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.

How I manage priapism due to sickle cell disease

“…Priapism is more common in homozygous sickle cell anaemia but can affect all the genotypes of the sickling disorders. In a report of paediatric and adolescent cases, there was a five‐fold increase in prevalence of homozygous cases compared with sickle cell haemoglobin C disease (Furtado et al , ) although no cases were seen in Sβ thalassaemia individuals. Priapism in SCD is almost always of the low flow ischaemic type, although isolated cases of high flow arterial priapism have been reported (Ramos et al , ).…”

Prevalence of priapism in individuals with sickle cell disease and implications on male sexual function

“…(18) Priapism in patients with SCD is a urologic emergency that requires immediate intervention to avoid erectile tissue damage. (11,20,21) In the present study, 11 patients sought emergency care because of painful and persistent erection. All patients were seen at local public health facilities, as there was no reference center for these emergencies in our region.…”

Endofalloprosthesis with a one-component semirigid prosthesis in a patient with an arteriovenous fistula of the cavernous artery and the development of priapism

“…человек в год [2,3]. У больных серповидноклеточной анемией, врожденным заболеванием, вызывающим хроническую гемолитическую анемию, распростра-ненность приапизма достигает 3,6 % в возрасте младше 18 лет [4] с увеличением показателей до 42 % в возрасте старше 18 лет [5][6][7].…”