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Gabos, Gabriella, Nadasan, Valentin, Mihaly, Eniko, Dobru, Daniela. (1399). Hereditary Angioedema Due to C1-Inhibitor Deficiency in Romania: First National Study, Diagnostic and Treatment Challenges. سامانه مدیریت نشریات علمی, 17(3), 226-235. doi: 10.22034/iji.2020.85416.1709
Gabriella Gabos; Valentin Nadasan; Eniko Mihaly; Daniela Dobru. "Hereditary Angioedema Due to C1-Inhibitor Deficiency in Romania: First National Study, Diagnostic and Treatment Challenges". سامانه مدیریت نشریات علمی, 17, 3, 1399, 226-235. doi: 10.22034/iji.2020.85416.1709
Gabos, Gabriella, Nadasan, Valentin, Mihaly, Eniko, Dobru, Daniela. (1399). 'Hereditary Angioedema Due to C1-Inhibitor Deficiency in Romania: First National Study, Diagnostic and Treatment Challenges', سامانه مدیریت نشریات علمی, 17(3), pp. 226-235. doi: 10.22034/iji.2020.85416.1709
Gabos, Gabriella, Nadasan, Valentin, Mihaly, Eniko, Dobru, Daniela. Hereditary Angioedema Due to C1-Inhibitor Deficiency in Romania: First National Study, Diagnostic and Treatment Challenges. سامانه مدیریت نشریات علمی, 1399; 17(3): 226-235. doi: 10.22034/iji.2020.85416.1709

Hereditary Angioedema Due to C1-Inhibitor Deficiency in Romania: First National Study, Diagnostic and Treatment Challenges

Iranian Journal of Immunology
دوره 17، شماره 3، آذر 2020، صفحه 226-235    اصل مقاله (891.51 K)
نوع مقاله: Original Article
شناسه دیجیتال (DOI): 10.22034/iji.2020.85416.1709
نویسندگان
Gabriella Gabos1؛ Valentin Nadasan* 2؛ Eniko Mihaly3؛ Daniela Dobru4
1Lotus Life Clinic, Târgu Mureș, Romania
2Romanian Network for Hereditary Angioedema; Department of Hygiene, George Emil Palade University of Medicine, Pharmacy, Science, and Technology of Targu Mures, Romania.
3Allergology and Immunology Department, Mures County Hospital, Targu Mures, Romania
4Gastroenterology Department, Mures County Hospital, Targu Mures, Romania; Department of Internal Medicine VII, George Emil Palade University of Medicine, Pharmacy, Science, and Technology of Targu Mures, Romania
چکیده
Background: Hereditary angioedema (HAE) is a rare genetic potentially life-threatening disease characterized by episodic non-pruritic subcutaneous and submucosal edema attacks in different parts of the body. Objective: To assess the status of Romanian HAE patients after the recent introduction of a new therapy through a nationwide program. Methods: This cross-sectional observational study included patients from the Romanian HAE Registry. Results: The study included 84 patients with HAE type I (91.7%) and type II (8.3%). The mean delay in diagnosis was 2.4 years in children and 16.7 years in adults (p=0.019). Stress and tiredness were the most frequent trigger factors. The majority of the HAE episodes involved subcutaneous (89.3%), abdominal (77.4%), genital (51.2%), facial (41.7%), and laryngeal (39.3%) symptoms during the preceding 12 months. One or several misdiagnoses were reported in 83.33% patients and 44.1 % of the patients were subjected to or proposed unnecessary surgery during abdominal episodes. Plasma-derived C1-INH (pdC1-INH) and recombinant C1-INH (rhC1-INH) were respectively used in 10 (11.9%) and 13 (15.5%) of the HAE patients for life-threatening attacks over the past 12 months. Fortythree (51.19%) patients practiced home treatment with subcutaneous injection of the bradykinin B2-receptor antagonist for acute HAE attacks. Conclusion: The significantly lower delay observed in children suggests an improvement in the awareness of C1-INH-HAE among physicians in recent years. The management of HAE in Romania has been somewhat enhanced as the majority of HAE patients have recently gained access to pdC1-INH, rhC1-INH, and bradykinin B2-receptor antagonist.
کلیدواژه‌ها
Angioedema؛ Diagnostic Errors؛ Hereditary؛ Romania
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