Urticaria-Deafness-Amyloidosis

Below you will find more information about Urticaria-Deafness-Amyloidosis from Medigest. If you believe that you are suffering from any of the symptoms of Urticaria-Deafness-Amyloidosis it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with Urticaria-Deafness-Amyloidosis and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to Urticaria-Deafness-Amyloidosis comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.

Definition

Also known as Muckle-Wells syndrome, Urticaria-deafness-amyloidosis (UDA) was first described in 1962 by Thomas James Muckle and Michael Vernon Wells. It is a rare autosomal disease, which causes sensorineural deafness, recurrent hives, and can lead to amyloidosis. MWS happens when a mutation in the CIAS1 gene leads to increased activity of the cryopyrin protein. The disorder is closely related to two other syndromes, familial cold urticaria and neonatal onset multisystem inflammatory disease.

Diagnosis

Diagnostic criteria are purely clinical but genetic diagnosis is considered currently feasible. There are only moderate non-specific signs of inflammation during the course of inflammatory attacks associated to the disorder. Biochemical analyses may be useful to exclude certain diseases that can be considered as a function of the clinical signs present.

Treatment

There is no known treatment to prevent urticarial attacks. To correct deafness, hearing aids may be used.

Symptoms and Signs

The common symptom for MWS is inflammation all over the body, which includes, fever, arthritis, and malaise. The chronic inflammation associated with MWS can lead to deafness. Prolonged inflammation can also lead to deposition of proteins in the kidney, a condition known as amyloidosis.

Causes

The disease is caused by a defect in the CIAS1 gene, which creates the protein cryopyrin.

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