Obstructive sleep apnea in Schwartz-Jampel syndrome
| Autor: | Steven P. Cook, Winslow J. Borkowski |
|---|---|
| Rok vydání: | 1997 |
| Předmět: |
Adult
Male Schwartz–Jampel syndrome medicine.medical_treatment Polysomnography Osteochondrodysplasias Sleep Apnea Syndromes Positive airway pressure medicine Humans medicine.diagnostic_test Respiratory distress business.industry Palate General Medicine Airway obstruction medicine.disease Myotonia respiratory tract diseases Tonsillectomy Obstructive sleep apnea stomatognathic diseases Otorhinolaryngology Anesthesia Pharynx Surgery business |
| Zdroj: | Archives of otolaryngology--headneck surgery. 123(12) |
| ISSN: | 0886-4470 |
| Popis: | Schwartz-Jampel syndrome (SJS) is a rare entity characterized by myotonia and skeletal abnormalities. Death and respiratory distress have previously been reported in newborns and young children with SJS. We describe a patient with SJS and snoring in whom polysomnography demonstrated obstructive sleep apnea and hypoxia. Although tonsillectomy with laser palatoplasty significantly widened the oropharyngeal introitus, obstructive sleep apnea persisted. Ultimate improvement occurred only after the institution of home therapy with bi-level positive airway pressure during the night. We also discuss the specific structural and neuromuscular features of SJS that may be responsible for upper airway obstruction. Arch Otolaryngol Head Neck Surg. 1997;123:1348-1350 |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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