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)(cholangiti...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.展开更多
基金Supported by University Malaya High Impact Research(UM.C/625/HIR/MOHE/CHAN/13/1)from Ministry of Higher Education,Malaysia
文摘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.
