ICD-11 code : MF81;
Preferred Label : Fibronectin glomerulopathy;
ICD-11 definition : Fibronectin glomerulopathy is a rare hereditary kidney disease in which fibronectin
(FN1) deposits are seen in the mesangium and subendotheliel space. The clinical picture
is characterized by proteinuria, type IV renal tubular acidosis, microscopic hematuria
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.;
Origin ID : 1877494378;
Currated CISMeF NLP mapping
Fibronectin glomerulopathy is a rare hereditary kidney disease in which fibronectin
(FN1) deposits are seen in the mesangium and subendotheliel space. The clinical picture
is characterized by proteinuria, type IV renal tubular acidosis, microscopic hematuria
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.
