腸栄養法として広く行われているが,まれな合併症として胃瘻造設後の胃軸捻転(以下,本症)が報告されている。本症は重症心身障害児の場合は呑気症による急性胃拡張を伴う胃軸捻転症であり,速やかに脱気や捻転解除を行わなければ胃の虚血壊死,穿孔を引き起こす。胃瘻造設後の胃軸捻転を発症した自験例を提示し,臨床的特徴と注意点について述べる。

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：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：English   Publisher：Hepatology Research  

Aim: To assess the preoperative disease characteristics and indications for living donor liver transplantation (LDLT), complications, patient survival, and prognosis after LDLT for fibropolycystic liver disease (FLD) in children. Methods: We undertook a cross-sectional survey of patients who underwent LDLT for FLD between January 2002 and December 2020. Results: A total of 35 patients (22 male and 13 female individuals) with FLD were included in this study, of whom 19 (54.3%) had isolated congenital hepatic fibrosis and 16 (45.6%) had Caroli syndrome. Refractory gastrointestinal bleeding was the most frequent symptom related to the indication for LDLT, being found in 48.6% of our patients, followed by uncontrollable cholangitis and ascites. The median age at the time of LDLT was 8.1 years old. Of the 27 patients presenting with renal involvement, 13 patients required kidney transplantation (KT). Overall, the renal function after LDLT decreased regardless of renal involvement; however, patients with renal involvement had a significantly lower estimated glomerular filtration rate than those without renal involvement throughout the course of this study (p < 0.01). The 5-year overall patient survival rate was 97.1%. Two patients died with a median follow-up of 8.9 years after LDLT; one died due to sepsis 2 weeks after simultaneous liver–kidney transplantation and the other committed suicide 10 years after LDLT. Conclusion: The prognosis of the pediatric patients who underwent LDLT for FLD was excellent. However, an individualized treatment approach based on the status of the renal function and liver disease is important, as a certain proportion of patients require KT.

DOI： 10.1111/hepr.14122

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Language：English   Publisher：Scientific Reports  

Phytotoxic air pollutants such as atmospheric nitrogen dioxide (NO<inf>2</inf>) are among the major stresses affecting tree photosynthesis in urban areas. We clarified the relationship between NO<inf>2</inf> concentrations and photosynthetic function for three major urban trees, Prunus × yedoensis, Rhododendron pulchrum, and Ginkgo biloba, planted in Kyoto and surrounding cities, combining our published data and new data collected from 2020 to 2023. High NO<inf>2</inf> increased long-term water use efficiency for all species. High NO<inf>2</inf> decreased photosynthesis in P. yedoensis and R. pulchrum, while for G. biloba, NO<inf>2</inf> imposed little effect on photosynthesis. We then focused on the decrease in NO<inf>2</inf> due to (1) air pollution control measures from 2005 to 2023 and (2) the economic recession caused by the COVID-19 pandemic, and examined whether these factors improved photosynthesis in urban trees. The historic decrease in NO<inf>2</inf> improved leaf photosynthesis for P. yedoensis and R. pulchrum, while the COVID-19 pandemic reduced NO<inf>2</inf> by only 0.3 ppb and did not further improve photosynthesis in these tree species. This report shows that air pollution control measures improved photosynthesis in urban trees over several years in Japan, and is valuable because it demonstrates that air pollution control measures can increase CO<inf>2</inf> uptake by urban trees.

DOI： 10.1038/s41598-024-85080-x

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Language：English   Publisher：Pediatrics International  

Background: Vitamin K (VK) prophylaxis refers to the administration of VK to newborns to prevent neonatal VK deficiency bleeding (VKDB), which is characterized by intracranial hemorrhage (ICH). This study investigated the relationship between VK prophylaxis methods and VKDB in biliary atresia (BA). Methods: The survey targeted 497 cases in the Japanese Biliary Atresia Registry between 2015 and 2019, of which 395 (79.5%) returned the questionnaire. Of the 395 patients, 289 were selected after excluding cases in which the gestational age was <36 weeks or the VK prophylaxis methods/feeding contents were unknown. The patients were categorized into two groups according to VK prophylaxis methods. We conducted a comparative study using propensity score matching. The prognosis of patients with or without ICH was also investigated. Results: In the analysis, no VKDB occurred in patients using the 3-month method. In the propensity score matching analysis, age at first visit and age at surgery were later in the three-times method (p = 0.018 and p = 0.022, respectively); VKDB was higher in the three-times method than in the 3-month method (p = 0.029). ICH, jaundice disappearance, cholangitis, and native liver survival rates (NLSRs) were not significantly different between groups. When examining the prognosis based on ICH occurrence, the two groups showed no significant differences in jaundice disappearance, cholangitis, and NLSRs. The ICH group had a greater number of cases of delayed mental and/or motor development. Conclusions: In BA, the 3-month method is effective in preventing VKDB, and early diagnosis is crucial.

DOI： 10.1111/ped.70075

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Language：English   Publisher：Pediatrics International  

Background: Portal cavernoma cholangiopathy (PCC) has recently been recognized as an abnormality of the intrahepatic and extrahepatic bile ducts caused by external compression by the portal cavernoma or ischemia in the biliary region. Although extrahepatic portal vein obstruction (EHPVO) is common in children with portal hypertension, the association between surgical treatment for EHPVO and the incidence of PCC in children is not well known. Methods: We retrospectively reviewed the medical records of 14 cases of childhood-onset EHPVO in our department. PCC was defined by intra- and extrahepatic bile duct irregularities on imaging, both symptomatic and asymptomatic. Results: There were six cases with PCC (Group A) and eight cases without PCC (Group B). EHPVO was diagnosed at 4.8 years and 4.1 years of age in Groups A and B, respectively. PCC was asymptomatic, except in one patient with repeated cholangitis. In Group A, 4/6 patients (66.7%) showed sufficient intrahepatic portal vein inflow via cavernoma at hilum, whereas in Group B, almost all cases (7/8 cases, 87.5%) showed poor intrahepatic portal vein delineation. PCC did not develop in 4/5 patients (80%) who underwent portosystemic shunt surgery, such as mesocaval shunting. Conclusions: PCC is a serious complication of EHPVO that affects its long-term prognosis. Although there are concerns about long-term problems such as hyperammonemia and pulmonary complications after portosystemic shunt surgery, it may also be beneficial as a prophylactic surgery for PCC.

DOI： 10.1111/ped.70061

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Language：English   Publisher：Pediatrics International  

DOI： 10.1111/ped.70012

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

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

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

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>The patient was a 17-year-old female who presented with lower abdominal pain. She was initially suspected of having a periappendiceal abscess or an appendiceal tumor, leading to a referral to our hospital for further assessment. A definitive diagnosis was not reached from preoperative test results; however, with the possibility of an appendiceal malignancy in mind, a laparoscopic ileocecal resection was performed. Rapid intraoperative histopathological examination revealed adenocarcinoma, prompting additional procedures, including a D2 lymph node dissection, right adnexectomy, and partial retroperitoneal resection to complete the surgery in a single session. Histopathological examination confirmed invasion up to the muscularis propria with no lymph node metastasis found. Although it is rare, it is crucial to consider the possibility of malignancy in young patients exhibiting atypical progression or imaging findings of appendicitis.</p>

DOI： 10.11164/jjsps.60.7_997

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

The article Artificial intelligence facilitates the potential of simulator training: An innovative laparoscopic surgical skill validation system using artificial intelligence technology, written by Atsuhisa Fukuta, Shogo Yamashita, Junnosuke Maniwa, Akihiko Tamaki, Takuya Kondo, Naonori Kawakubo, Kouji Nagata,Toshiharu Matsuura and Tatsuro Tajiri, was originally published electronically on the publisher’s internet portal on 19 August 2024 without open access. With the author(s)’ decision to opt for Open Choice the copyright of the article changed on 19 November 2024 to © The Author(s) 2024 and this article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The original article has been corrected.

