Abstract：
:Spinal muscular atrophy (SMA) is a relatively common, profoundly disabling and incurable disease that presents in early childhood with hypotonia, weakness and decreased movement. Without ventilatory support, premature death from respiratory insufficiency is universal in children with spinal muscular atrophy type 1 (SMA1). With mechanical ventilation, however, long-term survival in SMA1 has been reported from numerous international centres. Children kept alive by this means experience progressive paralysis and eventually become effectively 'locked in' on the ventilator, with no useful movements of the extremities, progressive facial and bulbar weakness, and complete inability to communicate. Prolongation of life by invasive ventilation in such cases is futile given the absence of curative treatments for infants with SMA1, and overly burdensome given the unacceptable quality of life of such children.
journal_name
Paediatr Respir Revjournal_title
Paediatric respiratory reviewsauthors
Ryan MMdoi
10.1016/j.prrv.2007.10.002subject
Has Abstractpub_date
2008-03-01 00:00:00pages
51-4; discussion 55-6issue
1eissn
1526-0542issn
1526-0550pii
S1526-0542(07)00117-0journal_volume
9pub_type
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pub_type： 杂志文章,评审
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journal_title：Paediatric respiratory reviews
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journal_title：Paediatric respiratory reviews
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journal_title：Paediatric respiratory reviews
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abstract：INTRODUCTION:Corticosteroids can be used prenatally to mature the fetal lungs and postnatally to treat or prevent chronic lung disease (CLD). Randomised controlled trials have been performed to evaluate the benefits and risks of perinatal corticosteroid therapy. METHODS:Systematic reviews of randomised controlled tria...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
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更新日期：2004-01-01 00:00:00
abstract：AIM:To describe the respiratory and nutritional supportive care and hospitalisations required in the real-world scenario in children with SMA type 1 treated with nusinersen. METHODS:Single-centre observational cohort study of children with SMA1 commencing nusinersen from November 2016 to September 2018. Motor, respira...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2020.09.002
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abstract：:Asthma is most frequent chronic disease in childhood. Childhood asthma frequently persists to adulthood and the available evidence suggests that the severity of childhood asthma predicts the severity of asthma in the adulthood. To prevent long term remodeling of the airways and improve prognosis, early diagnosis and t...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2006.04.171
更新日期：2006-01-01 00:00:00
