Bruno D, Wigfall D R, Zimmerman S A, Rosoff P M, Wiener J S
Division of Urologic Surgery, Department of Surgery, Duke University Medical Center, Durham, NC, USA.
J Urol. 2001 Sep;166(3):803-11.
In the last half century the molecular biology, pathophysiology and natural history of sickle cell disease have been well defined. Sickle cell disease causes microvascular occlusion, which is manifested in most organ systems. The genitourinary tract is most commonly affected by hematuria, urinary tract infection and priapism but other more serious sequelae have been identified.
We performed a computerized MEDLINE search from 1965 to the present and a bibliographic review of cross references. These references were analyzed for meaningful findings and case reports.
The diagnosis and management of sickle cell disease have advanced rapidly with a significant increase in the life expectancy of affected patients and recognition of a greater number of genitourinary complications. Renal function may be mildly altered or lost completely. Patients with sickle cell disease are at increased risk for urinary tract infection. Priapism is a painful complication of sickle cell disease that is poorly understood and challenging to treat and prevent. Testicular infarction has also been noted. Furthermore, renal medullary carcinoma, a highly lethal tumor, develops almost exclusively in young patients with sickle cell trait.
Heightened awareness of the genitourinary complications of sickle cell disease may prevent end stage disease, including renal failure and impotence. New forms of therapy for sickle cell disease, such as hydroxyurea, may prevent these complications in the future.
在过去的半个世纪里，镰状细胞病的分子生物学、病理生理学和自然史已得到明确界定。镰状细胞病会导致微血管阻塞，这在大多数器官系统中都有体现。泌尿生殖道最常受到血尿、尿路感染和阴茎异常勃起的影响，但也发现了其他更严重的后遗症。
我们对1965年至今的医学文献数据库（MEDLINE）进行了计算机检索，并对参考文献进行了文献综述。对这些参考文献进行分析以获取有意义的研究结果和病例报告。
随着受影响患者预期寿命的显著增加以及对更多泌尿生殖系统并发症的认识，镰状细胞病的诊断和治疗取得了迅速进展。肾功能可能会轻度改变或完全丧失。镰状细胞病患者发生尿路感染的风险增加。阴茎异常勃起是镰状细胞病的一种痛苦并发症，人们对其了解甚少，治疗和预防都具有挑战性。也有睾丸梗死的报道。此外，肾髓质癌是一种高度致命的肿瘤，几乎只发生在具有镰状细胞特征的年轻患者中。
提高对镰状细胞病泌尿生殖系统并发症的认识可能会预防终末期疾病，包括肾衰竭和阳痿。镰状细胞病的新治疗方法，如羟基脲，可能在未来预防这些并发症。
