Alagille syndrome and liver transplantation.
Alagille syndrome is a multisystem disorder in which progressive liver disease with persistent cholestasis and dramatic pruritus often warrant consideration for liver transplantation. The most important part of the transplant assessment is evaluation of the cardiac and renal involvement. Preoperatively, cardiac performance often must be tested with dynamic stress tests, mimicking hemodynamic changes during liver transplant. Many aspects of the syndrome including cholestasis, pruritus, and hypercholesterolemia improve posttransplant, but short stature is rarely significantly affected. One- and 5-year patient and graft survival after liver transplant is comparable to other elective indications, but effects of long-term immunosuppressants on evolution of other components of the syndrome, including vascular, bone, and renal disease, remain largely unknown.
Kamath
Binita M
BM
Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Schwarz
Kathleen B
KB
Hadzić
Nedim
N
D016738:Alagille Syndrome / Q000601:surgery*; D006085:Graft Survival; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016031:Liver Transplantation; D011300:Preoperative Care
D007166:Immunosuppressive Agents