Hereditary Angioedema (HAE) due to
C1-inhibitor (C1-INH) deficiency is a rare disease with an estimated prevalenceof 1:50,000 in the general population. It is characterized by recurrent oedema
of the subcutaneous and submucosal tissues.
These symptoms are episodic with a
high degree of variability, in frequency and severity, from patient to patient
and within the same patient from time to time. Subcutaneous locations result in
swelling of the extremities, face, and trunk. Mucosal edema develops in the
gastrointestinal wall or in the mouth, pharynx and larynx. The first causes
bowel sub occlusion, with severe pain vomiting and/or diarrhoea, the other
dysphagia and respiratory distress up to asphyxia. Read more>>>>>>>>
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