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Pediatr Nephrol ; 28(5): 823-6, 2013 May.
Article in English | MEDLINE | ID: mdl-23381011

ABSTRACT

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.


Subject(s)
Antibodies, Monoclonal, Murine-Derived/administration & dosage , Antibodies/blood , Coagulants/immunology , Factor IX/immunology , Glomerulonephritis, Membranous/therapy , Hemophilia B/drug therapy , Immunosuppressive Agents/administration & dosage , Mycophenolic Acid/analogs & derivatives , Nephrotic Syndrome/therapy , Biopsy , Child, Preschool , Coagulants/adverse effects , Coagulants/antagonists & inhibitors , Factor IX/adverse effects , Factor IX/antagonists & inhibitors , Glomerulonephritis, Membranous/diagnosis , Glomerulonephritis, Membranous/immunology , Hemophilia B/blood , Hemophilia B/immunology , Humans , Immune Tolerance , Male , Mycophenolic Acid/administration & dosage , Nephrotic Syndrome/diagnosis , Nephrotic Syndrome/immunology , Proteinuria/immunology , Proteinuria/therapy , Rituximab , Time Factors , Treatment Outcome
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