The first case of familial mediterranean fever associated with renal amyloidosis in Korea

Kyo Yeon Koo, Se Jin Park, Ji Young Wang, Jae Il Shin, Hyeon Joo Jeong, Beom Jin Lim, Jin Sung Lee

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Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by periodic episodes of fever and recurrent polyserositis. It is caused by a dysfunction of pyrin (or marenostrin) as a result of a mutation within the MEFV gene. It occurs mostly in individuals of Mediterranean origin; however, it has also been reported in non-Mediterranean populations. In this report, we describe the first case of FMF in a Korean child. As eight-year-old boy presented recurrent febrile attacks from an unknown cause, an acute scrotum and renal amyloidosis. He also showed splenomegaly, lymphadenopathy, pleural effusion, ascites and elevated acute phase reactants. After MEFV gene analysis, he was diagnosed as FMF combined with amyloidosis.

Original languageEnglish
Pages (from-to)454-458
Number of pages5
JournalYonsei medical journal
Issue number2
Publication statusPublished - 2012 Mar 1


All Science Journal Classification (ASJC) codes

  • Medicine(all)

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