α1-Antitrypsin: more than just deficiency

Stockley, R. A.
May 2004
Thorax;May2004, Vol. 59 Issue 5, p363
Academic Journal
Abnormal levels of α 1-antitrypsin represent a syndrome of clinical disease entities, some relating to a deficiency while others reflect an overload. With growing interest in the genetic nature of diseases and the clinical disorders associated with antitrypsin abnormalities, many countries developed national registries. This has resulted in the discovery of other clinical conditions that are more frequent in individuals with Pi ZZ antitrypsin, including vasculitis, Wegner's granulomatosis, glomerulonephritis and panniculitis. Thus the condition is associated with many clinical disease entities and is more representative of a syndrome.


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