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Saudi Journal of Kidney Diseases and Transplantation
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CASE REPORT Table of Contents   
Year : 2017  |  Volume : 28  |  Issue : 6  |  Page : 1416-1420
Fibronectin glomerulopathy - A sporadic case with unusual clinical manifestation

1 Department of Nephrology, NH Malla Reddy Narayana Multispeciality Hospital, Telangana, Hyderabad, India
2 Department of Histopathology, Apollo Hospital, Telangana, Hyderabad, India
3 Laboratory of Immunology and Genetics of Transplantation and Rare Diseases IRCCS - “Mario Negri” Institute for Pharmacological Research Ranica, Bergamo, Italy
4 Department of Pathology, Apollo Hospital, Telangana, Hyderabad, India

Correspondence Address:
Surya Narayan Mandal
Department of Nephrology, NH Malla Reddy Narayana Multispeciality Hospital, Telangana, Hyderabad
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DOI: 10.4103/1319-2442.220860

PMID: 29265057

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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.

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