CM Hollands, FJ Rivera-Pedrogo, R Gonzalez-Vallina, et al (Miami Children’s Hosp, Fla)
Ileal exclusion for Byler’s disease: An alternative surgical aprooach with promising early results for pruritus.
J Pediatr Surg 33:220-224, 1998.
Progressive familial intrahepatic cholestasis (PFIC) – also called Byler’s disease – is an uncommon cause of hepatocellular cholestasis. Affected children have intermittent, painless jaundice. Intense pruritus disproportionate to the degree of hyperbilirubinemia can lead to self-mutilation by scratching. Good results have been achieved with liver transplantation and cutaneous biliary diversion procedures, both of which have their complications. Ileal exclusion or bypass procedures offer some important potential advantages over these approaches.