Literature DB >> 26280938

Duchenne muscular dystrophy: clinical trials and emerging tribulations.

Perry B Shieh1.   

Abstract

PURPOSE OF REVIEW: This article reviewed the most recent clinical trials investigating potential treatments for Duchenne muscular dystrophy (DMD). In the development of these studies, investigators have encountered unexpected challenges that reveal limitations in our understanding of the clinical trial design for this disease. RECENT
FINDINGS: Recent clinical trials for DMD have revealed limitations in our understanding of the natural history of this disease. As a result of these revelations, there has been a significant effort to document the natural history of Duchenne and develop valid outcome measure that could be used in clinical trials in different subsets of patients.
SUMMARY: Treatment for DMD is a rapidly changing field. Some of the drugs that are currently under investigation will receive Food and Drug Administration approval in the near future. These would become part of clinical practice. This article summarizes the different therapeutic approaches under investigation and illustrates the importance of defining sound research methods that are specific to the disease and population under investigation.

Entities:  

Mesh:

Year:  2015        PMID: 26280938     DOI: 10.1097/WCO.0000000000000243

Source DB:  PubMed          Journal:  Curr Opin Neurol        ISSN: 1350-7540            Impact factor:   5.710


  12 in total

1.  Loss of α-actinin-3 confers protection from eccentric contraction damage in fast-twitch EDL muscles from aged mdx dystrophic mice by reducing pathological fibre branching.

Authors:  Leonit Kiriaev; Peter J Houweling; Kathryn N North; Stewart I Head
Journal:  Hum Mol Genet       Date:  2022-05-04       Impact factor: 5.121

2.  Beyond ambulation: Measuring physical activity in youth with Duchenne muscular dystrophy.

Authors:  Mary Killian; Maciej S Buchowski; Thomas Donnelly; W Bryan Burnette; Larry W Markham; James C Slaughter; Meng Xu; Kimberly Crum; Bruce M Damon; Jonathan H Soslow
Journal:  Neuromuscul Disord       Date:  2020-02-20       Impact factor: 4.296

3.  Longitudinal timed function tests in Duchenne muscular dystrophy: ImagingDMD cohort natural history.

Authors:  Harneet Arora; Rebecca J Willcocks; Donovan J Lott; Ann T Harrington; Claudia R Senesac; Kirsten L Zilke; Michael J Daniels; Dandan Xu; Gihan I Tennekoon; Erika L Finanger; Barry S Russman; Richard S Finkel; William T Triplett; Barry J Byrne; Glenn A Walter; H Lee Sweeney; Krista Vandenborne
Journal:  Muscle Nerve       Date:  2018-07-24       Impact factor: 3.217

4.  Health-related quality of life and functional changes in DMD: A 12-month longitudinal cohort study.

Authors:  Sonia Messina; Gian Luca Vita; Maria Sframeli; Stefania Mondello; Elena Mazzone; Adele D'Amico; Angela Berardinelli; Matteo La Rosa; Claudio Bruno; Maria Grazia Distefano; Giovanni Baranello; Costanza Barcellona; Marianna Scutifero; Sonia Marcato; Arianna Palmieri; Luisa Politano; Lucia Morandi; Tiziana Mongini; Elena Pegoraro; Maria Grazia D'Angelo; Marika Pane; Carmelo Rodolico; Carlo Minetti; Enrico Bertini; Giuseppe Vita; Eugenio Mercuri
Journal:  Neuromuscul Disord       Date:  2016-02-02       Impact factor: 4.296

5.  Evidence for ACTN3 as a genetic modifier of Duchenne muscular dystrophy.

Authors:  Marshall W Hogarth; Peter J Houweling; Kristen C Thomas; Heather Gordish-Dressman; Luca Bello; Elena Pegoraro; Eric P Hoffman; Stewart I Head; Kathryn N North
Journal:  Nat Commun       Date:  2017-01-31       Impact factor: 14.919

Review 6.  The golden retriever model of Duchenne muscular dystrophy.

Authors:  Joe N Kornegay
Journal:  Skelet Muscle       Date:  2017-05-19       Impact factor: 4.912

Review 7.  Using a meta-narrative literature review and focus groups with key stakeholders to identify perceived challenges and solutions for generating robust evidence on the effectiveness of treatments for rare diseases.

Authors:  Kylie Tingley; Doug Coyle; Ian D Graham; Lindsey Sikora; Pranesh Chakraborty; Kumanan Wilson; John J Mitchell; Sylvia Stockler-Ipsiroglu; Beth K Potter
Journal:  Orphanet J Rare Dis       Date:  2018-06-28       Impact factor: 4.123

8.  Assessment of disease progression in dysferlinopathy: A 1-year cohort study.

Authors:  Ursula Moore; Marni Jacobs; Meredith K James; Anna G Mayhew; Roberto Fernandez-Torron; Jia Feng; Avital Cnaan; Michelle Eagle; Karen Bettinson; Laura E Rufibach; Robert Muni Lofra; Andrew M Blamire; Pierre G Carlier; Plavi Mittal; Linda Pax Lowes; Lindsay Alfano; Kristy Rose; Tina Duong; Katherine M Berry; Elena Montiel-Morillo; Irene Pedrosa-Hernández; Scott Holsten; Mohammed Sanjak; Ai Ashida; Chikako Sakamoto; Takayuki Tateishi; Hiroyuki Yajima; Aurélie Canal; Gwenn Ollivier; Valerie Decostre; Juan Bosco Mendez; Nieves Sánchez-Aguilera Praxedes; Simone Thiele; Catherine Siener; Jeanine Shierbecker; Julaine M Florence; Bruno Vandevelde; Brittney DeWolf; Meghan Hutchence; Richard Gee; Juliana Prügel; Elke Maron; Heather Hilsden; Hanns Lochmüller; Ulrike Grieben; Simone Spuler; Carolina Tesi Rocha; John W Day; Kristi J Jones; Diana X Bharucha-Goebel; Emmanuelle Salort-Campana; Matthew Harms; Alan Pestronk; Sabine Krause; Olivia Schreiber-Katz; Maggie C Walter; Carmen Paradas; Jean-Yves Hogrel; Tanya Stojkovic; Shin'ichi Takeda; Madoka Mori-Yoshimura; Elena Bravver; Susan Sparks; Jordi Díaz-Manera; Luca Bello; Claudio Semplicini; Elena Pegoraro; Jerry R Mendell; Kate Bushby; Volker Straub
Journal:  Neurology       Date:  2019-01-09       Impact factor: 9.910

Review 9.  Current and emerging treatment strategies for Duchenne muscular dystrophy.

Authors:  Jean K Mah
Journal:  Neuropsychiatr Dis Treat       Date:  2016-07-22       Impact factor: 2.570

10.  Mitochondrial disease patient motivations and barriers to participate in clinical trials.

Authors:  Zarazuela Zolkipli-Cunningham; Rui Xiao; Amy Stoddart; Elizabeth M McCormick; Amy Holberts; Natalie Burrill; Shana McCormack; Lauren Williams; Xiaoyan Wang; John L P Thompson; Marni J Falk
Journal:  PLoS One       Date:  2018-05-17       Impact factor: 3.240

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