Immunology ReportsAntibiotic Prophylaxis, Immunoglobulin Substitution and Supportive Measures Prevent Infections in MECP2 Duplication SyndromeBauer, Michael MD*; Krüger, Renate MD*; Kölsch, Uwe MD†; Unterwalder, Nadine MD†; Meisel, Christian MD†,‡; Wahn, Volker MD*; von Bernuth, Horst MD*,†,‡,§Author Information From the *Pediatric Pneumology, Immunology and Intensive Care Medicine, Charité University Medicine, Berlin, Germany †Department of Immunology, Labor Berlin GmbH, Berlin, Germany ‡Brandenburg Center for Regenerative Therapies (BCRT), Charité University Medicine, Berlin, Germany §Sozialpädiatrisches Zentrum, Charité University Medicine, Berlin, Germany. Accepted for publication June 27, 2017. The authors have no funding or conflicts of interest to disclose. Address for correspondence: Michael Bauer, MD, Charité – Kinderklinik mit Schwerpunkt Pneumologie und Immunologie, Augustenburger Platz 1, 13353 Berlin, Germany. E-mail: [email protected]. The Pediatric Infectious Disease Journal: May 2018 - Volume 37 - Issue 5 - p 466-468 doi: 10.1097/INF.0000000000001799 Buy Metrics Abstract Respiratory infections are the main cause of early death in patients with MECP2 duplication syndrome. We report on a 20-year-old patient with MECP2 duplication syndrome, IgG2/IgG4/IgA/IgM deficiency and polysaccharide-specific antibody deficiency, who had 46 episodes of pneumonia in his first 13 8/12 years of life. Immunoglobulin substitution, daily antibiotic prophylaxis with two agents and supportive measures reduced occurrence of pneumonia to four episodes in the following 6 2/12 years of life. Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.