Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative Strategies

Sickle cell anemia is a common and disabling disorder profoundly affecting mortality as well as quality of life. Up to 35% of men with sickle cell disease are affected by painful, prolonged erections termed ischemic priapism. A priapic episode may result in fibrosis and permanent erectile dysfunctio...

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Main Authors: Genevieve M. Crane, Nelson E. Bennett
Format: Article
Language:English
Published: Wiley 2011-01-01
Series:Anemia
Online Access:http://dx.doi.org/10.1155/2011/297364
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author Genevieve M. Crane
Nelson E. Bennett
author_facet Genevieve M. Crane
Nelson E. Bennett
author_sort Genevieve M. Crane
collection DOAJ
description Sickle cell anemia is a common and disabling disorder profoundly affecting mortality as well as quality of life. Up to 35% of men with sickle cell disease are affected by painful, prolonged erections termed ischemic priapism. A priapic episode may result in fibrosis and permanent erectile dysfunction. The severity of sickle cell disease manifestations is variable dependent on a number of contributing genetic factors; however, priapism tends to cluster with other severe vascular complications including pulmonary hypertension, leg ulceration, and overall risk of death. The mechanisms underlying priapism in sickle cell disease have begun to be elucidated including hemolysis-mediated dysregulation of the nitric oxide signaling pathway and dysregulation of adenosine-mediated vasodilation. A better understanding of these mechanisms is leading toward novel preventative strategies. This paper will focus on the mechanisms underlying development of ischemic priapism in sickle cell disease, current acute and preventative treatment strategies, and future directions for improved management of this disorder.
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spelling doaj-art-ea937572f0a6418ba16c504f9eb4d4522025-08-20T02:22:02ZengWileyAnemia2090-12672090-12752011-01-01201110.1155/2011/297364297364Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative StrategiesGenevieve M. Crane0Nelson E. Bennett1Institute of Urology, Lahey Clinic, 41 Mall Road, Burlington, MA 01805, USAInstitute of Urology, Lahey Clinic, 41 Mall Road, Burlington, MA 01805, USASickle cell anemia is a common and disabling disorder profoundly affecting mortality as well as quality of life. Up to 35% of men with sickle cell disease are affected by painful, prolonged erections termed ischemic priapism. A priapic episode may result in fibrosis and permanent erectile dysfunction. The severity of sickle cell disease manifestations is variable dependent on a number of contributing genetic factors; however, priapism tends to cluster with other severe vascular complications including pulmonary hypertension, leg ulceration, and overall risk of death. The mechanisms underlying priapism in sickle cell disease have begun to be elucidated including hemolysis-mediated dysregulation of the nitric oxide signaling pathway and dysregulation of adenosine-mediated vasodilation. A better understanding of these mechanisms is leading toward novel preventative strategies. This paper will focus on the mechanisms underlying development of ischemic priapism in sickle cell disease, current acute and preventative treatment strategies, and future directions for improved management of this disorder.http://dx.doi.org/10.1155/2011/297364
spellingShingle Genevieve M. Crane
Nelson E. Bennett
Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative Strategies
Anemia
title Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative Strategies
title_full Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative Strategies
title_fullStr Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative Strategies
title_full_unstemmed Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative Strategies
title_short Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative Strategies
title_sort priapism in sickle cell anemia emerging mechanistic understanding and better preventative strategies
url http://dx.doi.org/10.1155/2011/297364
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