Current practice regarding the diagnosis and treatment of anorectal malformations in female patients: a multicenter questionnaire survey in Japan.

Purpose: This study aimed to investigate the current practices in the diagnosis and surgical management of anorectal malformations (ARMs) in female patients in Japan, specifically focusing on anovestibular fistula (AVF), rectovaginal fistula (RVF), and persistent cloaca (PC).

Methods: An anonymous online survey was conducted with 61 institutional members of the Japanese Study Group for Anorectal Anomalies.

Results: Sixty-one institutions (100%) completed the survey.

Optimal transplant strategy of pediatric liver transplantation for fibropolycystic liver disease: Multicenter retrospective study in Japan.

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.

The long-term post-surgical outcome of intermediate anorectal malformation in our department.

Background: Posterior sagittal anorectoplasty and laparoscopic-assisted anorectal pull-through are preferred for anorectal malformation (ARM) today, while careful pull-through procedures with sacroperineal approach yield excellent outcomes. This study focuses on a pull-through procedure emphasizing continence mechanism preservation and compares outcomes with historical studies with various procedures.

Methods: Bowel function of patients with intermediate ARM followed up for over 10 years post-surgically was assessed.

Deceased donor non-composite split liver and intestinal transplantation for children.

Children with intestinal failure suffer liver damage associated with parenteral nutrition: a condition known as intestinal failure-associated liver disease (IFALD), which requires transplantation of both liver and intestine. In many countries, simultaneous transplantation of these two organs is performed using grafts from a deceased donor, but there have been no such cases in Japan, and the details of the procedure are not clear. Recently, we performed simultaneous split liver and intestinal transplantation in two premature infants with IFALD, using organs from identical deceased donors and achieved good results.

Efficacy of steroid therapy for improving native liver survival after pediatric acute liver failure with immune activation.

Aim: Recent evidence suggests that acute liver failure (ALF) in some patients may reflect a dysregulated immune response, and that corticosteroids improve survival of the native liver in ALF patients with high serum alanine aminotransferase levels, which are an indication of liver inflammation. However, it is unclear whether steroids are effective for pediatric acute liver failure (PALF). The aim of this retrospective case-control study is to examine whether steroid therapy for PALF accompanied by immune activation improves the survival of native liver and to identify factors that predict responses to steroid treatment.

Donor Complications Following Living Donor Hepatectomy: 17-Year Experience of a Single Team in One Institution of Japan.

Objectives: Donor safety is paramount in living donor liver transplantation. However, there remains a risk of postoperative complications for some donors. Here, we provide a comprehensive assessment of donor morbidity by a single team with 17 years of experience at a single center.

Mesenchymal cells regulate enteric neural crest cell migration via RET-GFRA1b trans-signaling.

During early development, the enteric nervous system forms from the migration of enteric neural crest cells (ENCCs) from the foregut to the hindgut, where they undergo proliferation and differentiation facilitated by interactions with enteric mesenchymal cells (EMCs). This study investigates the impact on ENCC migration of EMC-ENCC communication mediated by GFRA1b expressed in EMCs. GFRA1-expressing cells in day 11-12 (E11-12) mouse embryos differentiated into smooth muscle cells from E12 onwards.

Rapidly growing intramuscular lipoma: a unique entity of benign lipomas in children.

This report presents a case of an intramuscular lipoma observed in the left back of a healthy female toddler. It was resected after 3 months of observation because of rapid enlargement, raising suspicion of malignancy. Histopathological examination confirmed a diagnosis of intramuscular lipoma without malignant and blastemal components.

Two cases of pararenal artery aortic aneurysm treatment after pancreaticoduodenectomy and abdominal aortic aneurysm stent grafting.

Background: Acute pancreatitis caused by surgical procedures may occur less frequently in surgeries for aortic aneurysm involving the abdominal branch. However, in such cases, the associated mortality rate increases significantly. There have been few reports on abdominal aortic aneurysm surgery after pancreatoduodenectomy; as such the incidence of postoperative pancreatitis remains unclear.

External Esophageal Stenting Technique in Palliation for Tracheal Agenesis in a Case of Esophageal Lung: A Lesson Learned from the Experience for Tracheomalacia.

Tracheal agenesis (TA) is a rare congenital anomaly with an incidence of 1 per 50,000 newborns. It appears at birth with severe respiratory distress, cyanosis, and inaudible crying. Prompt esophageal intubation and long-term management of the esophageal airway are essential to overcome this catastrophic condition.

Living donor liver transplantation for myocerebrohepatopathy spectrum due to POLG mutations.

Background: POLG is one of several nuclear genes associated with mitochondrial DNA maintenance defects and is a group of diseases caused by mitochondrial DNA deficiency that results in impaired adenosine triphosphate production and organ dysfunction. Myocerebrohepatopathy spectrum (MCHS) is the most severe and earliest presentation of POLG mutations, and liver transplantation (LT) for MCHS has never been reported.

Case Presentation: The patient was a 3-month-old boy with acute liver failure and no neurological manifestations (e.

Effects of Adding Congested Segment IV to the Left Lateral Graft on Short-term Outcomes in Pediatric Living-donor Liver-transplant Recipients.

Background: In some pediatric patients undergoing living-donor liver transplantation, segment IV without the middle hepatic vein can be added to a left lateral segment graft to obtain larger graft volume. Because no clear consensus on this technique exists, this study investigated the effects of congested areas on postoperative outcomes in pediatric patients with biliary atresia undergoing living-donor liver transplantation.

