Reductions in functional muscle mass and ability to ambulate in Duchenne muscular dystrophy from ages 4 to 24 years
Duchenne muscular dystrophy (DMD) results in a progressive loss of functional skeletal muscle mass (MM) and replacement with fibrofatty tissue. Accurate evaluation of MM in DMD patients has not previously been available. Our objective was to measure MM using the D3creatine (D3Cr) dilution method and...
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| creator | Evans, William J. Hellerstein, Marc Butterfield, Russell J. Smith, Edward Guglieri, Michela Katz, Natalie Nave, Brittany Branigan, Lauren Thera, Stephanie Vordos, Kalista L. Behar, Laura Schiava, Marianela James, Meredith K. Field, Tyler Mohammed, Hussein Shankaran, Mahalakshmi |
| description | Duchenne muscular dystrophy (DMD) results in a progressive loss of functional skeletal muscle mass (MM) and replacement with fibrofatty tissue. Accurate evaluation of MM in DMD patients has not previously been available. Our objective was to measure MM using the D3creatine (D3Cr) dilution method and determine its relationship with strength and functional capacity in patients with DMD over a wide range of ages. Subjects were recruited for participation in a 12 month, longitudinal, observational study. Here, we report the baseline data. A 20 mg dose of D3Cr dissolved in water was ingested by 92 patients with DMD (ages 4–25 years) followed later with a fasting urine sample. Enrichment of D3creatinine was determined by liquid chromatography‐mass spectrometry analysis. The North Star Ambulatory Assessment (NSAA) total score was determined for ambulatory participants, and the Performance of Upper Limb (PUL 2.0) total score and grip strength for all participants. We observed a significant age‐associated increase in body weight along with a substantial decrease in MM/body weight (%MM). MM and %MM were associated with PUL score (r = 0.517, P |
| doi_str_mv | 10.1113/JP287069 |
| format | Article |
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Key points
The non‐invasive D3creatine dilution method provides novel data on whole body functional muscle mass (MM) in a wide range of ages in patients with DMD and reveals profoundly low functional MM in older non‐ambulant patients.
The difference in %MM between ambulant and non‐ambulant subjects suggests a threshold for loss of ambulatory ability between 18 and 22% MM.
The data suggest that as functional MM declines with age, maintaining a lower body weight may help to conserve ambulatory ability.
figure legend D3creatine (D3Cr) dilution was used to non‐invasively determine total body muscle mass (MM) in subjects with Duchenne muscular dystrophy. After oral ingestion of urinary D3creatinine (D3Crn) enrichment is measured and used for the calculation of MM. A clear difference in MM/body weight was observed between ambulant and non‐ambulant subjects with a potential threshold for loss of ambulatory ability of close to 20%.</description><identifier>ISSN: 0022-3751</identifier><identifier>ISSN: 1469-7793</identifier><identifier>EISSN: 1469-7793</identifier><identifier>DOI: 10.1113/JP287069</identifier><identifier>PMID: 39216089</identifier><language>eng</language><publisher>England: Wiley Subscription Services, Inc</publisher><subject>Age ; ambulatory status ; Body weight ; D3creatine dilution ; Duchenne's muscular dystrophy ; functional capacity ; Liquid chromatography ; Mass spectroscopy ; muscle mass ; Muscular dystrophy ; Neuromuscular diseases ; Skeletal muscle</subject><ispartof>The Journal of physiology, 2024-10, Vol.602 (19), p.4929-4939</ispartof><rights>2024 The Author(s). published by John Wiley & Sons Ltd on behalf of The Physiological Society.</rights><rights>2024 The Author(s). The Journal of Physiology published by John Wiley & Sons Ltd on behalf of The Physiological Society.</rights><rights>2024. This article is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c2753-9d6368f1db0471820f79cff19faf42bf2a484c50f3c58d744a5eebc3264d981d3</cites><orcidid>0000-0003-0073-9377 ; 0000-0001-5098-8932 ; 0000-0001-9138-9610 ; 0000-0001-6757-1471 ; 0000-0001-8078-7744 ; 0000-0002-8455-0637 ; 0000-0002-2327-0834</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1113%2FJP287069$$EPDF$$P50$$Gwiley$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1113%2FJP287069$$EHTML$$P50$$Gwiley$$Hfree_for_read</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/39216089$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Evans, William J.