Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?
Background Progressive cardiomyopathy is a common cause of death in Duchenne muscular dystrophy (DMD), presumably secondary to fibrosis of the myocardium. The posterobasal and left lateral free wall of the left ventricle (LV) are initial sites of myocardial fibrosis pathologically. The purposes of t...
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| Veröffentlicht in: | The International Journal of Cardiovascular Imaging 2009, Vol.25 (1), p.57-63 |
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| description | Background
Progressive cardiomyopathy is a common cause of death in Duchenne muscular dystrophy (DMD), presumably secondary to fibrosis of the myocardium. The posterobasal and left lateral free wall of the left ventricle (LV) are initial sites of myocardial fibrosis pathologically. The purposes of this study were to assess whether cardiac magnetic resonance imaging (CMRI), utilizing late gadolinium enhancement (LGE), could identify fibrosis in selective areas of the myocardium, and to assess the relationship of the presence and extent of fibrosis to LV function.
Methods
The cardiology databases at Primary Children’s Medical Center and Cincinnati Children’s Hospital Medical Center were reviewed to identify patients with DMD who had undergone a CMRI within the last 2 years. Age, LV ejection fraction, LV mass, presence and location of LGE were documented. Volumes were measured using MASS (Medis, Inc.) to calculate ejection fraction and mass. LGE images were acquired and when positive, customized computer assisted sizing of the areas of late gadolinium enhancement were performed on all slices. Normal function was defined as LV ejection fraction >54%.
Results
A total of 74 patients with DMD had complete data sets (median age 13.7 years, range 7.7–26.4). Twenty-four patients (32%) had LGE involving the posterobasal region of the LV in a sub-epicardial distribution. Those patients with more involvement had spread to the inferior and left lateral free wall with progressive transmural fibrous replacement. There was relative sparing of the interventricular septum and right ventricle. Patients with LGE were significantly older than those without (mean age 16.4 vs 12.9 years,
P
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| doi_str_mv | 10.1007/s10554-008-9352-y |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_pubme</sourceid><recordid>TN_cdi_proquest_miscellaneous_66719844</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>66719844</sourcerecordid><originalsourceid>FETCH-LOGICAL-p209t-d8372952c490d1e62c7d3cb6ab44a94b4431d4796b0e9b38e07a95786520f90d3</originalsourceid><addsrcrecordid>eNpdkU1r3DAQhkVpaZJtfkAvReTQm9PRhyWrlxA2n7DQSwu5CVlWYgdbciXr4H9fLbsh0MvMwDzzMrwvQl8JXBIA-SMRqGteATSVYjWt1g_olNSSVSA5-7ifhapqqfgJOkvpFQAoUPYZnZBGNAIIOUVPO7M4_GK6MA5-yBN2vjfeusn55Seeo7M5phDxErA1sRvCtIbZLP2KB49vsu2d9w5POdk8moi7NS0xzP169QV9ejZjcufHvkF_7m5_bx-q3a_7x-31rpopqKXqGiapqqnlCjriBLWyY7YVpuXcKF4qIx2XSrTgVMsaB9KoWjaipvBcTtgGfT_ozjH8zS4tehqSdeNovAs5aSEkUU2R2aCL_8DXkKMvv2lajKKKUyjQtyOU28l1eo7DZOKq3wwrAD0Aqaz8i4vvKgT0PhV9SEWXVPQ-Fb2yf3pnfYw</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>215629420</pqid></control><display><type>article</type><title>Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?</title><source>MEDLINE</source><source>SpringerLink Journals - AutoHoldings</source><creator>Puchalski, Michael D. ; Williams, Richard V. ; Askovich, Bojana ; Sower, C. Todd ; Hor, Kan H. ; Su, Jason T. ; Pack, Nathan ; Dibella, Edward ; Gottliebson, William M.</creator><creatorcontrib>Puchalski, Michael D. ; Williams, Richard V. ; Askovich, Bojana ; Sower, C. Todd ; Hor, Kan H. ; Su, Jason T. ; Pack, Nathan ; Dibella, Edward ; Gottliebson, William M.</creatorcontrib><description>Background
Progressive cardiomyopathy is a common cause of death in Duchenne muscular dystrophy (DMD), presumably secondary to fibrosis of the myocardium. The posterobasal and left lateral free wall of the left ventricle (LV) are initial sites of myocardial fibrosis pathologically. The purposes of this study were to assess whether cardiac magnetic resonance imaging (CMRI), utilizing late gadolinium enhancement (LGE), could identify fibrosis in selective areas of the myocardium, and to assess the relationship of the presence and extent of fibrosis to LV function.
Methods
The cardiology databases at Primary Children’s Medical Center and Cincinnati Children’s Hospital Medical Center were reviewed to identify patients with DMD who had undergone a CMRI within the last 2 years. Age, LV ejection fraction, LV mass, presence and location of LGE were documented. Volumes were measured using MASS (Medis, Inc.) to calculate ejection fraction and mass. LGE images were acquired and when positive, customized computer assisted sizing of the areas of late gadolinium enhancement were performed on all slices. Normal function was defined as LV ejection fraction >54%.
Results
A total of 74 patients with DMD had complete data sets (median age 13.7 years, range 7.7–26.4). Twenty-four patients (32%) had LGE involving the posterobasal region of the LV in a sub-epicardial distribution. Those patients with more involvement had spread to the inferior and left lateral free wall with progressive transmural fibrous replacement. There was relative sparing of the interventricular septum and right ventricle. Patients with LGE were significantly older than those without (mean age 16.4 vs 12.9 years,
P
< 0.001). LGE was positively associated with BSA-adjusted LV mass, LV end-diastolic volume, LV end-systolic volume, and RV end-systolic volume but inversely correlated with ejection fraction of the LV (
P
< 0.001) and RV (
P
= 0.004).
Conclusions
LGE by CMRI is able to detect fibrosis in selective regions of myocardium in patients with DMD. Unfavorable LV remodeling, with a corresponding decreased ejection fraction, is associated with the presence of LGE. Serial studies are warranted to determine if LGE precedes a decrease in function, and if early medical management is useful in preventing progression once LGE is documented.</description><identifier>ISSN: 1569-5794</identifier><identifier>EISSN: 1573-0743</identifier><identifier>EISSN: 1875-8312</identifier><identifier>DOI: 10.1007/s10554-008-9352-y</identifier><identifier>PMID: 18686011</identifier><identifier>CODEN: IJCIBI</identifier><language>eng</language><publisher>Dordrecht: Springer Netherlands</publisher><subject>Adolescent ; Cardiac Imaging ; Cardiology ; Cardiomyopathies - diagnosis ; Cardiomyopathies - etiology ; Child ; Contrast Media ; Female ; Gadolinium ; Humans ; Image Enhancement - methods ; Image Interpretation, Computer-Assisted ; Imaging ; Linear Models ; Magnetic Resonance Imaging - methods ; Male ; Medicine ; Medicine & Public Health ; Muscular Dystrophy, Duchenne - complications ; Original Paper ; Radiology ; Young Adult</subject><ispartof>The International Journal of Cardiovascular Imaging, 2009, Vol.25 (1), p.57-63</ispartof><rights>Springer Science+Business Media, B.V. 2008</rights><rights>Springer Science+Business Media, B.V. 2009</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-p209t-d8372952c490d1e62c7d3cb6ab44a94b4431d4796b0e9b38e07a95786520f90d3</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/s10554-008-9352-y$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s10554-008-9352-y$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,777,781,27905,27906,41469,42538,51300</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18686011$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Puchalski, Michael D.</creatorcontrib><creatorcontrib>Williams, Richard V.</creatorcontrib><creatorcontrib>Askovich, Bojana</creatorcontrib><creatorcontrib>Sower, C. Todd</creatorcontrib><creatorcontrib>Hor, Kan H.</creatorcontrib><creatorcontrib>Su, Jason T.</creatorcontrib><creatorcontrib>Pack, Nathan</creatorcontrib><creatorcontrib>Dibella, Edward</creatorcontrib><creatorcontrib>Gottliebson, William M.</creatorcontrib><title>Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?</title><title>The International Journal of Cardiovascular Imaging</title><addtitle>Int J Cardiovasc Imaging</addtitle><addtitle>Int J Cardiovasc Imaging</addtitle><description>Background
Progressive cardiomyopathy is a common cause of death in Duchenne muscular dystrophy (DMD), presumably secondary to fibrosis of the myocardium. The posterobasal and left lateral free wall of the left ventricle (LV) are initial sites of myocardial fibrosis pathologically. The purposes of this study were to assess whether cardiac magnetic resonance imaging (CMRI), utilizing late gadolinium enhancement (LGE), could identify fibrosis in selective areas of the myocardium, and to assess the relationship of the presence and extent of fibrosis to LV function.
