Research Group Pediatric Hepatology - Björn Fischler
Pediatric hepatology is a young subspecialty where clinical development has been exceptionally rapid in recent decades. From a situation at the end of the 1980s where morbidity and mortality from liver diseases in children were both significantly high, today over 95 percent of these patients can be saved to a long life with a good quality of life.
Decisive steps have been the development of liver transplantation, improved medical and nutritional treatment methods, and refined diagnostics.
The pediatric liver service at Karolinska University Hospital, Huddinge has been a national specialty throughout this period and has been responsible for clinical and research efforts in favor of the rapid development. Today, approximately 200 new children with severe liver disease are received per year for investigation and treatment, of which around 10 children per year need to undergo a liver transplant, while the rest can be treated effectively with drugs and specially designed nutrition.
The group's research mainly concerns cholestatic liver disease, viral infections and liver transplantation.
Research studies
Summary of research programs from the application to CIMED 2024
Cholestasis in infants is a serious medical condition with high morbidity and mortality. Knowledge of the mechanisms of origin of the various underlying diseases is limited, which hampers the possibilities for targeted therapy. We are therefore studying the pathogenesis, course and treatment of three of these unusual diseases:
Biliary atresia (BA),
where cytomegalovirus (CMV) appears to be important for both onset and course, which we study here in more detail. We and others have demonstrated a potentially beneficial effect on the course of antiviral therapy in uncontrolled studies and we now want to provide such treatment in a randomized controlled multicenter trial.
Progressive familial intrahepatic cholestasis (PFIC),
where we are studying the long-term effect of the drug odevixibat, which inhibits bile acid uptake in the terminal ileum and thereby reduces the patients' difficult-to-treat and painful cholestatic itching, as well as lowers the level of bile acids in serum, which in turn is expected to give an improved outcome in the long term with reduced need for liver transplantation.
Alagille syndrome,
where the genetic defect severely disrupts the maturation of normal bile duct cells but also affects several other organ systems. In these patients, we want to study in detail the reasons for the increased tendency to serious infections, as well as the effect of drugs that inhibit bile acid absorption in the terminal ileum.
Members of the research group
Björn Fischler,
adjunct professor, university hospital senior consultant
Henrik Arnell, PhD,
senior consultant and head of clinical unit
Afrodite Psaros-Einberg,
PhD, senior consultant
Ulrika Liliemark,
specialist physician,
PhD student in the biliary atresia and CMV project (Supervisor Björn Fischler, Afrodite Psaros Einberg, Jan F Svensson)
Feven Dawit,
specialist physician,
PhD student in the Alagille syndrome project (Supervisor Björn Fischler, Henrik Arnell, Niklas Björkström, Kristina Teär Fahnehjelm)
Silvia Malenicka,
specialist physician,
PhD student in liver transplantation project (Supervisors Björn Fischler, Carl Jorns, Ulla Berg, Antal Nemeth)
Gandom Kharrazi,
assistant senior consultant,
PhD student in autoimmune liver disease project (Supervisor Afrodite Psaros Einberg, Thomas Casswall, Björn Fischler)