scholarly journals P60: INTRODUCTION OF BERINERT® SC (HUMAN C1‐ESTERASE INHIBITOR) ADMINISTRATION IN THE HOME SETTING TO SUPPORT PROPHYLACTIC TREATMENT OF HEREDITARY ANGIOEDEMA PATIENTS

2021 ◽  
Vol 51 (S4) ◽  
pp. 22-22
Keyword(s):  
Hereditary Angioedema ◽  
Prophylactic Treatment ◽  
Home Setting ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

Successful Long-Term Prophylactic Treatment With Subcutaneous C1 Esterase Inhibitor in a Patient With Hereditary Angioedema

2019 ◽  
Vol 33 (6) ◽  
pp. 907-911
Author(s):  
Janina Hahn ◽  
Melanie Nordmann-Kleiner ◽  
Susanne Trainotti ◽  
Thomas K. Hoffmann ◽  
Jens Greve
Keyword(s):  
Quality Of Life ◽  
Hereditary Angioedema ◽  
Prophylactic Treatment ◽  
Upper Airway ◽  
Venous Access ◽  
Current Standard ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

Background: Hereditary angioedema (HAE) patients suffer from recurrent swellings. Current standard therapy consists of C1 esterase inhibitor (C1-INH) and bradykinin receptor B2 antagonists. Severe courses require prophylactic treatment. For such patients, it has been demonstrated that the intravenous (IV) administration of C1-INH [C1-INH(IV)] is safe and effective. A new prophylactic option is subcutaneous (SC) treatment with C1-INH. Methods and Case: We present the case of an HAE patient placed on prophylactic C1-INH(IV) therapy due to frequent attacks when managed with on-demand therapy. An implanted port allowed the periodical and safe application of medication until the device was explanted due to an infection. Due to the poor venous access, repeated IV application failed. Therefore, we began a SC treatment with 1500 IU C1-INH [C1-INH(SC)] as long-term prophylaxis and analyzed the clinical course over 16 months. Results: Under the SC prophylaxis, the number of attacks were reduced to 1/month in comparison to 4.33/month with no prophylactic treatment and 1.83/month with C1-INH(IV). No severe attacks and no attack within the upper airway occurred over the 16 months of C1-INH(SC) treatment. As a result, quality of life improved, as measured by the Angioedema quality of life questionaire (AE-QoL). Conclusion: Self-administered SC prophylactic use of C1-INH over a period of 16 months seems to be a well tolerated and efficient. The patient’s quality of life improved, and by learning self-application, the patient gained independence.

Anaphylaxis in Response to C1 Esterase Inhibitor in a Patient with Hereditary Angioedema

2007 ◽  
Vol 19 (4) ◽  
pp. 159-163 ◽  
Author(s):  
Marco Cicardi ◽  
Lorenza Zingale ◽  
Andrea Zanichelli ◽  
Daniela Lambertenghi Deliliers
Keyword(s):  
Hereditary Angioedema ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

scholarly journals Recombinant human C1 esterase inhibitor for hereditary angioedema attacks: A European registry

2021 ◽  
Vol 14 (4) ◽  
pp. 100535
Author(s):  
Anna Valerieva ◽  
Maria T. Staevska ◽  
Vesna Grivcheva-Panovska ◽  
Milos Jesenak ◽  
Kinga Viktória Kőhalmi ◽  
...  
Keyword(s):  
Hereditary Angioedema ◽  
C1 Esterase Inhibitor ◽  
European Registry ◽  
Esterase Inhibitor

Escalating Doses of C1 Esterase Inhibitor (CINRYZE) for Prophylaxis in Patients With Hereditary Angioedema

2014 ◽  
Vol 2 (1) ◽  
pp. 77-84.e1 ◽  
Author(s):  
Jonathan A. Bernstein ◽  
Michael E. Manning ◽  
Henry Li ◽  
Martha V. White ◽  
James Baker ◽  
...  
Keyword(s):  
Hereditary Angioedema ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

scholarly journals Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

2011 ◽  
Vol 4 ◽  
pp. S9-S21 ◽  
Author(s):  
Richard G Gower ◽  
Paula J Busse ◽  
Emel Aygören-Pürsün ◽  
Amin J Barakat ◽  
Teresa Caballero ◽  
...  
Keyword(s):  
Hereditary Angioedema ◽  
Treatment Strategies ◽  
C1 Esterase Inhibitor ◽  
Esterase Inhibitor

Etiology and predictors of cluster attacks of hereditary angioedema that recur despite pharmaceutical treatment

2021 ◽  
Vol 42 (4) ◽  
pp. 317-324
Author(s):  
Felix. A. Johnson ◽  
Magdalena Wirth ◽  
Zhaojun Zhu ◽  
Janina Hahn ◽  
Jens Greve ◽  
...  
Keyword(s):  
Hereditary Angioedema ◽  
Upper Airway ◽  
Poor Quality ◽  
Prophylactic Therapy ◽  
On Demand ◽  
C1 Esterase Inhibitor ◽  
Physical Stimuli ◽  
Inhibitor Treatment ◽  
Esterase Inhibitor

Introduction: Hereditary angioedema (HAE) is a disease that leads to recurrent swelling of the skin and mucous membranes, including the upper airway tract. Apart from being deadly, these attacks can be debilitating, which leads to a poor quality of life in patients. Clinicians are occasionally confronted with patients who have recurrent attacks despite treatment with C1 esterase inhibitor concentrate or β2-receptor antagonists. The goal of this study was to investigate repeated attacks that occur 48 hours to 7 days (“cluster attacks”) after treatment, to determine why they occur and the factors that may be associated with them, and thus to prevent their occurrence. Methods: We conducted a multicenter mixed retrospective-prospective study with data acquired from all documented attacks in our patients with collective (n = 132) between 2015 and 2018. Results: Eighty-five percent (n = 132) of our total patient collective (N = 156) agreed to participate in the study. Nine percent of these patients (n = 12) had cluster attacks, with a total of 48 cluster attacks. The data procured from the patients were mixed retrospective‐prospective. Approximately 72% of all the cluster attacks were caused by exogenous stimuli (41% due to psychological stress, 29% due to physical stimuli, and 2% due to menstruation). Cluster attacks occurred in 7% of the patients who received prophylactic therapy in comparison with 12.5% of patients who received on-demand therapy. Cluster attacks comprised 48.4% of all the attacks that patients with cluster-attacks (n= 9) experienced. In addition, the patients who were underdosing their C1 esterase inhibitor treatment had cluster attacks more often. A lower “time to repeated attack” was seen in the patients who received on-demand therapy compared with those who received prophylactic therapy. Discussion: The percentage of the patients who had attacks as a result of exogenous triggers was higher in the cluster-attack group (70.5%) compared with the general HAE population (30‐42%). Repeated attacks, therefore, were strongly associated with external triggers. The patients who received prophylactic treatment and who experienced cluster attacks were highly likely to have been underdosing, which may explain the repeated attacks despite treatment. In the patients prone to cluster attacks, prophylaxis should be considered.

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