Priapism is a devastating complication of sickle cell disease (SCD) with no uniformly accepted prophylaxis. Hydroxyurea (HU) has shown benefit in preventing chronic complications of SCD. We sought to determine the association between HU therapy and the prevention of priapism episodes in men with SCD. A retrospective case series study was conducted between July 1 and September 30, 2014, recruiting men treated at the Sickle Cell Unit, The University of the West Indies, Mona, Jamaica, who had a history of priapism and exposure to HU therapy. A questionnaire on priapism was administered, and changes in the characteristics of priapism episodes relative to HU therapy were recorded. Ten men with a mean age of 26.2 ± 10.7 years consented for the study. The most frequent indication for HU use was prevention of recurrent painful crises (50%). Three patients had baseline priapism episodes occurring daily or on alternate days. The baseline duration of the priapism episodes was greater than two hours in 50% of those interviewed, with 30% lasting over five hours. Five (50%) of the patients had cessation of episodes while on HU. Of the patients who continued to have priapism while on HU therapy, four had episodes lasting less than one hour, with one patient having episodes lasting beyond five hours. Chronic HU therapy appeared to be associated with an amelioration of recurrent priapism episodes in men with SCD.
Objective: The prevalence of asymptomatic bacteriuria (ASB) in sickle cell disease (SCD) in Jamaica is 5.3%. This study sought to determine the association between ASB and anatomical urological abnormalities and symptomatic urinary tract infections (UTIs). Methods: A matched case-controlled study of 11 patients with a history of ASB from the Jamaican Sickle Cell Cohort and 11 controls who did not have a history of ASB or recurrent UTIs but who had SCD was conducted. Midstream urine collected aseptically was obtained for urinalysis and culture and sensitivity analysis. Serum creatinine was measured. Voiding cystourethrogram (VCUG) and kidney-ureter-bladder ultrasound were done. History of UTIs was recorded in all cases. Results: The mean ± sd age of the group (cases and controls) was 34.2 ± 7.2 years. All VCUGs were normal. There was no difference in mean cortical thickness or renal scarring between cases and controls. All serum creatinine values were normal: cases (mean ± sd: 55.9 ± 15.3; min-max: 32-90 µmol/L) and controls (mean ± sd: 59.6 ± 18.5; min-max: 41-95 µmol/L). There was no difference in symptomatic UTIs between cases and controls. Conclusion: There was no association between anatomical urinary tract abnormalities and ASB in patients with SCD. Asymptomatic bacteriuria in SCD was not associated with increased numbers of symptomatic UTIs.
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