原创力文档on demand treatment and home therapy of hereditary angioedema in germany - the frankfurt experience按需治疗和家庭治疗遗传性血管性水肿在德国法兰克福的经验.pdfVIP
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on demand treatment and home therapy of hereditary angioedema in germany - the frankfurt experience按需治疗和家庭治疗遗传性血管性水肿在德国法兰克福的经验
Aygören-Pürsün et al. Allergy, Asthma Clinical Immunology 2010, 6:21
/content/6/1/21 ALLERGY, ASTHMA CLINICAL
IMMUNOLOGY
RESEARCH Open Access
On demand treatment and home therapy of
hereditary angioedema in Germany - the
Frankfurt experience
*
Emel Aygören-Pürsün , Inmaculada Martinez-Saguer, Eva Rusicke, Thomas Klingebiel, Wolfhart Kreuz
Abstract
Background: Manifestation of acute edema in hereditary angioedema (HAE) is characterized by interindividual and
intraindividual variability in symptom expression over time. Flexible therapy options are needed.
Methods: We describe and report on the outcomes of the highly individualized approach to HAE therapy
practiced at our HAE center in Frankfurt (Germany).
Results: The HAE center at the Frankfurt University Hospital currently treats 450 adults with HAE or AAE and 107
pediatric HAE patients with highly individualized therapeutic approaches. 73.9% of the adult patients treat HAE
attacks by on-demand therapy with pasteurized pd C1-INH concentrate, 9.8% use additional prophylaxis with
attenuated androgens, 1% of the total patient population in Frankfurt has been treated with Icatibant up to now.
In addition adult and selected pediatric patients with a high frequency of severe attacks are instructed to apply
individual replacement therapy (IRT) with pasteurized pd C1-INH concentrate. Improvement on Quality of Life items
was shown for these patients compared to previous long-term danazol prophylaxis. Home treatment of HAE
patients was developed in the Frankfurt HAE center in line with experiences in hemophilia therapy and has so far
been implemented over a period of 28 years. At
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