Amylase Levels Are Useful for Diagnosing Omphalomesenteric Cysts: A Case Report.

Omphalomesenteric cysts are an exceedingly rare type of embryologic remnant of the omphalomesenteric duct. Owing to its rarity and unspecific imaging findings, it is occasionally difficult to diagnose preoperatively. Herein, we report the case of a 15-month-old female with an omphalomesenteric cyst that presented as a painful abdominal mass.

Infection dynamics of Toxoplasma gondii in gut-associated tissues after oral infection: The role of Peyer's patches.

Toxoplasma gondii is a common perorally transmitted parasite; however, its immunopathogenesis in gut-associated tissues remains unclear. Here, we compared disease manifestation in C57BL/6 immunocompetent wild type (WT) mice and immunocompromised interferon (IFN)-γ-deficient (GKO) mice after peroral infection (PI) with T. gondii cysts (Fukaya strain).

Significance and indications for reoperative portoenterostomy in biliary atresia in light of long-term outcome.

Background: Among biliary atresia (BA) patients undergoing revision following failed portoenterostomy, long-term native liver survival (NLS), physical condition, and indications for revision were explored.

Methods: From 1977-2015, 33 of 95 BA patients (35%) at our institution underwent revision. Ten- and 20-year NLS rates (NLSRs) and biochemical statuses of 20-year-old native liver survivors were compared between post-portoenterostomy BA patients with and without history of revision.

Prediction of respiratory collapse among pediatric patients with mediastinal tumors during induction of general anesthesia.

Purpose: Fatality resulting from respiratory collapse (RC) during general anesthesia (GA) induction in children with mediastinal tumors has been reported. We explored potentially useful parameters for predicting the risk of RC based on objective imaging results.

Methods: We retrospectively reviewed the records of 31 patients (median age: 4years; range: 4months-15years) with mediastinal tumors treated between 2000 and 2015.

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.

Significance of imaging modalities for preoperative evaluation of the pancreaticobiliary system in surgery for pediatric choledochal cyst.

Objective: Roles of magnetic resonance cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography (ERCP), drip infusion cholangiography with computed tomography (DIC-CT), and intraoperative cholangiopancreatography (IOCP) in visualizing pancreaticobiliary anatomy for pediatric choledocal cyst (CC) operations were examined.

Methods: From 1980-2013, 117 pediatric CC patients (median age, 3 years) underwent hepaticojejunostomy at our institution, with imaging modalities of ERCP (n = 81 over 34 years), MRCP, DIC-CT, and IOCP (n = 45, 20, and 45 cases over the last 12 years). First, visualization rates for pancreaticobiliary maljunction (PBM), common bile duct (CBD), pancreatic duct (PD), and intrahepatic bile duct (IHBD) were investigated.

Ellis-van Creveld syndrome associated with chronic intestinal pseudo-obstruction.

Ellis-van Creveld (EVC) syndrome is a rare autosomal recessive disorder characterized by hypoplastic nails, polydactyly, and achondroplasia. Patients usually exhibit normal cognitive function and no remarkable developmental delay. We herein present an unusual case of EVC syndrome.

Antibody-dependent cellular cytotoxicity toward neuroblastoma enhanced by activated invariant natural killer T cells.

Anti-ganglioside GD2 antibodies mainly work through antibody-dependent cellular cytotoxicity (ADCC) and have demonstrated clinical benefit for children with neuroblastoma. However, high-risk neuroblastoma still has a high recurrence rate. For further improvement in patient outcomes, ways to maximize the cytotoxic effects of anti-GD2 therapies with minimal toxicity are required.

Coexistence of neuroblastoma and ganglioneuroma in a girl with a hemizygous deletion of chromosome 11q14.1-23.3.

Constitutional 11q interstitial deletion syndrome presents with congenital anomalies including microcephaly with craniostenosis, minor dysmorphic features, vitreoretinopathy, and renal anomalies. This syndrome is occasionally associated with neuroblastoma (NB) as a life-threatening complication, which is important for clinical care. Although the corresponding locus to NB has been predicted to exist in 11q22-23 by previous deletion studies related to NB, the causative haploinsufficient genes have not yet been identified.

Risk Factors for Intestinal Obstruction After Ladd Procedure.

Intestinal obstruction is a common complication after Ladd procedure. Ninety-three cases who had undergone the Ladd procedure between 1977 and 2013 treated at our own institution were retrospectively reviewed to identify the causes and risk factors for intestinal obstruction. The Ladd procedure has been performed without any intestinal fixing.

Evidence for viral infection as a causative factor of human biliary atresia.

Objectives: To explore the evidence for viral infections triggering human biliary atresia (BA) by reviewing archival original articles that analyzed human samples via polymerase chain reaction (PCR) experiments, considering the recent experimental trend of extensive use of rotaviral BA animal models.

Methods: A PubMed search retrieved original articles that reported the results of PCR experiments for detecting viral DNA or RNA in patient samples as proof of past infection. Search terms included the often-debated DNA or RNA viruses and BA.

