Basic and clinical immunologyHereditary angioedema: A decade of human C1-inhibitor concentrate therapy
Section snippets
Patients
Relevant data of 112 patients treated at the center18 since 1996 were analyzed (Table I). At the initial visit, patients diagnosed with HAE—established by clinical manifestations and complement screening—were invited by mail to attend control visits, scheduled at 1-year intervals at least. The disease severity class of patients was determined according to the criteria for evaluating disease severity, developed by experts from the Novel Methods for Predicting, Preventing, and Treating Attacks in
Results
Of the 112 individuals managed at the center, 61 (including 22 children) had received hC1-INH concentrate intravenously during their life. Fifty-one patients had never been treated with this agent, and 5 had never had an edematous episode. These 2 subsets (ie, patients treated vs not treated with hC1-INH concentrate) were similar with regard to age, sex, ratio of children/adults, type of HAE, and the time of onset of initial symptoms. In the subset treated with hC1-INH concentrate, HAE was
Discussion
In many countries, C1-INH concentrate is still unavailable; therefore, patients with HAE receive either symptomatic treatments22 or fresh frozen plasma.23, 24, 25 However, the use of fresh frozen plasma is associated with a relatively high risk of alloimmunization,26 anaphylactoid reactions,27 transfer of viral infections,28 and transfusion-related acute lung injury.29 Moreover, human plasma contains a variety of components that are undesirable during use for this purpose. Administration of a
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Cited by (118)
Efficacy of human C1 esterase inhibitor concentrate for treatment of ACE-inhibitor induced angioedema
2023, American Journal of Emergency MedicineExperience with Intravenous Plasma-Derived C1-Inhibitor in Pregnant Women with Hereditary Angioedema: A Systematic Literature Review
2020, Journal of Allergy and Clinical Immunology: In PracticeCitation Excerpt :Of these, 69 articles were excluded because they did not have data about C1-INH use during pregnancy (51 articles) or were not written in English (16 articles). Two additional articles were preliminary reports of cases21,22 that were more completely described in later reports,17,23 so the preliminary reports were excluded. Of the 16 articles that were not written in English, 5 had no associated abstract, and 8 had an English abstract that did not include data about the use of C1-INH in pregnancy.
Repeated attacks of type III hereditary angioedema with factor XII mutation during pregnancy
2018, International Journal of Obstetric AnesthesiaEfficacy of C1 inhibitor concentrate in hereditary angioedema with C1 inhibitor deficiency: Analysis in a French cohort
2018, Annals of Allergy, Asthma and ImmunologyOverview of Laboratory Testing and Clinical Presentations of Complement Deficiencies and Dysregulation
2016, Advances in Clinical Chemistry
Disclosure of potential conflict of interest: The authors have declared that they have no conflict of interest.