Serological cytokine signature in paediatric patients with inflammatory bowel disease impacts diagnosis.

Endoscopy is a central tool for diagnosing and evaluating paediatric inflammatory bowel diseases (PIBD), but is too invasive to be frequently repeated in young children. Furthermore, it is challenging to distinguish Crohn's disease (CD) from ulcerative colitis (UC) endoscopically. This study aimed to determine biomarkers useful for the diagnosis of PIBD.

A Multicenter Prospective Survey on Early-Onset Inflammatory Bowel Disease in Japan.

Introduction: The incidence of early-onset inflammatory bowel disease is increasing in Japan.

Objective: This study aimed to analyze the treatment and progress of early-onset inflammatory bowel disease.

Methods: This prospective survey evaluated the data of 43 patients aged <8 years who were diagnosed with inflammatory bowel disease (IBD) from the time of diagnosis to 36 months after registration.

Infliximab for pediatric patients with ulcerative colitis: a phase 3, open-label, uncontrolled, multicenter trial in Japan.

Background: Pediatric ulcerative colitis (UC) is typically more extensive and has a more active disease course than adult UC, and requires early treatment augmentation to achieve and maintain disease remission. The present study aimed to investigate the efficacy, safety, and pharmacokinetic profile of infliximab (IFX) in pediatric patients with moderate-to-severe UC and inadequate response to existing treatment.

Methods: This open-label, uncontrolled, multicenter, Phase 3 trial was conducted at 17 centers in Japan between April 2012 and September 2014.

Current role of colonoscopy in infants and young children: a multicenter study.

Background: To evaluate the role of colonoscopy in infants and young children and clarify the distribution of colonoscopy-requiring diseases in this age group.

Methods: Cohorts of colonoscopies performed at three children's hospitals in Japan between April 2011 and March 2016 including infants and children younger than six years of age were retrospectively reviewed.

Results: In total, 453 colonoscopies were performed in 276 infants and young children.

Clinical, Pathologic, and Genetic Features of Neonatal Dubin-Johnson Syndrome: A Multicenter Study in Japan.

Objective: To clarify the clinical, pathologic, and genetic features of neonatal Dubin-Johnson syndrome.

Study Design: Ten patients with neonatal Dubin-Johnson syndrome were recruited from 6 pediatric centers in Japan between September 2013 and October 2016. Clinical and laboratory course, macroscopic and microscopic liver findings, and molecular genetic findings concerning ATP-binding cassette subfamily C member 2 (ABCC2) were retrospectively and prospectively examined.

Prostaglandin E-major Urinary Metabolite as a Biomarker for Pediatric Ulcerative Colitis Activity.

Objectives: Prostaglandin E-major urinary metabolite (PGE-MUM) is a useful biomarker for adult ulcerative colitis (UC) activity. In the present study, we evaluated whether PGE-MUM can also be a biomarker of pediatric UC activity and compared its efficacy in predicting UC activity with that of C-reactive protein and erythrocyte sedimentation rate.

Methods: Twenty-nine pediatric patients with UC (8-18 years) and 29 healthy age- and sex-matched subjects were enrolled.

Screening of carnitine and biotin deficiencies on tandem mass spectrometry.

Background: It is important to assess pediatric patients for nutritional deficiency when they are receiving specific interventions, such as enteral feeding. We focused on measurement of C0 and 3-hydroxyisovalerylcarnitine (C5-OH) with tandem mass spectrometry (MS/MS), which is performed as part of the newborn mass screening. The purpose of this study was to investigate the usefulness of MS/MS for screening carnitine and biotin deficiencies.

Difference between early onset and late-onset pediatric ulcerative colitis.

Background: Early onset pediatric ulcerative colitis (EO-UC) is distinguished from late-onset pediatric ulcerative colitis (LO-UC) by the effects of genetic predisposition, but there have been few reports on the clinical features of EO-UC in Asia.

Methods: To describe and compare the presentation and disease course of EO-UC (age range, 0-7 years) with those of LO-UC (age range, 8-15 years), we retrospectively analyzed 63 children with UC who had been diagnosed between January 2004 and March 2014 at Saitama Children's Medical Center in Japan.

Results: Ten patients (16%) had EO-UC, and 53 (84%) had LO-UC.

Successful colonoscopic approach in a child with intussusception associated with enterohemorrhagic Escherichia coli O157 infection.

