Document Detail

Long-term noninvasive ventilation in children and adolescents with neuromuscular disorders.
MedLine Citation:
PMID:  14582916     Owner:  NLM     Status:  MEDLINE    
The aim of the current study was to investigate the long-term impact of nocturnal noninvasive (positive-pressure) ventilation (NIV) on sleep, sleep-disordered breathing (SDB) and respiratory function in children and adolescents with progressive neuromuscular disorders (NMD). Thirty patients (12.3 +/- 4.1 yrs) with various inherited NMD were treated with NIV for ventilatory insufficiency (n=14) or symptomatic SDB (n=16). Patients were prospectively followed with sleep studies, spirometry and peak inspiratory muscle pressure. Ten patients were studied before and after 3 nights withdrawal from NIV. NIV normalised nocturnal gas exchange in all patients and diurnal gas exchange in patients with ventilatory insufficiency. The effects persisted over 25.3 +/- 12.7 months. Nocturnal transcutaneous partial pressure of carbon dioxide improved from (baseline versus latest control) 7.1 +/- 1.3 to 5.5 +/- 0.6 kPa (53.7 +/- 9.9 to 41.6 +/- 4.8 mmHg), diurnal carbon dioxide arterial tension from 6.3 +/- 1.6 to 5.4 +/- 0.5 kPa (47.5 +/- 11.9 to 40.6 +/- 3.6 mmHg). NIV improved respiratory disturbance index, arousals from sleep, nocturnal heart rate and sleep architecture. Vital capacity decreased in five adolescents with Duchenne muscular dystrophy -183 +/- 111 mL x yr(-1) but remained stable in 25 children with other conditions (8 +/- 78 mL x yr(-1)). Three nights withdrawal of NIV in 10 previously stable patients resulted in prompt deterioration of SDB and gas exchange back to baseline but could be instantly normalised by resumption of NIV. Noninvasive (positive-pressure) ventilation has favourable long-term impact on nocturnal and diurnal gas exchange and sleep and in patients with non-Duchenne neuromuscular disorders on vital capacity as well. It is indicated in children and adolescents with symptomatic sleep-disordered breathing or ventilatory insufficiency due to neuromuscular disorders.
U Mellies; R Ragette; C Dohna Schwake; H Boehm; T Voit; H Teschler
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Publication Detail:
Type:  Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The European respiratory journal     Volume:  22     ISSN:  0903-1936     ISO Abbreviation:  Eur. Respir. J.     Publication Date:  2003 Oct 
Date Detail:
Created Date:  2003-10-29     Completed Date:  2004-02-27     Revised Date:  2013-05-23    
Medline Journal Info:
Nlm Unique ID:  8803460     Medline TA:  Eur Respir J     Country:  Denmark    
Other Details:
Languages:  eng     Pagination:  631-6     Citation Subset:  IM    
Dept of Pediatrics and Neuropediatrics, University of Essen, Children's Hospital, Essen, Germany.
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MeSH Terms
Follow-Up Studies
Intermittent Positive-Pressure Ventilation*
Lung / physiopathology*
Neuromuscular Diseases / complications
Prospective Studies
Pulmonary Gas Exchange / physiology*
Respiratory Muscles / physiopathology*
Sleep Apnea Syndromes / etiology,  physiopathology*
Time Factors

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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