Low bone density and bone metabolism alterations in Duchenne muscular dystrophy: response to calcium and vitamin D treatment
Summary Boys with Duchenne muscular dystrophy often have reduced bone mass and increased fracture risk. In this prospective study on 33 patients, calcifediol (25-OH vitamin D₃) plus adjustment of dietary calcium to the recommended dose reduced bone resorption, corrected vitamin D deficiency, and inc...
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description | Summary Boys with Duchenne muscular dystrophy often have reduced bone mass and increased fracture risk. In this prospective study on 33 patients, calcifediol (25-OH vitamin D₃) plus adjustment of dietary calcium to the recommended dose reduced bone resorption, corrected vitamin D deficiency, and increased bone mass in about two-thirds of cases. Introduction Low BMC and BMD and bone metabolism alterations are frequent in boys with Duchenne muscular dystrophy (DMD), especially now that long-term glucocorticosteroid (GC) treatment is the standard of care. This prospective study was designed to evaluate the effects of a first-line treatment (25-OH vitamin D₃ [calcifediol] plus adjustment of dietary calcium to the recommended daily dose) on bone. Methods Thirty-three children with DMD on GC treatment were followed for 3 years: one of observation and two of treatment. Main outcome: spine and total body BMC and BMD increase; secondary outcome: changes in bone turnover markers (C-terminal [CTx] and N-terminal [NTx] telopeptides of procollagen type I; osteocalcin [OC]). Results During the observation year, BMC and BMD decreased in all patients. At baseline and after 12 months, serum CTx and urinary NTx were higher than normal; OC and parathyroid hormone at the upper limit of normal; 25-OH vitamin D₃ significantly lower than normal. After 2 years of calcifediol and calcium-rich diet, BMC and BMD significantly increased in over 65% of patients, and bone metabolism parameters and turnover markers normalized in most patients (78.8%). During the observation year, there were four fractures in four patients, while during the 2 years of treatment there were two fractures in two patients. Conclusions Calcifediol plus adequate dietary calcium intake seems to be an effective first-line approach that controls bone turnover, corrects vitamin D deficiency, and increases BMC and BMD in most patients with DMD. Lack of response seems related to persistently high bone turnover. |
doi_str_mv | 10.1007/s00198-010-1275-5 |
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fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_954600378</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2236705151</sourcerecordid><originalsourceid>FETCH-LOGICAL-c522t-76b2b168331c948dae261cce4a9030f7f76026e03ce3c8c330a2205da784f47b3</originalsourceid><addsrcrecordid>eNp9kUtv1DAUhS0EokPhB7ABC6liFbh-JHa6Qy0vaSQWUImd5Tg3barEHmwHNBI_Hg8ZqMSCjS1ff-fcax9CnjJ4xQDU6wTAWl0Bg4pxVVf1PbJhUoiKt019n2ygFapqJft6Qh6ldAtF07bqITnhIGtdK9iQn9vwg3bBI-3RpzHvqfX9Wpgx2y5MY5qpnTJGm8fgEx09vVzcDfoDsiS3TDbSfp9yDLub_TmNmHaFQ5oDdXZy4zL_9vw-ZjsfxDRHtHlGnx-TB4OdEj457qfk6t3bLxcfqu2n9x8v3mwrV3OeK9V0vGONFoK5VureIm-YcyhtCwIGNagGeIMgHAqnnRBgOYe6t0rLQapOnJKXq-8uhm8LpmzmMTmcJusxLMm0tWwAhNKFfPEPeRuW6MtwRgsllS6tCsRWyMWQUsTB7OI427g3DMwhGLMGY-BwLsGYumieHY2Xbsb-r-JPEgU4OwI2lW8bovVuTHec0Ly8XRSOr1wqV_4a492E_-v-fBUNNhh7HYvx1WcOTBSQq7KIX8Idr5A</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>837478760</pqid></control><display><type>article</type><title>Low bone density and bone metabolism alterations in Duchenne muscular dystrophy: response to calcium and vitamin D treatment</title><source>MEDLINE</source><source>SpringerLink Journals</source><creator>Bianchi, M. L ; Morandi, L ; Andreucci, E ; Vai, S ; Frasunkiewicz, J ; Cottafava, R</creator><creatorcontrib>Bianchi, M. L ; Morandi, L ; Andreucci, E ; Vai, S ; Frasunkiewicz, J ; Cottafava, R</creatorcontrib><description>Summary