title: Renal involvement in sickle cell-beta thalassemia.
abstract: Renal function studies were performed in 41 patients with sickle cell-beta thalassaemia (S/b thal) and compared to 14 normal controls and 8 sickle cell (SS) patients. Polyuria, hyposthenuria and mild proteinuria were common in both S/b thal and SS patients. A renal concentrating defect was manifest in all patients studied, and in 4 of the 7 S/b that patients tested, an abnormal acidification test was found. A statistically significant negative correlation (n = 19, r = -0.48, p less than 0.05) was noted between creatinine clearance (CCr) and age for the patients over 30 years. There was no correlation between hemoglobin and CCr; on the contrary, a statistically significant negative correlation was found between CCr and hemoglobin F (n = 29, r = -0.428, p less than 0.05) Our S/b thal and SS patients showed a decreased daily excretion of sodium, calcium, phosphate and magnesium and lower serum magnesium levels than the controls. One third of the S/b thal patients showed hyperuricosuria, and a statistically significant negative correlation was noted between serum uric acid and its fractional excretion in all S/b thal patients (n = 41, r = -0.450, p less than 0.01). Serum phosphate levels were independent of age. A statistically significant positive correlation was found between the tubular reabsorptive capacity for phosphate and the number of painful crises per year (n = 33, r = 0.836, p less than 0.001). We conclude that renal involvement in the double heterozygous state is as severe as in homozygous sickle cell disease.
authors.affiliation: Second Department of Medicine, University of Athens, Greece.
authors.forename: P
authors.initials: P
authors.lastname: Kontessis
authors.forename: D
authors.initials: D
authors.lastname: Mayopoulou-Symvoulidis
authors.forename: A
authors.initials: A
authors.lastname: Symvoulidis
authors.forename: I
authors.initials: I
authors.lastname: Kontopoulou-Griva
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000503:physiopathology* / Q000652:urine; D003404:Creatinine / Q000378:metabolism; D004573:Electrolytes / Q000097:blood / Q000652:urine; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis; D006441:Hemoglobin A / Q000032:analysis; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007668:Kidney / Q000502:physiology / Q000503:physiopathology*; D007677:Kidney Function Tests; D008297:Male; D010146:Pain; D011507:Proteinuria; D012016:Reference Values; D013789:Thalassemia / Q000097:blood / Q000503:physiopathology* / Q000652:urine
chemical_list: D004573:Electrolytes; D006454:Hemoglobins; D006441:Hemoglobin A; D005319:Fetal Hemoglobin; D003404:Creatinine