Treatment of attacks with plasma-derived C1-inhibitor concentrate in pediatric hereditary angioedema patients Henriette Farkas, MD, PhD, DSc, Dorottya.

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Treatment of attacks with plasma-derived C1-inhibitor concentrate in pediatric hereditary angioedema patients Henriette Farkas, MD, PhD, DSc, Dorottya Csuka, MSc, Zsuzsanna Zotter, MD, Erika Szabó, MD, Ibolya Czaller, MD, Lilian Varga, PhD, János Fejes, MD, George Füst, PhD, DSc, George Harmat, MD, PhD Journal of Allergy and Clinical Immunology Volume 131, Issue 3, Pages 909-911.e5 (March 2013) DOI: 10.1016/j.jaci.2012.08.036 Copyright © 2012 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Age at the first onset of HAE symptoms, at the diagnosis of HAE, and at the first administration of the pdC1-INH concentrate (years). Journal of Allergy and Clinical Immunology 2013 131, 909-911.e5DOI: (10.1016/j.jaci.2012.08.036) Copyright © 2012 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 2 Time to initial relief of symptoms (A) and time to complete resolution (B) of symptoms after the administration of the pdC1-INH concentrate (Berinert, CSL Behring) (minutes). Journal of Allergy and Clinical Immunology 2013 131, 909-911.e5DOI: (10.1016/j.jaci.2012.08.036) Copyright © 2012 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E1 The distribution of attacks by location. A, All attacks (n = 1392) observed during the study. B, Attacks (n = 152) treated with the pdC1-INH concentrate (Berinert, CSL Behring) during the study. Journal of Allergy and Clinical Immunology 2013 131, 909-911.e5DOI: (10.1016/j.jaci.2012.08.036) Copyright © 2012 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E2 Analysis of the relationship between the number of vials of the pdC1-INH concentrate (Berinert, CSL Behring) received and the titers of antibodies to C1-INH. Two groups (“1-3 vials of pdC1-INH” and “>3 vials of pdC1-INH”) were created by dividing the study population into 2 subsets according to median per capita pdC1-INH consumption during the observational period. Start, time of diagnosis (ie, the beginning of the observational period). End, the year when the patient turned 18 years old (ie, the end of the observational period). Horizontal lines represent the upper limit of the normal range for each antibody. Journal of Allergy and Clinical Immunology 2013 131, 909-911.e5DOI: (10.1016/j.jaci.2012.08.036) Copyright © 2012 American Academy of Allergy, Asthma & Immunology Terms and Conditions