title: Glomerulonephritis as late manifestation of severe alpha 1-antitrypsin deficiency.
abstract: An association of alpha 1-antitrypsin deficiency with glomerulonephritis is rare and has so far been observed only in children or young adults. We report a 63-year-old man with severe alpha 1-antitrypsin deficiency associated with pulmonary emphysema, cirrhosis of the liver, and mesangioproliferative glomerulonephritis with nephrotic syndrome. Following initial presentation with nephropathy, further work-up revealed alpha 1-antitrypsin deficiency of proteinase inhibitor Z. In the absence of glomerular alpha 1-antitrypsin deposits the relationship between renal disease and alpha 1-antitrypsin deficiency remains unclear. alpha 1-Antitrypsin deficiency should be considered in adults with abnormal renal function and chronic liver disease.
authors.affiliation: Medizinische Universitätsklinik, Karl-Franzens Universität, Graz, Austria.
authors.forename: R E
authors.initials: RE
authors.lastname: Stauber
authors.forename: J H
authors.initials: JH
authors.lastname: Horina
authors.forename: M
authors.initials: M
authors.lastname: Trauner
authors.forename: G J
authors.initials: GJ
authors.lastname: Krejs
authors.forename: M
authors.initials: M
authors.lastname: Ratschek
authors.forename: M
authors.initials: M
authors.lastname: Klimpfinger
mesh_terms: D017809:Fatal Outcome; D005922:Glomerulonephritis, IGA / Q000209:etiology*; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology; D008173:Lung Diseases, Obstructive / Q000150:complications; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D011656:Pulmonary Emphysema / Q000209:etiology; D012907:Smoking / Q000009:adverse effects; D019896:alpha 1-Antitrypsin Deficiency