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诊断难题:一名36岁α-1抗胰蛋白酶缺乏症女性的病例研究

A diagnostic dilemma: case study of a 36-year-old female with alpha-1-antitrypsin deficiency.

作者信息

Lawrence Remi A, Clark James P

机构信息

Dept. of Pediatrics, West Virginia University School of Medicine, Charleston Division, USA.

出版信息

W V Med J. 2005 Mar-Apr;101(2):73-4.

Abstract

Alpha-1-antitrypsin deficiency is a genetic disorder that presents as early-onset emphysema in its most severe form. A high index of clinical suspicion is needed in cases of lung or liver disease of unknown etiology or a suggestive history and physical examination. Low or absent serum levels of alpha-1-antitrypsin levels identify persons with the disease and phenotyping is the confirmatory test. The main goal of management is attempting to prevent or slowing the progression of damage to the lungs. Medical and surgical options for treatment include augmentation therapy that maintains protective levels of AAT in the lung and serum and lung transplantation may be necessary in severe cases. We present this case report of a patient with Alpha-1-antitrypsin deficiency in order to increase awareness of this condition since early diagnosis improves long-term prognosis and reduces the overall cost of therapy.

摘要

α-1抗胰蛋白酶缺乏症是一种遗传性疾病,最严重的形式表现为早发性肺气肿。对于病因不明的肺部或肝脏疾病患者,或有提示性病史及体格检查的患者,需要高度的临床怀疑。血清α-1抗胰蛋白酶水平低或缺乏可识别患有该疾病的人,表型分析是确诊试验。治疗的主要目标是试图预防或减缓肺部损伤的进展。治疗的医学和手术选择包括增强疗法,即维持肺部和血清中AAT的保护水平,严重病例可能需要进行肺移植。我们报告了这例α-1抗胰蛋白酶缺乏症患者的病例,以提高对这种疾病的认识,因为早期诊断可改善长期预后并降低总体治疗成本。

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