Objective To define the incidence, risk factors and complications of priapism in a large population of patients with sickle‐cell anaemia in five centres in the UK and Nigeria, as priapism is common among these patients, but the precise characteristics of the condition in this population are poorly documented.Patients and methods A questionnaire was developed and administered to patients with sickle‐cell disease. Questions were designed to define the incidence, nature, precipitants, duration, treatment and complications of priapism. A distinction was made between acute (severe) priapism and the recurrent, ‘stuttering’ type.Results The questionnaire was completed by 130 patients (mean age 25 years, sd 11, range 4–66) from the five centres; 102 (78%) were homozygous Hb SS genotype, 19 (15%) were Hb SC genotype and two (1.5%) were Hb Sα−thalassaemia. Of the patients, 46 (35%) reported a history of priapism, and of these, 33 (72%) had a history of stuttering priapism, while 24 (52%) had had an acute episode of priapism. The mean age of onset of priapism was 15 years, with 75% of patients having the first episode before their 20th birthday. Sexual activity was the most frequent precipitating factor, with fever and/or dehydration being the next most common. Of the 46 patients, 10 (21%) with a history of priapism reported having erectile dysfunction. A similar proportion reported dissatisfaction with sexual intercourse, including a fear of engaging in sexual activity.Conclusion The incidence of priapism among patients with sickle‐cell anaemia is high (35%). The implications of priapism for erectile and sexual function are significant and documented in this large series. The treatment of this condition in these patients remains unstandardised. This study highlights the need for an increased awareness of the problems associated with priapism among patients, families and medical professionals.
Anyaegbu CC, Okpala IE, Aken'Ova AY & Salimonu LS. Complement haemolytic activity, circulating immune complexes and the morbidity of sickle cell anaemia. APMIS 1999; 107:699-702.The aim of this study was to find out if the number of crises and complications of sickle cell anaemia (SCA) relate to complement function, or the levels of circulating immune complexes (CIC), complement factor B (Bf), C3 and C4. In 73 steady-state HbSS patients and SO HbAA control subjects, we determined the haemolytic activity of the alternative pathway of complement (AP50), of the classical pathway (CHS0); and the serum concentrations of Bf, C3, C4 and CIC. By clinical examination of each patient and review of the medical records, we determined the number of complications of SCA which had occurred and the mean number of crises per year over a minimum period of 3 years. The mean?SD APSO for the patients (1422 U/ml) was significantly lower than the control value of 16+3 U/ml (p<0.001). APSO had a significant inverse correlation with the number of crises (r=-0.30, p<0.02). Mean2SD CIC in patients (0.4520.38 g/l) was significantly higher than in controls: 0.2420. I S g/I (p<0.002). CIC showed a significant direct correlation with the number of complications of SCA (r=+0.28, p
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