Abstract | BACKGROUND: METHODS: Clinical data on 65 NPHS1 patients who received 77 kidney transplants between the years 1986 and 2006 was collected. Serum anti- nephrin antibodies were assayed with an enzyme-linked immunosorbent assay method, and the kidney biopsy samples were evaluated by light microscopy and immunohistochemistry. RESULTS: Twenty-three episodes of recurrent NS occurred in 19 grafts of 13 NPSH1 patients homozygous for Fin-major mutation. Six retransplantations were performed to four NPHS1 patients, who lost their graft because of recurrent NS, and heavy proteinuria developed immediately in all cases. Although 73% of the patients had detectable serum anti- nephrin antibodies, the kidney biopsy findings were minimal. Introduction of PE alongside cyclophosphamide proved effective in the treatment of the proteinuric episodes (one graft loss out of nine). If remission was achieved, recurrent NS did not significantly deteriorate the long term graft function. CONCLUSIONS: The clinical and pathological data suggest that anti- nephrin antibodies effectively impair the glomerular function in kidney grafts of NPHS1 patients homozygous for Fin-major mutation. Plasma exchange is a useful adjunct to the treatment of the recurrent NS.
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Authors | Arvi-Matti Kuusniemi, Erik Qvist, Yi Sun, Jaakko Patrakka, Kai Rönnholm, Riitta Karikoski, Hannu Jalanko |
Journal | Transplantation
(Transplantation)
Vol. 83
Issue 10
Pg. 1316-23
(May 27 2007)
ISSN: 0041-1337 [Print] United States |
PMID | 17519780
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Autoantibodies
- Membrane Proteins
- nephrin
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Topics |
- Autoantibodies
(blood)
- Child
- Child, Preschool
- Finland
- Follow-Up Studies
- Glomerular Filtration Rate
- Humans
- Kidney Transplantation
(immunology, pathology)
- Membrane Proteins
(immunology)
- Mutation
- Nephrotic Syndrome
(congenital, genetics, surgery)
- Plasma Exchange
- Recurrence
- Reoperation
- Retrospective Studies
- Treatment Outcome
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