[Chronic obstructive lung disease and alpha-2-macroglobulin deficiency in serum--case report].
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".
Krüger
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Reha-Klinik der BfA, Utersum auf Föhr.
D000328:Adult; D001797:Blood Protein Electrophoresis; D003131:Combined Modality Therapy; D006801:Humans; D008173:Lung Diseases, Obstructive / Q000097:blood* / Q000534:rehabilitation; D008297:Male; D011656:Pulmonary Emphysema / Q000097:blood / Q000534:rehabilitation; D000511:alpha-Macroglobulins / Q000172:deficiency*
D000511:alpha-Macroglobulins