Priapism: Epidemiology, Treatment, and Secondary Prevention

阴茎异常勃起，一种不受欢迎的阴茎勃起疼痛，是镰状细胞病的常见并发症 疾病在儿童中的试验数据表明，高达90%的男性镰状细胞性贫血患者将 在20岁之前至少经历一次阴茎异常勃起。如果阴茎异常勃起持续4小时或更长时间而没有消肿，则患者存在不可逆的缺血性阴茎损伤的风险，其可能以纤维化和阳痿终止。然而，发病率尚不清楚，管理仍然是轶事，治疗方法不一致，结果尚未得到全面评价。该研究项目包括三个具体目标，将通过一系列多中心研究来解决。(1)为了更好地了解哪些，何时，以及在什么情况下，个人发展阴茎异常勃起，我们将管理一个适合年龄和文化敏感的问卷调查，以大横截面的美国男性成人和儿童患者镰状细胞贫血。这将允许列举患病率，精算风险的发展，并详细说明镰状细胞病患者阴茎异常勃起的共同特征。参与本部分研究的患者将接受关于阴茎异常勃起的结构化教育。(2)确定阴茎海绵体抽吸和稀释肾上腺素冲洗在阴茎异常勃起长期发作期间诱导立即消肿的疗效和副作用。(3)确定两种不同药物在阴茎异常勃起二级预防中的有效性。(3a)将在一项双盲、安慰剂对照、交叉研究中评估口服伪麻黄碱降低前6个月内有2次或2次以上阴茎异常勃起发作的患者阴茎异常勃起复发频率和严重程度的能力。(3b)对于多次复发性阴茎异常勃起（严重干扰生活质量）的患者，将评估长效亮丙瑞林6个月疗程预防阴茎异常勃起的能力。将使用骨密度测量以及骨转换的血液和血清标志物来研究该治疗的潜在骨质减少。

Priapism, an undesired painful erection of the penis, is a common complication of sickle cell disease. Pilot data in children suggest that up to 90% of males with sickle cell anemia will experience priapism at least once by 20 years of age. If priapism persists 4 hours or more without detumescence the patient is at risk for irreversible ischemic penile injury, which may terminate in fibrosis and impotence. Yet, incidence is unclear, management remains anecdotal, treatment methods are inconsistent, and outcome has not been comprehensively evaluated. This research project includes three specific aims which will be addressed by a series of multi-center research studies. (1) To better understand which, when, and under what circumstances individuals develop priapism, we will administer an age appropriate and culturally sensitive questionnaire to a large cross section of American male adult and pediatric patients with sickle cell anemia. This will allow enumeration of prevalence, development of actuarial risk, and detailing of the common characteristics of priapism in persons with sickle cell disease. Patients participating in this part of the study will receive structured education about priapism. (2) To determine the efficacy and side effects of aspiration of the corpora cavernosa and irrigation with dilute epinephrine to induce immediate detumescence during prolonged episodes of priapism. (3) To determine the effectiveness of two different agents in secondary prevention of priapism. (3a) The ability of oral pseudoephedrine to decrease the frequency and severity of recurrent priapism in patients with two or more episodes of priapism in the preceding six months will be assessed in a double blind, placebo-controlled, crossover study. (3b) For patients with multiply recurrent episodes of priapism which interferes substantially with quality of life, the ability of a six-month course of depot leuprolide to prevent priapism will be assessed. Measurement of bone density as well as blood and serum markers of bone turnover will be used to investigate potential osteopenia from this therapy.