True about fibronectin nephropathy are all except:
Ans. (a) Autosomal recessive inheritance(Ref: Rosai 10th/pg 1153)Fibronectin glomerulopathyDefinitionCharacterized by massive fibronectin deposition in the glomeruli.Genetic basisCaused by mutations of FN1 gene on chromosome 2q34InheritanceHereditary autosomal dominant (option A) disease; Both sexes are equally affectedClinical featuresProteinuria (nephrotic range), microscopic hematuria & slow deterioration of renal function over a period of several years.Light microscopy* Glomeruli appear enlarged & lobulated with minimal degree of hypercellularity* Most characteristic feature: marked enlargement of mesangium & subendothelial (option D) space due to deposition of a homogeneous PAS +ve, Congo red -ve (option B) materialElectron microscopyDense granular appearance of the deposit in mesangium or subendotheliumImmunohisto-chemical studies* Strong positivity for fibronectin in the areas corresponding to the deposits.* Scanty immunoglobulins & complement factors may occasionally be seen, inconsistently. (option C)PrognosisPoor prognosisRecurrence after renal transplantThe disease has been reported to recur after renal transplantation.
Autosomal recessive inheritance
Glomerular enlargement with PAS+ trichrome+ mesangial deposit
Glomerulus do not consistently stain for Ig and complement
Ultrastructural feature is presence of large electron dense mesangialorsubendothelial deposit
Kidney