Juan J Escobar, Joaquín Aguirre, Samuel Ibáñez, Bárbara J Cid, Rolando Campillay, Ana María Gallardo, Masumi Grau, Rodrigo Hoyos-Bachiloglu
{"title":"普及遗传性血管水肿患者的按需治疗,智利经验。","authors":"Juan J Escobar, Joaquín Aguirre, Samuel Ibáñez, Bárbara J Cid, Rolando Campillay, Ana María Gallardo, Masumi Grau, Rodrigo Hoyos-Bachiloglu","doi":"10.1089/ped.2023.0083","DOIUrl":null,"url":null,"abstract":"<p><p><b><i>Background:</i></b> 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. <b><i>Methods:</i></b> Review of the LRS database between 2018 and 2021. <b><i>Results:</i></b> 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. <b><i>Conclusion:</i></b> 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.</p>","PeriodicalId":54389,"journal":{"name":"Pediatric Allergy Immunology and Pulmonology","volume":" ","pages":"130-132"},"PeriodicalIF":1.1000,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Universal Access to On-Demand Treatment of Patients with Hereditary Angioedema, the Chilean Experience.\",\"authors\":\"Juan J Escobar, Joaquín Aguirre, Samuel Ibáñez, Bárbara J Cid, Rolando Campillay, Ana María Gallardo, Masumi Grau, Rodrigo Hoyos-Bachiloglu\",\"doi\":\"10.1089/ped.2023.0083\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p><b><i>Background:</i></b> 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. <b><i>Methods:</i></b> Review of the LRS database between 2018 and 2021. <b><i>Results:</i></b> 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. <b><i>Conclusion:</i></b> 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.</p>\",\"PeriodicalId\":54389,\"journal\":{\"name\":\"Pediatric Allergy Immunology and Pulmonology\",\"volume\":\" \",\"pages\":\"130-132\"},\"PeriodicalIF\":1.1000,\"publicationDate\":\"2023-12-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Pediatric Allergy Immunology and Pulmonology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1089/ped.2023.0083\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2023/11/2 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"ALLERGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric Allergy Immunology and Pulmonology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1089/ped.2023.0083","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2023/11/2 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"ALLERGY","Score":null,"Total":0}
Universal Access to On-Demand Treatment of Patients with Hereditary Angioedema, the Chilean Experience.
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.
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