Department of Urology, University of Rennes, Service d'Urologie, 2 Rue Henri Le Guilloux, 35000, Rennes, France.
Department of NeuroUrology, Tenon Hospital, Paris, France.
Clin Auton Res. 2019 Sep;29(Suppl 1):65-74. doi: 10.1007/s10286-019-00627-7. Epub 2019 Aug 26.
We aimed to review the current knowledge on the epidemiology, diagnosis, and management of urinary and sexual dysfunction in patients with TTR amyloidosis (ATTR).
We performed a review of the literature, screening for randomized controlled trials, prospective and retrospective series, position papers, and guidelines on urinary and sexual dysfunction in ATTR patients published in PubMed and Embase.
Lower urinary tract dysfunction is present in up to 83% of patients with ATTR. Voiding symptoms are the most common, reported in 34.8-87.5% of patients, while urinary tract infections are reported in up to 50%. Urinary incontinence is observed in 16.7-37.5% of the ATTR population, mostly due to decreased urethral resistance. Sexual dysfunction affects over 40% of ATTR patients, with erectile dysfunction and sexual arousal disorder being the most common symptoms in male and female patients, respectively. In addition to a thorough clinical examination, invasive pressure-flow urodynamic testing is a cornerstone in the assessment of ATTR lower urinary tract dysfunction. The most common finding is detrusor underactivity and intrinsic sphincter deficiency. Poor bladder compliance can also be observed in patients, due to amyloid deposits on the bladder wall. Urinary tract imaging may be of interest to rule out upper urinary tract deterioration. Given the paucity of data in the ATTR population, treatment should be tailored to the individual patient.
Urinary and sexual dysfunction are highly prevalent in ATTR patients. Comprehensive assessment and multidisciplinary management are keys to avoiding upper urinary tract damage and improving patients' quality of life.
我们旨在回顾目前关于转甲状腺素蛋白淀粉样变性病（ATTR）患者泌尿和性功能障碍的流行病学、诊断和管理的知识。
我们对文献进行了回顾，筛选了在 PubMed 和 Embase 上发表的关于 ATTR 患者泌尿和性功能障碍的随机对照试验、前瞻性和回顾性系列研究、立场文件和指南。
下尿路功能障碍在多达 83%的 ATTR 患者中存在。排尿症状最为常见，报告发生率为 34.8-87.5%，而尿路感染发生率高达 50%。尿失禁在 16.7-37.5%的 ATTR 人群中观察到，主要是由于尿道阻力降低。超过 40%的 ATTR 患者存在性功能障碍，勃起功能障碍和性唤起障碍分别是男性和女性患者中最常见的症状。除了彻底的临床检查外，侵入性压力-流量尿动力学测试是评估 ATTR 下尿路功能障碍的基石。最常见的发现是逼尿肌活动低下和内括约肌缺陷。由于膀胱壁上的淀粉样沉积，患者的膀胱顺应性也可能较差。尿路影像学检查可能有助于排除上尿路恶化。鉴于 ATTR 人群中的数据有限，治疗应根据个体患者进行调整。
泌尿和性功能障碍在 ATTR 患者中非常普遍。全面评估和多学科管理是避免上尿路损伤和提高患者生活质量的关键。
