Successful management of factor IX inhibitor-associated nephrotic syndrome in a hemophilia B patient.

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.

Preventing risk and promoting resilience in radiation health.

Because risk assessment is fundamentally deficient in the face of unknown or unforeseeable events and disasters such as occurred in 2011 at the Fukushima Daiichi Nuclear Power Station in Japan, resilience thinking, which focuses on the ability of both natural and human-made systems to prepare for, absorb, and recover from an adverse event and to adapt to new conditions is an important additional consideration in decision making. Radiation contamination is an impediment to most critical functions of a community; resilience planning considers how those critical functions will be maintained in the event that radiation contamination does occur. Therefore, planning should begin with resilience-based thinking and should be complemented with risk assessment-based tools. Integr Environ Assess Manag 2016;12:677-679. Published 2016. This article is a U.S. Government work and is in the public domain in the USA.