DOI： 10.1007/s11548-024-03300-1

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

症例は17歳女性.下腹部痛を主訴に前医受診し,画像検査の結果,虫垂周囲膿瘍または虫垂腫瘤の疑いで当院紹介となった.術前検査からは確定診断に至らなかったが,虫垂悪性腫瘍の可能性を念頭におき腹腔鏡下回盲部切除術を行った.術中迅速組織検査の結果,adenocarcinomaであることが判明したため,D2リンパ節郭清術,右付属器切除術,後腹膜部分切除術を追加し,一期的に手術を完遂した.病理検査の結果,固有筋層までの浸潤に留まっておりリンパ節転移は認めなかった.頻度は非常に低いが,非典型的な経過や画像所見を認める患者においては,若年者であっても悪性腫瘍の可能性を考慮することが重要である.(著者抄録)

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

糖原病は,肝臓や筋組織へのグリコーゲン蓄積を生じる疾患で,Ia型では原疾患のコントロール不良,多発肝細胞腺腫を理由に肝移植が適応となる。症例は8歳女児。生後10ヵ月で糖原病Iaと診断され,服薬食事療法を継続していたが,コントロールは不良で,7歳で多発肝細胞腺腫が指摘された。腺腫は増大し,悪性化も疑われ,8歳時に父親をドナーとする生体肝移植を施行した。出血時間正常化のため,術前1週間はブドウ糖点滴投与を行い,術後合併症なく退院し,服薬食事療法も終了できた。糖原病Iaに対する肝移植は肝細胞腺腫が適応因子となることが多く,良好な成績が報告されている。本邦では,肝細胞腺腫に対しては肝切除が選択されることもあるが,残存腺腫の悪性化や,出血合併症が問題となる。不可逆的とされる腎機能障害を回避し得るメリットからも,小児期における肝移植という選択肢は有用であると考える。(著者抄録)

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

2018年の小児優先システム導入後における小児献体肝移植(pDDLT)患者のアウトカムについて検討した。1999～2021年に日本臓器移植ネットワークと日本肝移植学会に登録された18歳未満のpDDLT患者112例を対象に、小児優先システム導入前後のグラフト生存率などを比較した。検討の結果、pDDLT患者の原疾患は肝硬変が最も多く、原発性硬化性胆管炎(PSC)がこれに続き、肝移植ドナーの半数以上は小児であった。pDDLTの年間施行数は2010年以前が平均0.9例、2010～2018年が平均6.4例、2018年以降が平均14.8例と経年的に増加を示し、移植待機期間は2010年以前が中央値565日、2010～2018年が中央値195日、2018年以降が中央値120日と経年的に短くなっていた。1年グラフト生存率は86.6%、5年グラフト生存率は83.3%、1年患者生存率は88.4%、5年患者生存率は86.6%であった。多変量解析ではグラフト欠失の独立リスク因子としてレシピエントのICU入院、MELD(model for end-stage liver disease)・PELD(pediatric end-stage liver disease)スコア25以上、外傷によるドナー死亡、ドナーの総ビリルビンピーク値5mg/dL以上が抽出された。1年グラフト生存率は小児優先システム導入前が80.0%、導入後が95.7%と有意な改善が認められ、1年患者生存率もシステム導入後に改善が得られていた。

Language：English   Publisher：Journal of Hepato Biliary Pancreatic Sciences  

Background: In Japan, there has never been a national analysis of pediatric deceased donor liver transplantation (pDDLT) based on donor and recipient factors. We constructed a Japanese nationwide database and assessed outcomes of pDDLT focusing on the pediatric prioritization system introduced in 2018. Methods: We collected data on pDDLTs (<18 years) performed between 1999 and 2021 from the Japan Organ Transplant Network and Japanese Liver Transplantation Society, identified risk factors for graft survival and compared the characteristics and graft survival in pDDLTs conducted before and after the introduction of the pediatric prioritization system. Results: Overall, 112 cases of pDDLT were included, with a 1-year graft survival rate of 86.6%. Four poor prognostic factors were identified: recipient intensive care unit stay, model for end-stage liver disease/pediatric end-stage liver disease score, donor cause of death, and donor total bilirubin. After the introduction of the system, allografts from pediatric donors were more reliably allocated to pediatric recipients and the annual number of pDDLTs increased. The 1-year graft survival rate improved significantly as did pDDLT conditions indicated by the risk factors. Conclusions: Under the revised allocation system, opportunities for pDDLT increased, resulting in favorable recipient and donor conditions and improved survival.

DOI： 10.1002/jhbp.12062

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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 (CCl<inf>4</inf>)-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 CCl<inf>4</inf>-injured mice groups, while control and non-transplanted CCl<inf>4</inf>-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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Language：English   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   Publisher：Pediatric Transplantation  

Background: Although the outcomes of living donor liver transplantation (LDLT) for pediatric acute liver failure (PALF) have improved, patient survival remains lower than in patients with chronic liver disease. We investigated whether the poor outcomes of LDLT for PALF persisted in the contemporary transplant era. Methods: We analyzed 193 patients who underwent LDLT between December 2000 and December 2020. The outcomes of patients managed in 2000–2010 (era 1) and 2011–2020 (era 2) were compared. Results: The median age at the time of LDLT was 1.2 years both eras. An unknown etiology was the major cause in both groups. Patients in era 1 were more likely to have surgical complications, including hepatic artery and biliary complications (p = 0.001 and p = 0.013, respectively). The era had no impact on the infection rate after LDLT (cytomegalovirus, Epstein–Barr virus, and sepsis). The mortality rates of patients and grafts in era one were significantly higher (p = 0.03 and p = 0.047, respectively). The 1- and 5-year survival rates were 76.4% and 70.9%, respectively, in era 1, while they were 88.3% and 81.9% in era 2 (p = 0.042). Rejection was the most common cause of graft loss in both groups. In the multivariate analysis, sepsis during the 30 days after LDLT was independently associated with graft loss (p = 0.002). Conclusions: The survival of patients with PALF has improved in the contemporary transplant era. The early detection and proper management of rejection in patients, while being cautious of sepsis, should be recommended to improve outcomes further.

DOI： 10.1111/petr.14838

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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：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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PubMed

Language：English   Publisher：Leukemia  

Aggressive natural killer cell leukemia (ANKL) is a rare hematological malignancy with a fulminant clinical course. Our previous study revealed that ANKL cells proliferate predominantly in the liver sinusoids and strongly depend on transferrin supplementation. In addition, we demonstrated that liver-resident ANKL cells are sensitive to PPMX-T003, an anti-human transferrin receptor 1 inhibitory antibody, whereas spleen-resident ANKL cells are resistant to transferrin receptor 1 inhibition. However, the microenvironmental factors that regulate the iron dependency of ANKL cells remain unclear. In this study, we first revealed that the anti-neoplastic effect of PPMX-T003 was characterized by DNA double-strand breaks in a DNA replication-dependent manner, similar to conventional cytotoxic agents. We also found that the influx of extracellular amino acids via LAT1 stimulated sensitivity to PPMX-T003. Taken together, we discovered that the amount of extracellular amino acid influx through LAT1 was the key environmental factor determining the iron dependency of ANKL cells via adjustment of their mTOR/Myc activity, which provides a good explanation for the different sensitivity to PPMX-T003 between liver- and spleen-resident ANKL cells, as the liver sinusoid contains abundant amino acids absorbed from the gut. (Figure presented.).

DOI： 10.1038/s41375-024-02296-6

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PubMed

Language：English   Publisher：Cancer Research  

Triple-negative breast cancer (TNBC) chemoresistance hampers the ability to effectively treat patients. Identification of mechanisms driving chemoresistance can lead to strategies to improve treatment. Here, we revealed that protein arginine methyltransferase-1 (PRMT1) simultaneously methylates D-3-phosphoglycerate dehydrogenase (PHGDH), a critical enzyme in serine synthesis, and the glycolytic enzymes PFKFB3 and PKM2 in TNBC cells. ¹³C metabolic flux analyses showed that PRMT1-dependent methylation of these three enzymes diverts glucose toward intermediates in the serine-synthesizing and serine/glycine cleavage pathways, thereby accelerating the production of methyl donors in TNBC cells. Mechanistically, PRMT1-dependent methylation of PHGDH at R54 or R20 activated its enzymatic activity by stabilizing 3-phos-phoglycerate binding and suppressing polyubiquitination. PRMT1-mediated PHGDH methylation drove chemoresistance independently of glutathione synthesis. Rather, activation of the serine synthesis pathway supplied a-ketoglutarate and citrate to increase palmitate levels through activation of fatty acid synthase (FASN). Increased palmitate induced protein S-palmitoylation of PHGDH and FASN to further enhance fatty acid synthesis in a PRMT1-dependent manner. Loss of PRMT1 or pharmacologic inhibition of FASN or protein S-palmitoyltransferase reversed chemoresistance in TNBC. Furthermore, IHC coupled with imaging MS in clinical TNBC specimens substantiated that PRMT1-mediated methylation of PHGDH, PFKFB3, and PKM2 correlates with chemoresistance and that metabolites required for methylation and fatty acid synthesis are enriched in TNBC. Together, these results suggest that enhanced de novo fatty acid synthesis mediated by coordinated protein arginine methylation and protein S-palmitoylation is a therapeutic target for overcoming chemoresistance in TNBC. Significance: PRMT1 promotes chemoresistance in TNBC by methylating metabolic enzymes PFKFB3, PKM2, and PHGDH to augment de novo fatty acid synthesis, indicating that targeting this axis is a potential treatment strategy.

DOI： 10.1158/0008-5472.CAN-23-2266

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PubMed

Language：English   Publisher：Histochemistry and Cell Biology  

c-Jun NH<inf>2</inf>-terminal protein kinase (JNK) and p38 are stress-activated mitogen-activated protein kinases (MAPK) that are phosphorylated by various stimuli. It has been reported that the loss of desmoglein (DSG) 3, a desmosomal transmembrane core molecule, in keratinocytes impairs cell–cell adhesion accompanied by p38 MAPK activation. To understand the biological role of DSG3 in desmosomes and its relationship with stress-activated MAPKs, we established DSG3 knockout keratinocytes (KO cells). Wild-type cells showed a linear localization of DSG1 to cell–cell contacts, whereas KO cells showed a remarkable reduction despite the increased protein levels of DSG1. Cell–cell adhesion in KO cells was impaired over time, as demonstrated by dispase-based dissociation assays. The linear localization of DSG1 to cell–cell contacts and the strength of cell–cell adhesion were promoted by the pharmacological inhibition of JNK. Conversely, pharmacological activation of JNK, but not p38 MAPK, in wild-type cells reduced the linear localization of DSG1 in cell–cell contacts. Our data indicate that DSG1 and DSG2 in KO cells cannot compensate for the attenuation of cell–cell adhesion strength caused by DSG3 deficiency and that JNK inhibition restores the strength of cell–cell adhesion by increasing the linear localization of DSG1 in cell–cell contacts in KO cells. Inhibition of JNK signaling may improve cell–cell adhesion in diseases in which DSG3 expression is impaired.

