Donnelly Joseph P, Hanna Mazen, Sperry Brett W, Seitz William H
Department of Cardiovascular Medicine, Heart and Vascular Institute, Cleveland Clinic, Beachwood, OH.
Department of Cardiovascular Medicine, Heart and Vascular Institute, Cleveland Clinic, Beachwood, OH; Mid America Heart Institute, Saint Luke's Hospital of Kansas City, Kansas City, MO.
J Hand Surg Am. 2019 Oct;44(10):868-876. doi: 10.1016/j.jhsa.2019.06.016. Epub 2019 Aug 7.
Carpal tunnel syndrome (CTS) can be caused by the deposition and accumulation of misfolded proteins called amyloid and is often an early manifestation of systemic amyloidosis. In patients undergoing surgery for idiopathic CTS, a recent study identified amyloidosis by tenosynovial biopsy in 10.2% of men older than 50 years and women older than 60 years; all positive patients had bilateral symptoms. These findings have led to a renewed interest in amyloidosis as an etiology of CTS. The 2 most common systemic amyloidoses, immunoglobulin light chain and transthyretin amyloidosis, affect the heart, nerves, and other organ systems throughout the body including the soft tissues. Patients with cardiac involvement of amyloidosis have an especially poor prognosis if the disease remains unrecognized and untreated. Early diagnosis is paramount, and patients classically present with cardiac disease several years after being operated on by a hand surgeon for carpal tunnel release. Herein, we present a review of amyloidosis as it pertains to CTS and an algorithm for the detection of amyloidosis in patients undergoing carpal tunnel release. Implementation of this straightforward algorithm will allow for early diagnosis of amyloidosis, a group of progressive and lethal diseases.
腕管综合征（CTS）可由称为淀粉样蛋白的错误折叠蛋白的沉积和积累引起，且常常是全身性淀粉样变性的早期表现。在接受特发性CTS手术的患者中，一项近期研究通过腱鞘活检在50岁以上男性和60岁以上女性中发现淀粉样变性的比例为10.2%；所有阳性患者均有双侧症状。这些发现使得人们对淀粉样变性作为CTS的病因重新产生兴趣。两种最常见的全身性淀粉样变性，即免疫球蛋白轻链淀粉样变性和转甲状腺素蛋白淀粉样变性，会影响心脏、神经以及包括软组织在内的全身其他器官系统。淀粉样变性累及心脏的患者，如果疾病未被识别和治疗，预后会特别差。早期诊断至关重要，患者通常在手外科医生为其进行腕管松解手术后数年才出现心脏疾病。在此，我们对与CTS相关的淀粉样变性进行综述，并给出在接受腕管松解手术的患者中检测淀粉样变性的算法。实施这种简单直接的算法将有助于早期诊断淀粉样变性，这是一组进行性和致死性疾病。
