Hyperammonemia and acute liver failure associated with deferasirox in two adolescents with sickle cell disease.

Chronic red blood cell transfusion therapy (CRTT) prevents strokes and silent cerebral infarcts (SCIs) in children with sickle cell disease (SCD).[1] Deferasirox, a once-daily oral iron chelator, is used to manage transfusion-related iron overload. Deferasirox (Exjade, 25 mg/kg/day) was started in 2014 (ferritin 2232 ng/mL), held in 2015 (ferritin <500 ng/mL) and restarted in 2016 (ferritin 1050 ng/mL). Hyperammonemia and acute liver failure (ALF) have occurred in deferasirox-treated children with transfusion-dependent thalassaemia (TDT).[[2], [4]] We report two deferasirox-treated teens with SCD who developed hyperammonemia and ALF. [Extracted from the article]