Liver involvement and cholelithiasis are common complications of sickle-cell disease. The incidence of clinically evident hepatic damage reported in the literature for black people varies from 15% to 30%, while no data are reported for white people.
To evaluate the liver involvement in 40 patients with homozygous sickle cell anemia (the βsβs form of sickle-cell disease) and 102 patients with doubleheterozygous hemoglobin S and β-thalassemia (65 with the βsβ0th and 37 with the βsβ+th form of sickle-cell disease).
The Department of Pediatric Hematology and Oncology, University of Catania, Catania, Italy.
Outpatients with sickle-cell disease.
We found that, in our patients, liver disease seems to be clinically irrelevant: only 2 of the 142 patients examined had notable alterations in hepatic function. Cholelithiasis was found in 42.1% of the subjects with the βsβs form of sickle-cell disease and in 26.8% of the subjects with the βsβth form. Age-related analysis revealed a greater incidence of cholelithiasis during the first years of life in the patients with the βsβs form of the disease than in patients with the βsβth form.
Our data showed that liver involvement in sickle-cell disease is clinically irrelevant, reflecting the fact that the clinical expression of sickle-cell disease in Sicilian patients is moderate.Arch Pediatr Adolesc Med. 1996;150:1177-1180