The authors evaluated the efficacy of short-term danazol prophylaxis in 12 hereditary angioedema patients undergoing maxillofacial or dental procedures. Hereditary angioedema is an autosomal dominant disorder resulting from the deficiency of the C1 esterase inhibitor. Characteristic clinical manifestations include the formation of subcutaneous and submucosal edema. The latter can lead to fatal upper airway obstruction. Edematous attacks can be precipitated by tissue injury from operative procedures, dental surgery in particular. In patients undergoing surgical and diagnostic procedures performed in the cephalic and cervical regions, such attacks can be prevented by appropriate drug therapy. Short-term danazol prophylaxis was effective in all 12 patients with a history of edematous complications that had occurred after dental procedures; none of them developed postoperative edema. The serum levels of complement components determined pre- and postoperatively as well as at 6- and 12-hour intervals also demonstrated the efficacy of prophylactic therapy.
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Allergol Select
November 2024
Department of Oto-Rhino-Laryngology. Head and Neck Surgery, Ulm University Medical Center, Ulm.
Background And Objectives: Patients with rare diseases like hereditary angioedema (HAE) are usually referred to an angioedema center to ensure guideline-compliant and experience-based therapy. Even though there are established guidelines and several approved therapeutics, there are still open questions and situations in the daily care of HAE patients, where an exchange between centers is needed.
Materials And Methods: A survey was conducted among physicians from German angioedema centers regarding challenges and issues in everyday HAE treatment.
World Allergy Organ J
September 2023
Specialty in Internal Medicine, Allergy and Clinical Immunology Master's Degree in History. Allergy and Immunology Department, Centro Médico Nacional 20 de Noviembre, ISSSTE, CDMX, Mexico.
Introduction And Objectives: Due to the lack of structured and systematic information available, the aim of this study was to describe the epidemiology, diagnosis, healthcare processes, and treatment patterns of hereditary angioedema (HAE) in Mexico. To achieve this, different data sources were consulted regarding medical literature, structured health system databases, and angioedema-specialized physicians (AEP) opinion regarding HAE.
Material And Methods: A mixed methods approach was conducted in 4 phases: I) systematic literature review (SLR) and meta-analysis according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines; II) review of national health system (NHS) databases and systematic reports; III) physician survey; and IV) an epidemiologic model.
Asia Pac Allergy
June 2023
Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Hereditary angioedema (HAE) is an uncommon disorder characterized clinically by recurrent episodes of nonitchy subcutaneous and/or submucosal swellings. The estimated prevalence of HAE is ~ 1: 10,000 to 1: 50,000. There are no prevalence data from India, however, estimates suggest that there are 27,000 to 135,000 patients with HAE in India at present.
View Article and Find Full Text PDFRev Med Interne
July 2023
Sorbonne université, AP-HP, service de médecine interne, hôpital Saint-Antoine, 75012 Paris, France.
Hereditary angioedema, with or without deficient C1 inhibitor level or function, is a rare disease characterized by recurrent attacks of noninflammatory subcutaneous and/or submucosal edema. It may be life-threatening and substantially affects quality of life. Attacks may be spontaneous or induced, in a setting of emotional stress, by infections or physical trauma, in particular.
View Article and Find Full Text PDFExpert Opin Drug Saf
January 2023
Department of Allergy, Asthma, and Immunology, Professor of Medicine, Pediatrics, and Biomedical Sciences, Hershey, PA, USA.
Introduction: Hereditary Angioedema (HAE) attacks show an increased frequency and severity for pregnant and lactating females secondary to the hormonal changes. The diagnosis and management of HAE in pregnant and lactating females pose a challenge for physicians due to the rarity of the disease and the paucity of the data for specific management.
Areas Covered: In this manuscript, we discuss the diagnosis and special presentation of HAE types 1 and 2 in pregnant and lactating females, including acute management, short-term prophylaxis, long-term prophylaxis, and drugs that should be avoided.
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