Diseases of bile duct in children

Several diseases originate from bile duct pathology.Despite studies on these diseases,certain etiologies of some of them still cannot be concluded.The most common disease of the bile duct in newborns is biliary atresia,whose prognosis varies according to the age of surgical correction.Other diseases such as Alagille syndrome,inspissated bile duct syndrome,and choledochal cysts are also time-sensitive because they can cause severe liver damage due to obstruction.The majority of these diseases present with cholestatic jaundice in the newborn or infant period,which is quite difficult to differentiate regarding clinical acumen and initial investigations.Intraoperative cholangiography is potentially necessary to make an accurate diagnosis,and further treatment will be performed synchronously or planned as findings suggest.This article provides a concise review of bile duct diseases,with interesting cases.

Detection of reflux-symptom association in children with esophageal atresia by video-pH-impedance study

BACKGROUND Children with esophageal atresia(EA)have risk of gastroesophageal reflux disease(GERD),suggesting reflux monitoring for prompt management.AIM To evaluate GERD in children with EA and specific symptom association from combined Video with Multichannel Intraluminal Impedance and pH(MII-pH)study.METHODS Children diagnosed with EA with suspected GERD and followed up at King Chulalongkorn Memorial Hospital between January 2000 and December 2018 were prospectively studied.All underwent esophagogastroduodenoscopy with esophageal biopsy and Video MII-pH study on the same day.Symptoms of GERD which included both esophageal and extra-esophageal symptom were recorded from video monitoring and abnormal reflux from MII-pH study based on the statement from the European Paediatric Impedance Group.Prevalence of GERD was also reported by using histopathology as a gold standard.Endoscopic appearance was recorded using Los Angeles Classification and esophagitis severity was graded using Esohisto criteria.RESULTS Fifteen children were recruited with age of 3.1(2.2,9.8)years(40%,male)and the common type was C(93.3%).The symptoms recorded were cough(75.2%),vomiting(15.2%),irritability or unexplained crying(7.6%)and dysphagia(1.9%)with the symptom-reflux association of 45.7%,89%,71%and 0%,respectively.There were abnormal endoscopic appearance in 52.9%,esophagitis in 64.7%and high reflux score in 47.1%.Video MII-pH study has high diagnostic value with the sensitivity,specificity and accuracy of 72.7%,100%and 82.4%,respectively.CONCLUSION Prevalence of GERD in children with EA was high.Video MII-pH study to detect GERD in children with EA had high diagnostic value with the trend of specific symptom association.

Pediatric liver transplantation outcomes from a single center in Thailand

BACKGROUND Liver transplantation(LT)has become an acceptable curative method for children with several liver diseases,especially irreversible acute liver failure and chronic liver diseases.King Chulalongkorn Memorial Hospital is one of Thailand’s largest liver transplant centers and is responsible for many pediatric cases.AIM To report the experience with pediatric LT and evaluate outcomes of livingrelated vs deceased-donor grafts.METHODS This evaluation included children who underwent LT between August 2004 and November 2019.Data were retrospectively reviewed,including demographics,diagnoses,laboratory values of donors and recipients,the pediatric end-stage liver disease(PELD)or model for end-stage liver disease(MELD)score,graft source,wait time,perioperative course,postoperative complications,and survival rates.Continuous data were reported using the median and interquartile range.The Mann–Whitney U-test was used to compare the wait time between the living-related and deceased-donor groups.The chi-square or Fisher's exact test were used to compare the frequencies of between-group complications.Survival rates were calculated using the Kaplan–Meier method.RESULTS Ninety-four operated pediatric liver transplant patients were identified(54%were females).The median age at transplantation was 1.2(0.8-3.8)years.The median PELD and MELD scores were 20(13-26.8)and 19.5(15.8-26.3),respectively.Most grafts(81.9%)were obtained from living-related donors.The median wait time for the living donors was significantly shorter compared with the deceased donors at 1.6(0.3-3.1)mo vs 11.2(2.1-33.3)mo(P=0.01).Most patients were diagnosed with biliary atresia(74.5%),and infection was the most common complication within 30 d posttransplantation(14.9%).Without a desensitization protocol,9%of transplants were ABOincompatible.Eight hepatitis B core antibodies(anti-HBc)-negative recipients received positive anti-HBc grafts without different observed complications.The overall survival rate was 93.6%and 90.3%at 1 and 5 years,respectively.No graft loss during follow-up was noted among survivors.CONCLUSION A significant number of pediatric LT cases were reported in Thailand.Based on relatively comparable outcomes,ABO-incompatible and HBc antibody-positive grafts may be considered in an organ shortage situation.