A 47-year-old woman was admitted to the hospital for evaluation of proteinuria. Bilateral sensorineural deafness had been diagnosed previously at age 35. She had a positive family history of deafness going back three generations. A renal biopsy showed the presence of highly organized fibrillary deposits in the subendothelial mesangial areas. The microfibrils had a diameter of 11-16 nm, larger than the diameter of amyloid fibrils, and did not have a microtubular appearance. The renal specimen was negative for Congo-red staining. There was no clinical or serologic evidence of paraproteinaemia, cryoglobulinaemia, light-chain disease, or systemic lupus erythematosus. The diagnosis was fibrillary glomerulonephritis. This is the first known case of fibrillary glomerulonephritis in a patient with familial sensorineural deafness.
|Number of pages||4|
|Publication status||Published - Jan 1 1997|
- Electron microscopy
- Familial sensorineural deafness
- Fibrillary glomerulonephritis
- Renal biopsy
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