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Portal hypertension predicts liver transplantation later in biliary atresia course

Recent study results showed that cholangitis episodes and the presence of portal hypertension or gastroesophageal varices in adolescence may predict the need for liver transplantation in adulthood among pediatric patients with biliary atresia.

“By 16 years of age, departure from the pediatric services will be near approaching,” Vandana Jain, from King’s College Hospital in the United Kingdom, and colleagues wrote. “Hence, the identification of prognostic markers, at 16 years of age, for poor outcomes in [biliary atresia] native liver survivors during adulthood, would provide greater awareness and expertise for adult hepatologists, in order to manage this unique cohort and their disease spectrum.”

To identify laboratory, clinical and radiological parameters associated with an increased risk for liver transplantation after age 16 years, Jain and colleagues retrospectively reviewed data on 397 patients with biliary atresia who had received Kasai Portoenterostomy procedure between 1980 and 1996.

Patients who required liver transplantation in adulthood were significantly more likely to have experienced at least one episode of cholangitis around age 16 years (HR = 4.897; 95% CI, 1.973-12.158) compared with native liver survivors.

They were also more likely to have developed portal hypertension (HR = 7.054; 95% CI, 2.354-21.139) or gastroesophageal varices by age 16 years (HR = 8.597; 95% CI, 3.287-22.48) and to have increased spleen size (HR = 1.173; 95% CI, 1.062-1.295) compared with native liver survivors.

“Pediatric patients with [biliary atresia] who survive with their native livers into adulthood, are at high risk of complications, and need specialized management,” Jain and colleagues wrote. “Current adult liver disease severity scoring models are not appropriate for [young patients] with [biliary atresia].”

The researchers concluded that the derivation of a more appropriate ‘biliary atresia liver disease severity scoring model’ for young patients is vital, and the role for Mayo primary sclerosing cholangitis risk scores in patients with biliary atresia should be explored further. – by Talitha Bennett

Disclosure: The authors report no relevant financial disclosures.