The pathogenesis of intussusception caused by enterohemorrhagic Escherichia coli (E. coli) O157 infection is unknown. In our case, colonoscopy was useful for confirming O157 infection.

[Severe aplastic anemia following living-donor liver transplantation for fluminant hepatitis].

We report a case of a 15-year-old girl with severe aplastic anemia who underwent orthotopic liver transplantation 5 years ago for fulminant hepatic failure during the course of immunodeficiency of unknown etiology. She previously exhibited similar immunodeficiency and experienced recurrent viral infections. She developed jaundice at 9 years of age and was diagnosed with fulminant hepatitis.

Characteristics of inflammatory bowel disease with an onset before eight years of age: a multicenter epidemiological survey in Japan.

Background And Aim: Pediatric inflammatory bowel disease (IBD) has not been rare in Japan since the 1990s. The present study attempted to define the epidemiological and clinical characteristics of early-childhood IBD in Japan in comparison with results from Western countries.

Methods: Among children diagnosed as having IBD between January 1998 and December 2008, those showing onset before 8 years of age were investigated retrospectively.

Leukocytapheresis in pediatric patients with ulcerative colitis.

Objective: Leukocytapheresis (LCAP) is a nonpharmacologic therapy that has recently been used to treat ulcerative colitis (UC). This multicenter open-label study prospectively assessed the efficacy and safety of LCAP in pediatric patients with UC.

Patients And Methods: Twenty-three patients ages 8 to 16 years with moderate (n = 19) to severe (n = 4) steroid-resistant UC were enrolled.

Efficacy and safety of pulse steroid therapy in Japanese pediatric patients with ulcerative colitis: a survey of the Japanese Society for Pediatric Inflammatory Bowel Disease.

Aims: We have evaluated the therapeutic efficacy and safety of pulse steroid therapy for ulcerative colitis (UC) in a Japanese pediatric population by means of a survey.

Methods: A questionnaire on UC patients treated with therapy between 2002 and 2006 was sent to 37 members of the Japanese Society for Pediatric Inflammatory Bowel Disease.

Results: 21 of 62 cases in 6 of 19 centers registered in this study had been treated with pulse steroid therapy.

Peginterferon alpha-2b and ribavirin for the treatment of chronic hepatitis C in Japanese pediatric and young adult patients: a survey of the Japan Society of Pediatric Hepatology.

Objectives: Only a few studies on the treatment with peginterferon-2b and ribavirin are available in children with chronic hepatitis C virus (HCV). The aim of this study was to evaluate both the efficacy and the safety of the treatment in Japanese children and young adults.

Methods: Twenty-two of 41 members of the Japan Society of Pediatric Hepatology reported on 37 cases who were treated with peginterferon and ribavirin.

[Thalidomide therapy for infantile-onset Crohn's disease].

A 6-month-old boy was diagnosed as having Crohn's disease (CD) by the endoscopic examination. Primary immunodeficiency syndrome was initially suspected due to a refractory infection that occurred just after birth and a family history that his older brother died at the age of 3 months of septicemia associated with perirectal abscess. Thalidomide was used because conventional medical treatment by steroids and immunosuppressives was ineffective.

Apoptosis under hypercytokinemia is a possible pathogenesis in influenza-associated encephalopathy.

Background: Influenza-associated encephalopathy is reported to be frequent in Japan and East Asia. No evaluating markers except interleukin (IL)-6 and tumor necrosis factor (TNF)-alpha and no likely pathological mechanism for the disease have yet been elucidated.

Methods: In this study, influenza-associated encephalopathy was defined by clinical symptoms, and the use of an anti-influenza antibody test and/or influenza antigen detection kits, as well as computed tomography and/or magnetic resonance imaging.

Guidelines for treatment of ulcerative colitis in children.

This paper introduces the guidelines for treatment of ulcerative colitis in children, created by the working group of the Japanese Society for Pediatric Gastroenterology, Hepatology and Nutrition (Chair: Yuichiro Yamashiro) and the Japanese Society for Pediatric Inflammatory Bowel Disease (IBD) (Chair: Akio Kobayashi). The ideas of the working group, with regard to the fundamental differences in medical treatment between children and adults, included: (1) for children, intensive medical treatment including appropriate systemic management is important during the acute phase of illness. (2) Treatment with steroids, which can cause growth disturbances, should not be continued for long periods of time.