Location of Repository

Chronic active hepatitis in the type I polyglandular autoimmune syndrome.

By T. M. Michele, J. Fleckenstein, A. R. Sgrignoli and P. J. Thuluvath

Abstract

Type I polyglandular autoimmune syndrome is characterized by the triad of hypoparathyroidism, Addison's disease and chronic mucocutaneous candidiasis. Chronic active hepatitis has been associated with this syndrome but its incidence and severity have not been well documented. We describe a sibship of two patients with type I polyglandular autoimmune syndrome who presented with autoimmune chronic active hepatitis. The first patient presented to us with advanced disease and died despite an emergent liver transplant, while the second patient responded to steroids. Autoimmune chronic active hepatitis, a major cause of mortality in this syndrome, can present without symptoms or physical signs of liver disease. We suggest periodic screening of liver enzymes in subjects with this syndrome

Topics: Research Article
Publisher: BMJ Group
OAI identifier: oai:pubmedcentral.nih.gov:2397651
Provided by: PubMed Central
Download PDF:
Sorry, we are unable to provide the full text but you may find it at the following location(s):
  • http://www.pubmedcentral.nih.g... (external link)
  • Suggested articles


    To submit an update or takedown request for this paper, please submit an Update/Correction/Removal Request.