Glucose-6-phosphate dehydrogenase deficiency: an etiology for idiopathic priapism?
BACKGROUND
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.


OBJECTIVE
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.


METHODS
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
Arthur L
AL
Department of Urology, The James Buchanan Brady Urological Institute, The Johns Hopkins Hospital and The Johns Hopkins University School of Medicine, Baltimore, MD, USA. aburnett@jhmi.edu
Bivalacqua
Trinity J
TJ
D000328:Adult; D005954:Glucosephosphate Dehydrogenase / Q000378:metabolism; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D008297:Male; D010410:Penile Erection / Q000187:drug effects; D010726:Phosphodiesterase Inhibitors / Q000008:administration & dosage; D011317:Priapism / Q000188:drug therapy* / Q000209:etiology*; D016896:Treatment Outcome
D010726:Phosphodiesterase Inhibitors; D005954:Glucosephosphate Dehydrogenase