Membranous glomerulonephritis and nephrosis post factor IX infusions in hemophilia B

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...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 1998-10, Vol.12 (8), p.654-657
Main Authors: DHARNIDHARKA, V. R, TAKEMOTO, C, EWENSTEIN, B. M, ROSEN, S, HARRIS, H. W
Format: Article
Language:English
Subjects:
Biological and medical sciences
Child, Preschool
Drug toxicity and drugs side effects treatment
Factor IX - adverse effects
Factor IX - therapeutic use
Glomerulonephritis, Membranous - etiology
Glomerulonephritis, Membranous - pathology
Hemophilia B - therapy
Humans
Infant
Kidney - pathology
Kidney - ultrastructure
Male
Medical sciences
Microscopy, Electron
Nephrotic Syndrome - etiology
Nephrotic Syndrome - pathology
Pharmacology. Drug treatments
Toxicity: urogenital system
Citations: Items that cite this one
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Description
Summary: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.
ISSN:0931-041X
1432-198X
DOI:10.1007/s004670050522