A case of achondroplasia with severe pulmonary hypertension due to obstructive sleep apnea
| Autor: | Selman Vefa Yildirim, Alper Nabi Erkan, Cemile Durmaz, Mir Ali Pourbagher |
|---|---|
| Rok vydání: | 2005 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities medicine.medical_specialty Hypertension Pulmonary Hypoxemia Achondroplasia Adenoidectomy Internal medicine medicine Humans Tonsillectomy Sleep disorder Sleep Apnea Obstructive business.industry Respiratory disease General Medicine medicine.disease Pulmonary hypertension Hypotonia Hypoplasia respiratory tract diseases Obstructive sleep apnea Airway Obstruction Treatment Outcome Otorhinolaryngology Echocardiography Anesthesia Child Preschool Cardiology Female medicine.symptom business |
| Zdroj: | European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery. 263(8) |
| ISSN: | 0937-4477 |
| Popis: | Achondroplasia is the most common skeletal dysplasia in children. Achondroplasic patients have a short cranial face and midface hypoplasia. They often have sleep-related respiratory disturbances that lead to hypoxemia caused by midfacial hypoplasia, a small upper airway, hypotonia of airway muscles, or brain stem compression. It has been well described that obstructive sleep apnea can cause pulmonary hypertension (PH) through the mechanism of chronic hypoxemia. However, severe PH due to obstructive-type sleep disorder is rare in patients with achondroplasia. In this report, we describe a 5-year-old girl with achondroplasia whose severe PH was caused by upper-airway obstruction and was resolved gradually after adenotonsillectomy. |
| Databáze: | OpenAIRE |
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