Current management of hereditary angio-oedema (C'1 esterase inhibitor deficiency).
Hereditary angio-oedema is characterised by recurrent swellings in any part of the body and also by recurrent attacks of severe abdominal pain. The disease is inherited in an autosomal dominant manner but up to 25% of cases can occur as a spontaneous mutation. Attacks of swelling can be precipitated by trauma, certain drugs, and emotional stress. Treatment usually involves a combination of prophylaxis, using androgens or antifibrolytic drugs, and replacement with C'1 esterase inhibitor concentrate for acute attacks and before surgery or other traumatic procedures.
Fay
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Department of Immunology, Newcastle upon Tyne Hospitals NHS Trust, Newcastle upon Tyne NE1 4LP, UK. Fiona.Campbell@nuth.northy.nhs.uk
Abinun
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M
D000208:Acute Disease; D000328:Adult; D000799:Angioedema / Q000209:etiology / Q000235:genetics / Q000628:therapy*; D002648:Child; D002908:Chronic Disease; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency* / Q000627:therapeutic use; D005260:Female; D006801:Humans; D011247:Pregnancy; D011248:Pregnancy Complications / Q000628:therapy
D003174:Complement C1 Inactivator Proteins