Tnf Receptor-Associated Periodic Syndrome (Traps)

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(Familial Hibernian fever)

Tumor necrosis factor (TNF) receptor-associated periodic syndrome is an autosomal dominate genetic disease that causes recurring fever and painful, wandering myalgia with overlying erythema sensitive. The mirrors of the type 1 TNF receptors are lowered. The treatment is performed with corticosteroids and etanercept.

TRAPS was first described in the case of a family with Irish-Scottish descent, but also occurs in other ethnic groups before. The cause is a mutation in the gene which encodes the TNF receptor. The mutation leads to uncontrolled TNF signals that lead to inflammation, possibly because the secretion of TNF receptor is defective.

Tumor necrosis factor (TNF) receptor-associated periodic syndrome is an autosomal dominate genetic disease that causes recurring fever and painful, wandering myalgia with overlying erythema sensitive. The mirrors of the type 1 TNF receptors are lowered. The treatment is performed with corticosteroids and etanercept. TRAPS was first described in the case of a family with Irish-Scottish descent, but also occurs in other ethnic groups before. The cause is a mutation in the gene which encodes the TNF receptor. The mutation leads to uncontrolled TNF signals that lead to inflammation, possibly because the secretion of TNF receptor is defective. The attacks of this rare disease begin before age 20. You can take from 1-2 days to a week. The clearest sign of a seizure are wandering myalgia, and swelling of the extremities. The overlying skin is red and sensitive. Other symptoms may include headache, abdominal pain, diarrhea or constipation, nausea, painful conjunctivitis, joint pain, rash and testicular pain. Men tend to develop hernias. Amyloidosis of the kidneys occurs in a minority of families. With treatment, the prognosis is good, but patients with renal amyloidosis should be particularly observed. the diagnosis based on anamnesis, examination and a low type 1 TNF receptor levels (<1 ng / ml), which is determined during the attacks is provided diagnosis. Non-specific findings include granulocytosis, increasing the acute phase proteins and a polyclonal gammopathy during seizures. Patients should be regularly examined for proteinuria. Genetic testing for TRAPS is possible. Treatment The seizures are p.o. with at least 20 mg of prednisone once treated successfully every day. The dosage may need to be increased over time. When a prednisone treatment brings no relief from seizures, are etanercept, TNF binds and inactivates and anakinra possible options. The recommended dose of etanercept is 0.4 mg / kg s.c. in children and 50 mg s.c. in adults 2 times a week. Anakinra 1.5 mg / kg s.c. once daily can be effective in children.

Health Life Media Team

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