DOI： 10.1007/s00418-023-02264-8

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PubMed

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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PubMed

Language：English   Publisher：Physica Scripta  

Continuous-variable quantum key distribution (CV-QKD) has many practical advantages including compatibility with current optical communication technology. Implementation using heterodyne measurements is particularly attractive since it eliminates the need for active phase locking of the remote pair of local oscillators, but the full security of CV QKD with discrete modulation was only proved for a protocol using homodyne measurements. Here we propose an all-heterodyne CV-QKD protocol with binary modulation and prove its security against general attacks in the finite-key regime. Although replacing a homodyne measurement with a heterodyne measurement would be naively expected to incur a 3-dB penalty in the rate-distance curve, our proof achieves a key rate with only a 1-dB penalty.

DOI： 10.1088/1402-4896/ad1022

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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  

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Language：English  

Web of Science

Language：Japanese   Publisher：The Japan Society for Transplantation  

<p>【はじめに】乳幼児例の肝移植はグラフトが大きく、腹腔内に還納することでグラフトの血流障害や腹部コンパートメント症候群を起こすことがある。今回、当科の腹壁閉鎖困難例を検討した。【対象と方法】現在までに肝移植を施行した乳児例57例を対象とした。腹壁閉鎖困難例18例と腹壁閉鎖可能例39例を後方視的に比較検討した。腹壁閉鎖困難例はゴアテックスシートを用いて閉腹した。【結果】腹壁閉鎖困難例は閉鎖可能例と比較して、性別、移植時月齢に有意差は認めなかったが、有意に体重が小さかった(5,723 vs 6,425g)。グラフト重量、GRWR、GV/SLVはそれぞれ175 vs 202.5g, 3.03 vs 3.07%, 81.8 vs 86.3%と両群間に有意差は認めなかった。グラフトの種類は、減寸グラフトの割合が腹壁閉鎖困難例で有意に多かった(61 vs 26%)。血管合併症は腹壁閉鎖困難例で1例(5.6%)、閉鎖可能例で5例(8.8%)認め、再手術を要したが、有意差はなかった。ゴアテックスは中央値3カ月で摘出されており、全例腹壁閉鎖可能であった。手術時間は中央値130分であり、摘出までの期間が1カ月以内の症例は癒着が比較的軽度で、手術時間も短い傾向にあった。【結語】減寸グラフトを要する低体重児は腹壁困難の高リスクであった。全例摘出可能であったが、癒着例も多く、摘出時期に関しては更なる検討が必要である。</p>

DOI： 10.11386/jst.59.supplement_s339_3

CiNii Research

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

2007～2022年に小児神経芽腫群腫瘍(PNT:神経芽腫、神経節芽腫、神経節腫)に対して内視鏡手術が施行された24例(年齢1ヵ月齢～15歳)を対象に、PNTに対する内視鏡手術の適応について検討した。24例の内訳は神経芽腫11例、神経節腫10例、神経節芽腫3例であった。腫瘍の部位は副腎腫瘍18例、縦隔腫瘍5例、後腹膜腫瘍1例であった。術前のCT所見におけるimage-defined risk factor(IDRF)は8例が陽性であった(腎血管へのcontact 6例、主気管支の圧迫2例)。21例(IDRF陰性例14/16例[87.5%]、IDRF陽性例7/8例[87.5%])で完全切除が得られた。フォローアップ中に再発は認められなかった。これらの結果から、CT所見における腎血管へのcontactおよび主気管支の圧迫は不完全切除の指標にはならないと考えられた。小児PNTに対する内視鏡的アプローチは、厳密な患者選択を行えば実行可能かつ予後良好である。

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

DOI： 10.34410/jspbm.47.0_36

CiNii Research

Language：English   Publisher：Quantum  

We developed new concentration inequalities for a quantum state on an Nqudit system or measurement outcomes on it that apply to an adversarial setup, where an adversary prepares the quantum state. Our one-sided concentration inequalities for a quantum state require the N-qudit system to be permutation invariant and are thus de-Finetti type, but they are tighter than the one previously obtained. We show that the bound can further be tightened if each qudit system has an additional symmetry. Furthermore, our concentration inequality for the outcomes of independent and identical measurements on an N-qudit quantum system has no assumption on the adversarial quantum state and is much tighter than the conventional one obtained through Azuma's inequality. We numerically demonstrate the tightness of our bounds in simple quantum information processing tasks.

DOI： 10.22331/q-2024-11-27-1540

Scopus

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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PubMed

Language：English  

DOI： 10.1007/s13691-023-00621-x

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PubMed

Language：English   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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Language：English   Publisher：Pediatric Surgery International  

Introduction: Intestinal transplantation (ITx) is the ultimate treatment for intestinal failure (IF). In Japan, most cases of IF are a result of pediatric disease, including secondary or congenital intestinal disease or allied disorders of Hirschsprung’s disease. Here, we report the results of the Japanese ITx registry. Methods: A web-based survey form was completed. We investigated the number, age, sex, indication, surgical procedure, immunosuppressants, postoperative course, and the effects of transplantation in patients who underwent cadaveric or living-donor ITx. Results: By the end of 2022, 42 cases of ITx have been performed in 38 patients in Japan. The donor sources included cadavers (29 cases) and living donors (13 cases). The surgical method was isolated ITx (N = 40) and combined liver and ITx (n = 2). Survival rates were 92%, 73%, and 59% at 1 year, 5 years, and 10 years, respectively. Ninety percent of patients completely discontinued parenteral nutrition. Approximately 80% of the patients had a performance status of 1 or less, indicating that the QOL of patients after ITx was extremely good. Conclusion: The results of ITx are acceptable to treat IF patients and the QOL after transplantation is also good.

DOI： 10.1007/s00383-023-05552-5

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PubMed

Language：English   Publisher：Journal of Pediatric Gastroenterology and Nutrition  

OBJECTIVES: Our objective was to evaluate the short- and long-term safety and efficacy of teduglutide treatment in infants and children with short bowel syndrome with intestinal failure (SBS-IF). METHODS: Two open-label phase 3 studies and 1 extension study investigated the short- and long-term safety and efficacy of teduglutide (0.05 mg/kg/day) in infants and children with SBS-IF: NCT03571516, 24-week study of infants who were randomized to receive teduglutide or standard of care (SoC); NCT02980666, 24-week study of infants and children who all received teduglutide; and NCT03268811, 24-week extension study of patients who completed NCT02980666 (patients could receive up to 48 weeks of total treatment). RESULTS: Twelve infants and 8 children enrolled in the core studies, and 2 infants and 7 children in the extension study. After 24 weeks of treatment, parenteral support (PS) requirements reduced by ≥20% from baseline for 4 infants (57.1%) and 4 children (66.7%) receiving teduglutide and for 2 infants receiving SoC (50.0%). One infant (50.0%) and 4 children (80.0%) receiving teduglutide maintained the ≥20% reduction in PS at 48 weeks of treatment. Two children receiving teduglutide achieved enteral autonomy, after 12 weeks and 28 weeks of treatment, respectively. All adverse events (AEs) were in line with known impacts of SBS-IF and adverse reactions to teduglutide. Only one serious AE (abdominal pain) was considered related to teduglutide. CONCLUSIONS: Short- and long-term treatment with teduglutide resulted in clinically meaningful reductions in PS requirements for infants and children with SBS-IF. Teduglutide was well tolerated, and efficacy improved with longer-term treatment.

DOI： 10.1097/MPG.0000000000003867

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PubMed

Language：English  

Web of Science

Language：English   Publisher：Land Use Policy  

Rural households in developing countries often depend on non-timber forest products (NTFPs), including foods, traditional medicines, fuelwood, fodder, and construction and craft materials such as bamboo and rattan, for their livelihoods, but the forests that supply NTFPs are subject to competition from other land uses. NTFP collectors who are unable to sustain their livelihoods because of changes caused in the NTFP collection environment by deforestation and degradation may even convert surrounding forests to agricultural land to compensate for their reduced incomes. This feedback loop of deforestation and degradation can create further conflicts among natural resource users and undermine long-term development goals. Here, we aimed to advance the discussion of forest conservation interventions in protected areas in developing countries—particularly the discussion of patrolling strategies in and around existing wildlife sanctuaries—by presenting a new approach that uses the case of NTFP collection and a human geography perspective. We used a structured questionnaire composed of closed questions to measure the awareness of village households of the impacts of deforestation and/or illegal extraction of trees on NTFP collection over a short period of time (2014–2016) near the Prey Long Wildlife Sanctuary in the eastern part of the Kampong Thom Province, Cambodia. Our survey was based on the conceptual framework of Bohensky and Lynam (Ecol. Soc. 10, 11; 2005) that people in complex adaptive systems change their behaviour after becoming aware of the impact or consequences of a change. The probability of a village having an affected household (i.e., a household that was aware of the impact) was predicted by using generalized linear mixed models. By using identified geospatial indicators of environmental and socioeconomic aspects, including distance between the village and the wildlife sanctuary, size of deforested area per capita in 2014–2016 within a 10-km radius of the village and proportion of households in the village that collect NTFPs for cash income, we predicted the spatial distribution of villages with affected NTFP-collecting households. Priority areas for NTFP collection were then mapped with reference to the identified indicators. By using these results, we present an approach to breaking the deforestation feedback loop by identifying priority areas for patrolling and other forest conservation interventions in the wildlife sanctuary.

