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European Archives of Oto-Rhino-Laryngology

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01-08-2006 | Case Report

A case of achondroplasia with severe pulmonary hypertension due to obstructive sleep apnea

Authors: Selman Vefa Yıldırım, Cemile Durmaz, Mir Ali Pourbagher, Alper Nabi Erkan

Published in: European Archives of Oto-Rhino-Laryngology | Issue 8/2006

Abstract

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.

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Title: A case of achondroplasia with severe pulmonary hypertension due to obstructive sleep apnea
Authors: Selman Vefa Yıldırım
Cemile Durmaz
Mir Ali Pourbagher
Alper Nabi Erkan
Publication date: 01-08-2006
Publisher: Springer-Verlag
Published in: European Archives of Oto-Rhino-Laryngology / Issue 8/2006
Print ISSN: 0937-4477
Electronic ISSN: 1434-4726
DOI: https://doi.org/10.1007/s00405-006-0042-x

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A case of achondroplasia with severe pulmonary hypertension due to obstructive sleep apnea

Abstract

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

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