巴氯芬
这种γ-羟丁氨酸衍生物有松弛肌肉和抗痉挛的作用,有研究显示它可以治疗反复发作的反应性勃起。发生这种反射性勃起的患者多合并脊髓损伤或神经系统疾病。此型阴茎异常勃起的病理生理机制尚不清楚,一些报道称硬膜内注射巴氯芬可以获得不错的疗效。[50]Rourke KF, Fischler AH, Jordan GH. Treatment of recurrent idiopathic priapism with oral baclofen. J Urol. 2002;168:2552-2553.http://www.ncbi.nlm.nih.gov/pubmed/12441973?tool=bestpractice.com[51]Denys P, Mane M, Azouvi P, et al. Side effects of chronic intrathecal baclofen on erection and ejaculation in patients with spinal cord lesions. Arch Phys Med Rehabil. 1998;79:494-496.http://www.ncbi.nlm.nih.gov/pubmed/9596387?tool=bestpractice.com[52]D'Aleo G, Rifici C, Kofler M, et al. Favorable response to intrathecal, but not oral, baclofen of priapism in a patient with spinal cord injury. Spine (Phila Pa 1976). 2009;34:E127-E129.http://www.ncbi.nlm.nih.gov/pubmed/19179913?tool=bestpractice.com文献没有将此类型阴茎异常勃起归类为缺血性或非缺血性,因此还需要进行更多的研究去评估复发性反应性勃起和缺血性间歇发作的异同。[3]Broderick GA, Kadioglu A, Bivalacqua TJ, et al. Priapism: pathogenesis, epidemiology, and management. J Sex Med. 2010;7:476-500.http://www.ncbi.nlm.nih.gov/pubmed/20092449?tool=bestpractice.com巴氯芬在中枢神经系统发挥作用,同时也可以作用于球海绵体肌和坐骨海绵体肌。[53]van Driel MF, Beck JJ, Elzevier HW, et al. The treatment of sleep-related painful erections. J Sex Med. 2008;5:909-918.http://www.ncbi.nlm.nih.gov/pubmed/17971102?tool=bestpractice.com
磷酸二酯酶5抑制剂
常用的口服磷酸二酯酶5抑制剂如西地那非、他达那非常用于治疗勃起功能障碍,使阴茎恢复勃起功能,新的研究结果显示它在缓解复发性/间歇性阴茎异常勃起方面也有疗效。[54]Champion HC, Bivalacqua TJ, Takimoto E, et al. Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc Natl Acad Sci U S A. 2005;102:1661-1666.http://www.pnas.org/content/102/5/1661.longhttp://www.ncbi.nlm.nih.gov/pubmed/15668387?tool=bestpractice.com[30]Burnett AL. Pathophysiology of priapism: dysregulatory erection physiology thesis. J Urol. 2003;170:26-34.http://www.ncbi.nlm.nih.gov/pubmed/12796638?tool=bestpractice.com[49]Burnett AL, Bivalacqua TJ, Champion HC, et al. Long-term oral phosphodiesterase 5 inhibitor therapy alleviates recurrent priapism. Urology. 2006;67:1043-1048.http://www.ncbi.nlm.nih.gov/pubmed/16698365?tool=bestpractice.com[55]Burnett AL, Bivalacqua TJ, Champion HC, et al. Feasibility of the use of phosphodiesterase type 5 inhibitors in a pharmacologic prevention program for recurrent priapism. J Sex Med. 2006;3:1077-1084.http://www.ncbi.nlm.nih.gov/pubmed/17100941?tool=bestpractice.com[56]Bivalacqua TJ, Musicki B, Hsu LL, et al. Establishment of a transgenic sickle-cell mouse model to study the pathophysiology of priapism. J Sex Med. 2009;6:2494-2504.http://www.ncbi.nlm.nih.gov/pubmed/19523035?tool=bestpractice.com在一个小型病例系列中,每天使用 PDE5 抑制剂可以减少间歇性阴茎异常勃起男性患者的缺血性阴茎异常勃起发作次数。[55]Burnett AL, Bivalacqua TJ, Champion HC, et al. Feasibility of the use of phosphodiesterase type 5 inhibitors in a pharmacologic prevention program for recurrent priapism. J Sex Med. 2006;3:1077-1084.http://www.ncbi.nlm.nih.gov/pubmed/17100941?tool=bestpractice.com长期使用PDE5抑制剂可以减少特发性阴茎异常勃起的发作,也可以治疗由镰状细胞病引起的阴茎异常勃起,且不影响勃起功能。[49]Burnett AL, Bivalacqua TJ, Champion HC, et al. Long-term oral phosphodiesterase 5 inhibitor therapy alleviates recurrent priapism. Urology. 2006;67:1043-1048.http://www.ncbi.nlm.nih.gov/pubmed/16698365?tool=bestpractice.com[32]Bivalacqua TJ, Musicki B, Champion HC, et al. Phosphodiesterase type 5 inhibitor therapy for priapism. In: Carson CC III, Kirby RS, Goldstein R, et al, eds. Textbook of erectile dysfunction. 2nd ed. New York, NY: Informa Healthcare; 2009:428-433.在这些研究中,初始口服西地那非的剂量为每天25mg,逐渐增至每天50mg,他达那非的剂量为每天5-10mg,一周3次,都取得了不错的效果。PDE5抑制剂应该在阴茎疲软时开始使用,而不应该在勃起发作时使用。疗效通常在2到4周可以体现。