DOI： 10.1016/j.landusepol.2023.106637

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Language：English  

DOI： 10.1097/01.tp.0000946048.47062.b2

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Language：English   Publisher：Journal of Pediatric Surgery  

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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PubMed

Language：English   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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PubMed

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

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

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

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

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

Language：English   Publisher：Annals of Botany  

Background and Aims: For a comprehensive understanding of the mechanisms of changing plant photosynthetic capacity during plant evolutionary history, knowledge of leaf gas exchange and optical properties are essential, both of which relate strongly to mesophyll anatomy. Although ferns are suitable for investigating the evolutionary history of photosynthetic capacity, comprehensive research of fern species has yet to be undertaken in this regard. Methods: We investigated leaf optical properties, gas exchange and mesophyll anatomy of fern species with a wide range of divergence time, using 66 ferns from natural habitats and eight glasshouse-grown ferns. We used a spectroradiometer and an integrating sphere to measure light absorptance and reflectance by the leaves. Key Results: The more newly divergent fern species had a thicker mesophyll, a larger surface area of chloroplasts facing the intercellular airspaces (Sc), thicker cell walls and large light absorptance. Although no trend with divergence time was obtained in leaf photosynthetic capacity on a leaf-Area basis, when the traits were expressed on a mesophyll-Thickness basis, trends in leaf photosynthetic capacity became apparent. On a mesophyll-Thickness basis, the more newly divergent species had a low maximum photosynthesis rate, accompanied by a low Sc. Conclusions: We found a strong link between light capture, mesophyll anatomy and photosynthesis rate in fern species for the first time. The thick mesophyll of the more newly divergent ferns does not necessarily relate to the high photosynthetic capacity on a leaf-Area basis. Rather, the thick mesophyll accompanied by thick cell walls allowed the ferns to adapt to a wider range of environments through increasing leaf toughness, which would contribute to the diversification of fern species.

DOI： 10.1093/aob/mcad025

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PubMed

Language：English   Publisher：Den Open  

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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PubMed

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。

Language：English   Publisher：Pediatric Blood and Cancer  

DOI： 10.1002/pbc.30155

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PubMed

Language：English  

DOI： 10.1186/s40792-023-01618-7

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PubMed

Language：English  

Web of Science

Language：English   Publisher：International Journal of Surgery Case Reports  

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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PubMed

Language：English   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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PubMed

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

CiNii Research

Language：English   Publisher：Quantum  

Recent studies showed the finite-size security of binary-modulation CV-QKD protocols against general attacks. However, they gave poor key-rate scaling against transmission distance. Here, we extend the security proof based on complementarity, which is used in the discrete-variable QKD, to the previously developed binary-modulation CV-QKD protocols with the reverse reconciliation under the finite-size regime and obtain large improvements in the key rates. Notably, the key rate in the asymptotic limit scales linearly against the attenuation rate, which is known to be optimal scaling but is not achieved in previous finite-size analyses. This refined security approach may offer full-fledged security proofs for other discrete-modulation CV-QKD protocols.

DOI： 10.22331/Q-2023-08-29-1095

Scopus

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：English   Publisher：Pediatric Surgery International  

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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PubMed

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 CCl<inf>4</inf> 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 CCl<inf>4</inf> treated mice. Results: SHED-Hep transplantation (SHED-HepTx) improved trabecular bone loss and liver fibrosis in chronic CCl<inf>4</inf>-treated mice. SHED-HepTx reduced hepatic ROS production and interleukin 17 (Il-17) expression under chronic CCl<inf>4</inf> 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 CCl<inf>4</inf>-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 CCl<inf>4</inf>-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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PubMed

Language：English   Publisher：Comparative Clinical Pathology  

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

Scopus

Language：English   Publisher：Pediatric Surgery International  

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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PubMed

Language：English  

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Language：English  

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Language：English  

Web of Science

Language：English   Publisher：Journal of Cancer Survivorship  

Purpose: To characterize the issues regarding work and employment specific to allogeneic hematopoietic cell transplantation (allo-HCT) survivors, we conducted a nationwide cross-sectional questionnaire survey. Methods: We targeted allo-HCT survivors employed at diagnosis, aged 20–64 at survey, and survived ≥2 years without relapse. The questionnaire included the timing of and reasons for resignation (termination of employment contract), and patient-related, HCT-related, work-related, and HCT center-related factors. Results: A total of 1048 eligible participants were included in the analysis (response rate, 60%). The median time after allo-HCT was 5 years (range, 2–30) at the time of survey. After diagnosis, 41% of participants resigned from work throughout the course of treatment. The most frequent timing of the first resignation was “after discharge post-HCT” (46%), followed by “from diagnosis to initial treatment” (27%). Factors significantly associated with resignation included female gender, older age, and part-time employment. Favorable factors included the presence of occupational health staff at the workplace, employment of ≥10 years, and self-employed/freelance. After resignation, the overall incidence of return to work with some accommodations was 76% at 5 years after HCT, but it was 52% without any accommodation. Conclusions: Overall, the rate of resignation was 41%, and the most frequent timing of resignation was after discharge post-HCT, accounting for approximately half of the resignations (46%). Workplace accommodations increased the rate of return to work from 52% to 76%. Implications for Cancer survivors: Early detection of employment-related concerns and support throughout the treatment process are necessary for patients receiving allo-HCT.

DOI： 10.1007/s11764-021-01092-w

Scopus

Language：English   Publisher：Ja Clinical Reports  

Background: Carbamoyl phosphate synthetase deficiency (CPS1D) is a urea-cycle disorder (UCD). We report successful perioperative management of pediatric living donor liver transplantation (LDLT) in a CPS1D patient. Case presentation: A 10-year-old female patient with CPS1D underwent LDLT. Proper administration of dextrose 50% and 60 kcal/kg/day with l-arginine and l-carnitine resulted in the avoidance of intraoperative hyperammonemia induced by hypercatabolism. Serum ammonia level transiently increased to 61 mmol/L in the anhepatic phase and decreased to 44 mmol/L after reperfusion. Conclusions: We suggest anesthesia management with administration of dextrose to avoid hyperammonemia during LDLT in patients with CPS1D.

DOI： 10.1186/s40981-022-00558-9

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PubMed

Language：English   Publisher：Medical Molecular Morphology  

Tumor-associated macrophages (TAMs) have protumor functions in various cancers. However, their significance in hepatoblastoma, the most common liver tumor in children, remains unclear. The aim of this study was to explore the potential roles of TAMs in hepatoblastoma. Immunohistochemical analysis revealed that the density of CD204-positive TAMs was significantly higher in the embryonal component than in other histological subtypes of hepatoblastoma. An in vitro co-culture study with Huh6 cells and human monocyte-derived macrophages (HMDMs) showed that macrophage-colony-stimulating factor receptor (M-CSFR) was strongly up-regulated in the Huh6 cells that were directly co-cultured with HMDMs. The expressions of M-CSFR ligands (interleukin-34 and M-CSF) were also increased by co-culture with HMDMs. The proliferation of HepG2 cells (another hepatoblastoma cell line expressing M-CSFR) was inhibited by an M-CSFR inhibitor. M-CSFR was found to be highly expressed in the embryonal component and in recurrent lesions. The number of CD204-positive macrophages was also higher in the M-CSFR-positive areas than in the M-CSFR-negative areas. Thus, M-CSFR expression appeared to be induced by cell–cell contact with macrophages in hepatoblastoma cells, and M-CSFR inhibitor is potentially effective against M-CSFR-positive hepatoblastoma, especially recurrent cases.

