Congenital central hypoventilation syndrome and desogestrel: a call for caution: addendum to "C. Straus, H. Trang, M.H. Becquemin, P. Touraine, T. Similowski, Chemosensitivity recovery in Ondine's curse syndrome under treatment with desogestrel" [Respir. Physiol. Neurobiol. 171 (2010) 171-174]

Respir Physiol Neurobiol. 2011 Sep 15;178(2):357-8. doi: 10.1016/j.resp.2011.07.007. Epub 2011 Jul 23.

Abstract

Patients suffering from congenital central hypoventilation syndrome (CCHS) depend on mechanical ventilation during sleep, from birth and throughout life. They lack CO₂-chemosensitivity. Hope has recently been raised by serendipitous observations of chemosensitivity recovery under treatment by desogestrel, a very potent progestin (Straus et al., 2010). Caution is however needed, because this effect could depend on dose, idiosyncrasies, or be transient. Desogestrel should not be prescribed to CCHS patients with a respiratory purpose until the results of a pending clinical trial (clinicaltrials.gov. NCT01243697) are available.

Publication types

  • Review

MeSH terms

  • Carbon Dioxide
  • Chemoreceptor Cells / drug effects
  • Chemoreceptor Cells / physiology*
  • Contraindications
  • Desogestrel / therapeutic use*
  • Humans
  • Hypoventilation / congenital*
  • Hypoventilation / drug therapy
  • Hypoventilation / physiopathology
  • Recovery of Function / drug effects
  • Recovery of Function / physiology*
  • Sleep Apnea, Central / drug therapy*
  • Sleep Apnea, Central / physiopathology
  • Treatment Outcome

Substances

  • Carbon Dioxide
  • Desogestrel

Supplementary concepts

  • Congenital central hypoventilation syndrome

Associated data

  • ClinicalTrials.gov/NCT01243697