Premium
Fibrillary glomerulonephritis in a patient with familial sensorneural deafness
Author(s) -
ICHIKAWA Haruo,
IKEDA Shuji,
HASHIMOTO Masami,
NAGAKE Yoshio,
HIRONAKA Kazue,
SHIKATA Kenichi,
MAKINO Hirofumi
Publication year - 1997
Publication title -
nephrology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.752
H-Index - 61
eISSN - 1440-1797
pISSN - 1320-5358
DOI - 10.1111/j.1440-1797.1997.tb00259.x
Subject(s) - medicine , renal biopsy , proteinuria , pathology , glomerulonephritis , amyloidosis , serology , biopsy , dermatology , immunology , kidney , antibody
Summary: 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 Congored 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.