Priapism in sickle cell disease: Associations between NOS3 and EDN1 genetic polymorphisms and laboratory biomarkers

Abstract

Priapism is a urologic emergency characterized by an uncontrolled, persistent and painful erection in the absence of sexual stimulation, which can lead to penile fibrosis and impotence. It is highly frequent in sickle cell disease (SCD) associated with hemolytic episodes. Our aim was to investigate molecules that may participate in the regulation of vascular tone. Eighty eight individuals with SCD were included, of whom thirty-seven reported a history of priapism. Priapism was found to be associated with alterations in laboratory biomarkers, as well as lower levels of HbF. Patients with sickle cell anemia using hydroxyurea and those who received blood products seemed to be less affected by priapism. Multivariate analysis suggested that low HbF and NOm were independently associated with priapism. The frequency of polymorphisms in genes NOS3 and EDN1 was not statistically significant between the studied groups, and the presence of the variant allele was not associated with alterations in NOm and ET-1 levels in patients with SCD. The presence of the variant allele in the polymorphisms investigated did not reveal any influence on the occurrence priapism. Future studies involving larger samples, as well as investigations including patients in priapism crisis, could contribute to an enhanced understanding of the development of priapism in SCD.

The Risk of Cardiovascular and Cerebrovascular Disease in Men With a History of Priapism

“…22 For example, men with SCD and priapism have been shown to have lower nitric oxide metabolite levels and higher red blood cell aggregation scores compared to men without priapism. 23,24 Indeed, the current report found some attenuation after removing men with SCD from the primary analysis, but the elevated risk remains.…”

Genetic Variation and Sickle Cell Disease Severity

“…A total of 571 publications passed this screening (eFigure 1 in Supplement 1 , eTable 1 in Supplement 2 ). 8 , 11 , 12 , 13 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 ...…”

The Effectiveness of Priapism's Structured Educational Module on Nurses' Knowledge, Attitude, Beliefs, and Patient’s Profile

“…It is mainly happening in middle age 20 to 40 years in adults and impotence's outcome rates from 35-60% (5) . Despite priapism being a preventable condition, it is a serious and significant problem facing health care systems; increases in the hospital stay and cost negatively affect the patient's impact profile (6) . The condition is classified into three subtypes of priapism; ischemic (generally painful; low flow and veno-occlusive) in record cases, Stuttering (either recurrent or intermittent) considered by repetitive and painful occurrences of extended erections, and non-ischemic (generally non-painful; high arterial flow) (7) .…”

“…The condition is classified into three subtypes of priapism; ischemic (generally painful; low flow and veno-occlusive) in record cases, Stuttering (either recurrent or intermittent) considered by repetitive and painful occurrences of extended erections, and non-ischemic (generally non-painful; high arterial flow) (7) . Hematological disorders are the leading cause of priapism, estimated 40% of all the cases, include sickle-cell disease, hyperviscosity syndromes as seen with the myeloproliferative diseases, hypercoagulable states such as deficiencies of proteins C and S, antiphospholipid syndromes, and amyloidosis, while can be precipitated by trauma, neoplasm, neurologic conditions, and pharmacologic substances, e.g., Viagra (6,8) .…”