Open AccessFibronectin glomerulopathy - A sporadic case with unusual clinical manifestation
Author(s) -
Surya Narayan Mandal,
Sumita Shrivastava,
Rossella Piras,
Swarnalata Gowrishankar
Publication year - 2017
Publication title -
saudi journal of kidney diseases and transplantation/našrat amraḍ wa zira'aẗ al-kulaẗ
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.268
H-Index - 30
eISSN - 2320-3838
pISSN - 1319-2442
DOI - 10.4103/1319-2442.220860
Subject(s) - medicine , pathology , periodic acid–schiff stain , renal biopsy , eosinophilic , nephrocalcinosis , mesangium , gastroenterology , biopsy , immunohistochemistry , kidney , glomerulonephritis
A 22-year-old nondiabetic young Indian female presented with short history of dyspnea, anorexia, and bilateral leg swelling. Her laboratory evaluation showed severe anemia, serum creatinine of 11.89 mg/dL, nephrotic range proteinuria and microscopic hematuria with 6-8 red blood cell/high-power field. Renal biopsy showed brightly eosinophilic, periodic acid-Schiff (PAS) positive, silver negative, and fuschinophilic deposits in the mesangium extending around the capillary loops with thickening of the basement membrane. Immunohistochemistry was strongly positive for fibronectin (FN). There was no family history of renal disease. Genetic screening revealed absence of mutations in the FN1 gene. She was put on maintenance hemodialysis.