Open Access
Severe Chemotherapy-Related Hepatic Toxicity Associated with MZ Protease Inhibitor Phenotype
Author(s) -
Eduardo D. Ruchelli,
Marianna Horn,
Suzanne R. Taylor
Publication year - 1990
Publication title -
journal of pediatric hematology/oncology
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.388
H-Index - 78
eISSN - 1536-3678
pISSN - 1077-4114
DOI - 10.1097/00043426-199023000-00019
Subject(s) - medicine , fulminant hepatic failure , chemotherapy , phenotype , fulminant , gastroenterology , wilms' tumor , toxicity , protease inhibitor (pharmacology) , population , immunology , liver transplantation , transplantation , human immunodeficiency virus (hiv) , antiretroviral therapy , viral load , gene , biochemistry , chemistry , environmental health
We describe a 10 1/2-month-old boy in whom fulminant hepatic failure following chemotherapy for Wilms' tumor developed. He then received an orthotopic liver transplant. An unexpected finding was the accumulation of alpha 1-antitrypsin (AAT) in periportal hepatocytes. A pretransplant serum sample showed a Pi MZ phenotype. The rarity of hepatic failure following treatment for Wilms' tumor raises the possibility of an increased susceptibility to toxic injury in the presence of AAT accumulation. Determination of the frequency of protease inhibitor MZ phenotype in patients who have chemotherapy-related hepatotoxicity could be used to initiate a prospective study aimed at identifying an at-risk population for chemoradiotherapy-related hepatoxicity.