慢性肉芽肿病患儿和成人管理的综合方法。
A Comprehensive Approach to the Management of Children and Adults with Chronic Granulomatous Disease.
机构信息
Division of Pediatric Infectious Diseases, Department of Pediatrics, Vanderbilt Vaccine Research Program, Vanderbilt University School of Medicine, Nashville, Tenn.
Division of Pediatric Infectious Diseases, Department of Pediatrics, Vanderbilt Vaccine Research Program, Vanderbilt University School of Medicine, Nashville, Tenn.
出版信息
J Allergy Clin Immunol Pract. 2016 Nov-Dec;4(6):1082-1088. doi: 10.1016/j.jaip.2016.03.021. Epub 2016 May 10.
Chronic granulomatous disease (CGD), a disease characterized by inadequate neutrophil killing of microbial pathogens, affects 4 to 5 per million live births. For many decades following its description, CGD was a fatal disease in childhood. With the development of effective preventive therapies and the early recognition of infectious complications, 90% of children with CGD now survive into adulthood. The management of CGD in adults includes unique challenges and potential disease manifestations. In this article, the authors discuss the current approach to the management of CGD in both children and adults. This includes a focus on the importance of a comprehensive multidisciplinary approach in the care of CGD and its potential complications. In addition, a novel approach to improving education about CGD, and subsequently improving adherence to preventive therapies, is discussed.
慢性肉芽肿病（CGD）是一种以中性粒细胞不能有效杀灭微生物病原体为特征的疾病，每百万活产儿中有 4 至 5 例发病。在该病被描述后的几十年里，CGD 曾是儿童期的一种致命疾病。随着有效预防疗法的发展和对感染性并发症的早期认识，现在 90%的 CGD 患儿能够存活至成年。成人 CGD 的管理包括独特的挑战和潜在的疾病表现。本文作者讨论了目前在儿童和成人 CGD 管理方面的方法。这包括重点关注综合多学科方法在 CGD 及其潜在并发症治疗中的重要性。此外，还讨论了一种提高 CGD 教育水平、进而提高预防治疗依从性的新方法。
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