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- Title
Sickling Cells, Cyclic Nucleotides, and Protein Kinases: The Pathophysiology of Urogenital Disorders in Sickle Cell Anemia.
- Authors
Claudino, Mário Angelo; Yotsumoto Fertrin, Kleber
- Abstract
Sickle cell anemia is one of the best studied inherited diseases, and despite being caused by a single pointmutation 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 deleterious effects of sickling on the genitourinary tract and how the role of cyclic nucleotides signaling and protein kinases may help understand the pathophysiology underlying these manifestations and develop novel therapies in the setting of urogenital disorders in sickle cell disease.
- Subjects
SICKLE cell anemia; CYCLIC nucleotides; PROTEIN kinases; PATHOLOGICAL physiology; GENITOURINARY diseases; GENETIC disorders; POINT mutation (Biology)
- Publication
Anemia (20901267), 2012, p1
- ISSN
2090-1267
- Publication type
Article
- DOI
10.1155/2012/723520