Chronic liver disease is universal in children with biliary atresia living with native liver

AIM To examine the medical status of children with biliary atresia(BA) surviving with native livers.METHODS In this cross-sectional review,data collected included complications of chronic liver disease(CLD)(cholangitis in the preceding 12 mo,portal hypertension,variceal bleeding,fractures,hepatopulmonary syndrome,portopulmonary hypertension) and laboratory indices(white cell and platelet counts,total bilirubin,albumin,international normalized ratio,alanine aminotransferase,aspartate aminotransferase,γ-glutamyl transpeptidase). Ideal medical outcome was defined as absence of clinical evidence of CLD or abnormal laboratory indices. RESULTS Fifty-two children [females = 32,62%; median age 7.4 years,n = 35(67%) older than 5 years] with BA(median age at surgery 60 d,range of 30 to 148 d) survived with native liver. Common complications of CLD noted were portal hypertension(40%,n = 21; 2 younger than 5 years),cholangitis(36%) and bleeding varices(25%,n = 13; 1 younger than 5 years). Fifteen(29%) had no clinical complications of CLD and three(6%) had normal laboratory indices. Ideal medical outcome was only seen in 1 patient(2%). CONCLUSION Clinical or laboratory evidence of CLD are present in 98% of children with BA living with native livers after hepatoportoenterostomy. Portal hypertension and variceal bleeding may be seen in children younger than 5 years of age,underscoring the importance of medical surveillance for complications of BA starting at a young age.

Appropriateness, endoscopic findings and contributive yield of pediatric gastrointestinal endoscopy

AIM:To determine the predictability of the North American Society for Pediatric Gastroenterology,Hepatology and Nutrition(NASPGHAN)and American Society for Gastrointestinal Endoscopy(ASGE)guideline with regard to appropriate endoscopic practice in children,positive endoscopic findings and contributive yield in clinical practice.METHODS:This was a descriptive,retrospective analysis,conducted at the Department of Paediatrics,University Malaya Medical Centre,Malaysia.All children who had esophagogastroduodenoscopy(EGD)and colonoscopy from January 2008 to June 2011 were included.An endoscopy was considered appropriate when its indication complied with the NASPGHAN and ASGE guideline.All endoscopic findings were classified as either positive(presence of any endoscopic or histologic abnormality)or negative(no or minor abnormality,normal histology);effecting a positive contributive(a change in therapeutic decisions or prognostic consequences)or non-contributive yield(no therapeutic or prognostic consequences).RESULTS:Overall,76%of the 345 procedures(231EGD alone,26 colonoscopy alone,44 combined EGD and colonoscopy)performed in 301 children(median age 7.0 years,range 3 months to 18 years)had a positive endoscopic finding.Based on the NASPGHAN and ASGE guideline,99.7%of the procedures performed were considered as appropriate.The only inappropriate procedure(0.3%)was in a child who had EGD for assessment of the healing of gastric ulcer following therapy in the absence of any symptoms.The overall positive contributive yield for a change in diagnosis and/or management was 44%.The presence of a positive endoscopic finding was more likely to effect a change in the therapeutic plan than an alteration of the initial diagnosis.A total of 20(5.8%)adverse events were noted,most were minor and none was fatal.CONCLUSION:The NASPGHAN and ASGE guideline is more likely to predict a positive endoscopic finding but is less sensitive to effect a change in the initial clinical diagnosis or the subsequent therapeutic plan.

Variable outcome in infantile-onset inflammatory bowel disease in an Asian cohort

AIM Infantile-onset inflammatory bowel disease(IO-IBD) with the onset of disease before 12 mo of age, is a different disease entity from childhood IBD. We aimed to describe the clinical features, outcome and role of mutation in interleukin-10(IL-10) and interleukin-10 receptors(IL-10R) in Asian children with IO-IBD. METHODS All cases of IO-IBD, defined as onset of disease before 12 mo of age, seen at University Malaya Medical Center, Malaysia were reviewed. We performed mutational analysis for IL10 and IL10 R genes in patients with presenting clinical features of Crohn's disease(CD).RESULTS Six [13%; CD = 3, ulcerative colitis(UC) = 2, IBDunclassified(IBD-U) = 1] of the 48 children(CD = 25; UC = 23) with IBD have IO-IBD. At final review [median(range) duration of follow-up: 6.5(3.0-20) years], three patients were in remission without immunosuppression [one each for post-colostomy(IBD-U), after standard immunosuppression(CD), and after total colectomy(UC)]. Three patients were on immunosuppression:one(UC) was in remission while two(both CD) had persistent disease. As compared with later-onset disease, IO-IBD were more likely to present with bloody diarrhea(100% vs 55%, P = 0.039) but were similar in terms of an associated autoimmune liver disease(0% vs 19%, P = 0.31), requiring biologics therapy(50% vs 36%, P = 0.40), surgery(50% vs 29%, P = 0.27), or achieving remission(50% vs 64%, P = 0.40). No mutations in either IL10 or IL10 R in the three patients with CD and the only patient with IBD-U were identified.CONCLUSION The clinical features of IO-IBD in this Asian cohort of children who were negative for IL-10 or IL-10 R mutations were variable. As compared to childhood IBD with onset of disease after 12 mo of age, IO-IBD achieved remission at a similar rate.

Quality indicators in pediatric colonoscopy in a low-volume center:Implications for training

AIM To study implications of measuring quality indicators on training and trainees' performance in pediatric colonoscopy in a low-volume training center.METHODS We reviewed retrospectively the performance of pediatric colonoscopies in a training center in Malaysia over 5 years(January 2010-December 2015), benchmarked against five quality indicators: appropriateness of indications, bowel preparations, cecum and ileal examination rates, and complications. The European Society of Gastrointestinal Endoscopy guideline for pediatric endoscopy and North American Society for Pediatric Gastroenterology, Hepatology and Nutrition training guidelines were used as benchmarks.RESULTS Median(± SD) age of 121 children [males = 74(61.2%)] who had 177 colonoscopies was 7.0(± 4.6) years. On average, 30 colonoscopies were performed each year(range: 19-58). Except for investigations of abdominal pain(21/177, 17%), indications for colonoscopies were appropriate in the remaining 83%. Bowel preparation was good in 87%. One patient(0.6%) with severe Crohn's disease had bowel perforation. Cecum examination and ileal intubation rate was 95% and 68.1%. Ileal intubation rate was significantly higher in diagnosing or assessing inflammatory bowel disease(IBD) than non-IBD(72.9% vs 50.0%; P = 0.016). Performance of four trainees was consistent throughout the study period. Average cecum and ileal examination rate among trainees were 97% and 77%.CONCLUSION Benchmarking against established guidelines helps units with a low-volume of colonoscopies to identify area for further improvement.