familial Hibernian fever

(redirected from TNF receptor associated periodic syndrome)
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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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familial Hibernian fever

A rare autosomal dominant syndrome characterized by intermittent elevations of body temperature, muscle pains, abdominal pain, inguinal hernias, and rash.
Synonym: TNF receptor associated periodic syndrome
See also: fever
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