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淀粉样变性病27例临床分析 |
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李航 李学旺
摘 要：目的 探讨淀粉样变性病的临床特征。方法 对1980～1997年住院的27例活检后刚果红染色确诊为淀粉样变性病病人的临床表现及实验室检查进行分析总结。结果 原发性者14例，以男性为主，继发性者3例。心、肾、消化及神经系统是常见的受累脏器。四肢麻木、体位性低血压、原因不明的大量蛋白尿和顽固性心衰及肝大、舌大等是常见的临床症状和体征。病因治疗效果差。家族性遗传性淀粉样变性病2例，均以神经系统受累症状为主。局限性淀粉样变8例，分别累及皮肤、支气管、鼻咽部等，症状局限，病程长，预后好。结论 淀粉样变既可累及全身多脏器也可局限性累及单个脏器。原发性者病情复杂，误诊率高，预后差，值得临床医生加以重视。直肠粘膜、齿龈及骨髓等部位活检及刚果红染色有助诊断。
关键词：淀粉样变性病 活检
Clinic Study of Amyloidosis (A report of 27 cases)
Li Hang
(Dirision of Nephrology,Peking Union Hospital,Beijing 100730)
Li Xuewang
(Dirision of Nephrology,Peking Union Hospital,Beijing 100730)
Abstract：Study the clinical features of amyloidosis, 27 cases of amyloidosis (Primary amyloidosis 14 cases,Secondary amyloidosis 3 cases, Familial amyloidosis 3 cases, Localized amyloidosis 8 cases) were analyzed. The cardiac,renal, digest and nervous system are more likely to be affected in the primary amyloidosis.Limb numbness,orthostatic hypotesion,unexplained massive proteinuria,congestive heart failrue and hepatomagely,macroglossia were prominent features in most of primary amyloidosis with a poor prognosis.Two cases of familial amyloidosis were both neuropathic type. Localized amyloidosis only involve single organ such as skin, bronchus,nasopharynx without evidence of systemic disease.Amyloidosis can occur either in local forms,limited to particular organs and tissues,or systemically distributed throughout the body with a diversity of clincal manifestations.The clinicans should be alert to amyloidosis for it is a disease which frequently be misdiagnosied.The current treatment for amyloidosis is unsatisfactory.Biopsy of recuum,gingiva,bone marrow is reliable method for the diagnosis of amyloidosis.
Key words：Amyloidosis Biopsy▲
淀粉样变性病(amyloidosis)是一种以“淀粉样变”的无定形、嗜酸性物质在心脏、肾脏、消化道等脏器的细胞间浸润沉积所引起的疾病。淀粉样物质既可浸润全身多个脏器，也可仅局限性地浸润单个脏器，每一脏器浸润程度不同，其临床症状多种多样，误诊率高［1］。本文总结了我院确诊此病患者的临床特点、治疗及预后的情况，现报告如下。
对象与方法
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