Sharpsteen J R, Powars D, Johnson C, Rogers Z R, Williams W D, Posch R J
Department of Pediatrics, University of Southern California School of Medicine, Los Angeles.
Am J Med. 1993 Mar;94(3):289-95. doi: 10.1016/0002-9343(93)90061-s.
Priapism is an uncommon but debilitating complication of sickle cell disease (SCD). Recent observations among adult males regarding the abysmal failure of medical and surgical therapy encouraged us to review our 25-year experience identifying the prognostic features that might determine outcome.
As part of a prospective 25-year longitudinal demographic and clinical cohort study, a subset of 38 (8.2%) patients with priapism were identified among a cohort of 461 men with SCD. The patients with priapism were compared with the nonaffected men with respect to severity of disease expression, hematologic status, beta s globin gene haplotype, and the incidence of sickle-related major organ failure. The influence of the treatment modalities on outcome was also evaluated.
Priapism occurred as a single episode in 24 patients, and in 14 as temporally clustered repeat episodes. Eighty-seven percent of those with priapism had sickle cell anemia (SS), an increased risk as compared with other variants of SCD (p = < 0.05). There were two distinct age-related patterns of disease expression. Eight patients were prepubertal; they experienced shorter episodes, involvement of the corpora cavernosa only, few recurrent episodes, and a good prognosis for future erectile function. Non-surgical therapy in children was associated with excellent results. In contrast, the 29 postpubertal adults often had involvement of the corpora cavernosa and corpus spongiosa (tricorporal disease) and half had prolonged episodes that lasted longer than 8 days. One pubescent patient had repeated episodes and became impotent. Prolonged or repeated episodes eventuated in impotence in 56%. Surgical intervention was not beneficial. Sickle cell-related organ failure such as stroke, chronic restrictive lung disease, chronic renal failure, and nonhealing leg ulcers was observed more frequently in men who had priapism. Death occurred in nine adult patients (25%) within 5 years of the first episode of priapism.
Priapism in adult males identifies those at high risk for other sickle cell-related organ failure syndromes and, as such, is another complication indicative of severe disease. The dismal prognosis in SS adults requires better understanding of the precise pathophysiology of low-flow tricorporal priapism. Clarification of the mechanisms inducing the priapic state should lead to specific therapeutic maneuvers and an improved prognosis for this disabling condition.
阴茎异常勃起是镰状细胞病（SCD）一种罕见但使人衰弱的并发症。近期对成年男性患者的观察发现，药物和手术治疗效果极差，这促使我们回顾25年的经验，以确定可能决定预后的预测特征。
作为一项前瞻性25年纵向人口统计学和临床队列研究的一部分，在461名患有SCD的男性队列中，识别出38名（8.2%）阴茎异常勃起患者。将阴茎异常勃起患者与未受影响的男性在疾病表现严重程度、血液学状态、βs珠蛋白基因单倍型以及镰状细胞相关主要器官衰竭发生率方面进行比较。还评估了治疗方式对预后的影响。
24例患者阴茎异常勃起为单次发作，14例为时间上聚集的重复发作。阴茎异常勃起患者中87%患有镰状细胞贫血（SS），与SCD的其他变体相比风险增加（p = < 0.05）。存在两种与年龄相关的不同疾病表现模式。8例患者为青春期前；他们发作时间较短，仅累及海绵体，复发次数少，未来勃起功能预后良好。儿童非手术治疗效果极佳。相比之下，29例青春期后成年患者常累及海绵体和尿道海绵体（三 corporal 疾病），半数患者发作持续时间超过8天。1例青春期患者反复发并导致阳痿。56%的患者因发作时间延长或反复发最终导致阳痿。手术干预并无益处。阴茎异常勃起的男性中，镰状细胞相关器官衰竭如中风、慢性限制性肺病、慢性肾衰竭和不愈合的腿部溃疡更为常见。9例成年患者（25%）在阴茎异常勃起首次发作后5年内死亡。
成年男性阴茎异常勃起表明其有发生其他镰状细胞相关器官衰竭综合征的高风险，因此是严重疾病的另一种并发症。SS成年患者预后不佳，需要更好地了解低流量三 corporal 阴茎异常勃起的确切病理生理学。阐明导致阴茎异常勃起状态的机制应能带来特定的治疗手段，并改善这种致残状况的预后。
