The prevalence of priapism in children and adolescents with sickle cell disease in Brazil

摘要

To evaluate priapism rates in individuals 18 years of age with sickle cell disease (SCD) at a referral center. An evaluation was made of 599 consecutive male patients with SCD, separated according to type of hemoglobinopathy (HbSS, HbSC and HbS-β-thalassemia). Age at first episode and number of episodes were recorded. Cases of sickle cell trait were excluded. Mean age was similar in all groups. Overall, priapism occurred in 3.6 % of patients (5.6 % of those with HbSS and 1.1 % of those with HbSC; P = 0.01). In HbSS patients, the prevalence rate of priapism was from 3.5 (CI 95 % 0.94–13.4) when compared with patients with HbSC. No patient with β-thalassemia had priapism. Mean follow-up was 39.7 months (range 1–202 months). Since 91 % of patients with priapism had HbSS, this group was evaluated separately, revealing a rate of priapism of 1.6 % in patients 10 years and 8.3 % in those ≥10 years of age (P = 0.002). Regarding priapism in HbSS patients ≥10 years (8.3 %) when compared with patients 10 years (1.6 %), the prevalence rate was from 3.3 (CI 95 % 1.1–9.5). Duration of follow-up was not correlated with priapism (P = 0.774). Forty-seven patients were lost to follow-up. Telephone contact was successful with 14/22 patients with priapism, 50 % of whom had required hospital treatment. Most episodes (86 %) occurred at night, always during sleep. Medical interventions were required in 13 cases as follows: intravenous hydration (n = 4), corpora cavernosa puncture and drainage (n = 7) and corpus cavernosum–corpus spongiosum shunts (n = 2). The prevalence of priapism in children 18 years of age with SCD was 3.6 %, lower than previously reported. Prevalence was higher in HbSS patients, increasing in patients 10 years of age. Most episodes occurred at night and half of the patients required some form of urological procedure.