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Journal: NAUKA MOLODYKH (Eruditio Juvenium) (Vol.6, No. 3)

Publication Date:

Authors : ;

Page : 405-413

Keywords : Goodpasture's syndrome; rapidly progressing glomerulonephritis; antibodies to the basement membrane; clinical and morphological comparison; transmission electron microscopy; ultrastructure.;

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Goodpasture's syndrome first described by Goodpasture in 1919, is a combination of glomerulonephritis and alveolar bleeding in the presence of anti-GVM antibodies. The basis of the pathogenesis of Goodpasture's syndrome is formation of autoantibodies to the basement membrane of renal glomerular capillaries and alveoli. These antibodies belong to IgG class, they bind with antibodies of the basement membranes in the presence of C3 complement component, with subsequent development of immune inflammation of kidneys and lung alveoli. Goodpasture's syndrome most often develops in people with hereditary predisposition, but probable additional factors are viral infections of the respiratory tract. The results of a clinical observation of a patient of 8 years of age with Goodpasture's syndrome, started with a rapidly progressive glomerulonephritis type I, are presented. In the anamnesis there are many episodes of ARVI. The disease began with fever, followed by vomiting, loose stools, epistaxis, decreased diuresis up to complete anuria. Morphological changes at the terminal stage of autoimmune renal damage are described: thickening of glomerular basement membrane, its fragmentation, inflammatory infiltration around glomeruli, periglomerular sclerosis, dystrophic changes in tubular epithelium, linear deposition of IgG and C3 complement component along glomerular basal membranes. Ultrastructural changes in the basement glomerular membrane have been identified – sclerotic changes, thickening and, in some cases, fragmentation in all glomeruli of the preparation. The basement membrane of the glomeruli stratified and thickened due to deposition of antibodies and production of collagen fibers. Subendothelial deposits in the form of «humps» are detected, which are immune complexes and correspond to the IgG and C3 deposits found in the IF examination. Probable causes of pathology are discussed, and a differential series is constructed.

Last modified: 2019-01-09 17:27:00