Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2010 Jul 28;6(1):21.
doi: 10.1186/1710-1492-6-21.

On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience

Emel Aygören-Pürsün  1 , Inmaculada Martinez-Saguer, Eva Rusicke, Thomas Klingebiel, Wolfhart Kreuz
Affiliations

On demand treatment and home therapy of hereditary angioedema in Germany - the Frankfurt experience

Emel Aygören-Pürsün et al. Allergy Asthma Clin Immunol. .

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 present 248 (55%) of the adult patients and 26 (24%) of the pediatric patients are practicing home treatment either as on demand or IRT treatment.

Conclusions: In conclusion, the individualized home therapies provided by our HAE center, aim to limit the disruption to normal daily activities that occurs for many HAE patients. Furthermore, we seek to optimize the economic burden of the disease while offering a maximum quality of life to our patients.

PubMed Disclaimer

References

    1. Bowen T, Cicardi M, Bork K, Zuraw B, Frank M, Ritchie B, Farkas H, Varga L, Zingale LC, Binkley K, Wagner E, Adomaitis P, Brosz K, Burnham J, Warrington R, Kalicinsky C, Mace S, McCusker C, Schellenberg R, Celeste L, Hebert J, Valentine K, Poon MC, Serushago B, Neurath D, Yang W, Lacuesta G, Issekutz A, Hamed A, Kamra P, Dean J, Kanani A, Stark D, Rivard GE, Leith E, Tsai E, Waserman S, Keith PK, Page D, Marchesin S, Longhurst HJ, Kreuz W, Rusicke E, Martinez-Saguer I, Aygören-Pürsün E, Harmat G, Füst G, Li H, Bouillet L, Caballero T, Moldovan D, Späth PJ, Smith-Foltz S, Nagy I, Nielsen EW, Bucher C, Nordenfelt P, Xiang ZY. Hereditary angiodema: a current state-of-the-art review, VII: Canadian Hungarian 2007 International Consensus Algorithm for the Diagnosis, Therapy, and Managment of Hereditary Angioedema. Ann Allergy Asthma Immunol. 2008;100:S30–40. doi: 10.1016/S1081-1206(10)60584-4. - DOI - PubMed
    1. Kreuz W, Martinez-Saguer I, Aygören-Pürsün E, Rusicke E, Heller C, Klingebiel T. C1-inhibitor concentrate for individual replacement therapy in patients with severe hereditary angioedema refractory to danazol prophylaxis. Transfusion. 2009;49:1987–1995. doi: 10.1111/j.1537-2995.2009.02230.x. - DOI - PubMed
    1. Bork K, Barnstedt SE. Treatment of 193 episodes of laryngeal Edema With C1 Inhibitor Concentrate in patients With Hereditary Angioedema. Arch Intern Med. 2001;161:714–718. doi: 10.1001/archinte.161.5.714. - DOI - PubMed
    1. Bork K, Meng G, Staubach P, Hardt J. Treatment with C1 Inhibitor Concentrate in Abdominal Pain Attacks of Patients with Hereditary Angioedema. Transfusion. 2005;45:1774–1784. doi: 10.1111/j.1537-2995.2005.00602.x. - DOI - PubMed
    1. Bork K, Staubach P, Hardt J. Treatment of Skin Swellings with C1-Inhibitor Concentrate in patients with Hereditary Angio-oedema. Allergy. 2008;63:751–757. doi: 10.1111/j.1398-9995.2007.01577.x. - DOI - PubMed

LinkOut - more resources