C1-esterase inhibitor transfusions in patients with hereditary angioedema. 1998

D E Visentin, and W H Yang, and J Karsh
Section of Allergy and Clinical Immunology, Ottawa Civic Hospital, University of Ottawa, Ontario, Canada.

BACKGROUND Hereditary angioedema results from the deficiency of C1-esterase inhibitor (C1-INH), and C1-INH replacement would represent definitive treatment for angioedema attacks. In Canada, C1-INH is available only on a compassionate basis at select medical facilities. Our objective is to assess the efficacy of C1-INH transfusions during angioedema attacks at a single Canadian institution. METHODS A retrospective chart review of transfusion data between January 1, 1995 and June 30, 1996 was performed. Phone interviews with patients elicited their opinions of the treatment. Data collected included the number and duration of angioedema attacks, dose of transfused C1-INH, and side effects of treatment. RESULTS Of a cohort of 13 patients with hereditary angioedema, seven received transfusions with C1-INH. Attacks totaled 87, and more than 100,000 units of the product were transfused. The mean time for abatement of an attack after initiation of transfusion was 50 +/- 8 minutes (1 SD). There were no reports of adverse effects. Although patients were satisfied with the treatment, they raised concerns regarding long-term safety and availability. CONCLUSIONS C1-INH transfusion is a satisfactory means of treating angioedema attacks.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D001803 Blood Transfusion The introduction of whole blood or blood component directly into the blood stream. (Dorland, 27th ed) Blood Transfusions,Transfusion, Blood,Transfusions, Blood
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003174 Complement C1 Inactivator Proteins Serum proteins that inhibit, antagonize, or inactivate COMPLEMENT C1 or its subunits. Complement 1 Esterase Inhibitors,Complement C1 Inactivating Proteins,Complement C1 Inhibiting Proteins,Complement C1 Inhibitor Proteins,Complement C1r Protease Inhibitor Proteins,Complement C1s Esterase Inhibitor Proteins,Complement Component 1 Inactivator Proteins
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000799 Angioedema Swelling involving the deep DERMIS, subcutaneous, or submucosal tissues, representing localized EDEMA. Angioedema often occurs in the face, lips, tongue, and larynx. Quincke's Edema,Urticaria, Giant,Angioneurotic Edema,Angioedemas,Angioneurotic Edemas,Edema, Angioneurotic,Edema, Quincke's,Edemas, Angioneurotic,Giant Urticaria,Giant Urticarias,Quincke Edema,Quinckes Edema,Urticarias, Giant

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