Malignant rhabdoid tumor of the liver: a case report and literature review.

Malignant rhabdoid tumor (MRT) is a rare and aggressive malignancy associated with poor outcomes. MRT of the liver is even rarer, and little information has been described. We report the case of an 8-month-old boy with MRT of the liver.

Endoscopic management for congenital esophageal stenosis: A systematic review.

Congenital esophageal stenosis (CES) is an extremely rare malformation, and standard treatment have not been completely established. By years of clinical research, evidence has been accumulated. We conducted systematic review to assess outcomes of the treatment for CES, especially the role of endoscopic modalities.

Hybrid video-assisted thoracoscopic surgery lobectomy of fissureless congenital cystic adenomatoid malformation: a case report.

Introduction: Thoracoscopic lobectomy for congenital pulmonary airway malformation has been indicated from the neonatal period to adolescence. However, it is difficult to approach the pulmonary artery for lobectomy in congenital lung malformations with incomplete or absent interlobar fissures. Multidetector computed tomographic images and computed tomography pulmonary angiography gave us helpful information before the operation.

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.

Role of pediatric endoscopic retrograde cholangiopancreatography in an era stressing less-invasive imaging modalities.

Background: The significance of pediatric endoscopic retrograde cholangiopancreatography (ERCP) according to age or disease variation is inconclusive. This study aimed to evaluate the usefulness of pediatric ERCP in diagnosing or treating small children with pancreaticobiliary disorders, including choledochal cyst (CC) and biliary atresia (BA).

Methods: From 1980 to 2011, 235 ERCPs were performed in 220 pediatric patients (median age, 2 years) at our institution.

Urinary amylase/urinary creatinine ratio (uAm/uCr)--a less-invasive parameter for management of hyperamylasemia.

Background: The serum level of amylase (sAm) is commonly used as a biochemical marker for diagnosis and management of pancreatic disorders. However, the use of the urine level of amylase (uAm) is limited in practice, because the diagnostic ability of uAm is inferior to that of sAm. In the present study, the possible concordance of uAm-rerated parameters with sAm was investigated, and evaluate the usefulness of uAm for management of hyperamylasemia.

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.

Toll-like receptor mRNA expression in liver tissue from patients with biliary atresia.

Background And Aim: Inappropriate host immunological reactions against unknown ligands via the Toll-like receptor (TLR) cascades may trigger progressive inflammatory biliary destruction that manifests as biliary atresia (BA) in newborns or infants. The aim of the study was to clarify the role of the innate immune system in the development of BA.

Patients And Methods: Liver tissue was obtained from 49 patients with pediatric hepatobiliary diseases: 19 with BA, 21 with choledochal cysts, and 9 with other hepatobiliary diseases.

Hepatic expression of multidrug resistance protein 2 in biliary atresia.

Background: Biliary atresia (BA) is an idiopathic inflammatory obliterative cholangiopathy of neonates, leading to progressive biliary cirrhosis. Hepatoportoenterostomy (Kasai procedure) can cure jaundice in 30% to 80% of patients. Postoperative clearance of jaundice is one of the most important factors influencing long-term outcomes of BA patients.

Use of preoperative, 3-dimensional magnetic resonance cholangiopancreatography in pediatric choledochal cysts.

Background: Standard choledochal cyst (CC) operations involve dilated extrahepatic bile duct excision followed by biloenterostomy. However, biliary variants and associated intrahepatic bile duct (IHBD) stenoses or dilatations triggering postoperative sequelae require additional procedures. The usefulness of preoperative 3-dimensional magnetic resonance cholangiopancreatography (3D MRCP) and virtual cholangioscopy (VES) for observing biliary morphology and pancreaticobiliary maljunction (PBM) was evaluated.

Src kinase family inhibitor PP2 induces aggregation and detachment of neuroblastoma cells and inhibits cell growth in a PI3 kinase/Akt pathway-independent manner.

Purpose: Neuroblastoma (NB) is one of the most common extracranial solid tumors in children and is known for its clinical and biological heterogeneity. The aim of this study is to reveal the functional role of src family kinases in the biological behavior of NB by inhibiting their kinase activities with a specific inhibitor, PP2 (4-amino-5-(4-chloro-phenyl)-7-(t-butyl)pyrazolo[3,4-d]pyrimidine).

Methods: NB cell lines (SH-SY5Y, IMR32, RT-BM-1, CHP134, NLF, and LA-N-5) were treated with 0.

Use of multi-detector row CT for postoperative follow-up of biliary atresia patients with sequelae.

Background/aims: To evaluate the usefulness of multi-detector row computed tomography (MDCT) in the postoperative long-term follow-up of biliary atresia (BA) patients for detection of esophagogastric varices due to portal hypertension and intrahepatic bile duct (IHBD) dilatations. Well-developed esophagogastric varices can cause unexpected life-threatening rupture and contribute to the progression of hepatopulmonary vascular diseases. Dilated IHBDs can trigger cholangitis.