Complete remission of nephrotic syndrome in a woman with renal amyloidosis due to familial mediterranean fever.
نویسندگان
چکیده
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متن کاملFamilial Mediterranean Fever
Description Familial Mediterranean fever is an autosomal recessive disorder mostly prevalent in Mediterranean populations. It is characterized by recurrent selflimited episodes of fever, arthritis, serositis, and skin rash, with marked accumulations of polymorphonuclear leukocytes in affected areas during attacks. The most severe complication of the disorder is the development of amyloidosis, l...
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Secondary amyloidosis (AA) is characterized by the extracellular tissue deposition of fibrils composed of fragments of an acute-phase reactant protein, serum amyloid A (SAA), due to chronic inflammatory diseases, infections and several neoplasms. AA amyloidosis may also complicate several hereditary diseases, where genetic factors play a pivotal role in the expression of amyloidosis. Familial M...
متن کاملTherapeutic approach to patients with familial Mediterranean fever-related amyloidosis resistant to colchicine.
The frequency of FMF-related amyloidosis has been decreased by colchicine use over the past few decades. However, the beneficial effect of colchicine may differ in accordance with nephropathic stages. When used in proper doses and with compliance, colchicine is very effective in preclinical and proteinuric stages of FMF-related amyloidosis. Even so, a large number of patients with nephrotic ran...
متن کاملFamilial Mediterranean fever, Inflammation and Nephrotic Syndrome: Fibrillary Glomerulopathy and the M680I Missense Mutation
BACKGROUND Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by inflammatory serositis (fever, peritonitis, synovitis and pleuritis). The gene locus responsible for FMF was identified in 1992 and localized to the short arm of chromosome 16. In 1997, a specific FMF gene locus, MEFV, was discovered to encode for a protein, pyrin that mediates inflammation. To date...
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عنوان ژورنال:
- Nefrologia : publicacion oficial de la Sociedad Espanola Nefrologia
دوره 36 3 شماره
صفحات -
تاریخ انتشار 2016