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Respiratory difficulties and breathing disorders in achondroplasia.
MedLine Citation:
PMID:  23523391     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Respiratory difficulties and breathing disorders in achondroplasia are thought to underlie the increased risk for sudden infant death and neuropsychological deficits seen in this condition. This review evaluates literature regarding respiratory dysfunctions and their sequelae in patients with achondroplasia. The limited number of prospective studies of respiratory disease in achondroplasia means that observational studies and case series provide a large proportion of the data regarding the spectrum of respiratory diseases in achondroplasia and their treatments. Amongst clinical respiratory problems described, snoring is the commonest observed abnormality, but the reported incidence of obstructive sleep apnoea (OSA) shows wide variance (10% to 75%). Reported treatments of OSA include adenotonsillectomy, the use of CPAP, and surgical improvement of the airway, including mid-face advancement. Otolaryngologic manifestations are also common. Respiratory failure due to small thoracic volumes is reported, but uncommon. Mortality rate at all ages was 2.27 (CI: 1.7-3.0) with age-specific mortality increased at all ages. Sudden death was most common in infants and children. Cardiovascular events are the main cause of mortality in adults. Despite earlier recognition and treatment of respiratory complications of achondroplasia, increased mortality rates and other complications remain high. Future and ongoing evaluation of the prevalence and impact of respiratory disorders, particularly OSA, in achondroplasia is recommended.
Authors:
S Afsharpaiman; A Saburi; Karen A Waters
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-3-20
Journal Detail:
Title:  Paediatric respiratory reviews     Volume:  -     ISSN:  1526-0550     ISO Abbreviation:  Paediatr Respir Rev     Publication Date:  2013 Mar 
Date Detail:
Created Date:  2013-3-25     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100898941     Medline TA:  Paediatr Respir Rev     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2013. Published by Elsevier Ltd.
Affiliation:
Health Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran.
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