Familial glomerulopathy with giant fibrillar deposits

Proteinuria and microhaematuria were observed in three siblings and one first-degree cousin. Histological examination of three kidney biopsies and one autopsy specimen shows the same diffuse glomerular lesions in all patients, characterized by mainly subendothelial but frequently transmembranous and...

Full description

Saved in:
Bibliographic Details
Published in:Virchows Archiv A Pathological Anatomy and Histology 1980-01, Vol.388 (3), p.313-326
Main Authors: Bürgin, M, Hofmann, E, Reutter, F W, Gürtler, B A, Matter, L, Briner, J, Gloor, F
Format: Article
Language:English
Subjects:
Adult
Autopsy
Biopsy
Female
Fluorescent Antibody Technique
Glomerulonephritis - complications
Glomerulonephritis - genetics
Glomerulonephritis - pathology
Hematuria - complications
Humans
Kidney - pathology
Kidney Glomerulus - ultrastructure
Male
Microscopy, Electron
Pedigree
Proteinuria - complications
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Proteinuria and microhaematuria were observed in three siblings and one first-degree cousin. Histological examination of three kidney biopsies and one autopsy specimen shows the same diffuse glomerular lesions in all patients, characterized by mainly subendothelial but frequently transmembranous and mesangial deposits of a unique fibrillar structure, visible by electron microscopy. Examination by immunfluorescence gave inconstant findings. No serological abnormalities could be established. To our knowledge, such a pecular form of familial glomerulopathy has not been described so far.
ISSN:0340-1227
1432-2307
DOI:10.1007/bf00430861