Gbadoé Adama Dodji, Koffi Kouami Sédo, Akakpo-Maxwell Olivia, Lawson-Evi Koko, Guédéhoussou Têtê, Assimadi Jean Kossi
Unité d'infectiologie et d'onco-hématologie, Service de pédiatrie, CHU-Tokoin, BP 8881, Lomé, Togo.
Sante. 2002 Jul-Sep;12(3):343-7.
Priapism is a common complication of sickle cell anemia. Two different patterns are described: acute priapism, a prolonged painful erection generally lasting more than 6 hours, and stuttering priapism, which consist of brief repeated self-resolving episodes. Until 1990, priapism in sickle-cell patients has relied on measures aimed at lowering blood viscosity and acidosis and reducing the level of circulating hemoglobin S (alcalinization, hyperhydration, exsanguinotransfusion). But these means are not consistently successful. Surgical cavernous-venous shunt was proposed after 12 to 24 hours when conservative treatment failed. These therapeutic modalities are based on the pathophysiology of sickle-cell priapism. Priapism in sickle-cell disease may be due to sequestered sickled red cells in the corpus cavernosum with venous outflow obstruction. For some years, the treatment of priapism in sickle-cell anemia was changed by the use of alpha-adrenergic agonists. These therapeutics (mainly etilefrine and epinephrine) were first reserved for priapism resulting from intrapenile injections of vasoactive drugs which are used for the treatment of impotence. In acute priapism, alpha-adrenergic agonists are used in intracavernous injections (ICI). In stuttering priapism, treatment consists in an oral administration associated, if necessary, with self-administered ICI. ICI results mainly depend on when treatment occurs. Detumescence is achieved in patients treated within 30 hours, as opposed to the few patients treated beyond this delay. This finding is in agreement with experimental findings demonstrating histological evidence of necrosis of endothelial cells and cavernous smooth muscle fibers after 24 hours. Surgery is only used after failure of ICI. The result of oral treatment is not very satisfactory because many patients do not respond well or are dependent on ICI. However, self-administered ICI associated with the oral treatment protects patients with stuttering priapism against acute strokes. The safety of alpha-adrenergic agonists is good as both oral and ICI have few side-effects. The excellent efficacy of ICI in sickle-cell priapism leads to suggest that the pathogenic mechanism could involve a neuromuscular dysfunction.
阴茎异常勃起是镰状细胞贫血的常见并发症。其有两种不同类型：急性阴茎异常勃起，即长时间疼痛性勃起，通常持续超过6小时；以及间歇性阴茎异常勃起，由短暂反复自行消退的发作组成。直到1990年，镰状细胞病患者的阴茎异常勃起一直依赖于旨在降低血液粘度和酸中毒以及降低循环血红蛋白S水平的措施（碱化、过度水化、放血输血）。但这些方法并非总能成功。当保守治疗失败后，在12至24小时后建议进行手术性海绵体静脉分流。这些治疗方式基于镰状细胞性阴茎异常勃起的病理生理学。镰状细胞病中的阴茎异常勃起可能是由于镰状红细胞在海绵体中滞留并伴有静脉流出受阻。多年来，α-肾上腺素能激动剂的使用改变了镰状细胞贫血中阴茎异常勃起的治疗方法。这些治疗药物（主要是乙苯福林和肾上腺素）最初仅用于因阴茎内注射用于治疗阳痿的血管活性药物导致的阴茎异常勃起。在急性阴茎异常勃起中，α-肾上腺素能激动剂用于海绵体内注射（ICI）。在间歇性阴茎异常勃起中，治疗包括口服给药，必要时联合自行进行的ICI。ICI的效果主要取决于治疗时间。在30小时内接受治疗的患者可实现消肿，而超过此时间延迟接受治疗的患者则很少能消肿。这一发现与实验结果一致，实验结果表明24小时后存在内皮细胞和海绵体平滑肌纤维坏死的组织学证据。仅在ICI治疗失败后才使用手术。口服治疗的效果不太令人满意，因为许多患者反应不佳或依赖ICI。然而，自行进行的ICI联合口服治疗可保护间歇性阴茎异常勃起患者预防急性发作。α-肾上腺素能激动剂的安全性良好，因为口服和ICI的副作用都很少。ICI在镰状细胞性阴茎异常勃起中的卓越疗效表明其致病机制可能涉及神经肌肉功能障碍。
