Publisher：東京医学社  

DOI： 10.24479/ps.0000000975

CiNii Research

Publisher：診断と治療社  

DOI： 10.34433/pp.0000001202

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Language：English   Publisher：Pediatric Surgery International  

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

DOI： 10.1007/s00383-024-05807-9

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

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

DOI： 10.1007/s11548-024-03253-5

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000873

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Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

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

DOI： 10.11164/jjsps.60.4_719

CiNii Research

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

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

Language：English   Publisher：Frontiers in Pediatrics  

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

DOI： 10.3389/fped.2024.1305585

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Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

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

DOI： 10.11164/jjsps.60.2_147

CiNii Research

Publisher：金原出版  

DOI： 10.18888/sh.0000002991

CiNii Research

Language：English   Publisher：Molecular Genetics and Genomic Medicine  

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

DOI： 10.1002/mgg3.2427

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

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

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

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

Language：English   Publisher：Journal of Pediatric Surgery  

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

DOI： 10.1016/j.jpedsurg.2023.12.005

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Language：English   Publisher：Pediatrics International  

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

DOI： 10.1111/ped.15754

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

Publisher：東京医学社  

DOI： 10.24479/ps.0000000637

CiNii Research

Publisher：東京医学社  

DOI： 10.24479/peri.0000001159

CiNii Research

Publisher：東京医学社  

DOI： 10.24479/ps.0000000523

CiNii Research

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

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

DOI： 10.1016/j.jpedsurg.2023.01.052

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Language：Japanese   Publisher：(一社)日本臨床衛生検査技師会  

α-アマニチン(AMA)は主としてドクツルタケ(AV)等の毒キノコが有する自然毒成分である。我々はAMAの定性法であるMeixner試験の性能を検証した。過去の文献を参照したが,Meixner試験の詳細な手法に関する情報が乏しかった。そこで,我々は本試験の手順書を改めて独自に作製し,これを改良Meixner試験と名付けた。本研究の検体には,AMAを蒸留水(DW),ヒト尿,およびヒト血清にて調製した試料と,キノコの成分抽出試料を用いた。我々の手順書通りに改良Meixner試験を行った結果,DWで調製した200μg/mLのAMA試料を検体として陽性反応を得ることができた。一方,本試験に使用する塩酸の濃度を下げると検出感度が低下した。DWに加え,尿および血清で調製した200μg/mL AMA試料を検体としても,同様に陽性反応が得られた。なお,いずれの溶媒でも100μg/mL未満のAMA濃度では陰性となった。各検体を加熱(100℃,30分)しても,試験結果に影響はなかった。ヘモグロビン成分を添加した血清および尿試料では感度が低下し,200μg/mL未満のAMAで陰性となった。AVの成分抽出試料5検体のうち,陽性反応が得られたのは3検体であった。本研究において性能を検証した改良Meixner試験は,溶血試料や血尿試料を除き,≧100μg/mL程度のAMA検出感度を発揮することが示された。(著者抄録)

Publisher：東京医学社  

DOI： 10.24479/pm.0000000805

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

DOI： 10.1016/j.ijscr.2023.107885

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Language：Japanese   Publisher：(一社)日本臨床衛生検査技師会  

テトロドトキシン(TTX)は主としてフグ科魚類が有する神経毒である。TTXが原因の食中毒は近年でも多く見られるが,医療施設においてそれら食中毒患者の生体試料中TTXの測定はほとんど実施されていない。そこで,我々は陰イオン交換カラムを用いた高速液体クロマトグラフィー(HPLC)分析を活用して生体試料中のTTXを検出・定量する手法の構築を試みた。我々が構築したHPLC分析系では,保持時間が0.1-0.4minの分画において,TTXを示す波形が検出された。さらに,クエン酸緩衝液で調製したTTX試料は,1.0-50μg/mLの濃度範囲で測定が可能であり,測定結果に基づき検量線を作成することができた。続いて,ヒトの血清や尿で調製したTTX試料の検出と定量を試みた。血清試料の場合,除タンパク処理を施してもTTXを正確に検出・定量することは困難であった。一方,尿試料では,TTXは5.0-50μg/mLの濃度範囲でTTXを示す波形の検出が可能であったが,検量線に当てはめると実際のTTX濃度よりも低い値に算出された。そこで,尿を用いて調製したTTXの測定結果に基づき,改めて作成した検量線に当てはめることで,TTX濃度の算出値を補正することができた。本研究の結果から,我々が構築したHPLC分析系はTTX食中毒患者の尿試料に適応が可能と考えられた。(著者抄録)

Publisher：東京医学社  

DOI： 10.24479/ps.0000000319

CiNii Research

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

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

DOI： 10.11164/jjsps.58.7_978

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

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

Publisher：東京医学社  

DOI： 10.24479/ps.0000000246

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

Language：Japanese  

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000176

CiNii Research

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

中間位鎖肛に対する術式として, 恥骨直腸筋とそれに連続する縦走連合筋および外肛門括約筋をすべて縦切開するposterior sagittal anorectoplasty (PSARP)が広く行われている. 本法は, 肛門括約筋の全体像の把握が容易であり, 良好な視野展開が可能となる. 一方で, PSARP後に完全な排便機能が得られるのは37.5%にすぎず, 25%は便失禁を生じうる状態であり, 必ずしも良好な経過ではないとも報告されている. 外肛門括約筋皮下部および浅部のみ縦切開せず, その中心に直腸をpull-throughするPSARP変法の有用性も報告されているが, いまだ議論の余地がある. 当科では中間位鎖肛に対する術式として, 外肛門括約筋皮下部および浅部まですべて縦切開するPSARP原法を行っている. 本稿では, 当科における中間位鎖肛に対するPSARP法の術後肛門機能について評価し, 結果を含め考察を述べる.

