Abstract |
The clinical course of C1-INH deficiency is presently well established. There is an inherited form ( Hereditary Angioedema) characterized by recurrence of cutaneous and mucous swellings appearing early in life and usually accompanied by substantial family history, and an acquired form ( Acquired Angioedema) where identical symptoms start after the fourth decade of life without family history. The acquired form can be associated with other diseases, mainly B cell disorders, and/or with autoantibodies to C1-INH. The biochemical characteristic is the functional deficiency of C1-INH and of C4 and C2. Moreover a marked deficiency of C1 is present in most acquired forms, but never in the inherited ones. C1-INH deficiency can be corrected by attenuated androgens that increase C1-INH levels in a few days and are effective in the prophylaxis of attacks, or by substitutive therapy with C1-INH plasma concentrate that is the life-saving drug in laryngeal edema. Patients with the inherited form have a uniformly good response to both these treatments which are otherwise effective only in a minority of patients with the acquired deficiency. In these subjects C1-INH concentrate needs to be given in higher doses and prevention of attacks is obtained with antifibrinolytic agents ( Tranexamic acid).
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Authors | A Agostoni, M Cicardi, M Cugno, E Storti |
Journal | Behring Institute Mitteilungen
(Behring Inst Mitt)
Issue 93
Pg. 306-12
(Dec 1993)
ISSN: 0301-0457 [Print] Germany |
PMID | 8172581
(Publication Type: Journal Article)
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Chemical References |
- Androgens
- Complement C1 Inactivator Proteins
- Fibrinolytic Agents
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Topics |
- Adolescent
- Adult
- Aged
- Aged, 80 and over
- Androgens
(therapeutic use)
- Angioedema
(physiopathology, therapy)
- Child
- Child, Preschool
- Complement C1 Inactivator Proteins
(analysis, deficiency, therapeutic use)
- Fibrinolytic Agents
(therapeutic use)
- Humans
- Middle Aged
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