title: A Comprehensive Approach to the Management of Children and Adults with Chronic Granulomatous Disease.
abstract: 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.
authors.affiliation: Division of Pediatric Infectious Diseases, Department of Pediatrics, Vanderbilt Vaccine Research Program, Vanderbilt University School of Medicine, Nashville, Tenn. Electronic address: isaac.thomsen@vanderbilt.edu.
authors.forename: Isaac P
authors.initials: IP
authors.lastname: Thomsen
authors.affiliation: Division of Pediatric Infectious Diseases, Department of Pediatrics, Vanderbilt Vaccine Research Program, Vanderbilt University School of Medicine, Nashville, Tenn.
authors.forename: Meaghan A
authors.initials: MA
authors.lastname: Smith
authors.affiliation: Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
authors.affiliation: Division of Pediatric Infectious Diseases, Department of Pediatrics, Vanderbilt Vaccine Research Program, Vanderbilt University School of Medicine, Nashville, Tenn.
authors.forename: C Buddy
authors.initials: CB
authors.lastname: Creech
mesh_terms: D000328:Adult; D001424:Bacterial Infections / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D002648:Child; D006105:Granulomatous Disease, Chronic / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D009181:Mycoses / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology
keywords: Chronic granulomatous disease; IFN-γ; Multidisciplinary care