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Membranous glomerulonephritis and nephrosis post factor IX infusions in hemophilia B.

Authors
Type
Published Article
Journal
Pediatric nephrology (Berlin, Germany)
Publication Date
Volume
12
Issue
8
Pages
654–657
Identifiers
PMID: 9811389
Source
Medline
License
Unknown

Abstract

Children with hemophilia B who receive exogenous factor IX infusions may rarely develop inhibitors to the exogenous factor IX and require desensitization. Nephrotic syndrome has recently been described in some of these children. We report the renal clinicopathological findings in a child with severe factor IX deficiency, requiring induction of an immune tolerance protocol, who developed nephrotic syndrome. Renal biopsy revealed peripheral capillary wall thickening and a spike appearance consistent with membranous glomerulonephritis. Electron microscopy showed prominent deposits throughout the thickness of the basement membrane. Factor IX dose reduction was accompanied by reversal of the child's nephrotic syndrome without relapses.

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