Abstract
GBM of patients with Alport Syndrome(AS) does not bind anti-GBM (McCoy et al, Lab Invest 3:19, 1976). Diagnostic use of staining kidney biopsies of AS suspect children with antiGBM was evaluated. Kidneys of 21 autopsied patients without renal disease (Groups I-III) and 3 patients with AS (with characteristic electron microscopic lesions) were treated with serum of a patient with strong antiGBM, then with fluorochromed anti IgG. Results were as follows:
Young infants, like AS patients, appear to lack GBM antigen(s) normally present in older children and adults. Normal GBM increases from 950 Å at birth to 2844 Å by 3 years. Whether adult components are superimposed on fetal GBM or fetal GBM is replaced by adult GBM components is unknown. Failure of GBM to bind antiGBM is not diagnostic of AS in young infants, but is useful in older patients. These data may also partly explain absence of antiGBM nephritis in young children.
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Anand, S., Landing, B., Lieberman, E. et al. CHANGES IN GLOMERULAR BASEMENT MEMBRANE(GBM) ANTIGEN(S) WITH AGE AND DISEASE. Pediatr Res 11, 546 (1977). https://doi.org/10.1203/00006450-197704000-01055
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DOI: https://doi.org/10.1203/00006450-197704000-01055