Sickling Cells, Cyclic Nucleotides, and Protein Kinases: The Pathophysiology of Urogenital Disorders in Sickle Cell Anemia
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 ul...
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| Format: | Article |
| Language: | English |
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Wiley
2012-01-01
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| Series: | Anemia |
| Online Access: | http://dx.doi.org/10.1155/2012/723520 |
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| author | Mário Angelo Claudino Kleber Yotsumoto Fertrin |
| author_facet | Mário Angelo Claudino Kleber Yotsumoto Fertrin |
| author_sort | Mário Angelo Claudino |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-91917b8f50a24ea0a4e83e8402e72e71 |
| institution | Kabale University |
| issn | 2090-1267 2090-1275 |
| language | English |
| publishDate | 2012-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Anemia |
| spelling | doaj-art-91917b8f50a24ea0a4e83e8402e72e712025-02-03T05:47:16ZengWileyAnemia2090-12672090-12752012-01-01201210.1155/2012/723520723520Sickling Cells, Cyclic Nucleotides, and Protein Kinases: The Pathophysiology of Urogenital Disorders in Sickle Cell AnemiaMário Angelo Claudino0Kleber Yotsumoto Fertrin1Laboratory of Multidisciplinary Research, São Francisco University (USF), 12916-900 Bragança Paulista, SP, BrazilHematology and Hemotherapy Center, University of Campinas (UNICAMP), 13083-970 Campinas, SP, BrazilSickle 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.http://dx.doi.org/10.1155/2012/723520 |
| spellingShingle | Mário Angelo Claudino Kleber Yotsumoto Fertrin Sickling Cells, Cyclic Nucleotides, and Protein Kinases: The Pathophysiology of Urogenital Disorders in Sickle Cell Anemia Anemia |
| title | Sickling Cells, Cyclic Nucleotides, and Protein Kinases: The Pathophysiology of Urogenital Disorders in Sickle Cell Anemia |
| title_full | Sickling Cells, Cyclic Nucleotides, and Protein Kinases: The Pathophysiology of Urogenital Disorders in Sickle Cell Anemia |
| title_fullStr | Sickling Cells, Cyclic Nucleotides, and Protein Kinases: The Pathophysiology of Urogenital Disorders in Sickle Cell Anemia |
| title_full_unstemmed | Sickling Cells, Cyclic Nucleotides, and Protein Kinases: The Pathophysiology of Urogenital Disorders in Sickle Cell Anemia |
| title_short | Sickling Cells, Cyclic Nucleotides, and Protein Kinases: The Pathophysiology of Urogenital Disorders in Sickle Cell Anemia |
| title_sort | sickling cells cyclic nucleotides and protein kinases the pathophysiology of urogenital disorders in sickle cell anemia |
| url | http://dx.doi.org/10.1155/2012/723520 |
| work_keys_str_mv | AT marioangeloclaudino sicklingcellscyclicnucleotidesandproteinkinasesthepathophysiologyofurogenitaldisordersinsicklecellanemia AT kleberyotsumotofertrin sicklingcellscyclicnucleotidesandproteinkinasesthepathophysiologyofurogenitaldisordersinsicklecellanemia |