Document Detail

Successful management of factor IX inhibitor-associated nephrotic syndrome in a hemophilia B patient.
MedLine Citation:
PMID:  23381011     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Nephrotic syndrome (NS) is a recognized complication of immune tolerance induction (ITI) therapy, a treatment strategy used to treat inhibitors in patients with hemophilia B receiving factor IX concentrate.
CASE DIAGNOSIS/TREATMENT: We present a 4-year-old boy with hemophilia B and an inhibitor who underwent ITI, and developed NS 19 months into this therapy. A percutaneous renal biopsy was safely performed with factor IX (FIX) concentrate administration both preceding and following the procedure. The patient's inhibitor level had increased to 1.4-1.6 Bethesda Units just prior to the onset of proteinuria. Histology confirmed segmental membranous nephropathy (MGN). The patient was continued on FIX concentrate as ITI and also received 4 weekly doses of rituximab and ongoing immunosuppression with mycophenolate mofetil. This resulted in the complete resolution of his inhibitor and his NS. He continues with a modified ITI regimen and remains inhibitor-free without proteinuria >12 months post-biopsy.
CONCLUSIONS: Hemophilia B patients undergoing ITI should be regularly screened for NS. At first detection of proteinuria, with proper precautions, a percutaneous kidney biopsy can be performed safely in patients with low levels of inhibitor. Our patient had segmental MGN with complete remission of NS.
Priya Verghese; Stephen Darrow; Margaret H Kurth; Robyn C Reed; Youngki Kim; Susan Kearney
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2013-02-06
Journal Detail:
Title:  Pediatric nephrology (Berlin, Germany)     Volume:  28     ISSN:  1432-198X     ISO Abbreviation:  Pediatr. Nephrol.     Publication Date:  2013 May 
Date Detail:
Created Date:  2013-03-21     Completed Date:  2013-09-16     Revised Date:  2014-11-13    
Medline Journal Info:
Nlm Unique ID:  8708728     Medline TA:  Pediatr Nephrol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  823-6     Citation Subset:  IM    
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MeSH Terms
Antibodies / blood*
Antibodies, Monoclonal, Murine-Derived / administration & dosage*
Child, Preschool
Coagulants / adverse effects,  antagonists & inhibitors,  immunology*
Factor IX / adverse effects,  antagonists & inhibitors,  immunology*
Glomerulonephritis, Membranous / diagnosis,  immunology,  therapy*
Hemophilia B / blood,  drug therapy*,  immunology
Immune Tolerance
Immunosuppressive Agents / administration & dosage*
Mycophenolic Acid / administration & dosage,  analogs & derivatives*
Nephrotic Syndrome / diagnosis,  immunology,  therapy*
Proteinuria / immunology,  therapy
Time Factors
Treatment Outcome
Reg. No./Substance:
0/Antibodies; 0/Antibodies, Monoclonal, Murine-Derived; 0/Coagulants; 0/Immunosuppressive Agents; 0/rituximab; 9001-28-9/Factor IX; 9242ECW6R0/mycophenolate mofetil; HU9DX48N0T/Mycophenolic Acid

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

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