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). 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 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. 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.

[Congenital pulmonary lymphangiectasia with chylothorax. Different evolution of 2 cases with severe neonatal respiratory distress]. / Linfangectasia polmonare congenita con chilotorace. Differente evoluzione di due casi con grave distress respiratorio neonatale.

The Authors report on 2 cases of congenital pulmonary lymphangiectasia with chylothorax and severe respiratory distress which were characterized by a different clinic course. One of the newborns, in fact, died after a few days of life, while the other has survived with the complete regression of chylothorax. The Authors also add some physiopathologic remarks about pulmonary lymphatic circulation, in order to focus the different evolution of the 2 cases and the pulmonary lymphangiectasia treatment.