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Volume 2012, Article ID 723520, 13 pages
Review Article

Sickling Cells, Cyclic Nucleotides, and Protein Kinases: The Pathophysiology of Urogenital Disorders in Sickle Cell Anemia

1Laboratory of Multidisciplinary Research, São Francisco University (USF), 12916-900 Bragança Paulista, SP, Brazil
2Hematology and Hemotherapy Center, University of Campinas (UNICAMP), 13083-970 Campinas, SP, Brazil

Received 23 January 2012; Revised 16 April 2012; Accepted 22 April 2012

Academic Editor: Solomon F. Ofori-Acquah

Copyright © 2012 Mário Angelo Claudino and Kleber Yotsumoto Fertrin. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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 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.