Article
alpha 1-antitrypsin deficiency and idiopathic pulmonary fibrosis in a family.
Southern medical journal - 1 Oct 1996
Kim H, Lepler L, Daniels A, Phillips Y
Abstract excerpt
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease of unknown etiology with an estimated prevalence of 3 to 5 per 100,000. We describe a patient with a family history of both IPF and alpha 1-antitrypsin (AAT) deficiency. The patient had a 6-month history of worsening dyspnea when first seen, and he later died of multisystem organ failure and recalcitrant hypoxemia. He had IPF and AAT deficiency,...
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