Iranian Journal of Kidney Diseases, Vol 7, No 1 (2013)

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Amyloidosis in a Child With Hyperimmunoglobulinemia D Syndrome

Sibel Yel, Zubeyde Gunduz, Funda Bastug, Ruhan Dusunsel, Ismail Dursun, Hakan Poyrazoglu, Sebahat Tulpar, Kemal Deniz, Erkan Demirkaya


Hereditary periodic fever syndromes are a group of genetic diseases clinically characterized by recurrent febrile attacks. Patients are at variable risks for the development of systemic reactive (AA) amyloidosis, leading to the nephrotic syndrome and kidney failure. We present the first report of the occurrence of renal AA amyloidosis causing severe nephrotic syndrome in a Turkish child affected with hyperimmunoglobulinemia D syndrome.

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