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Sleep Breath. 2014 Mar;18(1):143-9. doi: 10.1007/s11325-013-0862-z. Epub 2013 May 22.

Home sleep study characteristics in patients with mucopolysaccharidosis.

Author information

1
Pediatric Metabolic Unit, Gazi University Hospital, Ankara, Turkey, cskasapkara@gmail.com.

Abstract

BACKGROUND:

Mucopolysaccharidoses (MPS) are a group of inherited lysosomal storage disorders caused by the deficiency of hydrolases involved in the degradative pathway of glycosaminoglycans. In MPS, upper airway obstruction may result from multiple causative factors which may impact severely upon morbidity and mortality.

METHODS:

We evaluated upper airway obstructive disease and related clinical findings through home sleep study in 19 patients (11 with MPS VI, 4 with MPS I, 4 with MPS II) with MPS followed at Gazi University Pediatric Metabolic Unit. Patients underwent home-based sleep measurements, and sleep respiratory problems were asked in a detailed clinical history. Measurements of apnea, apnea-hypopnea index (AHI), hypopnea index, oxygen desaturation index, and minimal oxygen saturation were obtained through home sleep study.

RESULTS:

For 19 children, the disorder was normal in 1, mild (AHI=1.5-5/h) in 5, moderate (AHI=5-10/h) in 2, and severe (AHI>10/h) in 11. The prevalence of OSA was 94.7 % (18/19) in patients with MPS. Snoring, witnessed apnea, pectus carinatum, and macroglossia were the main clinical findings. Echocardiograms showed evidence of pulmonary hypertension in 13 patients.

CONCLUSION:

Home sleep study is a quick and accessible screening test to determine the abnormalities of breathing during sleep and enables clinicians to take necessary action for patients with severe manifestations.

PMID:
23690022
DOI:
10.1007/s11325-013-0862-z
[Indexed for MEDLINE]

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