DOI： 10.1007/s00795-022-00323-y

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PubMed

Language：English   Publisher：Springer Berlin Heidelberg  

症例は10歳女児で、7歳時にカルバモイルリン酸合成酵素欠損症と診断され、9歳時に生体肝移植を受けていた。術前検査で特記すべき異常はなかった。肝機能はChild-Pugh分類クラスAであったが、血清アンモニア値は45μmol/Lであった。術中、60kcal/kg/日に相当するブドウ糖50%、L-アルギニン、L-カルニチンを投与して、高アンモニア血症を回避した。無肝期に血清アンモニア値が一時的に61mmol/Lまで上昇したが、再灌流後に44mmol/Lまで低下し、手術終了時は50mmol/Lであった。術後、ICUに入室し、L-カルニチンとブドウ糖の投与を継続した。血清アンモニア値は術後3日目に正常範囲に低下した。経過は良好で術後3日目に一般病棟に転棟し、27日目に退院した。

Language：English   Publisher：Journal of Pediatric Surgery  

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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PubMed

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

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

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

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

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

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

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

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

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

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

Language：English   Publisher：Hepatology Research  

Background: Biliary atresia (BA) is among the commonest indications for liver transplantation (LT) in children. We examined whether serum matrix metalloproteinase-7 (MMP-7) is useful for diagnosis of BA in Japanese infants, and whether serum MMP-7 concentrations before and after Kasai portoenterostomy (KP) predicted LT within a year. Methods: Subjects under 6 months old at eight pediatric centers in Japan were enrolled retrospectively, including patients with cholestasis and normal controls (NC) without liver disease. Patients with cholestasis were divided into groups representing BA versus cholestasis from other causes (non-BA). Serum samples were collected from patients with BA at diagnosis and 1 and 4 weeks after KP, as well as from non-BA and NC. Results: Serum MMP-7 concentrations were significantly higher in BA at diagnosis (median, 89.1 ng/ml) than in non-BA (11.0; p < 0.001) or NC (10.3; p < 0.001). Receiver operating characteristic (ROC) analysis of MMP-7 for BA versus non-BA yielded an area under the ROC curve of 0.99 (95% confidence interval, 0.96–1.00). An optimal cut-off value of 18.6 ng/ml for serum MMP-7 in diagnosing BA demonstrated sensitivity and specificity of 100% and 90%, respectively. Serum MMP-7 before and 1 week and 4 weeks after KP did not differ significantly between BA requiring only KP and BA requiring LT after KP. Conclusion: Serum MMP-7 is a useful marker for diagnosis of BA in Japanese infants, but it could not predict LT within a year.

DOI： 10.1111/hepr.13753

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PubMed

Language：English   Publisher：Scientific Reports  

Hirschsprung’s disease is a congenital entero-neuropathy that causes chronic constipation and intestinal obstruction. New treatments for entero-neuropathy are needed because current surgical strategies have limitations5. Entero-neuropathy results from enteric nervous system dysfunction due to incomplete colonization of the distal intestine by neural crest-derived cells. Impaired cooperation between the enteric nervous system and intestinal pacemaker cells may also contribute to entero-neuropathy. Stem cell therapy to repair these multiple defects represents a novel treatment approach. Dental pulp stem cells derived from deciduous teeth (dDPSCs) are multipotent cranial neural crest-derived cells, but it remains unknown whether dDPSCs have potential as a new therapy for entero-neuropathy. Here we show that intravenous transplantation of dDPSCs into the Japanese Fancy-1 mouse, an established model of hypoganglionosis and entero-neuropathy, improves large intestinal structure and function and prolongs survival. Intravenously injected dDPSCs migrate to affected regions of the intestine through interactions between stromal cell-derived factor-1α and C-X-C chemokine receptor type-4. Transplanted dDPSCs differentiate into both pacemaker cells and enteric neurons in the proximal colon to improve electrical and peristaltic activity, in addition to their paracrine effects. Our findings indicate that transplanted dDPSCs can differentiate into different cell types to correct entero-neuropathy-associated defects.

DOI： 10.1038/s41598-022-10077-3

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PubMed

Language：English   Publisher：Clinical and Experimental Hypertension  

Introduction: Increased sympathetic output contributes to cardiac hypertrophy. Sympathoexcitation is induced by activating the cardiac sympathetic afferent nerves through transient receptor potential vanilloid 1 (TRPV1) in cardiac afferent endings. Brainstem nucleus tractus solitarius (NTS) receives the sensory cardiac afferent inputs. Brain-derived neurotrophic factor (BDNF) is released within NTS from sensory neurons in an activity-dependent manner. Additionally, BDNF in NTS tonically regulates sympathetic activity. Therefore, we hypothesized that TRPV1-expressing cardiac afferent nerves contribute to cardiac hypertrophy in accompany with an increased BDNF expression in NTS. Methods and Results: Abdominal aortic banding (AB) or sham operation was conducted in wild-type C57BL/6 J (WT-AB) and TRPV1 knockout mice (TRPV1 KO-AB). At 8 weeks post-operation, echocardiographic left ventricular wall thickness and heart weight/body weight ratio were significantly greater in WT-AB than WT-Sham mice, and these hypertrophic indexes were attenuated in TRPV1 KO-AB mice. Among the groups, left ventricular fractional shortening was not different. The protein levels of TRPV1 in heart and BDNF in NTS were significantly increased in WT-AB compared to WT-Sham mice, whereas BDNF expression in NTS was not increased by AB in TRPV1-KO mice. Chemical ablation of TRPV1-expressing cardiac afferents attenuated the AB-induced cardiac hypertrophy and increase in BDNF in NTS. Sympathetic activity analyzed using heart rate variability, and sympathoexcitatory responses to the stimulation of cardiac afferents were increased in WT-AB compared to WT-Sham mice. Conclusion: TRPV1-expressing cardiac afferent nerves may contribute to pressure overload-induced cardiac hypertrophy in accompany with the increased BDNF within NTS.

DOI： 10.1080/10641963.2022.2029470

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PubMed

Language：English   Publisher：Cancer Medicine  

Hepatoblastoma is the most common pediatric liver tumor, but little research has been done on the role of macrophages in hepatoblastoma. The purpose of this study was to gain insight into potential roles for macrophages in hepatoblastoma. Paraffin-embedded specimens from 56 patients who underwent surgical resection were examined with immunohistochemical staining for the macrophage-specific markers, Iba1 and CD163. Significant differences were seen among histological subtypes. Significantly increased numbers of macrophages were detected in embryonal components compared to fetal components in the mixed epithelial type. In vitro studies using human monocyte-derived macrophages and two hepatoblastoma cell lines (HepG2 and Huh6) were performed. Conditioned medium from these cell lines induced increased CD163 expression in macrophages. Direct co-culture with macrophages induced tumor cell proliferation via induction of protumor cytokine secretion from macrophages. Direct co-culture with macrophages also induced interleukin (IL)-34 overexpression by Huh6 cells via Brd4 signaling. IL-34 overexpression promoted tumor cell proliferation and chemoresistance. High IL-34 and Brd4 expression was detected in embryonal components, which have potentially higher proliferation activity than fetal components. In conclusion, IL-34 expression in embryonal components may induce macrophage chemotaxis in a paracrine manner, and tumor cell proliferation and chemoresistance in an autocrine manner. IL-34 is a potential therapeutic target for hepatoblastoma.

DOI： 10.1002/cam4.4537

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PubMed

Language：English   Publisher：Pediatrics International  

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 cmH<inf>2</inf>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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PubMed

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  

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：English  

CiNii Research

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：English  

CiNii Research

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

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

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

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

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

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

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

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

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

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

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

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

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

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

PURPOSE OF REVIEW: Intestinal transplantation (ITx) activity remains low in East Asia. We conducted a multinational, retrospective study on patients who underwent ITx in Korea, Taiwan, and Japan, to provide an overview and to foresee future developments and collaborations in this region. RECENT FINDINGS: Total 71 ITx were performed in 67 patients. Living donor ITx was most commonly conducted in Japan (n = 13). Despite the low caseload, all three countries demonstrated acceptable patient survival rates of approximately 70% at 5 years. Over 70% of recipients with a functioning graft were free from total parenteral nutrition. SUMMARY: There is an urgent need to establish a nationwide and multinational registry of ITx recipients and patients with intestinal failure in East Asia. An efficient referral system to specialized intestinal rehabilitation and ITx centers and a multidisciplinary team approach is also warranted to provide state-of-the-art treatment for patients desperately waiting for a chance to survive.

DOI： 10.1097/MOT.0000000000000750

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

Background: Human deciduous pulp stem cells (hDPSCs) have remarkable stem cell potency associated with cell proliferation, mesenchymal multipotency, and immunosuppressive function and have shown beneficial effects in a variety of animal disease models. Recent studies demonstrated that hDPSCs exhibited in vivo anti-fibrotic and anti-inflammatory action and in vivo hepatogenic-associated liver regeneration, suggesting that hDPSCs may offer a promising source with great clinical demand for treating liver diseases. However, how to manufacture ex vivo large-scale clinical-grade hDPSCs with the appropriate quality, safety, and preclinical efficacy assurances remains unclear. Methods: We isolated hDPSCs from human deciduous dental pulp tissues formed by the colony-forming unit-fibroblast (CFU-F) method and expanded them under a xenogeneic-free and serum-free (XF/SF) condition; hDPSC products were subsequently stored by two-step banking including a master cell bank (MCB) and a working cell bank (WCB). The final products were directly thawed hDPSCs from the WCB. We tested the safety and quality check, stem cell properties, and preclinical potentials of final hDPSC products and hDPSC products in the MCB and WCB. Results: We optimized manufacturing procedures to isolate and expand hDPSC products under a XF/SF culture condition and established the MCB and the WCB. The final hDPSC products and hDPSC products in the MCB and WCB were validated the safety and quality including population doubling ability, chromosome stability, microorganism safety, and stem cell properties including morphology, cell surface marker expression, and multipotency. We also evaluated the in vivo immunogenicity and tumorigenicity and validated in vivo therapeutic efficacy for liver regeneration in a CCl₄-induced chronic liver fibrosis mouse model in the final hDPSC products and hDPSC products in the WCB. Conclusion: The manufacture and quality control results indicated that the present procedure could produce sufficient numbers of clinical-grade hDPSC products from a tiny deciduous dental pulp tissue to enhance clinical application of hDPSC products in chronic liver fibrosis.

