Document Detail


Membranous glomerulonephritis and nephrosis post factor IX infusions in hemophilia B.
MedLine Citation:
PMID:  9811389     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
V R Dharnidharka; C Takemoto; B M Ewenstein; S Rosen; H W Harris
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Pediatric nephrology (Berlin, Germany)     Volume:  12     ISSN:  0931-041X     ISO Abbreviation:  Pediatr. Nephrol.     Publication Date:  1998 Oct 
Date Detail:
Created Date:  1999-02-11     Completed Date:  1999-02-11     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8708728     Medline TA:  Pediatr Nephrol     Country:  GERMANY    
Other Details:
Languages:  eng     Pagination:  654-7     Citation Subset:  IM    
Affiliation:
Department of Medicine, Children's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
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MeSH Terms
Descriptor/Qualifier:
Child, Preschool
Factor IX / adverse effects*,  therapeutic use
Glomerulonephritis, Membranous / etiology*,  pathology
Hemophilia B / therapy*
Humans
Infant
Kidney / pathology,  ultrastructure
Male
Microscopy, Electron
Nephrotic Syndrome / etiology*,  pathology
Chemical
Reg. No./Substance:
9001-28-9/Factor IX

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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