</creatorcontrib><creatorcontrib>Hellerstein, Marc</creatorcontrib><creatorcontrib>Butterfield, Russell J.</creatorcontrib><creatorcontrib>Smith, Edward</creatorcontrib><creatorcontrib>Guglieri, Michela</creatorcontrib><creatorcontrib>Katz, Natalie</creatorcontrib><creatorcontrib>Nave, Brittany</creatorcontrib><creatorcontrib>Branigan, Lauren</creatorcontrib><creatorcontrib>Thera, Stephanie</creatorcontrib><creatorcontrib>Vordos, Kalista L.</creatorcontrib><creatorcontrib>Behar, Laura</creatorcontrib><creatorcontrib>Schiava, Marianela</creatorcontrib><creatorcontrib>James, Meredith K.</creatorcontrib><creatorcontrib>Field, Tyler</creatorcontrib><creatorcontrib>Mohammed, Hussein</creatorcontrib><creatorcontrib>Shankaran, Mahalakshmi</creatorcontrib><title>Reductions in functional muscle mass and ability to ambulate in Duchenne muscular dystrophy from ages 4 to 24 years</title><title>The Journal of physiology</title><addtitle>J Physiol</addtitle><description>Duchenne muscular dystrophy (DMD) results in a progressive loss of functional skeletal muscle mass (MM) and replacement with fibrofatty tissue. Accurate evaluation of MM in DMD patients has not previously been available. Our objective was to measure MM using the D3creatine (D3Cr) dilution method and determine its relationship with strength and functional capacity in patients with DMD over a wide range of ages. Subjects were recruited for participation in a 12 month, longitudinal, observational study. Here, we report the baseline data. A 20 mg dose of D3Cr dissolved in water was ingested by 92 patients with DMD (ages 4–25 years) followed later with a fasting urine sample. Enrichment of D3creatinine was determined by liquid chromatography‐mass spectrometry analysis. The North Star Ambulatory Assessment (NSAA) total score was determined for ambulatory participants, and the Performance of Upper Limb (PUL 2.0) total score and grip strength for all participants. We observed a significant age‐associated increase in body weight along with a substantial decrease in MM/body weight (%MM). MM and %MM were associated with PUL score (r = 0.517, P < 0.0001 and r = 0.764, P < 0.0001 respectively). The age‐associated decrease in MM and %MM was strongly associated with ambulatory status. We observed very little overlap in %MM between ambulant and non‐ambulant subjects, suggesting a threshold of 18–22% associated with loss of ambulation. MM is substantially diminished with advancing age and is highly related to clinically meaningful functional status. The D3Cr dilution method may provide a biomarker of disease progression and therapeutic efficacy in patients with DMD or other neuromuscular disorders.
Key points
The non‐invasive D3creatine dilution method provides novel data on whole body functional muscle mass (MM) in a wide range of ages in patients with DMD and reveals profoundly low functional MM in older non‐ambulant patients.
The difference in %MM between ambulant and non‐ambulant subjects suggests a threshold for loss of ambulatory ability between 18 and 22% MM.
The data suggest that as functional MM declines with age, maintaining a lower body weight may help to conserve ambulatory ability.
figure legend D3creatine (D3Cr) dilution was used to non‐invasively determine total body muscle mass (MM) in subjects with Duchenne muscular dystrophy. After oral ingestion of urinary D3creatinine (D3Crn) enrichment is measured and used for the calculation of MM. A clear difference in MM/body weight was observed between ambulant and non‐ambulant subjects with a potential threshold for loss of ambulatory ability of close to 20%.</description><subject>Age</subject><subject>ambulatory status</subject><subject>Body weight</subject><subject>D3creatine dilution</subject><subject>Duchenne's muscular dystrophy</subject><subject>functional capacity</subject><subject>Liquid chromatography</subject><subject>Mass spectroscopy</subject><subject>muscle mass</subject><subject>Muscular dystrophy</subject><subject>Neuromuscular diseases</subject><subject>Skeletal muscle</subject><issn>0022-3751</issn><issn>1469-7793</issn><issn>1469-7793</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><sourceid>24P</sourceid><recordid>eNp1kctKw0AUhgdRbK2CTyADbtykzi2ZzFLqtRQsUtdhMhebkkudySB5e9ObguDqcA7f-Rb_D8AlRmOMMb2dzknKUSKOwBCzREScC3oMhggRElEe4wE4836FEKZIiFMwoILgBKViCPyb0UG1RVN7WNTQhnq7yBJWwavSwEp6D2WtocyLsmg72DZQVnkoZWs2H_dBLU1dmy3fXx3UnW9ds1520LqmgvLDeMg2b4TBzkjnz8GJlaU3F_s5Au-PD4vJczR7fXqZ3M0iRXhMI6ETmqQW6xwxjlOCLBfKWiystIzklkiWMhUjS1Wcas6YjI3JFSUJ0yLFmo7Azc67ds1nML7NqsIrU5ayNk3w2SaMFHGRiB69_oOumuD6GHoK95FiKhD9FSrXeO-MzdauqKTrMoyyTRHZoYgevdoLQ14Z_QMeku-B8Q74KkrT_SvKFtM5Toig9Btd1JBN</recordid><startdate>20241001</startdate><enddate>20241001</enddate><creator>Evans, William J.</creator><creator>Hellerstein, Marc</creator><creator>Butterfield, Russell J.</creator><creator>Smith, Edward</creator><creator>Guglieri, Michela</creator><creator>Katz, Natalie</creator><creator>Nave, Brittany</creator><creator>Branigan, Lauren</creator><creator>Thera, Stephanie</creator><creator>Vordos, Kalista L.</creator><creator>Behar, Laura</creator><creator>Schiava, Marianela</creator><creator>James, Meredith K.</creator><creator>Field, Tyler</creator><creator>Mohammed, Hussein</creator><creator>Shankaran, Mahalakshmi</creator><general>Wiley Subscription Services, Inc</general><scope>24P</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7QP</scope><scope>7QR</scope><scope>7TK</scope><scope>7TS</scope><scope>8FD</scope><scope>FR3</scope><scope>P64</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0003-0073-9377</orcidid><orcidid>https://orcid.org/0000-0001-5098-8932</orcidid><orcidid>https://orcid.org/0000-0001-9138-9610</orcidid><orcidid>https://orcid.org/0000-0001-6757-1471</orcidid><orcidid>https://orcid.org/0000-0001-8078-7744</orcidid><orcidid>https://orcid.org/0000-0002-8455-0637</orcidid><orcidid>https://orcid.org/0000-0002-2327-0834</orcidid></search><sort><creationdate>20241001</creationdate><title>Reductions in functional muscle mass and ability to ambulate in Duchenne muscular dystrophy from ages 4 to 24 years</title><author>Evans, William J. ; Hellerstein, Marc ; Butterfield, Russell J. ; Smith, Edward ; Guglieri, Michela ; Katz, Natalie ; Nave, Brittany ; Branigan, Lauren ; Thera, Stephanie ; Vordos, Kalista L. ; Behar, Laura ; Schiava, Marianela ; James, Meredith K. ; Field, Tyler ; Mohammed, Hussein ; Shankaran, Mahalakshmi</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c2753-9d6368f1db0471820f79cff19faf42bf2a484c50f3c58d744a5eebc3264d981d3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Age</topic><topic>ambulatory status</topic><topic>Body weight</topic><topic>D3creatine dilution</topic><topic>Duchenne's muscular dystrophy</topic><topic>functional capacity</topic><topic>Liquid chromatography</topic><topic>Mass spectroscopy</topic><topic>muscle mass</topic><topic>Muscular dystrophy</topic><topic>Neuromuscular diseases</topic><topic>Skeletal muscle</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Evans, William J.</creatorcontrib><creatorcontrib>Hellerstein, Marc</creatorcontrib><creatorcontrib>Butterfield, Russell J.</creatorcontrib><creatorcontrib>Smith, Edward</creatorcontrib><creatorcontrib>Guglieri, Michela</creatorcontrib><creatorcontrib>Katz, Natalie</creatorcontrib><creatorcontrib>Nave, Brittany</creatorcontrib><creatorcontrib>Branigan, Lauren</creatorcontrib><creatorcontrib>Thera, Stephanie</creatorcontrib><creatorcontrib>Vordos, Kalista L.</creatorcontrib><creatorcontrib>Behar, Laura</creatorcontrib><creatorcontrib>Schiava, Marianela</creatorcontrib><creatorcontrib>James, Meredith K.</creatorcontrib><creatorcontrib>Field, Tyler</creatorcontrib><creatorcontrib>Mohammed, Hussein</creatorcontrib><creatorcontrib>Shankaran, Mahalakshmi</creatorcontrib><collection>Wiley Online Library Open Access</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Chemoreception Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>Physical Education Index</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>The Journal of physiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Evans, William J.</au><au>Hellerstein, Marc</au><au>Butterfield, Russell J.</au><au>Smith, Edward</au><au>Guglieri, Michela</au><au>Katz, Natalie</au><au>Nave, Brittany</au><au>Branigan, Lauren</au><au>Thera, Stephanie</au><au>Vordos, Kalista L.</au><au>Behar, Laura</au><au>Schiava, Marianela</au><au>James, Meredith K.