Boys with Duchenne muscular dystrophy often have reduced bone mass and increased fracture risk. In this prospective study on 33 patients, calcifediol (25-OH vitamin D₃) plus adjustment of dietary calcium to the recommended dose reduced bone resorption, corrected vitamin D deficiency, and increased bone mass in about two-thirds of cases. Introduction Low BMC and BMD and bone metabolism alterations are frequent in boys with Duchenne muscular dystrophy (DMD), especially now that long-term glucocorticosteroid (GC) treatment is the standard of care. This prospective study was designed to evaluate the effects of a first-line treatment (25-OH vitamin D₃ [calcifediol] plus adjustment of dietary calcium to the recommended daily dose) on bone. Methods Thirty-three children with DMD on GC treatment were followed for 3 years: one of observation and two of treatment. Main outcome: spine and total body BMC and BMD increase; secondary outcome: changes in bone turnover markers (C-terminal [CTx] and N-terminal [NTx] telopeptides of procollagen type I; osteocalcin [OC]). Results During the observation year, BMC and BMD decreased in all patients. At baseline and after 12 months, serum CTx and urinary NTx were higher than normal; OC and parathyroid hormone at the upper limit of normal; 25-OH vitamin D₃ significantly lower than normal. After 2 years of calcifediol and calcium-rich diet, BMC and BMD significantly increased in over 65% of patients, and bone metabolism parameters and turnover markers normalized in most patients (78.8%). During the observation year, there were four fractures in four patients, while during the 2 years of treatment there were two fractures in two patients. Conclusions Calcifediol plus adequate dietary calcium intake seems to be an effective first-line approach that controls bone turnover, corrects vitamin D deficiency, and increases BMC and BMD in most patients with DMD. Lack of response seems related to persistently high bone turnover.</description><identifier>ISSN: 0937-941X</identifier><identifier>EISSN: 1433-2965</identifier><identifier>DOI: 10.1007/s00198-010-1275-5</identifier><identifier>PMID: 20458570</identifier><language>eng</language><publisher>London: London : Springer-Verlag</publisher><subject>Adolescent ; Biological and medical sciences ; bone density ; Bone Density - drug effects ; Bone Density Conservation Agents - administration & dosage ; Bone Density Conservation Agents - pharmacology ; Bone mass ; Bone mineral content ; Bone mineral density ; Bone Remodeling - drug effects ; Bone resorption ; Bone Resorption - drug therapy ; Bone turnover ; Bones ; Calcifediol - administration & dosage ; Calcifediol - pharmacology ; Calcium ; Calcium (dietary) ; Calcium, Dietary - administration & dosage ; Calcium, Dietary - pharmacology ; Child ; Child, Preschool ; Children ; Collagen ; Collagen Type I - metabolism ; Corticoids ; Dietary supplements ; Diets ; Diseases of striated muscles. Neuromuscular diseases ; Diseases of the osteoarticular system ; Duchenne muscular dystrophy ; Duchenne's muscular dystrophy ; Endocrinology ; Fractures ; Glucocorticoids - adverse effects ; Humans ; Investigative techniques, diagnostic techniques (general aspects) ; Male ; Medical sciences ; Medicine ; Medicine & Public Health ; Muscular dystrophy ; Muscular Dystrophy, Duchenne - complications ; Muscular Dystrophy, Duchenne - drug therapy ; Neurology ; Nutrient deficiency ; Original Article ; Orthopedics ; Osteoarticular system. Muscles ; Osteocalcin ; Osteocalcin - blood ; Osteoporosis ; Osteoporosis. Osteomalacia. Paget disease ; Parathyroid hormone ; Parathyroid Hormone - blood ; Peptides - metabolism ; procollagen ; Prospective Studies ; Radiodiagnosis. Nmr imagery. Nmr spectrometry ; Rheumatology ; Risk assessment ; Spine ; Treatment Outcome ; Vitamin D ; Vitamin D - analogs & derivatives ; Vitamin D - blood ; Vitamin D Deficiency - drug therapy ; Vitamin D3</subject><ispartof>Osteoporosis international, 2011-02, Vol.22 (2), p.529-539</ispartof><rights>International Osteoporosis Foundation and National Osteoporosis Foundation 2010</rights><rights>2015 INIST-CNRS</rights><rights>International Osteoporosis Foundation and National Osteoporosis Foundation 2011</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c522t-76b2b168331c948dae261cce4a9030f7f76026e03ce3c8c330a2205da784f47b3</citedby><cites>FETCH-LOGICAL-c522t-76b2b168331c948dae261cce4a9030f7f76026e03ce3c8c330a2205da784f47b3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00198-010-1275-5$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00198-010-1275-5$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,776,780,27901,27902,41464,42533,51294</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=23829483$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/20458570$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Bianchi, M. L</creatorcontrib><creatorcontrib>Morandi, L</creatorcontrib><creatorcontrib>Andreucci, E</creatorcontrib><creatorcontrib>Vai, S</creatorcontrib><creatorcontrib>Frasunkiewicz, J</creatorcontrib><creatorcontrib>Cottafava, R</creatorcontrib><title>Low bone density and bone metabolism alterations in Duchenne muscular dystrophy: response to calcium and vitamin D treatment</title><title>Osteoporosis international</title><addtitle>Osteoporos Int</addtitle><addtitle>Osteoporos Int</addtitle><description>Summary Boys with Duchenne muscular dystrophy often have reduced bone mass and increased fracture risk. In this prospective study on 33 patients, calcifediol (25-OH vitamin D₃) plus adjustment of dietary calcium to the recommended dose reduced bone resorption, corrected vitamin D deficiency, and increased bone mass in about two-thirds of cases. Introduction Low BMC and BMD and bone metabolism alterations are frequent in boys with Duchenne muscular dystrophy (DMD), especially now that long-term glucocorticosteroid (GC) treatment is the standard of care. This prospective study was designed to evaluate the effects of a first-line treatment (25-OH vitamin D₃ [calcifediol] plus adjustment of dietary calcium to the recommended daily dose) on bone. Methods Thirty-three children with DMD on GC treatment were followed for 3 years: one of observation and two of treatment. Main outcome: spine and total body BMC and BMD increase; secondary outcome: changes in bone turnover markers (C-terminal [CTx] and N-terminal [NTx] telopeptides of procollagen type I; osteocalcin [OC]). Results During the observation year, BMC and BMD decreased in all patients. At baseline and after 12 months, serum CTx and urinary NTx were higher than normal; OC and parathyroid hormone at the upper limit of normal; 25-OH vitamin D₃ significantly lower than normal. After 2 years of calcifediol and calcium-rich diet, BMC and BMD significantly increased in over 65% of patients, and bone metabolism parameters and turnover markers normalized in most patients (78.8%). During the observation year, there were four fractures in four patients, while during the 2 years of treatment there were two fractures in two patients. Conclusions Calcifediol plus adequate dietary calcium intake seems to be an effective first-line approach that controls bone turnover, corrects vitamin D deficiency, and increases BMC and BMD in most patients with DMD. Lack of response seems related to persistently high bone turnover.</description><subject>Adolescent</subject><subject>Biological and medical sciences</subject><subject>bone density</subject><subject>Bone Density - drug effects</subject><subject>Bone Density Conservation Agents - administration & dosage</subject><subject>Bone Density Conservation Agents - pharmacology</subject><subject>Bone mass</subject><subject>Bone mineral content</subject><subject>Bone mineral density</subject><subject>Bone Remodeling - drug effects</subject><subject>Bone resorption</subject><subject>Bone Resorption - drug therapy</subject><subject>Bone turnover</subject><subject>Bones</subject><subject>Calcifediol - administration & dosage</subject><subject>Calcifediol - pharmacology</subject><subject>Calcium</subject><subject>Calcium (dietary)</subject><subject>Calcium, Dietary - administration & dosage</subject><subject>Calcium, Dietary - pharmacology</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Children</subject><subject>Collagen</subject><subject>Collagen Type I - metabolism</subject><subject>Corticoids</subject><subject>Dietary supplements</subject><subject>Diets</subject><subject>Diseases of striated muscles. Neuromuscular diseases</subject><subject>Diseases of the osteoarticular system</subject><subject>Duchenne muscular dystrophy</subject><subject>Duchenne's muscular dystrophy</subject><subject>Endocrinology</subject><subject>Fractures</subject><subject>Glucocorticoids - adverse effects</subject><subject>Humans</subject><subject>Investigative techniques, diagnostic techniques (general aspects)</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Muscular dystrophy</subject><subject>Muscular Dystrophy, Duchenne - complications</subject><subject>Muscular Dystrophy, Duchenne - drug therapy</subject><subject>Neurology</subject><subject>Nutrient deficiency</subject><subject>Original Article</subject><subject>Orthopedics</subject><subject>Osteoarticular system. Muscles</subject><subject>Osteocalcin</subject><subject>Osteocalcin - blood</subject><subject>Osteoporosis</subject><subject>Osteoporosis. Osteomalacia. Paget disease</subject><subject>Parathyroid hormone</subject><subject>Parathyroid Hormone - blood</subject><subject>Peptides - metabolism</subject><subject>procollagen</subject><subject>Prospective Studies</subject><subject>Radiodiagnosis. Nmr imagery. Nmr spectrometry</subject><subject>Rheumatology</subject><subject>Risk assessment</subject><subject>Spine</subject><subject>Treatment Outcome</subject><subject>Vitamin D</subject><subject>Vitamin D - analogs & derivatives</subject><subject>Vitamin D - blood</subject><subject>Vitamin D Deficiency - drug therapy</subject><subject>Vitamin D3</subject><issn>0937-941X</issn><issn>1433-2965</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BENPR</sourceid><recordid>eNp9kUtv1DAUhS0EokPhB7ABC6liFbh-JHa6Qy0vaSQWUImd5Tg3barEHmwHNBI_Hg8ZqMSCjS1ff-fcax9CnjJ4xQDU6wTAWl0Bg4pxVVf1PbJhUoiKt019n2ygFapqJft6Qh6ldAtF07bqITnhIGtdK9iQn9vwg3bBI-3RpzHvqfX9Wpgx2y5MY5qpnTJGm8fgEx09vVzcDfoDsiS3TDbSfp9yDLub_TmNmHaFQ5oDdXZy4zL_9vw-ZjsfxDRHtHlGnx-TB4OdEj457qfk6t3bLxcfqu2n9x8v3mwrV3OeK9V0vGONFoK5VureIm-YcyhtCwIGNagGeIMgHAqnnRBgOYe6t0rLQapOnJKXq-8uhm8LpmzmMTmcJusxLMm0tWwAhNKFfPEPeRuW6MtwRgsllS6tCsRWyMWQUsTB7OI427g3DMwhGLMGY-BwLsGYumieHY2Xbsb-r-JPEgU4OwI2lW8bovVuTHec0Ly8XRSOr1wqV_4a492E_-v-fBUNNhh7HYvx1WcOTBSQq7KIX8Idr5A</recordid><startdate>20110201</startdate><enddate>20110201</enddate><creator>Bianchi, M. L</creator><creator>Morandi, L</creator><creator>Andreucci, E</creator><creator>Vai, S</creator><creator>Frasunkiewicz, J</creator><creator>Cottafava, R</creator><general>London : Springer-Verlag</general><general>Springer-Verlag</general><general>Springer</general><general>Springer Nature B.V</general><scope>FBQ</scope><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7QP</scope><scope>7RV</scope><scope>7TS</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>KB0</scope><scope>M0S</scope><scope>M1P</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>7U7</scope><scope>C1K</scope></search><sort><creationdate>20110201</creationdate><title>Low bone density and bone metabolism alterations in Duchenne muscular dystrophy: response to calcium and vitamin D treatment</title><author>Bianchi, M. L ; Morandi, L ; Andreucci, E ; Vai, S ; Frasunkiewicz, J ; Cottafava, R</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c522t-76b2b168331c948dae261cce4a9030f7f76026e03ce3c8c330a2205da784f47b3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>Adolescent</topic><topic>Biological