Long-term survival of participants in the CENTAUR trial of sodium phenylbutyrate-taurursodiol in amyotrophic lateral sclerosis.


Journal

Muscle & nerve
ISSN: 1097-4598
Titre abrégé: Muscle Nerve
Pays: United States
ID NLM: 7803146

Informations de publication

Date de publication:
01 2021
Historique:
received: 04 09 2020
revised: 07 10 2020
accepted: 09 10 2020
pubmed: 17 10 2020
medline: 20 2 2021
entrez: 16 10 2020
Statut: ppublish

Résumé

An orally administered, fixed-dose coformulation of sodium phenylbutyrate-taurursodiol (PB-TURSO) significantly slowed functional decline in a randomized, placebo-controlled, phase 2 trial in ALS (CENTAUR). Herein we report results of a long-term survival analysis of participants in CENTAUR. In CENTAUR, adults with ALS were randomized 2:1 to PB-TURSO or placebo. Participants completing the 6-month (24-week) randomized phase were eligible to receive PB-TURSO in the open-label extension. An all-cause mortality analysis (35-month maximum follow-up post-randomization) incorporated all randomized participants. Participants and site investigators were blinded to treatment assignments through the duration of follow-up of this analysis. Vital status was obtained for 135 of 137 participants originally randomized in CENTAUR. Median overall survival was 25.0 months among participants originally randomized to PB-TURSO and 18.5 months among those originally randomized to placebo (hazard ratio, 0.56; 95% confidence interval, 0.34-0.92; P = .023). Initiation of PB-TURSO treatment at baseline resulted in a 6.5-month longer median survival as compared with placebo. Combined with results from CENTAUR, these results suggest that PB-TURSO has both functional and survival benefits in ALS.

Identifiants

pubmed: 33063909
doi: 10.1002/mus.27091
pmc: PMC7820979
doi:

Substances chimiques

Neuroprotective Agents 0
Phenylbutyrates 0
Taurochenodeoxycholic Acid 516-35-8
ursodoxicoltaurine 60EUX8MN5X
4-phenylbutyric acid 7WY7YBI87E

Types de publication

Journal Article Randomized Controlled Trial Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

31-39

Subventions

Organisme : NCATS NIH HHS
ID : UL1 TR001414
Pays : United States
Organisme : NCATS NIH HHS
ID : UL1 TR001420
Pays : United States

Commentaires et corrections

Type : CommentIn

Informations de copyright

© 2020 The Authors. Muscle & Nerve published by Wiley Periodicals LLC.

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Auteurs

Sabrina Paganoni (S)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
Department of Physical Medicine and Rehabilitation, Spaulding Rehabilitation Hospital, Harvard Medical School, Boston, Massachusetts.

Suzanne Hendrix (S)

Pentara Corporation, Millcreek, Utah.

Samuel P Dickson (SP)

Pentara Corporation, Millcreek, Utah.

Newman Knowlton (N)

Pentara Corporation, Millcreek, Utah.

Eric A Macklin (EA)

Department of Medicine, Biostatistics Center, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

James D Berry (JD)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Michael A Elliott (MA)

Swedish Neuroscience Institute, Seattle, Washington.

Samuel Maiser (S)

Departments of Neurology and Medicine, Hennepin Healthcare, Minneapolis, Minnesota.

Chafic Karam (C)

Department of Neurology, Oregon Health & Science University, Portland, Oregon.

James B Caress (JB)

Department of Neurology, Wake Forest School of Medicine, Winston-Salem, North Carolina.

Margaret Ayo Owegi (MA)

Department of Neurology, University of Massachusetts Memorial Medical Center, Worcester, Massachusetts.

Adam Quick (A)

Department of Neurology, The Ohio State University College of Medicine, Columbus, Ohio.

James Wymer (J)

Department of Neurology, University of Florida College of Medicine, Gainesville, Florida.

Stephen A Goutman (SA)

Department of Neurology, University of Michigan, Ann Arbor, Michigan.

Daragh Heitzman (D)

Texas Neurology, Dallas, Texas.

Terry D Heiman-Patterson (TD)

Department of Neurology, Lewis Katz School of Medicine, Temple University, Philadelphia, Pennsylvania.

