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

Abstract

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.

Keywords: Biliary atresia; Chronic liver disease; Medical status.

Figure 1

Boxplot showing distribution of weight-for-age and height-for-age z-score in 52 children with biliary atresia surviving with native livers. From top to bottom, the five horizontal lines represent the largest data point, which is not more than 1.5 times from the box, the third quartile, the median, the first quartile and the smallest data point which is not more than 1.5 times the interquartile range from the box, respectively.

Figure 2

Boxplot showing distribution of white cell count, platelet count, total serum bilirubin and liver enzymes in 52 children with biliary atresia surviving with native livers. From top to bottom, the five horizontal lines represent the largest data point, which is not more than 1.5 times from the box, the third quartile, the median, the first quartile and the smallest data point which is not more than 1.5 times the interquartile range from the box, respectively.