Nearly 40% of children with sickle cell illness have liver damage. Cholelithiasis is the most common complication. Acute hepatic crisis is the most serious consequence, with symptoms ranging from rising jaundice to multiple organ failure and death. The most effective and immediate therapy is exchange transfusion. Persistent cholangiopathy is becoming more well recognised, with autoimmune characteristics in the majority of patients, which are exacerbated by chronic ischemia.
Transfusion-related iron overload is not currently an issue in children, and iron chelator hepatotoxicity is uncommon. Researchers provide advice to avoid, investigate, and treat these problems. They stress the importance of strong coordination between hepatologists and sickle cell disease experts.