title: [Anatomical and pathophysiological bases of renal disorders in sickle cell syndrome patients and carriers of the trait].
abstract: The process of intraerythrocytic gelling of haemoglobin S is exacerbated by physiochemical and circulatory conditions which dominate in the renal medulla. In the first few years of life, the sickle cell disturbance is reversible by exchange transfusions. At about the age of 15, this is no longer reversible in homozygotes and is replaced by anatomical lesions which gradually destroy the renal functional capacity for concentrating urine, for excreting H+ and K+ ions and for secreting uric acid. The crises are acute rheological events which temporarily accentuate these disorders which appear to be the combined result of hyperviscosity and vascular occlusions. As the occurrence of hematuria and urinary infections is necessarily organically linked to the parenchymatous lesions, their frequency and severity pose great problems concerning treatment. Recent works emphasize the frequency and severity of renal disorders in sickle cell patients during the period of life which is over 30 years. A preventive disposition must be maintained from childhood: restriction of the use of nephrotoxic drugs, reduction of the frequency of crises, dehydration and urinary infections, and subsequently regular observation of kalemia, uricemia and proteinuria.
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000473:pathology / Q000503:physiopathology / Q000652:urine; D002648:Child; D006417:Hematuria / Q000209:etiology; D006801:Humans; D006859:Hydrogen / Q000652:urine; D007674:Kidney Diseases / Q000209:etiology* / Q000628:therapy; D011188:Potassium / Q000652:urine; D012805:Sickle Cell Trait / Q000150:complications*; D014552:Urinary Tract Infections / Q000209:etiology
chemical_list: D006859:Hydrogen; D011188:Potassium
vernacular_title: Bases anatomiques et physiopathologiques des altérations rénales chez les patients atteints de syndromes drépanocytaires et porteurs du trait.