Indian Journal of Sleep Medicine

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VOLUME 17 , ISSUE 1 ( January-March, 2022 ) > List of Articles

CASE REPORT

Management of Severe Obstructive Sleep Apnea in Pediatric Patients with Pycnodysostosis: A Report of Two Cases

Dhruv Jain, Sanjeev Datana, SS Agarwal, Indranil D Roy

Keywords : Obstructive sleep apnea, Oral appliance therapy, Pycnodysostosis

Citation Information : Jain D, Datana S, Agarwal S, Roy ID. Management of Severe Obstructive Sleep Apnea in Pediatric Patients with Pycnodysostosis: A Report of Two Cases. Indian Sleep Med 2022; 17 (1):22-31.

DOI: 10.5005/jp-journals-10069-0091

License: CC BY-NC 4.0

Published Online: 30-04-2022

Copyright Statement:  Copyright © 2022; The Author(s).


Abstract

Pycnodysostosis (PKND) is a rare autosomal recessive disorder caused by molecular mutation in cathepsin K (CTSK) gene, resulting in decreased bone turnover with enfeeblement of bone structure. This report addresses an array of systemic and craniofacial features of two siblings affected with PKND with an association of severe obstructive sleep apnea (OSA), managed conservatively with an oral mandibular advancement appliance.


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