Ischaemic Priapism and Glucose- 6- Phosphate Dehydrogenase Deficiency: A Mechanism of Increased Oxidative Stress?

Morrison, BF; Thompson, E. Aubrey; Shah, SD; Wharfe, GH

doi:10.7727/wimj.2013.294

Cited by 5 publications

(3 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…G6PD deficiency increases the risk of kernicterus and death from neonatal jaundice (Cunningham et al, 2016) and has also been associated with bipolar and schizoaffective disorders (Raj et al, 2014), erectile dysfunction (Morrison et al, 2014), and vitiligo (Namazi, 2015). Because G6PD plays an essential role in maintaining healthspan by protection against oxidative damage (Nóbrega-Pereira et al, 2016), the effects of G6PD deficiency on human health have likely been underestimated and thus it is expected that additional consequences of G6PD deficiency will be identified in the future (Spencer and Stanton, 2016; Stanton, 2012).…”

Section: Discussionmentioning

confidence: 99%

Coupling between Protein Stability and Catalytic Activity Determines Pathogenicity of G6PD Variants

et al. 2017

View full text Add to dashboard Cite

Summary G6PD deficiency, an enzymopathy affecting 7% of the world population, is caused by over 160 identified amino acid variants in glucose-6-phosphate dehydrogenase (G6PD). The clinical presentation of G6PD deficiency is diverse, likely due to the broad distribution of variants across the protein and the potential for multidimensional biochemical effects. In this study, we use bioinformatic and biochemical analyses to interpret the relationship between G6PD variants and their clinical phenotype. Using structural information and statistical analyses of known G6PD variants, we predict the molecular phenotype of five uncharacterized variants from a reference population database. Through multidimensional analysis of biochemical data, we demonstrate that the clinical phenotypes of G6PD variants are largely determined by a trade-off between protein stability and catalytic activity. This work expands the current understanding of the biochemical underpinnings of G6PD variant pathogenicity, and suggests a promising avenue for correcting G6PD deficiency by targeting essential structural features of G6PD.

show abstract

Section: Discussionmentioning

confidence: 99%

Coupling between Protein Stability and Catalytic Activity Determines Pathogenicity of G6PD Variants

et al. 2017

View full text Add to dashboard Cite

show abstract

“…Morrison et al have proposed a possible mechanism of priapism that in G6PD deficiency oxidative stress which leads to hemolysis, endothelial injury and nitric oxide depletion. NADPH levels are reduced which causes reduced and aberrant nitric oxide signaling [4]. In most situations of oxidative stress when patients with G6PD deficiency present with acute hemolysis, the levels of G6PD enzyme are usually normal as the cells with low levels of the enzyme have lyzed but interestingly all of the cases described in literature who had priapism with G6PD desease had low G6PD levels but had no signs of acute hemolysis at the time of presentation [4][5][6].…”

Section: Discussionmentioning

confidence: 99%

G6PD Deficiency and Priapism: Is There a Link Between the Two Entities?

2019

View full text Add to dashboard Cite

Glucose 6 phosphate dehydrogenase (G6PD) is called the house keeping enzyme essential for all cells survival. G6PD deficiency is best studied for its role in hemolysis as this enzyme is the only source of nicotinamide adenine dinucleotide phosphate (NADPH) in red blood cells (RBCs) and is the cells defense against reactive oxygen species. We present one of the rare complications of G6PD deficiency in this case where all other causes of priapism were ruled out.

show abstract

“…For that reason, it is important to treat it in time and in correct way, but also to identify the possible risk factors. Three cases of priapism in patients with G6PD deficiency have been described in literature (2). We report an unique case of "high flow" priapism without fistula in a patient with G6PD deficiency.…”

Section: Introductionmentioning

confidence: 99%

Priapism and glucose-6-phosphate dehydrogenase deficiency: An underestimated correlation?

Rose¹,

Mantica²,

Tosi³

et al. 2016

Arch Ital Urol Androl

View full text Add to dashboard Cite

Priapism is a rare clinical condition characterized by a persistent erection unrelated to sexual excitement. Often the etiology is idiopathic. Three cases of priapism in glucose-6-phosphate dehydrogenase (G6PD) deficiency patients have been described in literature. We present the case of a 39-year-old man with glucose-6-phosphate dehydrogenase deficiency, who reached out to our department for the arising of a non-ischemic priapism without arteriolacunar fistula. We suggest that the glucose-6-phosphate dehydrogenase deficiency could be an underestimated risk factor for priapism.KEY WORDS: Non-ischemic; Glucose-6-phosphate dehydrogenase (G6PD) deficiency. obliged him to go to the emergency department. He reported a normal and spontaneous erectile function before the arising of the disease. He denied having any gender of trauma and he admitted to have a G6PD deficiency, documented by laboratory tests. He denied having taken any drugs, phosphodiesterase type 5 (PDE5) inhibitors, intracavernosal agents or any other pharmacological agents. He denied any other risk factor for priapism. At clinical examination he presented a painless erection. Systemic hemoglobin was 13.6 g/dL. Intracorporal blood resulted as arterial at gas analysis (P0 2 = 76 mmHg; PC0 2 = 44.5 mmHg; pH = 7.35). The Color Doppler ultrasonography didn't point out any arterocavernosa fistula. After a period of observation in which there wasn't the resolution of the priapism, we decided to submit him to a pelvic arteriography (Figure 1). Panoramic arteriography of the aorto-iliac and selective of internal pudendal artery showed a bilateral increase of the arterial level of the corpora cavernosa, higher in the left one, without a fistula. We performed a temporary embolization with Spongostan TM of the distal left internal pudendal artery. A complete detumescence was obtained and the next arteriography confirmed the procedure was a success. The patient was released. After 2 weeks of a state of good health, he presented a new episode of prolonged erection, after which he returned to the emergency department of our Institute.

show abstract

Ischaemic Priapism and Glucose- 6- Phosphate Dehydrogenase Deficiency: A Mechanism of Increased Oxidative Stress?

Abstract: Ischaemic priapism is a devastating urological condition that has

Cited by 5 publications

References 11 publications

Coupling between Protein Stability and Catalytic Activity Determines Pathogenicity of G6PD Variants

Coupling between Protein Stability and Catalytic Activity Determines Pathogenicity of G6PD Variants

G6PD Deficiency and Priapism: Is There a Link Between the Two Entities?

Priapism and glucose-6-phosphate dehydrogenase deficiency: An underestimated correlation?

Contact Info

Product

Resources

About