Clinical and morphological features of fibronectin glomerulopathy: a report of ten patients from a single institution

Fibronectin glomerulopathy complicated with persistent cloaca and congenital esophageal atresia: a case report and literature review

“…Seventy-five cases of fibronectin glomerulopathy have been reported, so far with full clinical data (Table 1 ) [ 1 , 2 , 5 – 8 , 15 – 22 ]. Proteinuria was recorded in 69 cases, of which 35 had nephrotic-range proteinuria (>3.5 g/24 h).…”

“…There have been numerous reports of cases with detailed family histories, but few reports of sporadic cases, such as the present patient [ 5 , 19 , 20 , 22 ]. Eighteen of the 75 reported cases (24%) had no apparent family history of renal diseases.…”

Hypertension and proteinuria—the needle in the haystack?: Answers

“…In a recent series, 10 cases were identified from among 35,088 kidney biopsies performed from 2003 to 2012 [1]. Fibronectin is an extracellular protein involved in cell-matrix interactions and exists in two forms: a cellular form produced by mesangial cells and a circulating form produced by the liver.…”

“…The characteristic biopsy findings, illustrated by this case, include: marked eosinophilic and PAS positive mesangial and subendothelial deposits with glomerulomegaly and lobulation of tufts, negative Jones and Congo Red stains, and granular electron-dense mesangial and subendothelial deposits on EM, which may appear fibrillary. Progression to end-stage kidney disease within 15-20 years is frequent [1,2,5,6]. Recurrence after renal transplantation has been reported [6,7].…”

Fibronectin Glomerulopathy

“…Electron microscopy indicated focal fibrils or fibrillar subendothelial deposits with 12–16 nm fibrils, and immunoelectron microscopy confirmed that colloidal gold particles marked fibronectin assembled in the electron-dense deposits in the mesangial matrix and along the glomerular capillary loops. [ 3 ]…”