Alpha 1-antitrypsin deficiency presenting as cryptogenic cirrhosis in adults over 50

J Clin Gastroenterol. 1985 Oct;7(5):405-8. doi: 10.1097/00004836-198510000-00006.

Abstract

We report cirrhosis and alpha 1-antitrypsin deficiency in two patients over 50 years of age who had an initial diagnosis of cryptogenic cirrhosis. Serum alpha 1-antitrypsin levels were in the homozygous range and a liver biopsy demonstrated cirrhosis with periodic acid-Schiff-positive, diastase-resistant globules in both. Red intracytoplasmic inclusion globules seen on trichrome staining in the first patient, and a decreased alpha-1 fraction on serum protein electrophoresis in the second suggested the diagnosis. We propose that the diagnosis of alpha 1-antitrypsin deficiency be considered in all patients, regardless of age, and that trichrome staining be part of the routine histologic evaluation in all patients with cirrhosis of uncertain etiology.

Publication types

  • Case Reports

MeSH terms

  • Age Factors
  • Aged
  • Azo Compounds*
  • Coloring Agents
  • Diagnosis, Differential
  • Eosine Yellowish-(YS)*
  • Humans
  • Immunoenzyme Techniques
  • Liver Cirrhosis / diagnosis*
  • Liver Cirrhosis / etiology
  • Liver Cirrhosis / pathology
  • Male
  • Methyl Green*
  • Middle Aged
  • alpha 1-Antitrypsin Deficiency*

Substances

  • Azo Compounds
  • Coloring Agents
  • trichrome stain
  • Methyl Green
  • Eosine Yellowish-(YS)