Liver involvement in white patients with sickle-cell disease.
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 beta 5 beta 5 form of sickle-cell disease) and 102 patients with double-heterozygous hemoglobin S and beta-thalassemia (65 with the beta 5 beta 0th and 37 with the beta 5 beta +th form of sickle-cell disease).


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


METHODS
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 beta 5 beta 5 form of sickle-cell disease and in 26.8% of the subjects with the beta 5 beta th form. Age-related analysis revealed a greater incidence of cholelithiasis during the first years of life in the patients with the beta 5 beta 5 form of the disease than in patients with the beta 5 beta th form.


CONCLUSIONS
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.
Department of Pediatric Hematology and Oncology, University of Catania, Italy.
P
P
Samperi
C
C
Consalvo
V
V
Romano
S
S
Gelardi
D
D
Di Bella
G
G
Schilirò
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D002769:Cholelithiasis / Q000209:etiology; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000097:blood / Q000209:etiology*; D008875:Middle Aged