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
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.
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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