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November 1996

Liver Involvement in White Patients With Sickle-cell Disease

Author Affiliations

From the Department of Pediatric Hematology and Oncology (Drs Samperi, Consalvo, Romano, and Schilirò); and the Service of Pediatric Radiology (Drs Gerardi and Di Bella), University of Catania, Catania, Italy.

Arch Pediatr Adolesc Med. 1996;150(11):1177-1180. doi:10.1001/archpedi.1996.02170360067011

Background:  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.

Objective:  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).

Setting:  The Department of Pediatric Hematology and Oncology, University of Catania, Catania, Italy.

Patients:  Outpatients with sickle-cell disease.

Results:  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.

Conclusion:  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