Abstract: Fibronect  in glomerular disease (FNG) is a rare autosomal dominant  in herited disease characterized by a large amount of fibronectin deposited in the glomerular subendothelial and mesangium.  The clinical manifestations are proteinuria, a different level of hematuria, hypertension, and slowly progressing renal dysfunction.And a small number of patients have renal tubular acidosis. The diagnosis is mainly based on the results of renal puncture, and the fibronectin staining is positive.There is no specific treatment, and more symptomatic treatment should be used. FNG is rare, and the complication, monoclonal immunoglobulinemia (MGRS) with renal significance, is rarely reported. This article reported a case of FNG accompanied by MGRS and literature were reviewed.

Key words: connexins, blood protein disorders, biopsy, needle