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

J Sex Med. 2008 Jan;5(1):237-40. doi: 10.1111/j.1743-6109.2007.00631.x. Epub 2007 Oct 25.

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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Glucosephosphate Dehydrogenase / metabolism
  • Glucosephosphate Dehydrogenase Deficiency / complications*
  • Glucosephosphate Dehydrogenase Deficiency / drug therapy
  • Humans
  • Male
  • Penile Erection / drug effects
  • Phosphodiesterase Inhibitors / administration & dosage
  • Priapism / drug therapy*
  • Priapism / etiology*
  • Treatment Outcome

Substances

  • Phosphodiesterase Inhibitors
  • Glucosephosphate Dehydrogenase