Abstract
Although renal involvement is common in LMBBS, glomerular ultrastructural (EM) changes have not been delineated. We have studied glomeruli from 3 patients with LMBBS and have found similar EM changes in the glomerular basement membranes (GBM). Each had retinopathy, obesity and polydactyly; 2 were retarded. Two had decreased renal function and hypertension; one had normal function and blood pressure; none had proteinuria. Intravenous urography revealed reduced concentration and distorted calyces in two cases. The third was normal.
Light microscopy revealed segmental increases in mesangial cells and matrix of all glomeruli; this ranged from mild proliferation to sclerosis. Marked changes in the GBM were seen by EM. There was diffuse effacement of the trilaminar architecture and segmental and irregular thickening alternating with thinner regions, and rarefaction. There were also accumulations of granular and fibrillary material in the inner third of the GBM. Spherical dense particles were also seen. In some areas the GBM was split and mesangium was interposed. No deposits were seen by EM or immunofluorescence.
These GBM changes were found in each case and were independent of the degree of uremia or hypertension; these changes may therefore be the primary or earliest glomerular abnormality seen in LMBBS.
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Price, D., Gartner, J. & Kaplan, B. ULTRASTRUCTURAL LESIONS OF THE GLOMERULAR BASEMENT MEMBRANE IN LAURENCE-MOON-BIEDL-BARDET SYNDROME (LMBBS). Pediatr Res 14, 984 (1980). https://doi.org/10.1203/00006450-198008000-00067
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DOI: https://doi.org/10.1203/00006450-198008000-00067