Strategies to improve the outcome of biliary atresia – lessons from the Dutch national database

PhD ceremony: Ms. W. de Vries, 14.45 uur, Aula Academiegebouw, Broerstraat 5, Groningen

Dissertation: Strategies to improve the outcome of biliary atresia – lessons from the Dutch national database

Promotor(s): prof. H.J. Verkade

Faculty: Medical Sciences

Biliary atresia (BA) is a severe but rare liver disease of infancy (1:18,000 life births), characterized by complete fibrotic obliteration of the bile ducts and cholestasis, with unknown cause. Treatment is surgical via Kasai’s porto-enterostomy. This technique results in longstanding biliary drainage in 25% of the patients. In the remaining group of patients, liver damage continues and sooner or later a liver transplantation will be necessary.

Using the Dutch national BA patient database, De Vries searched for strategies to improve treatment outcome of BA patients. The pre-transplant mortality appeared to be relatively high in the Netherlands. Referral for liver transplantation in a too advanced state of disease appeared to be an important factor. She also found strong suggestions that early Kasai surgery (before the age of 60 days) and the use of post-Kasai antibiotic prophylaxis may increase the transplant-free survival. In addition, post-Kasai BA patients who have reached adulthood were studied. One fifth of all BA patients survives into adulthood without signs of liver cirrhosis. Furthermore, De Vries studied the health-related quality of life in adult BA patients and the achievement of developmental milestones into adulthood.