DOI： 10.1186/s13287-020-01630-w

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

Purpose: Mesenchymal stem cell (MSC)-based cell therapies have emerged as a promising treatment option for various diseases. Due to the superior survival and higher differentiation efficiency, three-dimensional spheroid culture systems have been an important topic of MSC research. Stem cells from human exfoliated deciduous teeth (SHED) have been considered an ideal source of MSCs for regenerative medicine. Thus, in the present study, we introduce our newly developed method for fabricating SHED-based micro-hepatic tissues, and demonstrate the therapeutic effects of SHED-based micro-hepatic tissues in mouse disease models. Methods: SHED-converted hepatocyte-like cells (SHED-HLCs) were used for fabricating spherical micro-hepatic tissues. The SHED-HLC-based spheroids were then transplanted both into the liver of mice with CCl₄-induced chronic liver fibrosis and the kidney of factor VIII (F8)-knock-out mice. At 4 weeks after transplantation, the therapeutic efficacy was investigated. Results: Intrahepatic transplantation of SHED-HLC-spheroids improved the liver dysfunction in association with anti-fibrosis effects in CCl₄-treated mice. Transplanted SHED-converted cells were successfully engrafted in the recipient liver. Meanwhile, renal capsular transplantation of the SHED-HLC-spheroids significantly extended the bleeding time in F8-knock-out mice. Conclusions: These findings suggest that SHED-HLC-based micro-hepatic tissues might be a promising source for treating pediatric refractory diseases, including chronic liver fibrosis and hemophilia A.

DOI： 10.1007/s00383-019-04564-4

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

Background: Indocyanine green (ICG) fluorescence imaging is a promising tool for intraoperative decision-making. The aim of this study was to evaluate the utility of near-infrared fluorescence cholangiography (NIR-FCG) with ICG in primary surgery for biliary atresia (BA). Methods: We performed NIR-FCG with ICG in 10 BA patients and observed the fluorescence of their hilar micro-bile ducts and hilar exudate in order to assess the appropriate level at which to dissect the hilar fibrous corn. We compared the jaundice outcome of 10 patients using NIR-FCG (Group A) to that of 35 historical patients in whom NIR-FCG had not been used (Group B). Results: The mean age of patients was 74.8 days. The classification of BA was type I in two cases and type-III in eight cases. NIR-FCG visualized the hilar micro-bile ducts, and the incidence of positive fluorescence was 80%. The ratio of postoperative normalization of hyperbilirubinemia in Group A was significantly higher than that in Group B (1.0 vs. 0.65, p < 0.05). Conclusion: NIR-FCG provided important objectifiable information about the biliary structures in surgery of BA. Although the number of cases was small, our results suggest that NIR-FCG may be useful for improving the outcome of primary surgery for BA. Type of Study: Study of Diagnostic Test. Level of Evidence: Level III.

DOI： 10.1016/j.jpedsurg.2019.08.029

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

Objectives: Wound dehiscence is a common surgical complication, especially among pediatric liver trans - plant recipients in our center. In 2013, we introduced negative pressure wound therapy as a preventive treatment. We herein report the clinical outcomes of this intervention. Materials and Methods: We conducted a retrospective review of the 26 pediatric liver transplant recipients in our center since 2011. We excluded 1 girl whose wound could not be closed due to bowel edema. The first 13 of the 25 remaining patients were treated with conventional wound management (conventional group). The latter 12 were treated with prophylactic negative pressure wound therapy (prophylactic group). Incidences of surgical complications and patient characteristics were compared between groups. Results: Wound dehiscence occurred in 7 of the 13 patients in the conventional group and 3 of the 12 patients in the prophylactic group. When restricted to dehiscence that required surgical debridement, there were 6 cases in the conventional group and no cases in the prophylactic group. Although background data showed that liver insufficiency in the prophylactic group was more severe, this group had a lower incidence of wound dehiscence (P = .015). Conclusions: Prophylactic negative pressure wound therapy is thought to be effective for preventing wound dehiscence among pediatric liver transplant recipients.

DOI： 10.6002/ect.2018.0076

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

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

Background: Technology for detecting liver tumors and identifying the bile ducts using indocyanine green (ICG) has recently been developed. However, the usefulness and limitations of ICG navigation surgery for hepatoblastoma (HB) have not been fully clarified. We herein report our experiences with surgical navigation using ICG for in HB patients. Methods: In 5 HB patients, 10 ICG navigation surgeries were performed using a 10-mm infrared fluorescence imaging scope after the injection of 0.5 mg/kg ICG intravenously. The surgical and clinical features were collected retrospectively. Results: Navigation surgery using ICG was performed for primary liver tumors in 4 cases, and the timing of ICG injection was 90.5 ± 33.7 h before the operation. All tumors exhibited intense fluorescence from the liver surface. ICG navigation for the primary liver tumor was useful for detecting the residual tumor at the stump and invasion to the diaphragm during surgery. Six lung surgeries using ICG navigation were performed. The timing of ICG injection was 21.8 ± 3.4 h before the operation. The size of the metastatic tumor was 7.4 ± 4.1 mm (1.2–15 mm). Of 11 metastatic tumors detected by computed tomography (CT), 10—including the smallest tumor (1.2 mm)—were able to be detected by ICG from the lung surface. The depth of the 10 ICG-positive tumors from the lung surface was 0.9 ± 1.9 mm (0–6 mm), and the depth of the single ICG-negative tumor was 12 mm. One lesion not detected by CT showed ICG false positivity. Conclusion: Navigation surgery using ICG for patients with HB was useful for identifying tumors and confirming complete resection. However, in ICG navigation surgery, we must be aware of the limitations with regard to the tumor size and the depth from the surface.

DOI： 10.1007/s00383-019-04458-5

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

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

Stem cells from human exfoliated deciduous teeth (SHEDs), being a type of mesenchymal stem cell, are an ideal cell source for regenerative medicine. They have minimal risk of oncogenesis, high proliferative capacity, high multipotency, and immunosuppressive ability. Stem cell transplantation using SHED has been found to have an anti-fibrotic effect on liver fibrosis in mice. SHED transplantation and the bio 3D printer, which can create scaffold-free 3-D images of the liver and diaphragm, provide a new innovative treatment modality for intractable pediatric surgical diseases such as biliary atresia and diaphragmatic hernia.

DOI： 10.1007/s00595-019-01783-z

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

Background/Purpose: Intracranial hemorrhage (ICH) is a severe complication of biliary atresia (BA). We aimed to compare the clinical data of BA patients with and without ICH. Methods: Sixty-three BA patients who underwent Kasai portoenterostomy were included in this study. We retrospectively reviewed their clinical records, and compared the ICH and non-ICH groups. Results: ICH occurred in seven patients (11.1%). The patients with ICH were significantly older at the time of Kasai portoenterostomy (median age: 90.0 vs 65.5 days). The hepatobiliary enzyme levels of the patients with ICH were significantly lower in comparison to the patients without ICH (T-Bil 6.7 vs 9.8 mg/dl; AST 95 vs 194 U/L; ALT 44 vs 114 U/L). On the other hand, the coagulation test values of the patients with ICH were significantly higher in comparison to the patients without ICH (PT 50.0 vs 12.4 s; APTT 200.0 vs 36.9 s). Although the survival rates did not differ to a statistically significant extent, persistent neurological sequelae occurred in two patients in the ICH group. Conclusions: The hepatobiliary enzyme levels of the patients with ICH were significantly lower than those without ICH. However, coagulopathy was found to be significantly more progressive in patients with ICH. Levels of Evidence: Level III.

DOI： 10.1016/j.jpedsurg.2018.04.031

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

Background: Donor age for intestinal transplantation (ITx) is somewhat younger than that for other solid organs. Clear criteria for adequate donors have not been established. There is a donor scarcity for ITx in Japan due to the shortage of young donors. Methods: We reviewed outcomes associated with ITx in Japan based on donor age for cadaveric and living donation. Results: Standardized report forms were sent to all known ITx programs, asking for information on ITxs performed between 1996 and 2016. All programs responded. Patient and graft survival estimates were obtained using the Kaplan-Meier method. Five institutions provided data on 27 grafts in 24 patients. There were 14 cadaveric and 13 living donor transplants. Median donor age for ITxs was 40 (range, 17–60) years. Graft survival at 5 years was 66% for patients >40 years old (n = 18) and 47% for those <40 years old (n = 9), not a statistically significant difference (P =.49). Graft survival at 5 years was 60% in those >50 years old (n = 5) and 57% for those <50 years old (n = 22), again not a significant difference (P =.27). Conclusion: There is no difference in survival between for those with donor age <40 vs >40 years. Donor age for ITx can be extended from >40 to up to 50 years, which may help to mitigate the donor shortage. It will be necessary to clarify the donor criteria for ITx through accumulation of further data on ITx.

