THE BETA-1C GLOBULIN IN CHILDHOOD NEPHROTIC SYNDROME: LABORATORY DIAGNOSIS OF PROGRESSIVE GLOMERULONEPHRITIS.
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Biomedical subjects
Publications and source records attributed to F S ROSEN.
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Serums of patients with hereditary angioneurotic edema lack inhibitory activity against the esterase derived from the first component of complement. In one group of patients this lack appears to result from failure to synthesize the esterase inhibitor of the first component of complement, whereas in another group of patients an abnormal, nonfunctional protein is synthesized.
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"Natural" antibodies to representative species of Enterobacteriaceae were found to be principally associated with the gamma(1)-macroglobulin prepared from Cohn fraction III-I of pooled human plasma. The amount of antibodies present was estimated by measuring the bactericidal, hemagglutinating, mouse-protective, and opsonophagocytic activities of this fraction. Comparison with the antibody activity of gamma(2)-globulin of Cohn fraction II showed that up to 100 times more antibody activity on a weight basis was present in the macroglobulin fraction. These findings suggest that differences between natural and immune antibody result from the physicochemical properties of 2 different classes of immune globulin.
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Lymph nodes and splenic tissue from patients with congenital agammaglobulinemia and dysgammaglobulinemia and from normal subjects were studied with the use of immunofluorescence and histochemical stains to determine the site of synthesis of the 19S gamma(1)-globulins. The two patients with dysgammaglobulinemia had high serum concentrations of the 19S gamma(1)-globulins and a marked deficit of the 7S gamma-globulins. These patients, as well as agammaglobulinemic children, had only rare or no plasma cells in their tissues. Cells were identified in sections of spleen from a dysgammaglobulinemic child as well as from normal individuals which exhibited specific fluorescence with an anti-19S gamma-globulin antiserum adsorbed with 7S gamma(2)-globulins and which stained positively with PAS and methyl green pyronine. These cells resembled the transitional cells described by Fagraeus.
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