Cholestatic liver injury caused by sirolimus in a child with kaposiform hemangioendothelioma

An 8-month-old female infant received propranolol 2 mg orally twice daily and prednisone 7.5 mg orally once daily for kaposiform hemangioendothelioma. Due to poor efficacy, propranolol and prednisone were discontinued and sirolimus 0.2 mg was given orally twice daily. The child′s liver function was normal before sirolimus treatment. On day 10 of sirolimus treatment, the laboratory tests showed alanine aminotransferase(ALT) 58 U/L, aspartate aminotransferase(AST) 116 U/L, γ- glutamyltransfarase(γ-GT) 84 U/L, alkaline phosphatase(ALP) 181 U/L, total bile acid(TBA) 109.2 μmol/L, total bilirubin(TBil) 57.4 μmol/L, and direct bilirubin(DBil) 38.0 μmol/L. At the same time, the child developed decrease of appetite, milk refusal, and cry and scream all the time. The physical examination showed slightly yellow on her skin and sclera. Cholestatic liver injury caused by sirolimus was considered. Sirolimus was discontinued and liver-protective therapy and drugs for anti-jaundice were given. On day 6 of drug withdrawal, the laboratory tests showed ALT 58 U/L, AST 116 U/L, γ-GT 84 U/L, ALP 181 U/L, TBA 109.2 μmol/L, TBil 41.5 μmol/L, and DBil 20.8 μmol/L; the child′s appetite recovered, yellowish skin and sclera improved markedly. On day 20 of drug withdrawal, the child was discharged. No abnormalities were found in the child′s liver function after 15 days of discharge. Key words: Hemangioendothelioma; Sirolimus; Cholestasis, intrahepatic; Chemical and drug induced liver injury