Prevalence Of Priapism In Children With Sickle Cell Disease. A Study In A Reference Center In Brazil
PURPOSE To report the prevalence of priapism in a reference center for treatment of sickle cell disease(SCA) in Brazil. We also evaluate if there are risk factors of priapism attacks. MATERIAL AND METHODS We studied 603 consecutive male children and adolescents with HbSS (388),HbSC(178), Hb-Talassemia(11), HbAS(6), other hemoglobin disease(22) who were treated from October 1990 to May 2008 in the reference center of hematologic disease in a State of around 14 million habitants. RESULTS Priapism was present in 22 patients (3.4%): 20 (5.2%) in HbSS and 2(1.1%) in HbSC. In univariate analysis of HbSS patients time of follow-up, urianalisis, loss of weight, bone pain, headache, hematologic alterations, urinary tract infection, use of pholate or iron, level of seric hemoglobin, hematocrit, reticulocites and latic dehidrogenase were not associated with priapism. Only age proved to be statistically significant. The prevalence of priapism was 1.6% of patients under 10 years of age and 8.3% of those 10 years old or more (p=0.002). Of the 22 patients with priapism, 14 could be reached by phone. Erectile dysfunction was reported in 5 patients. Ten patients that could not be reached by phone had the charts reviewed and erectile dysfunction was reported by one. CONCLUSIONS The prevalence of priapism in children with SCD is 3.4% and is higher in patients with HbSS. The rate of erectile dysfunction in those patients with priapism was 27%. Only age was associated with occurrence of priapism. To our knowledge we reported the largest study of prevalence of priapism in the pediatric population with SCD in the literature.