Evaluation Of Different Methods Of Treatment In Children With Byler Disease (Progressive Familial Intrahepatic Cholestasis - PFIC)

Ursodeoxycholic acid treatment in children with Byler disease

“…The pruritus is the prominent clinical feature of hepatic cholestasis and remains a therapeutic challenge with significant morbidity and poorly defined pathogenesis. Several treatment modalities, including rifampicin, phenobarbital, UDCA, nalaxone, plasmapheresis and phototherapy, have been used for pruritus 5 , 13 . There is currently no effective medical therapy for children with Byler disease.…”

“…Medical therapy for Byler disease has been disappointing. Ursodeoxycholic acid (UDCA), cholestyramine, phenobarbital and rifampin therapy have all been tried with various degrees of success 5 . The aim of the present study is to show the effect of UDCA in children with Byler disease.…”

“…The disease is transmitted as an autosomal recessive trait with variable expression 4 . Medical therapy does little to control pruritus and has no effect on the liver failure 5 . The pathogenesis of Byler disease is not exactly known.…”