At the Beatrix Children’s Hospital of the University Medical Centre Groningen children from the Netherlands and beyond are treated for rare liver diseases. A recent study of Ruben de Kleine, in collaboration with the University of Groningen, sheds new light on two of these conditions: choledochal malformation (CM) and paediatric acute liver failure (pALF).
Choledochal malformation is a congenital dilation of the bile ducts. The study indicates that children with CM can safely undergo surgery at a later age, which may reduce complications. Furthermore, it was found that bile duct cancer was not present in children who had undergone surgery, whereas in adults with CM, this occurs in 11 per cent of cases. A specialised surgical procedure that removes the affected bile duct provides the necessary protection.
In the case of acute liver failure, a severe condition often of unknown origin, the research demonstrates that specific components of the immune system may play a role. The study examined a cluster of five Dutch children with pALF and concludes that early recognition and specialised care are crucial.
These findings assist doctors in developing better treatment strategies and offer hope for patients suffering from these rare but serious liver diseases.
