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Drug-Induced Sleep Endoscopy (DISE) with Target Controlled Infusion (TCI) and Bispectral Analysis in Obstructive Sleep Apnea
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Disordered breathing during sleep in patients with mucopolysaccharidoses

S E Leighton1, B Papsin, A Vellodi

  • 1Department of Otolaryngology, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH, UK. kate.king@virgin.net

International Journal of Pediatric Otorhinolaryngology
|March 30, 2001
PubMed

Insights

Obstructive sleep apnoea (OSA) is common in children with mucopolysaccharidoses (MPS), varying by MPS type. Objective sleep studies are crucial for accurate OSA assessment in these patients.

Area of Science:

  • Pediatric Pulmonology
  • Sleep Medicine
  • Rare Genetic Diseases

Background:

  • Obstructive sleep apnoea (OSA) is a known complication in children with mucopolysaccharidoses (MPS).
  • The precise incidence and severity of OSA across different MPS types remain poorly understood.
  • Objective measurement of OSA is needed for effective management.

Purpose of the Study:

  • To objectively quantify the degree of OSA in children with various MPS syndromes.
  • To compare OSA severity across different types of MPS.
  • To evaluate the accuracy of clinical history in assessing OSA severity.

Main Methods:

  • A cross-sectional study involving 26 children with MPS.
  • Cardiopulmonary sleep studies were conducted during unsedated sleep over two years.
  • OSA severity was scored using both clinical history and objective sleep study data.

Main Results:

  • OSA was diagnosed in 24 out of 26 patients (92%), with severity ranging from mild to severe.
  • OSA was most pronounced in MPS type I H (Hurler syndrome), followed by types I HS (Hurler-Scheie syndrome) and II (Hunter syndrome).
  • Clinical history underestimated severe OSA in 5 cases and overestimated mild OSA in 4 cases, showing poor agreement (65%) with objective findings.

Conclusions:

  • Obstructive respiratory issues are highly prevalent in MPS patients, with significant variation in OSA severity among MPS types.
  • Clinical assessment alone is insufficient for accurately determining OSA severity in MPS.
  • Objective sleep studies are essential for evaluating MPS patients, monitoring outcomes, and assessing therapeutic interventions.
Abstract

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