DOI： 10.1016/j.transproceed.2018.04.021

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

Intestinal transplantation (ITx) is a treatment for refractory intestinal failure (IF). However, the indications for and timing of ITx are still controversial because the course of IF is unknown. We performed a prospective multi-institutional cohort study to identify the prognostic factors for referral to an ITx facility. Patients under 18 years of age in Japan who suffered from IF and had received parenteral nutrition for longer than 6 months were enrolled in this study. They were followed up for 3 years. Seventy-two patients were followed. The mean age at the beginning of the study was 7.0 years. Diagnoses were short gut syndrome (n = 25), motility disorder (n = 45), and other (n = 2). The overall 3-year survival rate was 95%. The 3-year survival rate was 86% in patients with intestinal-failure-associated liver disease (IFALD) (n = 6) compared to 97% in those without IFALD (n = 66) (P =.0003). Furthermore, the 3-year survival rates of patients who did and did not meet the criteria for ITx were 82% (n = 11) and 97% (n = 62), respectively (P =.034). Six (44%) of 14 patients whose performance status (PS) was ≥3 at enrollment were dead or still had a PS ≥ 3 at 3 years. This study indicates that IFALD is a poor prognostic factor in pediatric patients with IF. Our indication for ITx, namely the presence of IFALD or loss of more than 2 parenteral nutrition access sites, seems to be applicable.

DOI： 10.1016/j.transproceed.2018.03.067

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

A graft volume/standard liver volume ratio (GV/SLV) > 35% or graft/recipient weight ratio (GRWR) > 0.8% has been considered as a standard criteria of graft selection. Even if the graft size meets these selection criteria, small-for-size syndrome can still occur depending on the portal venous flow (PVF). The aim of this study was to identify other factors contributing to portal hyperperfusion and the post-transplant course, focusing on the graft volume-to-spleen volume ratio (GV/SV). Thirty-seven BA patients who underwent living donor liver transplantation were reviewed retrospectively. First, we evaluated the preoperative factors contributing to portal hyperperfusion. Second, we evaluated the factors contributing to post-transplant complications, such as thrombocytopenia, hyperbilirubinemia, and coagulopathy. The GV/SLV was >35% in all cases; however, portal hyperperfusion (≥250 ml/min/100 g graft) was found in 12 recipients (35.3%). Furthermore, although the GRWR was >0.8% in over 90% of cases, portal hyperperfusion was found in 10 recipients (32.3%). In contrast, the GV/SV showed a significant correlation with the PVF after reperfusion. If the GV/SV was <0.88, about 80% of recipients developed portal hyperperfusion. Furthermore, the GV/SV also showed a significant correlation with post-transplant persistent thrombocytopenia and hyperbilirubinemia. The GV/SV < 0.88 predicts portal hyperperfusion, post-transplant persistent thrombocytopenia, and hyperbilirubinemia.

DOI： 10.1111/tri.13131

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

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

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

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

Background: No protocol has been established for the diagnosis and management of chylous ascites after liver transplantation (LT). In this study, we retrospectively reviewed our cases of posttransplant chylous ascites (PTCA) and aimed to propose a diagnostic and management protocol. Patients and methods: We retrospectively reviewed the clinical records of 96 LT recipients who underwent LT at our department. The incidence of PTCA and the associated risk factors were analyzed and our protocol for chylous ascites was evaluated. Results: PTCA occurred in 6 (6.3%) patients (mean age: 10.7 ± 11.0 years) at a mean of 10.8 ± 3.6 days after LT. The primary disease in all of PTCA cases was biliary atresia (BA). The periportal lymphadnopathy was an independent risk factor for PTCA. In all cases PTCA successfully resolved according to our protocol. Octreotide was administered in 4 of our 6 PTCA cases. The mean postoperative hospital stay was 40.2 ± 8.4 days, which was similar to that of cases without PTCA. Conclusions: The incidence of PTCA in LT patients, especially in those with BA, is relatively high. Our diagnostic criteria and our management protocol were helpful for patients with refractory ascites after LT. Type of study: Diagnostic test: Level II. Treatment study: Level III.

DOI： 10.1016/j.jpedsurg.2017.06.004

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

The mortality rate of giant omphalocele has improved; however long-term follow-up has revealed umbilical defects and deformities after primary closure. We herein report the efficacy of a new abdominal wall reconstruction strategy combining a component separation technique with delayed natural and deep umbilicoplasty.

DOI： 10.1016/j.epsc.2018.02.018

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

Severe portopulmonary hypertension (POPH) is a contraindication for liver transplantation (LT) because of the high risk of postoperative heart failure. The early detection of POPH is important for patients with biliary atresia (BA). Brain natriuretic peptide (BNP) is known to be correlated with liver fibrosis in patients with liver cirrhosis. The aim of this study was to elucidate the efficacy of BNP measurement for the follow-up of patients with BA. Thirty-two patients with BA were identified from September 2011 to December 2016. As indices of liver fibrosis/cirrhosis, APRI (P < .0001), FIB-4 (P < .0001), Child-Pugh score (P < .0001), IV collagen (P = .0005), and hyaluronic acid (P = .0291) had high or moderate correlations with BNP. Patients with splenomegaly, esophageal varices, liver fibrosis, and collateral veins had significantly higher BNP levels than those without. Patients diagnosed with POPH had significantly higher BNP levels in comparison with those patients without (P = .0068). In contrast, PELD/MELD scores showed an almost negligible correlation with the BNP level. LT was successful in 3 asymptomatic BA patients with POPH who had high BNP levels despite the low PELD/MELD scores. In conclusion, routine serum BNP surveillance can be easy to predict asymptomatic POPH. This may help to identify POPH before it reaches a stage that would contraindicate LT.

DOI： 10.1111/petr.13203

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

Background: The diagnosis of biliary atresia (BA) is still challenging. The aim of this study was to analyze the clinical features and parameters that contribute to a diagnosis of BA. Methods: From January 2000 to December 2013, 37 patients who underwent operative cholangiography were evaluated retrospectively. The patients were divided into two groups: a BA group, and a group with other cholestatic diseases (non-BA group). The demographic data and preoperative liver function test results were analyzed. Results: Of the 37 patients, 29 patients were confirmed to have BA. Age at operation was not significantly different between the two groups (P = 0.77). On preoperative liver function tests, only the level of γ-glutamyl transpeptidase (γ-GTP) was significantly higher in the BA group (P = 0.015). The predominant non-BA disease was inspissated bile syndrome (IBS). In the IBS patients, the jaundice was relieved after lavage of the biliary tree. Conclusion: The preoperative differentiation of cholestasis is difficult based on laboratory data and imaging. Preoperative γ-GTP may be useful for diagnosing BA, but operative cholangiography should be performed when BA is suspected and cannot be ruled out by other methods, given that the most common non-BA disease may be IBS.

DOI： 10.1111/ped.13392

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

Cell-based therapy has been proposed as an alternative to orthotopic liver transplantation. The novel transplantation of an in vitro-generated liver bud might have therapeutic potential. In vivo and ex vivo methods for growing a liver bud are essential for paving the way for the clinical translation of liver bud transplantation. We herein report a novel transplantation method for liver buds that are grown in vivo involving orthotopic transplantation on the transected parenchyma of the liver, which showed long engraftment and marked growth in comparison to heterotopic transplantation. Furthermore, this study demonstrates a method for rapidly fabricating scalable liver-like tissue by fusing hundreds of liver bud-like spheroids using a 3D bioprinter. Its system to fix the shape of the 3D tissue with the needle-array system enabled the fabrication of elaborate geometry and the immediate execution of culture circulation after 3D printing-thereby avoiding an ischemic environment ex vivo. The ex vivo-fabricated human liver-like tissue exhibited self-tissue organization ex vivo and engraftment on the liver of nude rats. These achievements conclusively show both in vivo and ex vivo methods for growing in vitro-generated liver buds. These methods provide a new approach for in vitro-generated liver organoids transplantation.

DOI： 10.1038/s41598-017-14542-2

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

DOI： 10.1016/j.pedneo.2017.05.001

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

Large-for-size syndrome is defined by inadequate tissue oxygenation, which results in vascular complications and graft compression after abdominal closure in living donor liver transplantation recipients. An accurate graft reduction that matches the optimal liver volume for the recipient is essential. We herein initially present the feasibility and safety of graft reduction using a powered stapler to obtain an optimal graft size. From October 1996 to October 2015, a total of eight graft reductions were performed using a powered stapler (group A; n=4) or by the conventional method using a cavitron ultrasonic surgical aspirator and portal triad suturing (group B; n=4). The background, intraoperative findings and the post-operative outcomes of these eight patients were retrospectively investigated. There were no statistically significant differences in the background of the patients in the two groups. Graft reduction was successfully achieved without any intraoperative complications in group A, whereas intraoperative complications, such as bleeding and bile leakage, occurred in two patients of group B. No post-operative surgical complications were detected on computed tomography; moreover, the serum aspartate aminotransferase level normalized significantly earlier in group A (P<.05). In summary, graft reduction using a powered stapler was feasible and safe in comparison with the conventional method.