and medical sciences</topic><topic>bone density</topic><topic>Bone Density - drug effects</topic><topic>Bone Density Conservation Agents - administration & dosage</topic><topic>Bone Density Conservation Agents - pharmacology</topic><topic>Bone mass</topic><topic>Bone mineral content</topic><topic>Bone mineral density</topic><topic>Bone Remodeling - drug effects</topic><topic>Bone resorption</topic><topic>Bone Resorption - drug therapy</topic><topic>Bone turnover</topic><topic>Bones</topic><topic>Calcifediol - administration & dosage</topic><topic>Calcifediol - pharmacology</topic><topic>Calcium</topic><topic>Calcium (dietary)</topic><topic>Calcium, Dietary - administration & dosage</topic><topic>Calcium, Dietary - pharmacology</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Children</topic><topic>Collagen</topic><topic>Collagen Type I - metabolism</topic><topic>Corticoids</topic><topic>Dietary supplements</topic><topic>Diets</topic><topic>Diseases of striated muscles. Neuromuscular diseases</topic><topic>Diseases of the osteoarticular system</topic><topic>Duchenne muscular dystrophy</topic><topic>Duchenne's muscular dystrophy</topic><topic>Endocrinology</topic><topic>Fractures</topic><topic>Glucocorticoids - adverse effects</topic><topic>Humans</topic><topic>Investigative techniques, diagnostic techniques (general aspects)</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Muscular dystrophy</topic><topic>Muscular Dystrophy, Duchenne - complications</topic><topic>Muscular Dystrophy, Duchenne - drug therapy</topic><topic>Neurology</topic><topic>Nutrient deficiency</topic><topic>Original Article</topic><topic>Orthopedics</topic><topic>Osteoarticular system. Muscles</topic><topic>Osteocalcin</topic><topic>Osteocalcin - blood</topic><topic>Osteoporosis</topic><topic>Osteoporosis. Osteomalacia. Paget disease</topic><topic>Parathyroid hormone</topic><topic>Parathyroid Hormone - blood</topic><topic>Peptides - metabolism</topic><topic>procollagen</topic><topic>Prospective Studies</topic><topic>Radiodiagnosis. Nmr imagery. Nmr spectrometry</topic><topic>Rheumatology</topic><topic>Risk assessment</topic><topic>Spine</topic><topic>Treatment Outcome</topic><topic>Vitamin D</topic><topic>Vitamin D - analogs & derivatives</topic><topic>Vitamin D - blood</topic><topic>Vitamin D Deficiency - drug therapy</topic><topic>Vitamin D3</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Bianchi, M. L</creatorcontrib><creatorcontrib>Morandi, L</creatorcontrib><creatorcontrib>Andreucci, E</creatorcontrib><creatorcontrib>Vai, S</creatorcontrib><creatorcontrib>Frasunkiewicz, J</creatorcontrib><creatorcontrib>Cottafava, R</creatorcontrib><collection>AGRIS</collection><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Nursing & Allied Health Database</collection><collection>Physical Education Index</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Public Health Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Nursing & Allied Health Premium</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>Toxicology Abstracts</collection><collection>Environmental Sciences and Pollution Management</collection><jtitle>Osteoporosis international</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Bianchi, M. L</au><au>Morandi, L</au><au>Andreucci, E</au><au>Vai, S</au><au>Frasunkiewicz, J</au><au>Cottafava, R</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Low bone density and bone metabolism alterations in Duchenne muscular dystrophy: response to calcium and vitamin D treatment</atitle><jtitle>Osteoporosis international</jtitle><stitle>Osteoporos Int</stitle><addtitle>Osteoporos Int</addtitle><date>2011-02-01</date><risdate>2011</risdate><volume>22</volume><issue>2</issue><spage>529</spage><epage>539</epage><pages>529-539</pages><issn>0937-941X</issn><eissn>1433-2965</eissn><abstract>Summary