One hundred and four patients affected by hereditary angioedema belonging to 31 families have been studied. Twenty-two percent had the variant form related to the deficiency of the functional activity of serum C1 esterase inhibitor. The remaining 78% of patients had the predominant form, characterized by low antigenic levels and low functional activity of serum C1 esterase inhibitor. Attacks of swelling affected the subcutaneous tissue in 86% of patients; the upper airways in 76% of patients, and the bowel mucose in 75% of patients. Before treatment was available the mortality rate was 56%. One or more attacks a month were present in 46% of cases. The infusion of C1 inhibitor concentrate promptly reversed 14 severe attacks without any side effect. Twenty-nine patients were given long term prophylactic treatment with androgen derivatives with full success. Tranexamic acid reduced the frequency of swelling of 70% of the patients.

Hereditary angioedema: an appraisal of 104 cases / M. Cicardi, L. Bergamaschini, B. Marasini, G. Boccassini, A. Tucci, A. Agostoni. - In: THE AMERICAN JOURNAL OF THE MEDICAL SCIENCES. - ISSN 0002-9629. - 284:1(1982), pp. 2-9.

Hereditary angioedema: an appraisal of 104 cases

M. Cicardi
Primo
;
L. Bergamaschini
Secondo
;
B. Marasini;A. Agostoni
Ultimo
1982

Abstract

One hundred and four patients affected by hereditary angioedema belonging to 31 families have been studied. Twenty-two percent had the variant form related to the deficiency of the functional activity of serum C1 esterase inhibitor. The remaining 78% of patients had the predominant form, characterized by low antigenic levels and low functional activity of serum C1 esterase inhibitor. Attacks of swelling affected the subcutaneous tissue in 86% of patients; the upper airways in 76% of patients, and the bowel mucose in 75% of patients. Before treatment was available the mortality rate was 56%. One or more attacks a month were present in 46% of cases. The infusion of C1 inhibitor concentrate promptly reversed 14 severe attacks without any side effect. Twenty-nine patients were given long term prophylactic treatment with androgen derivatives with full success. Tranexamic acid reduced the frequency of swelling of 70% of the patients.
Settore MED/09 - Medicina Interna
1982
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/192998
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