Membranous glomerulonephritis and nephrosis post factor IX infusions in hemophilia B

Vikas R. Dharnidharka, Clifford M Takemoto, Bruce M. Ewenstein, Seymour Rosen, H. William Harris

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)654-657
Number of pages4
JournalPediatric Nephrology
Volume12
Issue number8
DOIs
StatePublished - Oct 1998
Externally publishedYes

    Fingerprint

Keywords

  • Hemophilia B
  • Membranous glomerulonephritis
  • Nephrotic syndrome

ASJC Scopus subject areas

  • Nephrology
  • Pediatrics, Perinatology, and Child Health

Cite this

Dharnidharka, V. R., Takemoto, C. M., Ewenstein, B. M., Rosen, S., & Harris, H. W. (1998). Membranous glomerulonephritis and nephrosis post factor IX infusions in hemophilia B. Pediatric Nephrology, 12(8), 654-657. https://doi.org/10.1007/s004670050522