Glucose-6-Phosphate Dehydrogenase Deficiency: An Etiology for Idiopathic Priapism?

Authors

  • Arthur L. Burnett MD,

    1. Department of Urology, The James Buchanan Brady Urological Institute, The Johns Hopkins Hospital and The Johns Hopkins University School of Medicine, Baltimore, MD, USA
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  • Trinity J. Bivalacqua MD, PhD

    1. Department of Urology, The James Buchanan Brady Urological Institute, The Johns Hopkins Hospital and The Johns Hopkins University School of Medicine, Baltimore, MD, USA
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Arthur L. Burnett, MD, Department of Urology, The John Hopkins Hospital, 600 North Wolfe Street/Marburg 407, Baltimore, MD 21287-2411 USA. Tel: 410-614-3986; Fax: 410-614-3695; E-mail: aburnett@jhmi.edu

ABSTRACT

Introduction.  Efforts to identify the health risk associations for priapism may reveal pathophysiologic mechanisms for the disorder and suggest a scientifically rational approach for correcting it.

Aim.  We describe a clinical presentation of idiopathic recurrent priapism in a patient with glucose-6-phosphate dehydrogenase (G6PD) deficiency and consider a possible nitric oxide (NO)-dependent mechanistic basis from which the medical condition causes priapism.

Methods.  The case report profiled a 35-year-old African-American man with G6PD deficiency who presented with a rapid progression of recurrent priapism episodes. He was outwardly healthy and did not have sickle cell disease or trait by hematologic screening. His management featured use of a long-term, continuous phosphodiesterase type 5 (PDE5) inhibitor therapeutic regimen.

Main Outcome Measures.  Clinical history data and response to PDE5 inhibitor therapy.

Results.  After a 3-month duration of PDE5 inhibitor therapy, priapism recurrences were sufficiently resolved and the patient discontinued therapy. At 18-month clinical follow-up, he experienced only minor priapism recurrences and retention of full erectile ability.

Conclusions.  G6PD deficiency offers an explanation for idiopathic priapism. The medical condition generates a pathophysiologic milieu consistent with aberrant NO signaling and heightened oxidative stress in the penis. Burnett AL, and Bivalacqua TJ. Glucose-6-phosphate dehydrogenase deficiency: An etiology for idiopathic priapism? J Sex Med 2008;5:237–240.

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