Sickling cells, cyclic nucleotides, and protein kinases: the pathophysiology of urogenital disorders in sickle cell anemia.

ABSTRACT: Sickle cell anemia is one of the best studied inherited diseases, and despite being caused by a single point mutation in the HBB gene, multiple pleiotropic effects of the abnormal hemoglobin S production range from vaso-occlusive crisis, stroke, and pulmonary hypertension to osteonecrosis and leg ulcers. Urogenital function is not spared, and although priapism is most frequently remembered, other related clinical manifestations have been described, such as nocturia, enuresis, increased frequence of lower urinary tract infections, urinary incontinence, hypogonadism, and testicular infarction. Studies on sickle cell vaso-occlusion and priapism using both in vitro and in vivo models have shed light on the pathogenesis of some of these events. The authors review what is known about the deleter

SUBMITTER: Claudino MA 

PROVIDER: S-EPMC3382378 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature