Systematic review: interventions in sickle cell disease-related priapism in reducing overall morbidity, recurrence, and side effects

Systematic review: interventions in sickle cell disease-related priapism in reducing overall morbidity, recurrence, and side effects

Juliana Arenas 1 , Hugo E. López-Ramos 2, Maria P. Abaunza-Camacho 2

1 Department of Urology, Pontificia Universidad Javeriana, Hospital Universitario San Ignacio, Bogotá, Colombia; 2 Department of Urology, Hospital Universitario San Ignacio, Pontificia Universidad Javeriana, Bogotá, Colombia

*Correspondence: Hugo E. López-Ramos, Email not available

Abstract

Pediatric priapism is a rare, underreported urological emergency. Sickle cell disease (SCD)-related priapism cases should be assessed considering specific pathophysiologic characteristics. Chronic control of the disease has an impact on a patient’s quality of life. A systematic review in different databases was done, for defining if management in All-type priapism in SCD patients was equally effective in reducing overall morbidity, disability and comorbidities, recurrence, and therapy-related side effects. Qualitative analysis was performed. Nine studies were included in the study; the majority were case series, and 300 patients were included in the analysis. The interventions and outcomes were clinically heterogeneous but displayed perspectives for further studies. This is the first analysis approach for a consensus for SCD-related priapism treatment. Studies with internal validity and causality designs are needed, to evaluate further causality relationships and establish evidence- based approaches. Etilefrine, Pseudoephedrine/etilefrine-5 inhibitors, and finasteride are promising strategies. Quality of life scores should be applied to these patients, and molecular studies should be developed for pharmacological designs.

Keywords: Priapism. Sickle cell disease. Sickle cell anemia. Recurrence. Quality of life.

Contents

DOI: 10.24875/RUC.23000082
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    DOI: 10.24875/RUC.23000082

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