Publication

Glomerulopathy associated with predominant fibronectin deposits: a newly recognized hereditary disease

Journal Paper/Review - Jul 1, 1995

Units
PubMed

Citation
Strøm E, Riess R, Neuweiler J, Gloor F, Monga G, Mazzucco G, Abt A, Krapf R, Banfi G, Stosiek P. Glomerulopathy associated with predominant fibronectin deposits: a newly recognized hereditary disease. Kidney international 1995; 48:163-70.
Type
Journal Paper/Review (English)
Journal
Kidney international 1995; 48
Publication Date
Jul 1, 1995
Issn Print
0085-2538
Pages
163-70
Brief description/objective

A newly recognized type of familial glomerulopathy observed in patients of both sexes in six families is reported. Proteinuria, often within the nephrotic range, microscopic hematuria, hypertension and a slowly decreasing renal function over several years were common. No underlying systemic diseases were identified. Generally, light microscopy showed enlarged glomeruli with minimal hypercellularity and with extensive deposits in the mesangium and subendothelial space. By electron microscopy, granular deposits with some admixture of fibrils were most common. In one family, the deposits were predominantly fibrillary. Immunoglobulins and complement factors were inconstant or lacking. A main finding was a strong immune reactivity to fibronectin, corresponding to the distribution of the deposits. In one patient, the deposits recurred in a renal transplant. There was no indication of systemic deposition. Abnormalities in the metabolism of circulating fibronectin may play a pathogenetic role in this disease of probably autosomal dominant inheritance.