Antibiotic Prophylaxis, Immunoglobulin Substitution and Supportive Measures Prevent Infections in MECP2 Duplication Syndrome

Pediatr Infect Dis J. 2018 May;37(5):466-468. doi: 10.1097/INF.0000000000001799.

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.

Publication types

  • Case Reports

MeSH terms

  • Administration, Intravenous
  • Anti-Bacterial Agents / administration & dosage
  • Antibiotic Prophylaxis*
  • Cefuroxime / administration & dosage
  • Humans
  • IgA Deficiency
  • IgG Deficiency
  • Immunoglobulin M / deficiency
  • Immunoglobulins / administration & dosage*
  • Levofloxacin / administration & dosage
  • Male
  • Mental Retardation, X-Linked / drug therapy*
  • Mental Retardation, X-Linked / microbiology
  • Pneumonia / microbiology
  • Pneumonia / prevention & control*
  • Sepsis
  • Young Adult

Substances

  • Anti-Bacterial Agents
  • Immunoglobulin M
  • Immunoglobulins
  • Levofloxacin
  • Cefuroxime

Supplementary concepts

  • Lubs X-linked mental retardation syndrome