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A Rare Cause of Lymphadenopathy in Children: Autoinflammatory Disease - Familial Mediterranean Fever
Author(s) -
Gonca Keskindemirci,
Nuray Aktay Ayaz,
Deniz Tuğcu,
Mustafa Çakan,
Gönül Aydoğan,
Ali Er,
Ferhan Akıcı,
Hamide Sevinç Genç,
Serdar Sander
Publication year - 2015
Publication title -
turkish journal of pediatric disease
Language(s) - English
Resource type - Journals
eISSN - 1307-4490
pISSN - 2148-3566
DOI - 10.12956/tjpd.2015.186
Subject(s) - familial mediterranean fever , medicine , dermatology , abdominal pain , rash , mefv , arthritis , rare disease , chest pain , pediatrics , disease , biochemistry , chemistry , gene mutation , mutation , gene
Familial Mediterranean fever (FMF) is an autosomal recessively inherited autoinflammatory disorder that is characterized by recurrent attacks of fever and polyserositis. Approximately 90% of patients are diagnosed in childhood. Typical attacks last for 1-3 days and there is an attack-free period without any complaints. Most common symptoms are fever and abdominal pain. Other common symptoms are chest pain and arthritis due to pleural and synovial involvement. Rash, eye involvement, epididymo-orchitis and glomerulonephritis are rare manifestations of FMF. Herein, we present a case of FMF whose initial symptoms were fever, fatigue and weight loss with high acute phase reactants and abdominal lympadenopathy that was mimicking a malignant process.

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