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Respiratory and sleep disorders in mucopolysaccharidosis

Kenneth I Berger1, Simone C Fagondes, Roberto Giugliani

  • 1Department Medicine, Physiology and Neuroscience, André Cournand Pulmonary Physiology Laboratory, Bellevue Hospital, New York University School of Medicine, New York, NY, USA. Kenneth.Berger@nyumc.org

Journal of Inherited Metabolic Disease
|November 16, 2012
PubMed

Insights

Mucopolysaccharidoses (MPS) cause glycosaminoglycan (GAG) buildup, leading to common ear, nose, throat (ENT), and respiratory issues. This review details these manifestations and their management in MPS patients.

Area of Science:

  • Biochemistry
  • Genetics
  • Pediatrics

Background:

  • Mucopolysaccharidoses (MPS) are rare genetic lysosomal storage disorders.
  • Characterized by the accumulation of glycosaminoglycans (GAGs) in various tissues.
  • This accumulation leads to progressive multi-systemic clinical manifestations.

Purpose of the Study:

  • To provide a comprehensive overview of Ear, Nose, and Throat (ENT) and respiratory problems in MPS.
  • To discuss the evaluation and management strategies for these manifestations.
  • To highlight the early and common occurrence of ENT and respiratory symptoms in MPS patients.

Main Methods:

  • Literature review of existing studies on MPS.
  • Analysis of clinical manifestations related to ENT and respiratory systems.
  • Discussion of diagnostic and therapeutic approaches.

Main Results:

  • ENT and respiratory issues are highly prevalent in MPS, often presenting early.
  • Common manifestations include airway obstruction, restrictive pulmonary disease, chronic infections, and sleep apnea.
  • These symptoms significantly impact quality of life and can lead to severe complications like respiratory failure.

Conclusions:

  • ENT and respiratory problems are critical components of MPS clinical presentation.
  • Timely evaluation and appropriate management are essential for improving patient outcomes.
  • Further research into specialized interventions for these manifestations is warranted.

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