familial Hibernian fever


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familial Hibernian fever

a periodic syndrome associated with a mutation in a receptor for tumor necrosis factor. This defect causes recurrent high fevers, rash, and abdominal pain. Mean age of first episode is 3 years but varies from patient to patient.

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.

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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