Unusual abnormalities in adolescent siblings with α1-antitrypsin deficiency

J. S. Wagener, R. E. Sobonya, L. M. Taussig, R. J. Lemen

Research output: Contribution to journalArticle

8 Scopus citations

Abstract

We studied, over a four-year period, two adolescents with α1-antitrypsin (AAT) deficiency who subsequently died from complications of hepatic cirrhosis. Serial pulmonary function studies indicated mild obstructive lung disease involving peripheral airways in both patients. Postmortem histologic and pulmonary morphometric studies indicated mild diffuse airspace and bronchial gland enlargement, and slight dilation of small airways. This airspace enlargement may represent the early stage of lung disease in AAT-deficient subjects and suggests that pulmonary anatomic changes may occur long before the onset of clinically and pathologically significant emphysema.

Original languageEnglish (US)
Pages (from-to)464-468
Number of pages5
JournalCHEST
Volume83
Issue number3
DOIs
StatePublished - Jan 1 1983

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ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

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