title: The prevalence of priapism in children and adolescents with sickle cell disease in Brazil.
abstract: 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.
authors.affiliation: Bahiana University of Bahia, Salvador, Brazil.
authors.forename: Paulo Sampaio
authors.initials: PS
authors.lastname: Furtado
authors.forename: Milena Paiva
authors.initials: MP
authors.lastname: Costa
authors.forename: Flávia
authors.initials: F
authors.lastname: Ribeiro do Prado Valladares
authors.forename: Leandro
authors.initials: L
authors.lastname: Oliveira da Silva
authors.forename: Maurício
authors.initials: M
authors.lastname: Lordêlo
authors.forename: Isa
authors.initials: I
authors.lastname: Lyra
authors.forename: Ubirajara
authors.initials: U
authors.lastname: Barroso
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications*; D001938:Brazil / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D015995:Prevalence; D011317:Priapism / Q000453:epidemiology* / Q000209:etiology*