familial periodic fever


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Related to familial periodic fever: familial periodic paralysis

familial

 [fah-mil´e-al]
occurring in or affecting members of a family more than would be expected by chance.
familial Mediterranean fever a hereditary disease usually occurring in Armenians and Sephardic Jews, and marked by short recurrent attacks of fever with pain in the abdomen, chest, or joints, and erythema resembling that seen in erysipelas; it is sometimes complicated by amyloidosis.
familial periodic fever a rare autosomal dominant syndrome that includes an abnormality on the cell receptor for tumor necrosis factor; characteristics include periodic fever with any of various skin disorders lasting for four days to three weeks, as well as mild systemic manifestations such as abdominal pain, headache, and chest pain. Called also tumor necrosis factor receptor–associated periodic syndrome.

familial periodic fever

a rare autosomal-dominant syndrome that includes an abnormality on the cell receptor for tumor necrosis factor, characterized by periodic fever with any of various skin disorders lasting for 4 days to 3 weeks and mild systemic manifestations, such as abdominal pain, headache, and chest pain. Also called tumor necrosis factor receptor-associated periodic syndrome (TRAPS).

periodic fever, familial, autosomal dominant

A hereditary periodic fever syndrome (OMIM:142680) characterised by recurrent fever, abdominal pain, localised tender skin lesions and myalgia. The main complication is amyloidosis, which occurs in 25% of cases.
 
Molecular pathology
Caused by defects of TNFRSF1A, which encodes a major receptor for TNF-alpha that activates NF-kappaB, mediates apoptosis and regulates inflammation.
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