A recurrent fibronectin glomerulopathy in a renal transplant patient: A case report

Yasuhiro Otsuka, Asami Takeda, Keiji Horike, Daijo Inaguma, Norihiko Goto, Yoshihiko Watarai, Kazuharu Uchida, Michael J. Mihatsch, Kensuke Joh, Kunio Morozumi

Research output: Contribution to journalArticle

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Abstract

Fibronectin glomerulopathy (FNG) is a rare, autosomal dominant renal disease with massive mesangial, and subendothelial fibronectin deposits. It presents proteinuria, often in the nephrotic range in the third to fourth decade, and slow progression to end-stage renal disease. The risk of recurrent disease in renal allograft is uncertain. A Japanese female with end-stage renal disease because of unknown origin received a renal transplant and was referred with proteinuria and mild deterioration of renal function four months after transplantation. Five allograft biopsies were underwent from one h to 12 months after the transplantation, including a biopsy 19 d after the transplantation, which revealed dense deposits suggesting fibronectin. A biopsy 134 d after the transplantation showed a feature of lobular glomerulonephritis corresponding FNG. The diagnosis was confirmed by IST4 positive and IST9 negative immunostaining together with typical fibrillary dense deposits in the mesangium and subendothelial spaces in electron microscopy. This is the first report of recurrent FNG in Japan.

Original languageEnglish
Pages (from-to)58-63
Number of pages6
JournalClinical Transplantation
Volume26
Issue numberSUPPL.24
DOIs
Publication statusPublished - 01-07-2012
Externally publishedYes

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Transplantation
Transplants
Kidney
Biopsy
Proteinuria
Fibronectins
Chronic Kidney Failure
Allografts
Glomerulonephritis
Electron Microscopy
Japan
Glomerulopathy with fibronectin deposits

All Science Journal Classification (ASJC) codes

  • Transplantation

Cite this

Otsuka, Yasuhiro ; Takeda, Asami ; Horike, Keiji ; Inaguma, Daijo ; Goto, Norihiko ; Watarai, Yoshihiko ; Uchida, Kazuharu ; Mihatsch, Michael J. ; Joh, Kensuke ; Morozumi, Kunio. / A recurrent fibronectin glomerulopathy in a renal transplant patient : A case report. In: Clinical Transplantation. 2012 ; Vol. 26, No. SUPPL.24. pp. 58-63.
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Otsuka, Y, Takeda, A, Horike, K, Inaguma, D, Goto, N, Watarai, Y, Uchida, K, Mihatsch, MJ, Joh, K & Morozumi, K 2012, 'A recurrent fibronectin glomerulopathy in a renal transplant patient: A case report', Clinical Transplantation, vol. 26, no. SUPPL.24, pp. 58-63. https://doi.org/10.1111/j.1399-0012.2012.01644.x

A recurrent fibronectin glomerulopathy in a renal transplant patient : A case report. / Otsuka, Yasuhiro; Takeda, Asami; Horike, Keiji; Inaguma, Daijo; Goto, Norihiko; Watarai, Yoshihiko; Uchida, Kazuharu; Mihatsch, Michael J.; Joh, Kensuke; Morozumi, Kunio.

In: Clinical Transplantation, Vol. 26, No. SUPPL.24, 01.07.2012, p. 58-63.

Research output: Contribution to journalArticle

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N2 - Fibronectin glomerulopathy (FNG) is a rare, autosomal dominant renal disease with massive mesangial, and subendothelial fibronectin deposits. It presents proteinuria, often in the nephrotic range in the third to fourth decade, and slow progression to end-stage renal disease. The risk of recurrent disease in renal allograft is uncertain. A Japanese female with end-stage renal disease because of unknown origin received a renal transplant and was referred with proteinuria and mild deterioration of renal function four months after transplantation. Five allograft biopsies were underwent from one h to 12 months after the transplantation, including a biopsy 19 d after the transplantation, which revealed dense deposits suggesting fibronectin. A biopsy 134 d after the transplantation showed a feature of lobular glomerulonephritis corresponding FNG. The diagnosis was confirmed by IST4 positive and IST9 negative immunostaining together with typical fibrillary dense deposits in the mesangium and subendothelial spaces in electron microscopy. This is the first report of recurrent FNG in Japan.

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