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.

Treatment guidelines for persistent cloaca, cloacal exstrophy, and Mayer-Rokitansky-Küster-Häuser syndrome for the appropriate transitional care of patients.

We developed treatment guidelines (TGs) for appropriate transitional care of the genitourinary system in patients with persistent cloaca (PC), cloacal exstrophy (CE), or Mayer-Rokitansky-Küster-Häuser syndrome (MRKH). These TGs are in accordance with the Medical Information Network Distribution Service (Minds), published in 2014 in Japan. Clinical questions (CQs) concerning treatment outcomes of the genitourinary system, pregnancy and delivery, and quality of life in adulthood were prepared as six themes for PC and CE and five themes for MRKH.

Acquired isolated hypoganglionosis as a distinct entity: results from a nationwide survey.

Purpose: Acquired isolated hypoganglionosis (A-IH) is a late-onset intestinal pseudo-obstruction disorder and shows different pathophysiological findings from congenital isolated hypoganglionosis (C-IH). In this study, we retrospectively examined five cases of A-IH and investigated the features of A-IH.

Methods: Five cases of A-IH were extracted from a nationwide retrospective cohort study in 10 years, from which totally 355 cases of Allied Disorders of Hirschsprung's Disease (ADHD) were collected.

Usefulness of Tc-DTPA galactosyl human serum albumin liver scintigraphy for evaluating hepatic functional reserve after Kasai procedure.

Background/purpose: We investigated whether Tc-DTPA galactosyl human serum albumin (GSA) liver scintigraphy could help evaluate post-Kasai procedure hepatic functional reserve in order to inform therapeutic strategies, including liver transplantation.

Methods: GSA scintigraphy was performed post-Kasai procedure. Patients were classified as having favorable (native liver survival) or poor (liver transplantation or death) prognoses.

Systemic and Local Cytokine Profile in Biliary Atresia.

 Systemic and local immune environments in human biliary atresia (BA) were analyzed.  Plasma concentrations of 19 inflammatory components in 16 preoperative BA patients (median age, 51 days), 13 normal controls (NCs) (44 days), and 15 cholestatic controls (CC) (23 days) were measured using flow cytometry, and compared according to post-Kasai outcomes in BA patients. Hepatic mRNA levels of representative helper T (Th) cell cytokines and forkhead box protein 3 (FoxP3) quantified by real-time reverse transcription polymerase chain reaction were compared between BA and non-BA.

Histological features of primary tumors after induction or high-dose chemotherapy in high-risk neuroblastoma.

Purpose: In the recent years in Japan, an increasing number of patients with neuroblastoma (NB) are being treated by the "delayed local treatment (DL)" policy, undergoing surgery after the completion of high-dose chemotherapy with hematopoietic stem cell rescue (HDC). We reviewed the histopathological findings of second-look operations, including those of patients treated with DL.

Patients: From 1998 to 2013, 26 patients with high-risk NB underwent radical operation following chemotherapy.

Diagnosis of Biliary Atresia Can not be Excluded by Declining Trend of Serum Direct Bilirubin.

The aim of this paper is to investigate the clinical courses of patients with biliary atresia (BA) during neonatal period. We examined 19 patients with BA, who underwent blood tests including direct bilirubin (D-Bil) within 20 days of age, in 3 tertiary hospitals in Japan. The first blood sample was collected at 8.

Optimal surgical treatment and urological outcomes in boys with pelvic and urogenital rhabdomyosarcomas and soft tissue sarcomas.

Background: Soft tissue sarcomas (STS) of pelvic origin in boys often involve the urogenital organs. The optimal extensiveness of radical surgery has long been an issue of discussion, since exenterative surgeries result in severe urogenital adverse effects. We conducted a retrospective review of patients with pelvic STS treated in two regional center hospitals and assessed the radicality of surgery and the functional outcome of the bladder.

Etiology of neonatal gastric perforation: a review of 20 years' experience.

Purpose: Gastric perforation (GP) of the newborn is a rare, serious, and life-threatening problem, and its etiology remains unclear. Although historically GP has often been described as "spontaneous'', some cases are non-spontaneous. The aim of the present study was to review cases of GP and to discuss its etiology in a single prefecture in Japan over a period of 20 years.