Siegel J F, Rich M A, Brock W A
Department of Urology, Schneider Children's Hospital, Long Island Jewish Medical Center, New Hyde Park, New York.
J Urol. 1993 Nov;150(5 Pt 1):1480-2. doi: 10.1016/s0022-5347(17)35817-2.
Priapism and acute neurological events are believed to be unrelated complications of sickle cell hemoglobinopathy. We describe a syndrome based on our experience and a review of the literature of significant neurological events after partial exchange transfusion to treat priapism in sicklemic patients. Severe headache is often the initiating symptom of this complex. The ensuing neurological events range from seizure activity to obtundation requiring ventilatory support. The proposed pathophysiology of these neurological events is related to cerebral ischemia after an acute increase in per cent total hemoglobin, concomitant decrease in per cent hemoglobin S and subsequent release of vasoactive substances during penile detumescence. We have termed this constellation of events the ASPEN syndrome, an eponym for association of sickle cell disease, priapism, exchange transfusion and neurological events. Early recognition and aggressive medical management resulted in complete reversal of neurological sequela.
阴茎异常勃起和急性神经事件被认为是镰状细胞血红蛋白病无关的并发症。我们根据自身经验并回顾文献，描述了一种在镰状细胞病患者中进行部分换血治疗阴茎异常勃起后发生的重大神经事件综合征。严重头痛往往是这种综合征的首发症状。随后发生的神经事件范围从癫痫发作到需要通气支持的昏迷。这些神经事件的推测病理生理学与总血红蛋白百分比急性增加、血红蛋白S百分比随之降低以及阴茎消肿过程中血管活性物质的释放后导致的脑缺血有关。我们将这一系列事件称为ASPEN综合征，这是镰状细胞病、阴茎异常勃起、换血治疗和神经事件关联的一个专用名词。早期识别和积极的药物治疗导致神经后遗症完全逆转。