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

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

Language：Japanese  

中間位鎖肛に対する術式として, 恥骨直腸筋とそれに連続する縦走連合筋および外肛門括約筋をすべて縦切開するposterior sagittal anorectoplasty (PSARP)が広く行われている. 本法は, 肛門括約筋の全体像の把握が容易であり, 良好な視野展開が可能となる. 一方で, PSARP後に完全な排便機能が得られるのは37.5%にすぎず, 25%は便失禁を生じうる状態であり, 必ずしも良好な経過ではないとも報告されている. 外肛門括約筋皮下部および浅部のみ縦切開せず, その中心に直腸をpull-throughするPSARP変法の有用性も報告されているが, いまだ議論の余地がある. 当科では中間位鎖肛に対する術式として, 外肛門括約筋皮下部および浅部まですべて縦切開するPSARP原法を行っている. 本稿では, 当科における中間位鎖肛に対するPSARP法の術後肛門機能について評価し, 結果を含め考察を述べる.

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Language：Japanese   Publisher：(一社)日本臨床衛生検査技師会  

テトロドトキシン(TTX)が原因の食中毒は近年においても見られるが、TTXの測定を目的とした臨床検査は実施されていない。そこで、我々はTTXの検出と定量に有用な酵素結合免疫吸着法(ELISA)の構築を試みた。まず、TTXに対する抗体を用いて構築したELISAにより、TTXの希釈系列を測定し、検量線を作成した。その結果から、ELISA構築における測定性に優れた2種類の抗体の組み合わせを決定した。次に各種溶媒で100μg/mLに調製したTTX試料14本を同時測定した。クエン酸緩衝液で希釈したTTX試料の測定結果はばらつきが少なく、概ね正確な測定が可能であった。一方、尿試料で平均37.36μg/mLと調製濃度よりも低く、血清試料で平均249.86μg/mLと高いTTX濃度が算出された。この結果は試料中の共存物質が影響していると思われたため、尿や血清で調製したTTX希釈系列を測定、その結果から検量線を作成し、補正を試みた。その後、TTX試料14本の同時測定の結果は、尿試料では調製TTX濃度に近づいたが、血清試料では平均162.92μg/mLであり、調製濃度との乖離が見られた。そこで、TTX調製血清を除タンパク処理した試料の測定を試みた結果、平均111.29μg/mLと改善が認められた。検体の前処理法や測定工程には課題が残るが、本ELISAは生体試料中TTXの測定にある程度有用と思われた。(著者抄録)

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DOI： 10.1089/lap.2019.0162.

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DOI： 10.1016/j.ijscr.2020.09.021.

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DOI： 10.11164/jjsps.51.6_1

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DOI： 10.11164/jjsps.51.5_868

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DOI： 10.1016/j.epsc.2014.12.006

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DOI： 10.1007/s00383-014-3570-2.

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DOI： 10.1007/s00383-014-3571-1

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DOI： 10.11164/jjsps.50.2_217

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症例は13歳の女児.下腹部痛を主訴に当院救急部を受診した.腹部レントゲン検査の結果,便秘症の診断にて加療されていたが,下腹部痛が持続するため3病日にMRI検査を施行した.MRIにて骨盤内に嚢胞性病変を認め,重複腸管,卵管水腫捻転,メッケル憩室捻転などが疑われ当科紹介となった.腹腔鏡下緊急手術の結果,骨盤内に360度捻転し,暗褐色に腫大した左卵管を認めた.両側卵巣,右卵管には異常を認めなかった.左卵管水腫が原因の卵管捻転と診断し,腹腔鏡下に捻転解除および水腫を含めた左卵管切除術を行った.小児の卵管捻転は稀な疾患であるが,今回,診断および治療に腹腔鏡が非常に有用であった卵管捻転の1例を経験したので文献的考察を加えて報告する.

DOI： 10.11164/jjsps.48.2_229

Language：Japanese  

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

症例は17生日の男児.出生時より左下顎部に拇指頭大の腫瘤を認めていたが,徐々に増大してきたため,17生日に生検目的にて当科紹介入院となった.入院時,左下顎部に小児手拳大の腫瘤を認め,血液所見では血小板数1.6×10^4/μlと減少していた.腫瘤生検の結果,腫瘤はcapillary hemangiomaと判明した.血管腫に合併したKasabach-Merritt症候群(KMS)と診断し,血小板輸血に加えステロイド治療,放射線治療を行ったが効果なく,129生日に70～80%の腫瘍部分摘出術を施行した.術後,血小板減少は著明に改善した.2年経過した現在,残存血管腫は縮小し血小板数も正常値を保っている.KMSは稀な疾患であるが新生児に発症すると高い死亡率が報告されている.今回,新生児期にKMSを伴って増大した頸部血管腫に対して腫瘍部分摘出術が有効であった1例を経験したので文献的考察を加えて報告する.

DOI： 10.11164/jjsps.47.2_246

Event date： 2024.5

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

Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)  

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

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：(株)東京医学社  

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

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