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[慢性阻塞性肺疾病与血清α-2-巨球蛋白缺乏——病例报告]

[Chronic obstructive lung disease and alpha-2-macroglobulin deficiency in serum--case report].

作者信息

Krüger U

机构信息

Reha-Klinik der BfA, Utersum auf Föhr.

出版信息

Pneumologie. 1993 Sep;47(9):531-4.

PMID:7694273
Abstract

The protease-antiprotease concept has contributed substantially to the pathogenetic understanding of different internistic disease patterns including the generalised, histologically mostly panlobular pulmonary emphysema. Attention is presently focused on alpha-1-antitrypsin protease inhibitor deficiency for which substitute solutions are already available. We present a case report on a patient of 40 years of age suffering from a severe, basally located pulmonary emphysema (as was evident on x-ray examination) with respiratory global insufficiency and an obstructive component that was only partially reversible under medication. Laboratory chemistry revealed that this was associated with an alpha-2-macroglobulin deficiency. Possibly the deficiency in this protease inhibitor of low specificity but broad spectrum of action may contribute to a better understanding of some of the types of emphysema that had so far been subclassified as cryptogenic although bearing traits of "proteinase inhibitor deficiency".

摘要

蛋白酶 - 抗蛋白酶概念对包括广泛性、组织学上多为全小叶型肺气肿在内的不同内科疾病模式的发病机制理解做出了重大贡献。目前的注意力集中在α-1-抗胰蛋白酶蛋白酶抑制剂缺乏症上,对此已有替代解决方案。我们报告一例40岁患者,患有严重的、位于肺底部的肺气肿(X线检查显示明显),伴有呼吸功能整体不全以及阻塞性成分,药物治疗下仅部分可逆。实验室化学检查显示这与α-2-巨球蛋白缺乏有关。这种低特异性但作用谱广泛的蛋白酶抑制剂缺乏可能有助于更好地理解一些迄今为止虽具有“蛋白酶抑制剂缺乏”特征但被归类为隐源性的肺气肿类型。

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