PDF(Pubmed)
Abstract：
Priapism is a urologic emergency requiring prompt management. There are three types of priapism: stuttering (intermittent), non-ischemic (high-flow/arterial), and ischemic (low-flow/veno-occlusive). Here, we present the first case of an infant with recurrent non-ischemic priapism as the first sign of severe hypertension. An 11-month-old infant was admitted to the hospital for high-flow priapism. On admission, he was found to have severe hypertension that required a combination of five antihypertensive drugs; abdominal ultrasound showed polycystic kidneys, splenomegaly, and a parenchymal liver lesion. The priapism resolved spontaneously and did not recur again after the initiation of antihypertensive treatment. Genetic analysis confirmed autosomal recessive polycystic kidney disease (ARPKD). We found no other explanation for the priapism, such as genital trauma, hematologic disease, or anything else. Decreased nitric oxide (NO) bioavailability seen in patients with hypertension seems to be the principal mechanism of hypertension causing priapism. This hypothesis is supported by animal models of genetically modified mice lacking nitric oxide synthase. The same mechanism is thought to be the genesis of priapism and other complications, such as pulmonary hypertension, in patients with sickle cell disease. We present a case of severe hypertension-associated priapism in a child with unrecognized ARPKD. The endothelial dysfunction with decreased NO bioavailability seen in patients with hypertension may be the principal pathogenic mechanism.
摘要：
阴茎异常勃起是一种泌尿科急症，需要及时管理。有三种类型的阴茎异常勃起：口吃（间歇性），非缺血性（高流量/动脉），和缺血性（低流量/静脉闭塞）。这里,我们介绍了第一例婴儿复发性非缺血性阴茎异常勃起作为严重高血压的首发症状。一名11个月大的婴儿因高流量阴茎异常勃起入院。一入场,他被发现患有严重的高血压，需要五种抗高血压药物的组合；腹部超声显示多囊肾，脾肿大,和肝实质病变.在开始抗高血压治疗后，阴茎异常勃起自发消退，并且没有再次复发。遗传分析证实常染色体隐性遗传性多囊肾病（ARPKD）。我们没有找到其他关于阴茎异常勃起的解释,比如生殖器创伤,血液病,或其他任何东西。高血压患者的一氧化氮（NO）生物利用度降低似乎是高血压引起阴茎异常勃起的主要机制。缺乏一氧化氮合酶的转基因小鼠的动物模型支持了这一假设。同样的机制被认为是阴茎异常勃起和其他并发症的起源，比如肺动脉高压，镰状细胞病患者。我们介绍了一例患有未识别的ARPKD儿童的严重高血压相关阴茎异常勃起的病例。高血压患者的内皮功能障碍和NO生物利用度降低可能是主要的致病机制。