</au><au>Field, Tyler</au><au>Mohammed, Hussein</au><au>Shankaran, Mahalakshmi</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Reductions in functional muscle mass and ability to ambulate in Duchenne muscular dystrophy from ages 4 to 24 years</atitle><jtitle>The Journal of physiology</jtitle><addtitle>J Physiol</addtitle><date>2024-10-01</date><risdate>2024</risdate><volume>602</volume><issue>19</issue><spage>4929</spage><epage>4939</epage><pages>4929-4939</pages><issn>0022-3751</issn><issn>1469-7793</issn><eissn>1469-7793</eissn><abstract>Duchenne muscular dystrophy (DMD) results in a progressive loss of functional skeletal muscle mass (MM) and replacement with fibrofatty tissue. Accurate evaluation of MM in DMD patients has not previously been available. Our objective was to measure MM using the D3creatine (D3Cr) dilution method and determine its relationship with strength and functional capacity in patients with DMD over a wide range of ages. Subjects were recruited for participation in a 12 month, longitudinal, observational study. Here, we report the baseline data. A 20 mg dose of D3Cr dissolved in water was ingested by 92 patients with DMD (ages 4–25 years) followed later with a fasting urine sample. Enrichment of D3creatinine was determined by liquid chromatography‐mass spectrometry analysis. The North Star Ambulatory Assessment (NSAA) total score was determined for ambulatory participants, and the Performance of Upper Limb (PUL 2.0) total score and grip strength for all participants. We observed a significant age‐associated increase in body weight along with a substantial decrease in MM/body weight (%MM). MM and %MM were associated with PUL score (r = 0.517, P < 0.0001 and r = 0.764, P < 0.0001 respectively). The age‐associated decrease in MM and %MM was strongly associated with ambulatory status. We observed very little overlap in %MM between ambulant and non‐ambulant subjects, suggesting a threshold of 18–22% associated with loss of ambulation. MM is substantially diminished with advancing age and is highly related to clinically meaningful functional status. The D3Cr dilution method may provide a biomarker of disease progression and therapeutic efficacy in patients with DMD or other neuromuscular disorders.
Key points
The non‐invasive D3creatine dilution method provides novel data on whole body functional muscle mass (MM) in a wide range of ages in patients with DMD and reveals profoundly low functional MM in older non‐ambulant patients.
The difference in %MM between ambulant and non‐ambulant subjects suggests a threshold for loss of ambulatory ability between 18 and 22% MM.
The data suggest that as functional MM declines with age, maintaining a lower body weight may help to conserve ambulatory ability.
figure legend D3creatine (D3Cr) dilution was used to non‐invasively determine total body muscle mass (MM) in subjects with Duchenne muscular dystrophy. After oral ingestion of urinary D3creatinine (D3Crn) enrichment is measured and used for the calculation of MM. A clear difference in MM/body weight was observed between ambulant and non‐ambulant subjects with a potential threshold for loss of ambulatory ability of close to 20%.</abstract><cop>England</cop><pub>Wiley Subscription Services, Inc</pub><pmid>39216089</pmid><doi>10.1113/JP287069</doi><tpages>11</tpages><orcidid>https://orcid.org/0000-0003-0073-9377</orcidid><orcidid>https://orcid.org/0000-0001-5098-8932</orcidid><orcidid>https://orcid.org/0000-0001-9138-9610</orcidid><orcidid>https://orcid.org/0000-0001-6757-1471</orcidid><orcidid>https://orcid.org/0000-0001-8078-7744</orcidid><orcidid>https://orcid.org/0000-0002-8455-0637</orcidid><orcidid>https://orcid.org/0000-0002-2327-0834</orcidid><oa>free_for_read</oa></addata></record> |
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| source | Wiley Online Library Journals Frontfile Complete |
| subjects | Age ambulatory status Body weight D3creatine dilution Duchenne's muscular dystrophy functional capacity Liquid chromatography Mass spectroscopy muscle mass Muscular dystrophy Neuromuscular diseases Skeletal muscle |
| title | Reductions in functional muscle mass and ability to ambulate in Duchenne muscular dystrophy from ages 4 to 24 years |
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