Boys with Duchenne muscular dystrophy often have reduced bone mass and increased fracture risk. In this prospective study on 33 patients, calcifediol (25-OH vitamin D₃) plus adjustment of dietary calcium to the recommended dose reduced bone resorption, corrected vitamin D deficiency, and increased bone mass in about two-thirds of cases. Introduction Low BMC and BMD and bone metabolism alterations are frequent in boys with Duchenne muscular dystrophy (DMD), especially now that long-term glucocorticosteroid (GC) treatment is the standard of care. This prospective study was designed to evaluate the effects of a first-line treatment (25-OH vitamin D₃ [calcifediol] plus adjustment of dietary calcium to the recommended daily dose) on bone. Methods Thirty-three children with DMD on GC treatment were followed for 3 years: one of observation and two of treatment. Main outcome: spine and total body BMC and BMD increase; secondary outcome: changes in bone turnover markers (C-terminal [CTx] and N-terminal [NTx] telopeptides of procollagen type I; osteocalcin [OC]). Results During the observation year, BMC and BMD decreased in all patients. At baseline and after 12 months, serum CTx and urinary NTx were higher than normal; OC and parathyroid hormone at the upper limit of normal; 25-OH vitamin D₃ significantly lower than normal. After 2 years of calcifediol and calcium-rich diet, BMC and BMD significantly increased in over 65% of patients, and bone metabolism parameters and turnover markers normalized in most patients (78.8%). During the observation year, there were four fractures in four patients, while during the 2 years of treatment there were two fractures in two patients. Conclusions Calcifediol plus adequate dietary calcium intake seems to be an effective first-line approach that controls bone turnover, corrects vitamin D deficiency, and increases BMC and BMD in most patients with DMD. Lack of response seems related to persistently high bone turnover.</abstract><cop>London</cop><pub>London : Springer-Verlag</pub><pmid>20458570</pmid><doi>10.1007/s00198-010-1275-5</doi><tpages>11</tpages></addata></record> |
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subjects | Adolescent Biological and medical sciences bone density Bone Density - drug effects Bone Density Conservation Agents - administration & dosage Bone Density Conservation Agents - pharmacology Bone mass Bone mineral content Bone mineral density Bone Remodeling - drug effects Bone resorption Bone Resorption - drug therapy Bone turnover Bones Calcifediol - administration & dosage Calcifediol - pharmacology Calcium Calcium (dietary) Calcium, Dietary - administration & dosage Calcium, Dietary - pharmacology Child Child, Preschool Children Collagen Collagen Type I - metabolism Corticoids Dietary supplements Diets Diseases of striated muscles. Neuromuscular diseases Diseases of the osteoarticular system Duchenne muscular dystrophy Duchenne's muscular dystrophy Endocrinology Fractures Glucocorticoids - adverse effects Humans Investigative techniques, diagnostic techniques (general aspects) Male Medical sciences Medicine Medicine & Public Health Muscular dystrophy Muscular Dystrophy, Duchenne - complications Muscular Dystrophy, Duchenne - drug therapy Neurology Nutrient deficiency Original Article Orthopedics Osteoarticular system. Muscles Osteocalcin Osteocalcin - blood Osteoporosis Osteoporosis. Osteomalacia. Paget disease Parathyroid hormone Parathyroid Hormone - blood Peptides - metabolism procollagen Prospective Studies Radiodiagnosis. Nmr imagery. Nmr spectrometry Rheumatology Risk assessment Spine Treatment Outcome Vitamin D Vitamin D - analogs & derivatives Vitamin D - blood Vitamin D Deficiency - drug therapy Vitamin D3 |
title | Low bone density and bone metabolism alterations in Duchenne muscular dystrophy: response to calcium and vitamin D treatment |
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