Abstract:
BACKGROUND:Recurrent nephrotic syndrome (NS) is a severe problem after renal transplantation in patients with congenital nephrotic syndrome of the Finnish type (NPHS1). The NPHS1 kidneys do not express nephrin, and antibodies against this major glomerular filter protein have been observed in NPHS1 children with recurrent NS. We evaluated here the use of plasma exchange (PE) therapy and kidney retransplantation in NPHS1 patients with recurrent NS and extended our studies on the pathogenesis of the recurrence. 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.
journal_name
Transplantationjournal_title
Transplantationauthors
Kuusniemi AM,Qvist E,Sun Y,Patrakka J,Rönnholm K,Karikoski R,Jalanko Hdoi
10.1097/01.tp.0000262569.27890.64subject
Has Abstractpub_date
2007-05-27 00:00:00pages
1316-23issue
10eissn
0041-1337issn
1534-6080pii
00007890-200705270-00005journal_volume
83pub_type
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