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Is survival improve...
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Burkhardt, Christian
(författare)
Is survival improved by the use of NIV and PEG in amyotrophic lateral sclerosis (ALS)? : A post-mortem study of 80 ALS patients
- Artikel/kapitelEngelska2017
Förlag, utgivningsår, omfång ...
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2017-05-23
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Public Library of Science (PLoS),2017
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electronicrdacarrier
Nummerbeteckningar
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LIBRIS-ID:oai:DiVA.org:umu-136332
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https://urn.kb.se/resolve?urn=urn:nbn:se:umu:diva-136332URI
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https://doi.org/10.1371/journal.pone.0177555DOI
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Språk:engelska
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Sammanfattning på:engelska
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Ämneskategori:ref swepub-contenttype
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Ämneskategori:art swepub-publicationtype
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Background: Non-invasive ventilation (NIV) and percutaneous gastrostomy (PEG) are guideline-recommended interventions for symptom management in amyotrophic lateral sclerosis (ALS). Their effect on survival is controversial and the impact on causes of death is unknown.Objective: To investigate the effect of NIV and PEG on survival and causes of death in ALS patients.Methods: Eighty deceased ALS patients underwent a complete post mortem analysis for causes of death between 2003 and 2015. Forty-two of these patients consented for genetic testing. Effects of NIV and PEG on survival and causes of death were analyzed in a multivariable Cox proportional hazard regression.Results: Six patients, who requested assisted suicide causing drug-induced hypoxia, were excluded from final analysis. Respiratory failure was the main cause of death in 72 out of 74 patients. Fifteen out of 74 died of aspiration pneumonia 23/74 of bronchopneumonia and 8/74 of a combination of aspiration pneumonia and bronchopneumonia. Twenty died of hypoxia without concomitant infection, and six patients had pulmonary embolism alone or in combination with pneumonia. NIV (p = 0.01) and PEG (p<0.01) had a significant impact on survival. In patients using NIV bronchopneumonia was significantly more frequent (p <0.04) compared to non-NIV patients. This effect was even more pronounced in limb onset patients (p<0.002). Patients with C9orf72 hexanucleotide repeat expansions showed faster disease progression and shorter survival (p = 0.01).Conclusion: The use of NIV and PEG prolongs survival in ALS. This study supports current AAN and EFNS guidelines which recommend NIV and PEG as a treatment option in ALS. The risk of bronchopneumonia as cause of death may be increased by NIV.
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Biuppslag (personer, institutioner, konferenser, titlar ...)
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Neuwirth, Christoph
(författare)
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Sommacal, Andreas
(författare)
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Andersen, Peter M.Umeå universitet,Klinisk neurovetenskap(Swepub:umu)pean0001
(författare)
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Weber, Markus
(författare)
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Umeå universitetKlinisk neurovetenskap
(creator_code:org_t)
Sammanhörande titlar
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Ingår i:PLOS ONE: Public Library of Science (PLoS)12:51932-6203
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