MF81

ICD-11 MMS 2026-01

Fibronectin glomerulopathy

Description

Fibronectin glomerulopathy is a rare hereditary kidney disease in which fibronectin (FN1) deposits are seen in the mesangium and subendothelial space. The clinical picture is characterised by proteinuria, type IV renal tubular acidosis, microscopic haematuria and hypertension that may lead to end-stage renal failure in the second to sixth decade of life. This disease may be associated with mutations in the FN1 gene.

Index terms

  • Fibronectin glomerulopathy

Exclusions from above levels

  • 19 – Certain conditions originating in the perinatal period
  • JA66 – Clinical findings on antenatal screening of mother

Sibling codes

  • MF80 – Diffuse mesangial sclerosis
  • MF82 – Lipoprotein glomerulopathy
  • MF83 – Diabetic glomerular changes
  • MF84 – Pauci-immune proliferative glomerulonephritis
  • MF85 – Anti-glomerular basement membrane antibody mediated disease
  • MF8Y – Other specified clinical findings in specimens from the urinary system
  • MF8Z – Clinical findings in specimens from the urinary system, unspecified

Foundation entity

Fibronectin glomerulopathy – http://id.who.int/icd/entity/1877494378

Part of: 21: Symptoms, signs or clinical findings, not elsewhere classified