{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T21:58:04Z","timestamp":1790805484575,"version":"4.1.0"},"reference-count":11,"publisher":"BMJ","issue":"8","license":[{"start":{"date-parts":[[2022,5,16]],"date-time":"2022-05-16T00:00:00Z","timestamp":1652659200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"ALS Finding a Cure","award":["Not Applicable"],"award-info":[{"award-number":["Not Applicable"]}]},{"DOI":"10.13039\/100000971","name":"The ALS Association","doi-asserted-by":"crossref","award":["Not Applicable"],"award-info":[{"award-number":["Not Applicable"]}],"id":[{"id":"10.13039\/100000971","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Amylyx Pharmaceuticals, Inc.","award":["Not Applicable"],"award-info":[{"award-number":["Not Applicable"]}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"published-print":{"date-parts":[[2022,8]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Coformulated sodium phenylbutyrate\/taurursodiol (PB\/TURSO) was shown to prolong survival and slow functional decline in amyotrophic lateral sclerosis (ALS).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Determine whether PB\/TURSO prolonged tracheostomy\/ventilation-free survival and\/or reduced first hospitalisation in participants with ALS in the CENTAUR trial.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Adults with El Escorial Definite ALS \u226418 months from symptom onset were randomised to PB\/ TURSO or placebo for 6 months. Those completing randomised treatment could enrol in an open-label extension (OLE) phase and receive PB\/TURSO for \u226430 months. Times to the following individual or combined key events were compared in the originally randomised treatment groups over a period spanning trial start through July 2020 (longest postrandomisation follow-up, 35 months): death, tracheostomy, permanent assisted ventilation (PAV) and first hospitalisation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Risk of any key event was 47% lower in those originally randomised to PB\/TURSO (n=87) versus placebo (n=48, 71% of whom received delayed-start PB\/TURSO in the OLE phase) (HR=0.53; 95%\u2009CI 0.35 to 0.81; p=0.003). Risks of death or tracheostomy\/PAV (HR=0.51; 95%\u2009CI 0.32 to 0.84; p=0.007) and first hospitalisation (HR=0.56; 95%\u2009CI 0.34 to 0.95; p=0.03) were also decreased in those originally randomised to PB\/TURSO.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Early PB\/TURSO prolonged tracheostomy\/PAV-free survival and delayed first hospitalisation in ALS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Trial registration number<\/jats:title>\n                    <jats:p>\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"clintrialgov\" xlink:href=\"NCT03127514\">NCT03127514<\/jats:ext-link>\n                      ;\n                      <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"clintrialgov\" xlink:href=\"NCT03488524\">NCT03488524<\/jats:ext-link>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/jnnp-2022-329024","type":"journal-article","created":{"date-parts":[[2022,5,23]],"date-time":"2022-05-23T13:35:26Z","timestamp":1653312926000},"page":"871-875","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":66,"title":["Effect of sodium phenylbutyrate\/taurursodiol on tracheostomy\/ventilation-free survival and hospitalisation in amyotrophic lateral sclerosis: long-term results from the CENTAUR trial"],"prefix":"10.1136","volume":"93","clinical-trial-number":[{"clinical-trial-number":"nct03127514","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03488524","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03127514","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03488524","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct05021536","registry":"10.18810\/clinical-trials-gov"}],"author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0505-1168","authenticated-orcid":false,"given":"Sabrina","family":"Paganoni","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suzanne","family":"Hendrix","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel 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