Mortality during early adulthood has decreased among patients with pediatric chronic hepatobiliary disease, but the transition of these patients to adult services remains a challenge, according to a retrospective regional cohort study recently published in the Journal of Paediatrics and Child Health.

Pediatric chronic hepatobiliary disease can be caused by a heterogeneous group of diseases, including Alagille syndrome (ALGS), biliary atresia, and viral hepatitis.

The survival of patients with pediatric-onset chronic liver disease has consistently increased over the last 4 decades, leading to the establishment of standard protocols for the transfer of these patients to adult health services. However, data regarding patient outcomes during early adulthood remain scarce, with most studies focusing on specific conditions rather than on this heterogeneous group as a whole.

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The authors of the present study used the database from the Royal Hospital for Children and Young People in Edinburgh, Scotland, and selected 63 patients with a history of pediatric-onset chronic liver disease who had been transferred to adult services. The aim was to assess basic health parameters, including initial diagnosis, mortality, fertility, mental health, and mortality.

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The cohort included 27 different diagnoses, ranging from biliary atresia (13 patients) and ALGS (3 patients) to blunt liver trauma (1 patient). The median age was 26 years; no patient included in the study was formally discharged from follow-up for chronic liver disease.

Transplant prevalence was over 40%, with more than 30% of patients exhibiting transplant-related complications, including portal vein thrombosis, chronic ascites, and opportunistic infection. Although the majority of patients underwent an initial liver transplant during childhood, 2 patients (with ALGS and fulminant liver failure of unknown cause, respectively) underwent retransplant during adulthood.

Within the cohort, 6 patients with different diagnoses, including ALGS, hepatitis C, biliary atresia, and primary sclerosing cholangitis, experienced pregnancies, with 5 resulting in miscarriage or medical termination before 24 weeks. More than 40% of patients had a history of consultation with psychiatric services during follow-up.

“Using a population-based cohort study design, we have demonstrated the heterogeneous nature of pediatric-onset chronic liver disease and health-care issues encountered following handover to adult services,” the authors concluded.


Lau NSM, Henderson P. Outcomes of paediatric patients with chronic liver disease in early adulthood: a heterogeneous, but representative, regional cohort study. J Paediatr Child Health. Published online June 27, 2022. doi:10.1111/jpc.16091