Carlayne E Jackson (CE)

Department of Neurology, The University of Texas Health Science Center at San Antonio, San Antonio, Texas.

Colin Quinn (C)

Department of Neurology, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania.

Jeffrey D Rothstein (JD)

Brain Science Institute and Department of Neurology, Johns Hopkins University, Baltimore, Maryland.

Edward J Kasarskis (EJ)

Department of Neurology, University of Kentucky College of Medicine, Lexington, Kentucky.

Jonathan Katz (J)

California Pacific Medical Center Research Institute and Forbes Norris MDA/ALS Research and Treatment Center, San Francisco, California.

Liberty Jenkins (L)

California Pacific Medical Center Research Institute and Forbes Norris MDA/ALS Research and Treatment Center, San Francisco, California.

Shafeeq Ladha (S)

Department of Neurology, Gregory W. Fulton ALS Center, Barrow Neurological Institute, Phoenix, Arizona.

Timothy M Miller (TM)

Department of Neurology, Washington University School of Medicine in St. Louis, St. Louis, Missouri.

Stephen N Scelsa (SN)

Department of Neurology, Mount Sinai Beth Israel, Icahn School of Medicine at Mount Sinai, New York, New York.

Tuan H Vu (TH)

Department of Neurology, University of South Florida Morsani College of Medicine, Tampa, Florida.

Christina N Fournier (CN)

Departments of Neurology and Pathology, Emory University School of Medicine, Atlanta, Georgia.

Jonathan D Glass (JD)

Departments of Neurology and Pathology, Emory University School of Medicine, Atlanta, Georgia.

Kristin M Johnson (KM)

Department of Neurology, Ochsner Health System, New Orleans, Louisiana.

Andrea Swenson (A)

Department of Neurology, University of Iowa Carver College of Medicine, Iowa City, Iowa.

Namita A Goyal (NA)

Department of Neurology, University of California, Irvine School of Medicine, Irvine, California.

Gary L Pattee (GL)

Neurology Associates, Lincoln, Nebraska.

Patricia L Andres (PL)

Independent Consultant, Nobleboro, Maine.

Suma Babu (S)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Marianne Chase (M)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Derek Dagostino (D)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Meghan Hall (M)

Department of Neurology, Gregory W. Fulton ALS Center, Barrow Neurological Institute, Phoenix, Arizona.

Gale Kittle (G)

Department of Neurology, Gregory W. Fulton ALS Center, Barrow Neurological Institute, Phoenix, Arizona.

Matthew Eydinov (M)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Michelle McGovern (M)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Joseph Ostrow (J)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Lindsay Pothier (L)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Rebecca Randall (R)

Department of Neurology, Gregory W. Fulton ALS Center, Barrow Neurological Institute, Phoenix, Arizona.

Jeremy M Shefner (JM)

Department of Neurology, Gregory W. Fulton ALS Center, Barrow Neurological Institute, Phoenix, Arizona.

Alexander V Sherman (AV)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Maria E St Pierre (ME)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Eric Tustison (E)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Prasha Vigneswaran (P)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Jason Walker (J)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Hong Yu (H)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

James Chan (J)

Department of Medicine, Biostatistics Center, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Janet Wittes (J)

Statistics Collaborative, Inc., Washington, District of Columbia.

Zi-Fan Yu (ZF)

Statistics Collaborative, Inc., Washington, District of Columbia.

Joshua Cohen (J)

Amylyx Pharmaceuticals, Inc., Cambridge, Massachusetts.

Justin Klee (J)

Amylyx Pharmaceuticals, Inc., Cambridge, Massachusetts.

Kent Leslie (K)

Amylyx Pharmaceuticals, Inc., Cambridge, Massachusetts.

Rudolph E Tanzi (RE)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Walter Gilbert (W)

Harvard University, Cambridge, Massachusetts.

Patrick D Yeramian (PD)

Amylyx Pharmaceuticals, Inc., Cambridge, Massachusetts.

David Schoenfeld (D)

Department of Medicine, Biostatistics Center, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

Merit E Cudkowicz (ME)

Sean M. Healey & AMG Center for ALS & the Neurological Clinical Research Institute, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.

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Classifications MeSH