Pulmonary function testing: Difference between revisions

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===Pediatric neuromuscular disorders===

Neuromuscular disorders such as [[Duchenne muscular dystrophy]] are associated with gradual loss of muscle function over time. Involvement of respiratory muscles results in poor ability to cough and decreased ability to breathe well and leads to [[atelectasis]] (the inability of the lungs to gain oxygen) and an overall insufficiency in lung strength.<ref>Finder JD, Birnkrant D, Carl J, et al. Respiratory care of the patients with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med.2004;170 (4):456– 465</ref> A combination of reduced lung compliance caused by generalized and widespread microatelectasis and chest wall deformity caused by increased chest wall compliance4compliance results in increased work of breathing and chronic respiratory insufficiency.<ref>Wang CH, Finkel RS, Bertini ES, et al. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol.2007;22 (8):1027–1049</ref> Musculoskeletal deformities such as kyphoscoliosis contribute to restrictive lung disease.

Pulmonary function testing in patients with neuromuscular disorders helps to evaluate the respiratory status of patients at the time of diagnosis, monitor their progress and course, evaluate them for possible surgery, and gives an overall idea of the prognosis.<ref name="pmid19420147">{{cite journal| author=Sharma GD| title=Pulmonary function testing in neuromuscular disorders. | journal=Pediatrics | year= 2009 | volume= 123 Suppl 4 | issue= | pages= S219-21 | pmid=19420147 | doi=10.1542/peds.2008-2952D | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19420147 }}</ref>