Sleep-disordered breathing and nocturnal hypoventilation in children with the MECP2 duplication syndrome: A case series and review of the literature

Am J Med Genet A. 2020 Oct;182(10):2437-2441. doi: 10.1002/ajmg.a.61790. Epub 2020 Aug 23.

Abstract

There is limited knowledge on the occurrence of respiratory manifestations and sleep-disordered breathing in particular in children with the MECP2 duplication syndrome. Although sleep-disordered breathing and nocturnal hypoventilation are currently not cited as an important symptom in these children, we present three cases who all had an abnormal breathing during sleep. In view of the consequences associated with sleep apnea and hypoventilation, we advise to perform a polysomnography in children with MECP2 duplication. Different treatment modalities (ENT surgery, CPAP, and non-invasive ventilation) can be applied to successfully treat these conditions.

Keywords: MECP2 duplication syndrome; hypoventilation; non-invasive ventilation; obstructive sleep apnea; respiratory infections; sleep-disordered breathing.

Publication types

  • Case Reports

MeSH terms

  • Genetic Predisposition to Disease
  • Humans
  • Hypoventilation / diagnostic imaging
  • Hypoventilation / genetics*
  • Hypoventilation / pathology
  • Infant
  • Infant, Newborn
  • Male
  • Mental Retardation, X-Linked / diagnostic imaging
  • Mental Retardation, X-Linked / genetics*
  • Mental Retardation, X-Linked / pathology
  • Methyl-CpG-Binding Protein 2 / genetics*
  • Sleep Apnea Syndromes / diagnostic imaging
  • Sleep Apnea Syndromes / genetics*
  • Sleep Apnea Syndromes / pathology

Substances

  • MECP2 protein, human
  • Methyl-CpG-Binding Protein 2

Supplementary concepts

  • Lubs X-linked mental retardation syndrome