The Indian Journal of Sleep Medicine
  • Year: 2006
  • Volume: 1
  • Issue: 3

OSA in a patient with mucopolysaccharidosis MPS is (Scheie Syndrome)

  • Author:
  • D. Bhattacharya, N. K. Gupta, S. Chakraborti, M. K. Sen, J. C. Suri
  • Total Page Count: 5
  • Page Number: 161 to 165

Department of Pulmonary, Critical Care & Sleep Medicine, Vardhman Mahavir Medical College & Safdarjang Hospital, New Delhi.

Abstract

A rare case of metabolic disease - mucopoly saccharidosis in a 10 years old child with SDB is being presented. There was history of snoring, irritability and inattentiveness. Though ENT examination revealed retrognathia and a large tongue with bulky pharyngeal walls and pillars, there was no evidence of tonsils or adenoids. Polysomnography done revealed features of significant sleep disordered breathing.

Keywords

MPS: Mucopolysaccharidosis, PSG: polysomnography, OSA: Obstructive sleep apnea, AHI: Apnea hypopnea index