| Successful management of factor IX inhibitor-associated nephrotic syndrome in a hemophilia B patient. | |
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MedLine Citation:
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PMID: 23381011 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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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. |
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Authors:
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Priya Verghese; Stephen Darrow; Margaret H Kurth; Robyn C Reed; Youngki Kim; Susan Kearney |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2013-2-6 |
Journal Detail:
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Title: Pediatric nephrology (Berlin, Germany) Volume: - ISSN: 1432-198X ISO Abbreviation: Pediatr. Nephrol. Publication Date: 2013 Feb |
Date Detail:
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Created Date: 2013-2-5 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8708728 Medline TA: Pediatr Nephrol Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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University of Minnesota - Pediatric Nephrology, 2450 Riverside Avenue, MB 680, Minneapolis, MN, 55437, USA, pverghes@umn.edu. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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