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The association of familial liver disease, subepidermal immunoproteins, and membranoproliferative glomerulonephritis.

Abstract
Herein we report a new familial form of hepatic disease. Each of the four patients had splenomegaly, hypersplenism, a small liver, biochemical evidence of hepatic excretory dysfunction and hepatocellular damage, kidneys without demonstrable cysts, and normal blood pressue. An evaluation of serum immunoproteins, autoantibodies, histocompatibility antigens, and mixed lymphocyte reactivity further defined the immunologic features of this syndrome. Extrahepatic manifestations included a papulosquamous dermatitis with deposition of immunoglobulins and complement in both normal and abnormal skin, a membranoproliferative glomerulonephritis with subendothelial deposits, arthritis, and pericardial, pleural, and synovial effusions.
AuthorsR S Dobrin, J R Hoyer, T E Nevins, H Sharp, W C Gentry, R L Vernier
JournalThe Journal of pediatrics (J Pediatr) Vol. 90 Issue 6 Pg. 901-9 (Jun 1977) ISSN: 0022-3476 [Print] United States
PMID870658 (Publication Type: Case Reports, Journal Article, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Autoantibodies
  • Histocompatibility Antigens
  • Immunoglobulin A
  • Immunoglobulin M
  • Immunoglobulins
Topics
  • Adolescent
  • Adult
  • Autoantibodies (analysis)
  • Child
  • Female
  • Glomerulonephritis (etiology, immunology, pathology)
  • Histocompatibility Antigens (analysis)
  • Humans
  • Immunoglobulin A (analysis)
  • Immunoglobulin M (analysis)
  • Immunoglobulins (analysis)
  • Kidney (pathology)
  • Liver Diseases (complications, congenital, genetics, immunology, pathology)
  • Male
  • Skin (pathology)

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