Deflazacort vs prednisone treatment for Duchenne muscular dystrophy: A meta-analysis of disease progression rates in recent multicenter clinical trials.


Journal

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

Informations de publication

Date de publication:
01 2020
Historique:
received: 01 02 2019
revised: 02 10 2019
accepted: 03 10 2019
pubmed: 11 10 2019
medline: 31 3 2020
entrez: 11 10 2019
Statut: ppublish

Résumé

In this study we characterized disease progression over 48 weeks among boys receiving deflazacort vs prednisone/prednisolone placebo arm treatment in two recent Duchenne muscular dystrophy (DMD) clinical trials. Ambulatory boys with DMD receiving placebo in the phase 3 ataluren (N = 115) and tadalafil (N = 116) trials were included. The trials required at least 6 months of prior corticosteroid use and stable baseline dosing. Associations between corticosteroid use and 48-week changes in ambulatory function were estimated using mixed models. Adjusted differences between corticosteroid groups were pooled in a meta-analysis. In the meta-analysis, deflazacort-treated patients vs prednisone/prednisolone-treated patients experienced, on average, lower declines of 28.3 meters on 6-minute walk distance (95% confidence interval [CI], 5.7, 50.9; 2.9 seconds on rise from supine [95% CI, 0.9, 4.9 seconds]; 2.3 seconds on 4-stair climb [95% CI, 0.5, 4.1 seconds]; and 2.9 [95% CI, 0.1, 5.8] points on the North Star Ambulatory Assessment linearized score). Deflazacort-treated patients experienced significantly lower functional decline over 48 weeks.

Identifiants

pubmed: 31599456
doi: 10.1002/mus.26736
pmc: PMC6973289
doi:

Substances chimiques

Anti-Inflammatory Agents 0
Pregnenediones 0
Prednisolone 9PHQ9Y1OLM
deflazacort KR5YZ6AE4B
Prednisone VB0R961HZT

Types de publication

Journal Article Meta-Analysis Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

26-35

Informations de copyright

© 2019 The Authors. Muscle & Nerve published by Wiley Periodicals, Inc.

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Auteurs

Craig M McDonald (CM)

Physical Medicine and Rehabilitation Pediatrics, University of California Davis Health System, Sacramento, California.

Gautam Sajeev (G)

Analysis Group, Inc, Boston, Massachusetts.

Zhiwen Yao (Z)

Analysis Group, Inc, Boston, Massachusetts.

Erin McDonnell (E)

Analysis Group, Inc, Boston, Massachusetts.

Gary Elfring (G)

PTC Therapeutics, Inc, South Plainfield, New Jersey.

Marcio Souza (M)

PTC Therapeutics, Inc, South Plainfield, New Jersey.

Stuart W Peltz (SW)

PTC Therapeutics, Inc, South Plainfield, New Jersey.

Basil T Darras (BT)

Department of Neurology, Boston Children's Hospital, Boston, Massachusetts.

Perry B Shieh (PB)

Neurology, University of California at Los Angeles, Los Angeles, California.

David A Cox (DA)

Eli Lilly and Company, Indianapolis, Indiana.

John Landry (J)

Eli Lilly and Company, Toronto, Ontario, Canada.

James Signorovitch (J)

Analysis Group, Inc, Boston, Massachusetts.

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Classifications MeSH