Universal Access to On-Demand Treatment of Patients with Hereditary Angioedema, the Chilean Experience.

IF 1.1 4区医学 Q4 ALLERGY

Pediatric Allergy Immunology and Pulmonology Pub Date : 2023-12-01 Epub Date: 2023-11-02 DOI:10.1089/ped.2023.0083

Juan J Escobar, Joaquín Aguirre, Samuel Ibáñez, Bárbara J Cid, Rolando Campillay, Ana María Gallardo, Masumi Grau, Rodrigo Hoyos-Bachiloglu

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引用次数: 0

Abstract

Background: In Chile, patients with hereditary angioedema (HAE) type I and type II are protected under Ley Ricarte Soto (LRS), which guarantees access to on demand plasma-derived C1-INH (pdC1-INH) since 2018. We aimed to analyze the first 3 years of LRS. Methods: Review of the LRS database between 2018 and 2021. Results: During the study period, 154 patients were covered by LRS, with an estimated prevalence of HAE in Chile at 0.8:100,000 inhabitants. A delay in diagnosis of 22 years was noted, 50 patients received epinephrine during an attack before the diagnosis of HAE. Mean number of attacks per year was 8, with 50% of adults and 42% of children experiencing more than 1 attack per month. Conclusion: Disease awareness must improve to reduce the diagnostic delay of HAE. Long-term prophylactic medications should be included in LRS to treat patients with high attack rates and control the costs of frequent on-demand treatment with pdC1-INH.

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普及遗传性血管水肿患者的按需治疗，智利经验。

背景：在智利，遗传性血管性水肿（HAE）I型和II型患者受到Ley Ricarte Soto（LRS）的保护，该药物自2018年以来保证了获得按需血浆来源的C1-INH（pdC1-ISH）。我们旨在分析LRS的前3年。方法：回顾2018年至2021年间的LRS数据库。结果：在研究期间，154名患者被LRS覆盖，估计智利HAE的患病率为0.8:100000居民。诊断延迟了22年，50名患者在诊断为HAE之前的一次发作中接受了肾上腺素治疗。每年平均发作次数为8次，50%的成年人和42%的儿童每月发作1次以上。结论：必须提高疾病意识，减少HAE的诊断延误。LRS中应包括长期预防性药物，以治疗高发病率的患者，并控制pdC1 INH频繁按需治疗的成本。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Allergy Immunology and Pulmonology ALLERGY-IMMUNOLOGY

CiteScore

2.00

自引率

0.00%

发文量

审稿时长

>12 weeks

期刊介绍： Pediatric Allergy, Immunology, and Pulmonology is a peer-reviewed journal designed to promote understanding and advance the treatment of respiratory, allergic, and immunologic diseases in children. The Journal delivers original translational, clinical, and epidemiologic research on the most common chronic illnesses of children—asthma and allergies—as well as many less common and rare diseases. It emphasizes the developmental implications of the morphological, physiological, pharmacological, and sociological components of these problems, as well as the impact of disease processes on families. Pediatric Allergy, Immunology, and Pulmonology coverage includes: -Functional and genetic immune deficiencies- Interstitial lung diseases- Both common and rare respiratory, allergic, and immunologic diseases- Patient care- Patient education research- Public health policy- International health studies