Methods
The cardiology databases at Primary Children’s Medical Center and Cincinnati Children’s Hospital Medical Center were reviewed to identify patients with DMD who had undergone a CMRI within the last 2 years. Age, LV ejection fraction, LV mass, presence and location of LGE were documented. Volumes were measured using MASS (Medis, Inc.) to calculate ejection fraction and mass. LGE images were acquired and when positive, customized computer assisted sizing of the areas of late gadolinium enhancement were performed on all slices. Normal function was defined as LV ejection fraction >54%.
Results
A total of 74 patients with DMD had complete data sets (median age 13.7 years, range 7.7–26.4). Twenty-four patients (32%) had LGE involving the posterobasal region of the LV in a sub-epicardial distribution. Those patients with more involvement had spread to the inferior and left lateral free wall with progressive transmural fibrous replacement. There was relative sparing of the interventricular septum and right ventricle. Patients with LGE were significantly older than those without (mean age 16.4 vs 12.9 years,
P
< 0.001). LGE was positively associated with BSA-adjusted LV mass, LV end-diastolic volume, LV end-systolic volume, and RV end-systolic volume but inversely correlated with ejection fraction of the LV (
P
< 0.001) and RV (
P
= 0.004).
Conclusions
LGE by CMRI is able to detect fibrosis in selective regions of myocardium in patients with DMD. Unfavorable LV remodeling, with a corresponding decreased ejection fraction, is associated with the presence of LGE. Serial studies are warranted to determine if LGE precedes a decrease in function, and if early medical management is useful in preventing progression once LGE is documented.</description><subject>Adolescent</subject><subject>Cardiac Imaging</subject><subject>Cardiology</subject><subject>Cardiomyopathies - diagnosis</subject><subject>Cardiomyopathies - etiology</subject><subject>Child</subject><subject>Contrast Media</subject><subject>Female</subject><subject>Gadolinium</subject><subject>Humans</subject><subject>Image Enhancement - methods</subject><subject>Image Interpretation, Computer-Assisted</subject><subject>Imaging</subject><subject>Linear Models</subject><subject>Magnetic Resonance Imaging - methods</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Muscular Dystrophy, Duchenne - complications</subject><subject>Original Paper</subject><subject>Radiology</subject><subject>Young Adult</subject><issn>1569-5794</issn><issn>1573-0743</issn><issn>1875-8312</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2009</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><recordid>eNpdkU1r3DAQhkVpaZJtfkAvReTQm9PRhyWrlxA2n7DQSwu5CVlWYgdbciXr4H9fLbsh0MvMwDzzMrwvQl8JXBIA-SMRqGteATSVYjWt1g_olNSSVSA5-7ifhapqqfgJOkvpFQAoUPYZnZBGNAIIOUVPO7M4_GK6MA5-yBN2vjfeusn55Seeo7M5phDxErA1sRvCtIbZLP2KB49vsu2d9w5POdk8moi7NS0xzP169QV9ejZjcufHvkF_7m5_bx-q3a_7x-31rpopqKXqGiapqqnlCjriBLWyY7YVpuXcKF4qIx2XSrTgVMsaB9KoWjaipvBcTtgGfT_ozjH8zS4tehqSdeNovAs5aSEkUU2R2aCL_8DXkKMvv2lajKKKUyjQtyOU28l1eo7DZOKq3wwrAD0Aqaz8i4vvKgT0PhV9SEWXVPQ-Fb2yf3pnfYw</recordid><startdate>2009</startdate><enddate>2009</enddate><creator>Puchalski, Michael D.</creator><creator>Williams, Richard V.</creator><creator>Askovich, Bojana</creator><creator>Sower, C. Todd</creator><creator>Hor, Kan H.</creator><creator>Su, Jason T.</creator><creator>Pack, Nathan</creator><creator>Dibella, Edward</creator><creator>Gottliebson, William M.</creator><general>Springer Netherlands</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>3V.</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FD</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>M7Z</scope><scope>P64</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>7X8</scope></search><sort><creationdate>2009</creationdate><title>Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?</title><author>Puchalski, Michael D. ; Williams, Richard V. ; Askovich, Bojana ; Sower, C. Todd ; Hor, Kan H. ; Su, Jason T. ; Pack, Nathan ; Dibella, Edward ; Gottliebson, William M.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p209t-d8372952c490d1e62c7d3cb6ab44a94b4431d4796b0e9b38e07a95786520f90d3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2009</creationdate><topic>Adolescent</topic><topic>Cardiac Imaging</topic><topic>Cardiology</topic><topic>Cardiomyopathies - diagnosis</topic><topic>Cardiomyopathies - etiology</topic><topic>Child</topic><topic>Contrast Media</topic><topic>Female</topic><topic>Gadolinium</topic><topic>Humans</topic><topic>Image Enhancement - methods</topic><topic>Image Interpretation, Computer-Assisted</topic><topic>Imaging</topic><topic>Linear Models</topic><topic>Magnetic Resonance Imaging - methods</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Muscular Dystrophy, Duchenne - complications</topic><topic>Original Paper</topic><topic>Radiology</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Puchalski, Michael D.</creatorcontrib><creatorcontrib>Williams, Richard V.</creatorcontrib><creatorcontrib>Askovich, Bojana</creatorcontrib><creatorcontrib>Sower, C. Todd</creatorcontrib><creatorcontrib>Hor, Kan H.</creatorcontrib><creatorcontrib>Su, Jason T.</creatorcontrib><creatorcontrib>Pack, Nathan</creatorcontrib><creatorcontrib>Dibella, Edward</creatorcontrib><creatorcontrib>Gottliebson, William M.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>ProQuest Central (Corporate)</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>Technology Research 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>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Biochemistry Abstracts 1</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>MEDLINE - Academic</collection><jtitle>The International Journal of Cardiovascular Imaging</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Puchalski, Michael D.</au><au>Williams, Richard V.</au><au>Askovich, Bojana</au><au>Sower, C. Todd</au><au>Hor, Kan H.</au><au>Su, Jason T.</au><au>Pack, Nathan</au><au>Dibella, Edward</au><au>Gottliebson, William M.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy?</atitle><jtitle>The International Journal of Cardiovascular Imaging</jtitle><stitle>Int J Cardiovasc Imaging</stitle><addtitle>Int J Cardiovasc Imaging</addtitle><date>2009</date><risdate>2009</risdate><volume>25</volume><issue>1</issue><spage>57</spage><epage>63</epage><pages>57-63</pages><issn>1569-5794</issn><eissn>1573-0743</eissn><eissn>1875-8312</eissn><coden>IJCIBI</coden><abstract>Background
Progressive cardiomyopathy is a common cause of death in Duchenne muscular dystrophy (DMD), presumably secondary to fibrosis of the myocardium. The posterobasal and left lateral free wall of the left ventricle (LV) are initial sites of myocardial fibrosis pathologically. The purposes of this study were to assess whether cardiac magnetic resonance imaging (CMRI), utilizing late gadolinium enhancement (LGE), could identify fibrosis in selective areas of the myocardium, and to assess the relationship of the presence and extent of fibrosis to LV function.
Methods
The cardiology databases at Primary Children’s Medical Center and Cincinnati Children’s Hospital Medical Center were reviewed to identify patients with DMD who had undergone a CMRI within the last 2 years. Age, LV ejection fraction, LV mass, presence and location of LGE were documented. Volumes were measured using MASS (Medis, Inc.) to calculate ejection fraction and mass. LGE images were acquired and when positive, customized computer assisted sizing of the areas of late gadolinium enhancement were performed on all slices. Normal function was defined as LV ejection fraction >54%.
Results
A total of 74 patients with DMD had complete data sets (median age 13.7 years, range 7.7–26.4). Twenty-four patients (32%) had LGE involving the posterobasal region of the LV in a sub-epicardial distribution. Those patients with more involvement had spread to the inferior and left lateral free wall with progressive transmural fibrous replacement. There was relative sparing of the interventricular septum and right ventricle. Patients with LGE were significantly older than those without (mean age 16.4 vs 12.9 years,
P
< 0.001). LGE was positively associated with BSA-adjusted LV mass, LV end-diastolic volume, LV end-systolic volume, and RV end-systolic volume but inversely correlated with ejection fraction of the LV (
P
< 0.001) and RV (
P
= 0.004).
Conclusions
LGE by CMRI is able to detect fibrosis in selective regions of myocardium in patients with DMD. Unfavorable LV remodeling, with a corresponding decreased ejection fraction, is associated with the presence of LGE. Serial studies are warranted to determine if LGE precedes a decrease in function, and if early medical management is useful in preventing progression once LGE is documented.</abstract><cop>Dordrecht</cop><pub>Springer Netherlands</pub><pmid>18686011</pmid><doi>10.1007/s10554-008-9352-y</doi><tpages>7</tpages></addata></record> |
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| subjects | Adolescent Cardiac Imaging Cardiology Cardiomyopathies - diagnosis Cardiomyopathies - etiology Child Contrast Media Female Gadolinium Humans Image Enhancement - methods Image Interpretation, Computer-Assisted Imaging Linear Models Magnetic Resonance Imaging - methods Male Medicine Medicine & Public Health Muscular Dystrophy, Duchenne - complications Original Paper Radiology Young Adult |
| title | Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy? |
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