[3]Broderick GA, Kadioglu A, Bivalacqua TJ, et al. Priapism: pathogenesis, epidemiology, and management. J Sex Med. 2010;7:476-500.http://www.ncbi.nlm.nih.gov/pubmed/20092449?tool=bestpractice.com在PDE5抑制剂的说明书上目前提示此药治疗间歇性缺血性阴茎异常勃起属于禁忌,所以目前此药在阴茎异常勃起的治疗方向仍处在探索阶段。[1]Montague DK, Jarow J, Broderick GA, et al; American Urological Association. Guideline on the management of priapism. 2003. http://www.auanet.org (last accessed 22 April 2016).http://www.auanet.org/common/pdf/education/clinical-guidance/Priapism.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/14501756?tool=bestpractice.com
酮康唑联合泼尼松
它是一种对于未发育成熟和希望保留生育功能患者的有效治疗方法。酮康唑是一种经典的抗真菌药,它还有一个作用是可以减少睾酮水平。[57]Yuan J, Desouza R, Westney OL, Wang R. Insights of priapism mechanism and rationale treatment for recurrent priapism. Asian J Androl. 2008;10:88-101.http://www.asiaandro.com/archive/1008-682X/10/88.htmhttp://www.ncbi.nlm.nih.gov/pubmed/18087648?tool=bestpractice.com[58]DeCastro BJ, Costabile RA, McMann LP, Peterson AC. Oral ketoconazole for prevention of postoperative penile erection: a placebo controlled, randomized, double-blind trial. J Urol. 2008;179:1930-2.http://www.ncbi.nlm.nih.gov/pubmed/18353393?tool=bestpractice.com[59]Evans KC, Peterson AC, Ruiz HE, Costabile RA. Use of oral ketoconazole to prevent postoperative erections following penile surgery. Int J Impot Res. 2004;16:346-9.http://www.ncbi.nlm.nih.gov/pubmed/14973533?tool=bestpractice.com正是这种副作用让它能够治疗转移性前列腺癌,预防术后勃起,治疗库欣病,同样也可以治疗复发性阴茎异常勃起。[57]Yuan J, Desouza R, Westney OL, Wang R. Insights of priapism mechanism and rationale treatment for recurrent priapism. Asian J Androl. 2008;10:88-101.http://www.asiaandro.com/archive/1008-682X/10/88.htmhttp://www.ncbi.nlm.nih.gov/pubmed/18087648?tool=bestpractice.com[58]DeCastro BJ, Costabile RA, McMann LP, Peterson AC. Oral ketoconazole for prevention of postoperative penile erection: a placebo controlled, randomized, double-blind trial. J Urol. 2008;179:1930-2.http://www.ncbi.nlm.nih.gov/pubmed/18353393?tool=bestpractice.com[59]Evans KC, Peterson AC, Ruiz HE, Costabile RA. Use of oral ketoconazole to prevent postoperative erections following penile surgery. Int J Impot Res. 2004;16:346-9.http://www.ncbi.nlm.nih.gov/pubmed/14973533?tool=bestpractice.com应对患者严密监测,泼尼松与酮康唑同时给药,因为酮康唑能够阻断肾上腺类固醇的产生。在一个小样本研究中,作者进行了1.5年的随访,发现酮康唑联合泼尼松可以在保留性功能的同时预防阴茎异常勃起的发作。[60]Abern MR, Levine LA. Ketoconazole and prednisone to prevent recurrent ischemic priapism. J Urol. 2009;182:1401-6.http://www.ncbi.nlm.nih.gov/pubmed/19683289?tool=bestpractice.com这些研究结果均显示患者可以耐受这种治疗,并且效价比较高,但是这种治疗方式要想得到推广,仍需进行随机对照研究。[60]Abern MR, Levine LA. Ketoconazole and prednisone to prevent recurrent ischemic priapism. J Urol. 2009;182:1401-6.http://www.ncbi.nlm.nih.gov/pubmed/19683289?tool=bestpractice.com酮康唑可以引起严重肝损伤和肾上腺功能不全。在2013年7月,欧洲药物管理委员会的人用药品委员会不建议口服酮康唑治疗真菌感染,因为它的风险比获益要大。因此,目前在一些国家口服酮康唑已经禁止或受限。此推荐不适用于其他疾病。[46]Medicines and Healthcare Products Regulatory Agency. Press release: oral ketoconazole-containing medicines should no longer be used for fungal infections. July 2013. http://www.mhra.gov.uk/ (last accessed 22 April 2016).http://webarchive.nationalarchives.gov.uk/20141205150130/http://www.mhra.gov.uk/NewsCentre/Pressreleases/CON297530[47]European Medicines Agency. European Medicines Agency recommends suspension of marketing authorisations for oral ketoconazole. July 2013. http://www.ema.europa.eu/ema/ (last accessed 22 April 2016).http://www.ema.europa.eu/ema/index.jsp?curl=pages/news_and_events/news/2013/07/news_detail_001855.jsp&mid=WC0b01ac058004d5c1酮康唑在合并肝病的患者中禁用。如果使用此药,治疗前和治疗期间需严密监测肝功能和肾上腺功能。