Genitourinary complications of sickle cell disease

Purpose: In the last half century the molecular biology, pathophysiology an d natural history of sickle cell disease have been well defined. Sickle cel l disease causes microvascular occlusion, which is manifested in most organ systems. The genitourinary tract is most commonly affected by hematuria, u rinary tract infection and priapism but other more serious sequelae have be en identified. Materials and Methods: We performed a computerized MEDLINE search from 1965 to the present and a bibliographic review of cross references. These refer ences were analyzed for meaningful findings and case reports. Results: The diagnosis and management of sickle cell disease have advanced rapidly with a significant increase in the life expectancy of affected pati ents and recognition of a greater number of genitourinary complications. Re nal function may be mildly altered or lost completely. Patients with sickle cell disease are at increased risk for urinary tract infection. Priapism i s a painful complication of sickle cell disease that is poorly understood a nd challenging to treat and prevent. Testicular infarction has also been no ted. Furthermore, renal medullary carcinoma, a highly lethal tumor, develop s almost exclusively in young patients with sickle cell trait. Conclusions: Heightened awareness of the genitourinary complications of sic kle cell disease may prevent end stage disease, including renal failure and impotence. New forms of therapy for sickle cell disease, such as hydroxyur ea, may prevent these complications in the future.