Methods: We retrospectively reviewed data of recipients with biliary atresia aged ≤15 y who had undergone living-donor liver transplantation at Kyoto University Hospital between 2006 and 2021 and with graft-to-recipient weight ratios (GRWR) of ≤2%.

The protective association of HLA-C*12:02 and HLA-DQB1*06:01 with severe acute hepatitis of unknown origin in the Japanese population.

Global surveillance has been conducted to elucidate the pathogenesis of acute hepatitis of unknown origin (AHUO), However, the factors associated with the aggravation of this serious disease are unclear. Therefore, we conducted a HLA association study to identify HLA alleles or haplotypes predisposing or protective against Japanese AHUO. The HLA 5 locus (HLA-A, HLA-B, C, DRB1, and DQB1) 4-digit genotyping results of 72 AHUO patients who underwent liver transplantation at our institution between 2000 and 2021 were compared to those of 873 healthy Japanese controls.

Pretransplant Renal Dysfunction Negatively Affects Prognosis After Living Donor Liver Transplantation: A Single-Center Retrospective Study.

Background: To evaluate the influence of preoperative renal function on prognosis after living donor liver transplantation (LDLT).

Methods: Living donor liver transplantation cases were categorized into 3 groups as follows: renal failure with hemodialysis (HD; n = 42), renal dysfunction (RD; n = 94) (glomerular filtration rate <60 mL/min/1.73 m), and normal renal function (NF; n = 421).

The Junction Between the Peristaltic and Non-peristaltic Bowel (Shore Break) is Found in the Transition Zone in Hirschsprung's Disease.

Background: The junction between the peristaltic and non-peristaltic bowel, which is named the "shore break" (SB), observed on endoscopy is thought to be the boundary between normal and abnormal motility in Hirschsprung's disease (HD). The transition zone (TZ), which is the histopathological border, does not have normal motility and should be resected. This study aimed to evaluate the histopathological findings of the SB and determine the association between the SB and TZ.

Percutaneous Transhepatic Biliary Intervention in Adult Biliary Atresia Patients After Kasai Portoenterostomy.

Kasai portoenterostomy (KP) is a standard treatment for patients with biliary atresia (BA). After KP, patients with BA occasionally develop biliary complications, such as recurrent cholangitis, biliary stricture, and cystic dilatation of the intrahepatic bile duct. Percutaneous transhepatic biliary drainage is one of the treatment options for these biliary complications.

Hemodynamic and Clinical Response to Liver Transplantation in Children and Young Adults POPH Patients.

Portopulmonary hypertension is an intractable form of pulmonary hypertension. Although liver transplantation is recommended for patients who respond poorly to treatments, the mechanisms by which liver transplantation improves pulmonary hypertension remain unclear. The present study investigated these mechanisms by retrospectively evaluating patients' data.

Two Cases of Possible Exacerbation of Chronic Rejection After Anti-SARS-CoV-2 Messenger RNA Vaccination: A Case Report.

In post-liver transplant recipients, SARS-CoV-2 infection is a health threat, and novel messenger RNA vaccines such as Pfizer BioNTech BNT162b2 and Moderna mRNA-1273 are aggressively recommended. However, there are few reports on their adverse effects, some of which may be potentially fatal. We have experienced 2 post-liver transplant recipients with exacerbated chronic rejection after vaccination, one of whom had to undergo retransplant and the other who is still in the process of liver function without improvement.

Differences in isolated bacteria between perforated and non-perforated appendicitis: an analysis of 680 consecutive appendicectomies in a single institution.

Purpose: Escherichia coli and Bacteroides species are the most frequently detected species in ascites in perforated appendicitis and are generally sensitive to non-empiric cephalosporins like cefazolin or cefmetazole. However, monotherapy with such antibiotics is mostly insufficient for perforated appendicitis. To investigate this issue, this study aimed to compare bacterial floras in ascites culture between perforated and non-perforated appendicitis.

Recipient colon preoperative treatment with type I collagenase and fibronectin promotes the growth of transplanted enteric neural crest cells into Auerbach's plexus.

Purpose: Cell-based therapy is a potential treatment option for neurointestinal diseases by serving as a source of neural progenitor cells to replace missing or abnormal enteric neurons. Using an ex vivo transplantation model, we recently demonstrated that treatment with collagenase and fibronectin promotes infiltration of transplanted enteric neural crest cells (ENCCs) toward the colon lumen. The aim of this study was to determine whether this new method also promotes colonization of transplanted ENCCs in vivo.

The New Challenge in Pediatric Liver Transplantation: Chronic Antibody-Mediated Rejection.

Antibody-mediated rejection (AMR) of liver allograft transplantation was considered as anecdotal for many decades. However recently, AMR has gained clinical awareness as a potential cause of chronic liver injury, leading to liver allograft fibrosis and eventual graft failure. (1) Methods: Literature on chronic AMR (cAMR) in pediatric post-liver transplant patients was reviewed for epidemiologic data, physiopathology, diagnosis, and treatment approaches.

The impact of human leukocyte antigen mismatch on recipient outcomes in living-donor liver transplantation.

Donor-recipient human leukocyte antigen (HLA) compatibility has not been considered to significantly affect liver transplantation (LT) outcomes; however, its significance in living-donor LT (LDLT), which is mostly performed between blood relatives, remains unclear. This retrospective cohort study included 1954 LDLTs at our institution (1990-2020). The primary and secondary endpoints were recipient survival and the incidence of T cell-mediated rejection (TCMR) after LDLT, respectively, according to the number of HLA mismatches at all five loci: HLA-A, HLA-B, HLA-C, HLA-DR, and HLA-DQ.