Literature DB >> 33231165

Successful treatment of a child with Schwartz-Jampel syndrome using rapid maxillary expansion and CPAP.

Supakit Peanchitlertkajorn1, Thanate Assawakawintip1, Mevadee Pibulniyom2, Panida Srisan3, Patimaporn Pungchanchaikul4, Rasintra Jaroenying5.   

Abstract

STUDY
OBJECTIVES: We reported an 8-year-old male patient with Schwartz-Jampel syndrome, severe obstructive sleep apnea, constricted maxilla and moderate tonsillar hypertrophy. The syndrome is characterized by myotonia, skeletal dysplasia, and facial dysmorphism.
METHODS: CPAP was initially prescribed, but he was not able to tolerate due to a high pressure setting. Rapid maxillary expansion alone reduced AHI to 10.4 events/h.
RESULTS: When combined with CPAP, AHI is further reduced to 2.4 events/h. The patient has a better compliance with CPAP following rapid maxillary expansion therapy as the pressure setting decreased.
CONCLUSIONS: This is the first report utilizing a combination of rapid maxillary expansion and CPAP therapy to successfully treat severe pediatric OSA.
© 2021 American Academy of Sleep Medicine.

Entities:  

Keywords:  Schwartz-Jampel syndrome; orthodontic treatment; pediatric OSA; rapid maxillary expansion; rapid palatal expansion

Mesh:

Year:  2021        PMID: 33231165      PMCID: PMC7927341          DOI: 10.5664/jcsm.9028

Source DB:  PubMed          Journal:  J Clin Sleep Med        ISSN: 1550-9389            Impact factor:   4.062


  10 in total

1.  Long-term effects of orthodontic therapy on the maxillary dental arch and nasal cavity.

Authors:  Nanci L O De Felippe; Neeta Bhushan; Adriana C Da Silveira; Grace Viana; Bonnie Smith
Journal:  Am J Orthod Dentofacial Orthop       Date:  2009-10       Impact factor: 2.650

Review 2.  Update on pathophysiology and treatment of childhood obstructive sleep apnea syndrome.

Authors:  Justin C Brockbank
Journal:  Paediatr Respir Rev       Date:  2017-06-12       Impact factor: 2.726

3.  Perlecan, the major proteoglycan of basement membranes, is altered in patients with Schwartz-Jampel syndrome (chondrodystrophic myotonia).

Authors:  S Nicole; C S Davoine; H Topaloglu; L Cattolico; D Barral; P Beighton; C B Hamida; H Hammouda; C Cruaud; P S White; D Samson; J A Urtizberea; F Lehmann-Horn; J Weissenbach; F Hentati; B Fontaine
Journal:  Nat Genet       Date:  2000-12       Impact factor: 38.330

Review 4.  Rapid maxillary expansion for pediatric obstructive sleep apnea: A systematic review and meta-analysis.

Authors:  Macario Camacho; Edward T Chang; Sungjin A Song; Jose Abdullatif; Soroush Zaghi; Paola Pirelli; Victor Certal; Christian Guilleminault
Journal:  Laryngoscope       Date:  2016-10-31       Impact factor: 3.325

5.  Schwartz-Jampel syndrome (chondrodystrophic myotonia).

Authors:  D Viljoen; P Beighton
Journal:  J Med Genet       Date:  1992-01       Impact factor: 6.318

6.  Obstructive sleep apnea in Schwartz-Jampel syndrome.

Authors:  S P Cook; W J Borkowski
Journal:  Arch Otolaryngol Head Neck Surg       Date:  1997-12

7.  Obstructive Sleep Apnoea Syndrome (OSAS) and rhino-tubaric disfunction in children: therapeutic effects of RME therapy.

Authors:  Paola Pirelli; Maurizio Saponara; Giuseppe Attanasio
Journal:  Prog Orthod       Date:  2005       Impact factor: 2.750

8.  Adherence to and effectiveness of positive airway pressure therapy in children with obstructive sleep apnea.

Authors:  Carole L Marcus; Gerald Rosen; Sally L Davidson Ward; Ann C Halbower; Laura Sterni; Janita Lutz; Pamela J Stading; Daisy Bolduc; Nancy Gordon
Journal:  Pediatrics       Date:  2006-03       Impact factor: 7.124

9.  NREM sleep instability changes following rapid maxillary expansion in children with obstructive apnea sleep syndrome.

Authors:  Silvia Miano; Alessandra Rizzoli; Melania Evangelisti; Oliviero Bruni; Raffaele Ferri; Jacopo Pagani; Maria Pia Villa
Journal:  Sleep Med       Date:  2008-08-26       Impact factor: 3.492

10.  Clinico-pathogenetic findings and management of chondrodystrophic myotonia (Schwartz-Jampel syndrome): a case report.

Authors:  Nicola C Ho; Stacey Sandusky; Victor Madike; Clair A Francomano; Marinos C Dalakas
Journal:  BMC Neurol       Date:  2003-07-02       Impact factor: 2.474

  10 in total

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