The First Case of Familial Mediterranean Fever Associated with Renal Amyloidosis in Korea
Yonsei Medical Journal
;
: 454-458, 2012.
Article
in English
| WPRIM
| ID: wpr-114989
ABSTRACT
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Familial Mediterranean Fever
/
Amyloidosis
/
Kidney Diseases
/
Korea
Limits:
Child
/
Humans
/
Male
Country/Region as subject:
Asia
Language:
English
Journal:
Yonsei Medical Journal
Year:
2012
Type:
Article
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