DOI： 10.1111/petr.12985

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

Background: In liver transplantation (LT) for adult biliary atresia (BA), we often encounter a cirrhotic deformation of the native liver. We aimed to investigate a morphological study of the removed livers and the patient's clinical status. Methods: We examined 8 BA patients who had undergone LT in adulthood at our hospital. The presence of hypertrophic or atrophic areas of the removed liver was recorded macroscopically. We graded the microscopic findings in the porta hepatis area, a hypertrophic area, and an atrophic area, respectively. Moreover, we investigated the relationship between these morphological findings and the pre-transplant clinical status (MELD score). Results: Macroscopically, a hypertrophic area existed in central liver in all cases (8/8 cases), while an atrophic area was existed in peripheral liver (7/8 cases). Microscopically, an atrophic area was the most severely impaired, while the porta hepatis and hypertrophic area were relatively intact. The pathological score in a compensatory hypertrophic area was strongly correlated with the MELD score. Conclusions: This study suggests that the partial shrinking is not uncommon in BA cirrhotic liver. It may be due to the imbalance of bile drainage by the different segment. The patient's pre-transplant status depends on the compensatory hypertrophic liver.

DOI： 10.1007/s00383-012-3183-6

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

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

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

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

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

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

BACKGROUND AND AIM: The prophylactic oral administration of vitamin K to newborns has markedly reduced the incidence of vitamin K deficiency (VKD); however, intracranial hemorrhage (ICH) is still one of the complications found in biliary atresia (BA) patients and is associated with VKD bleeding. Therefore, we aimed to investigate the incidence and long-term outcome of ICH in patients with BA who previously received prophylactic vitamin K during the neonatal period. METHODS: Eighty-eight consecutive infants with BA were treated and followed up at Kyushu University Hospital from 1979 to 2009. The clinical records and imaging study results were retrospectively reviewed in the infants with BA who presented with ICH. RESULTS: ICH occurred in 7.95% of patients with BA. The onset of ICH occurred at 47 to 76 days after birth, before the patients underwent surgery for BA (9-37 days after the onset of ICH). Coagulopathy was found upon admission in all of the cases with available data and improved after intravenous administration of vitamin K. A craniotomy was required in 2 cases before the surgery for BA. During the 22 to 278 months of follow-up, some neurologic sequelae persisted in 5 of 7 cases. Follow-up head computed tomography scans showed a low-density area in the left hemisphere in 5 cases. CONCLUSIONS: Although vitamin K prophylaxis had been given during the neonatal period, ICH-associated VKD bleeding was still found in 7.95% of patients with BA. Persistent neurologic sequelae were found in 5 of 7 cases, with low-density area in the left hemisphere.

DOI： 10.1097/MPG.0b013e3182421878

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

Background: Portal vein thrombosis (PVT) or stenosis (PVS) often requires challenging techniques for reconstruction in living donor liver transplantation (LDLT). Materials and Methods: A total of 57 LDLTs were performed between October 1996 and December 2010. There were 16 cases (28%) with PVT/PVS that underwent modified portal vein anastomosis (m-PVa). The m-PVa techniques were classified into 3 groups: patch graft (Type-1), interposition graft (Type-2), and using huge shunt vessels (Type-3). The reconstruction patterns were evaluated with regard to age, graft vessels, PV flow, and complication rate. Results: The m-PVas were Type-1 in 10 cases, Type-2 in 3 cases, and Type-3 in 3 cases. The vessel graft in Type-1 was the inferior mesenteric vein (IMV) in 8 and the jugular vein in 2 cases, whereas the vessel graft in Type-2 was IMV in 2 and the saphenous vein in 1 case; in Type-3, the vessel grafts were renoportal, gonadal-portal, and coronary-portal anastomoses, respectively. The postoperative PV flow was sufficient in all types and slightly higher in Type-3. The postoperative complications occurred in 20% of the patients who underwent Type-1, in 33% who underwent Type-2, and in 0% who underwent Type-3. Conclusion: The m-PVa was effective to overcome the surgical difficulty during transplantation. Pretransplant planning for the selection of the type of reconstruction is important for recipients with PVT/PVS.

DOI： 10.1016/j.jpedsurg.2011.09.015

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

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

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

Event date： 2011.6

Venue：高松   Country：Japan  

Event date： 2011.6

Venue：横浜   Country：Japan  

Event date： 2011.6

Venue：京都   Country：Japan  

Event date： 2011.6

Venue：熊本   Country：Japan  

Event date： 2010.5

Venue：Kobe   Country：Japan  

Event date： 2010.5

Venue：横浜   Country：Japan  

Event date： 2010.5

Event date： 2010.3

Venue：富山   Country：Japan  

Event date： 2010.3

Venue：東京   Country：Japan  

Event date： 2010.2

Venue：Kuala Lumpur   Country：Malaysia  

Event date： 2009.3 - 2010.3

Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2008.9

Country：Japan  

Event date： 2007.9

Venue：Santa Monica   Country：United States  

Event date： 2005.6

Venue：Brussels   Country：Belgium  

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Language：Japanese   Publisher：(株)診断と治療社  

＜文献概要＞▽近年,高解像度内圧検査が開発され,食道運動機能を連続的かつ視覚的に評価可能となった.▽食道内圧検査に基づいたシカゴ分類が食道運動異常症の診断・治療に重要である.▽慢性便秘の原因精査として,直腸肛門内圧検査は有用な検査法である.▽特に,Hirschsprung病における直腸肛門内圧検査は,診断価値が高く汎用されている.

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

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

Language：Japanese   Publisher：金原出版(株)  

＜文献概要＞腸管機能不全患児において長期静脈栄養管理が必要となる場合,カテーテル関連血流感染症,中心静脈の血栓閉塞・損傷,腸管不全関連肝障害などをきたし,その予後は不良である.そのため治療にあたっては適切な経口摂取,経腸栄養,静脈栄養,中心静脈カテーテルの管理,薬物療法,外科治療,小腸移植や肝移植まで多岐にわたる治療を適切なタイミングで行っていく必要がある.腸管機能不全患児の予後改善,QOLの向上,社会復帰の実現を目的として,関連診療科および多職種が連携して行うチーム医療が重要である.本稿では,栄養管理法(経静脈栄養と経腸栄養),手術(腸管延長術,小腸移植)を中心に概説する.

Language：Japanese   Publisher：(株)メディカ出版  

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

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

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

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

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

＜Key Points＞(1)短腸症候群は,小腸大量切除に伴う消化吸収障害の状態である。(2)早期は下痢による水分や電解質喪失に対する完全静脈栄養管理,その後は長期にわたり,経腸栄養と静脈栄養管理が必要となる。(3)治療の目標は静脈栄養からの離脱であり,症例によっては腸管延長術や小腸移植などの外科的治療介入も有効な場合がある。(著者抄録)

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

＜Key Points＞(1)小児,とくに新生児・乳児期の便秘の原因の一つとしてHirschsprung病類縁疾患は考慮すべき疾患である。(2)Hirschsprung病類縁疾患は,日本では腸管壁内神経節細胞の形態や症状などにより7つの疾患に分けられ,予後が比較的よいものと悪いものとがあるため,その鑑別は重要である。(3)とくに予後の悪いIsolated hypoganglionosis,Megacystis microcolon intestinal hypoperistalsis syndrome,Chronic idiopathic intestinal pseudo-obstructionに対する薬物治療として,漢方薬(大建中湯)やプロバイオティクスは有効とする報告があるためまず試してもよい薬剤である。(著者抄録)

Language：Japanese   Publisher：金原出版(株)  

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

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

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

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

Language：Japanese   Publisher：(公社)日本リハビリテーション医学会  

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。(著者抄録)

Language：Japanese   Publisher：(株)医学書院  

＜文献概要＞ポイント　◆肝臓外科術後の門脈吻合部狭窄は,稀な合併症ではあるが,肝切除に付随して門脈腫瘍栓などによる門脈合併切除再建後,そして肝移植における門脈吻合再建後に認められることは周知のことである.◆手術技術的に早期に門脈吻合部狭窄が認められる場合と晩期に血管の線維化や腫瘍再発,浸潤,門脈血栓などによって門脈吻合部狭窄が認められる場合に大きく分けられる.◆周術期における門脈吻合部狭窄は,腸管うっ血・難治性腹水・肝機能障害などの原因となり,周術期管理において不利益となる.急速に門脈血流が障害されると,肝不全・播種性血管内凝固症候群といった重篤な病態に陥ってしまうため,早期発見・早期治療が不可欠である.また慢性期に移行し,血栓が基質化すると,門脈圧亢進症を発症し,食道および胃静脈瘤を発生させ,静脈瘤出血を引き起こす.◆診断は非侵襲的で感度も高い超音波検査を中心に,採血およびCT/MRI,直接門脈血管造影も併用して総合的に行う.◆治療としては,門脈ステントによる血管内治療,血栓症であれば抗凝固療法や外科的治療も選択肢となるが,依然確立された治療方針はない.本稿ではこれまでの報告に基づき,門脈吻合部狭窄・血栓症について概説したい.

Type：Academic society, research group, etc. 

Type：Academic society, research group, etc. 

Number of participants：700

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Type：Academic society, research group, etc. 

Number of participants：500

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Number of participants：1,000

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Type：Academic society, research group, etc. 

Number of participants：700

Type：Academic society, research group, etc. 

Number of participants：200

Type：Academic society, research group, etc. 

Type：Academic society, research group, etc. 

Type：Academic society, research group, etc. 

Number of participants：700

Type：Academic society, research group, etc. 

Type：Academic society, research group, etc. 

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc.