Loading…
Predictors of Survival in Friedreich's Ataxia: A Prospective Cohort Study
ABSTRACT Background Friedreich's ataxia (FA) is a rare multisystemic disorder which can cause premature death. Objectives To investigate predictors of survival in FA. Methods Within a prospective registry established by the European Friedreich's Ataxia Consortium for Translational Studies...
Saved in:
| Published in: | Movement disorders 2024-03, Vol.39 (3), p.510-518 |
|---|---|
| Main Authors: | , , , , , , , , , , , , , , , , , , , , , , |
| Format: | Article |
| Language: | English |
| Subjects: | |
| Citations: | Items that this one cites Items that cite this one |
| Online Access: | Get full text |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| cited_by | cdi_FETCH-LOGICAL-c3887-7253815e0715cfe39766e84ad6a447d55849b4ca2df5d5af3740f604159b4e653 |
|---|---|
| cites | cdi_FETCH-LOGICAL-c3887-7253815e0715cfe39766e84ad6a447d55849b4ca2df5d5af3740f604159b4e653 |
| container_end_page | 518 |
| container_issue | 3 |
| container_start_page | 510 |
| container_title | Movement disorders |
| container_volume | 39 |
| creator | Indelicato, Elisabetta Reetz, Kathrin Maier, Sarah Nachbauer, Wolfgang Amprosi, Matthias Giunti, Paola Mariotti, Caterina Durr, Alexandra Rivera Garrido, Francisco J.R. Klopstock, Thomas Schöls, Ludger Klockgether, Thomas Bürk, Katrin Pandolfo, Massimo Didszun, Claire Grobe‐Einsler, Marcus Nanetti, Lorenzo Nenning, Lukas Kiechl, Stefan Dichtl, Wolfgang Ulmer, Hanno Schulz, Jörg B. Boesch, Sylvia |
| description | ABSTRACT
Background
Friedreich's ataxia (FA) is a rare multisystemic disorder which can cause premature death.
Objectives
To investigate predictors of survival in FA.
Methods
Within a prospective registry established by the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS; ClinicalTrials.gov identifier NCT02069509) we enrolled genetically confirmed FA patients at 11 tertiary centers and followed them in yearly intervals. We investigated overall survival applying the Kaplan–Meier method, life tables, and log‐rank test. We explored prognostic factors applying Cox proportional hazards regression and subsequently built a risk score which was assessed for discrimination and calibration performance.
Results
Between September 2010 and March 2017, we enrolled 631 FA patients. Median age at inclusion was 31 (range, 6–76) years. Until December 2022, 44 patients died and 119 terminated the study for other reasons. The 10‐year cumulative survival rate was 87%. In a multivariable analysis, the disability stage (hazard ratio [HR] 1.51, 95% CI 1.08–2.12, P = 0.02), history of arrhythmic disorder (HR 2.93, 95% CI 1.34–6.39, P = 0.007), and diabetes mellitus (HR 2.31, 95% CI 1.05–5.10, P = 0.04) were independent predictors of survival. GAA repeat lengths did not improve the survival model. A risk score built on the previously described factors plus the presence of left ventricular systolic dysfunction at echocardiography enabled identification of four trajectories to prognosticate up to 10‐year survival (log‐rank test P |
| doi_str_mv | 10.1002/mds.29687 |
| format | article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2905528901</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2973885584</sourcerecordid><originalsourceid>FETCH-LOGICAL-c3887-7253815e0715cfe39766e84ad6a447d55849b4ca2df5d5af3740f604159b4e653</originalsourceid><addsrcrecordid>eNp10EFLwzAYBuAgipvTg39AAh7UQ7ekbZrU25hOhYmD6TlkScoy2mUm7XT_3sxOD4KnwMfD-315ATjHqI8RigeV8v04zxg9AF1MEhyxmNBD0EWMkSjBjHTAifdLhDAmODsGnYThFDEUd8HT1GllZG2dh7aAs8ZtzEaU0Kzg2BmtnDZyceXhsBafRtzCIZw669da1maj4cgurKvhrG7U9hQcFaL0-mz_9sDb-P519BhNXh6eRsNJJBPGaERjErYTjSgmstBJTrNMs1SoTKQpVYSwNJ-nUsSqIIqIIqEpKjKUYhLGOiNJD1y3uWtn3xvta14ZL3VZipW2jedxjgiJWY5woJd_6NI2bhWuC4qGc3bbgrpplQw_804XfO1MJdyWY8R3_fLQL__uN9iLfWIzr7T6lT-FBjBowYcp9fb_JP58N2sjvwDaaoH6</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2973885584</pqid></control><display><type>article</type><title>Predictors of Survival in Friedreich's Ataxia: A Prospective Cohort Study</title><source>Wiley-Blackwell Read & Publish Collection</source><creator>Indelicato, Elisabetta ; Reetz, Kathrin ; Maier, Sarah ; Nachbauer, Wolfgang ; Amprosi, Matthias ; Giunti, Paola ; Mariotti, Caterina ; Durr, Alexandra ; Rivera Garrido, Francisco J.R. ; Klopstock, Thomas ; Schöls, Ludger ; Klockgether, Thomas ; Bürk, Katrin ; Pandolfo, Massimo ; Didszun, Claire ; Grobe‐Einsler, Marcus ; Nanetti, Lorenzo ; Nenning, Lukas ; Kiechl, Stefan ; Dichtl, Wolfgang ; Ulmer, Hanno ; Schulz, Jörg B. ; Boesch, Sylvia</creator><creatorcontrib>Indelicato, Elisabetta ; Reetz, Kathrin ; Maier, Sarah ; Nachbauer, Wolfgang ; Amprosi, Matthias ; Giunti, Paola ; Mariotti, Caterina ; Durr, Alexandra ; Rivera Garrido, Francisco J.R. ; Klopstock, Thomas ; Schöls, Ludger ; Klockgether, Thomas ; Bürk, Katrin ; Pandolfo, Massimo ; Didszun, Claire ; Grobe‐Einsler, Marcus ; Nanetti, Lorenzo ; Nenning, Lukas ; Kiechl, Stefan ; Dichtl, Wolfgang ; Ulmer, Hanno ; Schulz, Jörg B. ; Boesch, Sylvia ; European Friedreich's Ataxia Consortium for Translational Studies (EFACTS) ; the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS)</creatorcontrib><description>ABSTRACT
Background
Friedreich's ataxia (FA) is a rare multisystemic disorder which can cause premature death.
Objectives
To investigate predictors of survival in FA.
Methods
Within a prospective registry established by the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS; ClinicalTrials.gov identifier NCT02069509) we enrolled genetically confirmed FA patients at 11 tertiary centers and followed them in yearly intervals. We investigated overall survival applying the Kaplan–Meier method, life tables, and log‐rank test. We explored prognostic factors applying Cox proportional hazards regression and subsequently built a risk score which was assessed for discrimination and calibration performance.
Results
Between September 2010 and March 2017, we enrolled 631 FA patients. Median age at inclusion was 31 (range, 6–76) years. Until December 2022, 44 patients died and 119 terminated the study for other reasons. The 10‐year cumulative survival rate was 87%. In a multivariable analysis, the disability stage (hazard ratio [HR] 1.51, 95% CI 1.08–2.12, P = 0.02), history of arrhythmic disorder (HR 2.93, 95% CI 1.34–6.39, P = 0.007), and diabetes mellitus (HR 2.31, 95% CI 1.05–5.10, P = 0.04) were independent predictors of survival. GAA repeat lengths did not improve the survival model. A risk score built on the previously described factors plus the presence of left ventricular systolic dysfunction at echocardiography enabled identification of four trajectories to prognosticate up to 10‐year survival (log‐rank test P < 0.001).
Conclusions
Arrhythmias, progressive neurological disability, and diabetes mellitus influence the overall survival in FA. We built a survival prognostic score which identifies patients meriting closer surveillance and who may benefit from early invasive cardiac monitoring and therapy. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.</description><identifier>ISSN: 0885-3185</identifier><identifier>EISSN: 1531-8257</identifier><identifier>DOI: 10.1002/mds.29687</identifier><identifier>PMID: 38140802</identifier><language>eng</language><publisher>Hoboken, USA: John Wiley & Sons, Inc</publisher><subject>Ataxia ; cardiomyopathy ; Diabetes ; Diabetes mellitus ; disability stage ; Echocardiography ; Friedreich's ataxia ; Heart ; Life tables ; Medical prognosis ; Movement disorders ; Neurological complications ; Survival</subject><ispartof>Movement disorders, 2024-03, Vol.39 (3), p.510-518</ispartof><rights>2023 The Authors. published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.</rights><rights>2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.</rights><rights>2023. This article is published under http://creativecommons.org/licenses/by-nc-nd/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><citedby>FETCH-LOGICAL-c3887-7253815e0715cfe39766e84ad6a447d55849b4ca2df5d5af3740f604159b4e653</citedby><cites>FETCH-LOGICAL-c3887-7253815e0715cfe39766e84ad6a447d55849b4ca2df5d5af3740f604159b4e653</cites><orcidid>0000-0002-1808-2134 ; 0000-0003-1657-7368 ; 0000-0003-0217-8630 ; 0000-0002-9730-9228 ; 0000-0003-2405-3564</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/38140802$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Indelicato, Elisabetta</creatorcontrib><creatorcontrib>Reetz, Kathrin</creatorcontrib><creatorcontrib>Maier, Sarah</creatorcontrib><creatorcontrib>Nachbauer, Wolfgang</creatorcontrib><creatorcontrib>Amprosi, Matthias</creatorcontrib><creatorcontrib>Giunti, Paola</creatorcontrib><creatorcontrib>Mariotti, Caterina</creatorcontrib><creatorcontrib>Durr, Alexandra</creatorcontrib><creatorcontrib>Rivera Garrido, Francisco J.R.</creatorcontrib><creatorcontrib>Klopstock, Thomas</creatorcontrib><creatorcontrib>Schöls, Ludger</creatorcontrib><creatorcontrib>Klockgether, Thomas</creatorcontrib><creatorcontrib>Bürk, Katrin</creatorcontrib><creatorcontrib>Pandolfo, Massimo</creatorcontrib><creatorcontrib>Didszun, Claire</creatorcontrib><creatorcontrib>Grobe‐Einsler, Marcus</creatorcontrib><creatorcontrib>Nanetti, Lorenzo</creatorcontrib><creatorcontrib>Nenning, Lukas</creatorcontrib><creatorcontrib>Kiechl, Stefan</creatorcontrib><creatorcontrib>Dichtl, Wolfgang</creatorcontrib><creatorcontrib>Ulmer, Hanno</creatorcontrib><creatorcontrib>Schulz, Jörg B.</creatorcontrib><creatorcontrib>Boesch, Sylvia</creatorcontrib><creatorcontrib>European Friedreich's Ataxia Consortium for Translational Studies (EFACTS)</creatorcontrib><creatorcontrib>the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS)</creatorcontrib><title>Predictors of Survival in Friedreich's Ataxia: A Prospective Cohort Study</title><title>Movement disorders</title><addtitle>Mov Disord</addtitle><description>ABSTRACT
Background
Friedreich's ataxia (FA) is a rare multisystemic disorder which can cause premature death.
Objectives
To investigate predictors of survival in FA.
Methods
Within a prospective registry established by the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS; ClinicalTrials.gov identifier NCT02069509) we enrolled genetically confirmed FA patients at 11 tertiary centers and followed them in yearly intervals. We investigated overall survival applying the Kaplan–Meier method, life tables, and log‐rank test. We explored prognostic factors applying Cox proportional hazards regression and subsequently built a risk score which was assessed for discrimination and calibration performance.
Results
Between September 2010 and March 2017, we enrolled 631 FA patients. Median age at inclusion was 31 (range, 6–76) years. Until December 2022, 44 patients died and 119 terminated the study for other reasons. The 10‐year cumulative survival rate was 87%. In a multivariable analysis, the disability stage (hazard ratio [HR] 1.51, 95% CI 1.08–2.12, P = 0.02), history of arrhythmic disorder (HR 2.93, 95% CI 1.34–6.39, P = 0.007), and diabetes mellitus (HR 2.31, 95% CI 1.05–5.10, P = 0.04) were independent predictors of survival. GAA repeat lengths did not improve the survival model. A risk score built on the previously described factors plus the presence of left ventricular systolic dysfunction at echocardiography enabled identification of four trajectories to prognosticate up to 10‐year survival (log‐rank test P < 0.001).
Conclusions
Arrhythmias, progressive neurological disability, and diabetes mellitus influence the overall survival in FA. We built a survival prognostic score which identifies patients meriting closer surveillance and who may benefit from early invasive cardiac monitoring and therapy. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.</description><subject>Ataxia</subject><subject>cardiomyopathy</subject><subject>Diabetes</subject><subject>Diabetes mellitus</subject><subject>disability stage</subject><subject>Echocardiography</subject><subject>Friedreich's ataxia</subject><subject>Heart</subject><subject>Life tables</subject><subject>Medical prognosis</subject><subject>Movement disorders</subject><subject>Neurological complications</subject><subject>Survival</subject><issn>0885-3185</issn><issn>1531-8257</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><sourceid>24P</sourceid><recordid>eNp10EFLwzAYBuAgipvTg39AAh7UQ7ekbZrU25hOhYmD6TlkScoy2mUm7XT_3sxOD4KnwMfD-315ATjHqI8RigeV8v04zxg9AF1MEhyxmNBD0EWMkSjBjHTAifdLhDAmODsGnYThFDEUd8HT1GllZG2dh7aAs8ZtzEaU0Kzg2BmtnDZyceXhsBafRtzCIZw669da1maj4cgurKvhrG7U9hQcFaL0-mz_9sDb-P519BhNXh6eRsNJJBPGaERjErYTjSgmstBJTrNMs1SoTKQpVYSwNJ-nUsSqIIqIIqEpKjKUYhLGOiNJD1y3uWtn3xvta14ZL3VZipW2jedxjgiJWY5woJd_6NI2bhWuC4qGc3bbgrpplQw_804XfO1MJdyWY8R3_fLQL__uN9iLfWIzr7T6lT-FBjBowYcp9fb_JP58N2sjvwDaaoH6</recordid><startdate>202403</startdate><enddate>202403</enddate><creator>Indelicato, Elisabetta</creator><creator>Reetz, Kathrin</creator><creator>Maier, Sarah</creator><creator>Nachbauer, Wolfgang</creator><creator>Amprosi, Matthias</creator><creator>Giunti, Paola</creator><creator>Mariotti, Caterina</creator><creator>Durr, Alexandra</creator><creator>Rivera Garrido, Francisco J.R.</creator><creator>Klopstock, Thomas</creator><creator>Schöls, Ludger</creator><creator>Klockgether, Thomas</creator><creator>Bürk, Katrin</creator><creator>Pandolfo, Massimo</creator><creator>Didszun, Claire</creator><creator>Grobe‐Einsler, Marcus</creator><creator>Nanetti, Lorenzo</creator><creator>Nenning, Lukas</creator><creator>Kiechl, Stefan</creator><creator>Dichtl, Wolfgang</creator><creator>Ulmer, Hanno</creator><creator>Schulz, Jörg B.</creator><creator>Boesch, Sylvia</creator><general>John Wiley & Sons, Inc</general><general>Wiley Subscription Services, Inc</general><scope>24P</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7TK</scope><scope>8FD</scope><scope>FR3</scope><scope>K9.</scope><scope>NAPCQ</scope><scope>P64</scope><scope>RC3</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-1808-2134</orcidid><orcidid>https://orcid.org/0000-0003-1657-7368</orcidid><orcidid>https://orcid.org/0000-0003-0217-8630</orcidid><orcidid>https://orcid.org/0000-0002-9730-9228</orcidid><orcidid>https://orcid.org/0000-0003-2405-3564</orcidid></search><sort><creationdate>202403</creationdate><title>Predictors of Survival in Friedreich's Ataxia: A Prospective Cohort Study</title><author>Indelicato, Elisabetta ; Reetz, Kathrin ; Maier, Sarah ; Nachbauer, Wolfgang ; Amprosi, Matthias ; Giunti, Paola ; Mariotti, Caterina ; Durr, Alexandra ; Rivera Garrido, Francisco J.R. ; Klopstock, Thomas ; Schöls, Ludger ; Klockgether, Thomas ; Bürk, Katrin ; Pandolfo, Massimo ; Didszun, Claire ; Grobe‐Einsler, Marcus ; Nanetti, Lorenzo ; Nenning, Lukas ; Kiechl, Stefan ; Dichtl, Wolfgang ; Ulmer, Hanno ; Schulz, Jörg B. ; Boesch, Sylvia</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3887-7253815e0715cfe39766e84ad6a447d55849b4ca2df5d5af3740f604159b4e653</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Ataxia</topic><topic>cardiomyopathy</topic><topic>Diabetes</topic><topic>Diabetes mellitus</topic><topic>disability stage</topic><topic>Echocardiography</topic><topic>Friedreich's ataxia</topic><topic>Heart</topic><topic>Life tables</topic><topic>Medical prognosis</topic><topic>Movement disorders</topic><topic>Neurological complications</topic><topic>Survival</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Indelicato, Elisabetta</creatorcontrib><creatorcontrib>Reetz, Kathrin</creatorcontrib><creatorcontrib>Maier, Sarah</creatorcontrib><creatorcontrib>Nachbauer, Wolfgang</creatorcontrib><creatorcontrib>Amprosi, Matthias</creatorcontrib><creatorcontrib>Giunti, Paola</creatorcontrib><creatorcontrib>Mariotti, Caterina</creatorcontrib><creatorcontrib>Durr, Alexandra</creatorcontrib><creatorcontrib>Rivera Garrido, Francisco J.R.</creatorcontrib><creatorcontrib>Klopstock, Thomas</creatorcontrib><creatorcontrib>Schöls, Ludger</creatorcontrib><creatorcontrib>Klockgether, Thomas</creatorcontrib><creatorcontrib>Bürk, Katrin</creatorcontrib><creatorcontrib>Pandolfo, Massimo</creatorcontrib><creatorcontrib>Didszun, Claire</creatorcontrib><creatorcontrib>Grobe‐Einsler, Marcus</creatorcontrib><creatorcontrib>Nanetti, Lorenzo</creatorcontrib><creatorcontrib>Nenning, Lukas</creatorcontrib><creatorcontrib>Kiechl, Stefan</creatorcontrib><creatorcontrib>Dichtl, Wolfgang</creatorcontrib><creatorcontrib>Ulmer, Hanno</creatorcontrib><creatorcontrib>Schulz, Jörg B.</creatorcontrib><creatorcontrib>Boesch, Sylvia</creatorcontrib><creatorcontrib>European Friedreich's Ataxia Consortium for Translational Studies (EFACTS)</creatorcontrib><creatorcontrib>the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS)</creatorcontrib><collection>Wiley Open Access</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Neurosciences Abstracts</collection><collection>Technology Research Database</collection><collection>Engineering Research Database</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Nursing & Allied Health Premium</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Movement disorders</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Indelicato, Elisabetta</au><au>Reetz, Kathrin</au><au>Maier, Sarah</au><au>Nachbauer, Wolfgang</au><au>Amprosi, Matthias</au><au>Giunti, Paola</au><au>Mariotti, Caterina</au><au>Durr, Alexandra</au><au>Rivera Garrido, Francisco J.R.</au><au>Klopstock, Thomas</au><au>Schöls, Ludger</au><au>Klockgether, Thomas</au><au>Bürk, Katrin</au><au>Pandolfo, Massimo</au><au>Didszun, Claire</au><au>Grobe‐Einsler, Marcus</au><au>Nanetti, Lorenzo</au><au>Nenning, Lukas</au><au>Kiechl, Stefan</au><au>Dichtl, Wolfgang</au><au>Ulmer, Hanno</au><au>Schulz, Jörg B.</au><au>Boesch, Sylvia</au><aucorp>European Friedreich's Ataxia Consortium for Translational Studies (EFACTS)</aucorp><aucorp>the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS)</aucorp><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Predictors of Survival in Friedreich's Ataxia: A Prospective Cohort Study</atitle><jtitle>Movement disorders</jtitle><addtitle>Mov Disord</addtitle><date>2024-03</date><risdate>2024</risdate><volume>39</volume><issue>3</issue><spage>510</spage><epage>518</epage><pages>510-518</pages><issn>0885-3185</issn><eissn>1531-8257</eissn><abstract>ABSTRACT
Background
Friedreich's ataxia (FA) is a rare multisystemic disorder which can cause premature death.
Objectives
To investigate predictors of survival in FA.
Methods
Within a prospective registry established by the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS; ClinicalTrials.gov identifier NCT02069509) we enrolled genetically confirmed FA patients at 11 tertiary centers and followed them in yearly intervals. We investigated overall survival applying the Kaplan–Meier method, life tables, and log‐rank test. We explored prognostic factors applying Cox proportional hazards regression and subsequently built a risk score which was assessed for discrimination and calibration performance.
Results
Between September 2010 and March 2017, we enrolled 631 FA patients. Median age at inclusion was 31 (range, 6–76) years. Until December 2022, 44 patients died and 119 terminated the study for other reasons. The 10‐year cumulative survival rate was 87%. In a multivariable analysis, the disability stage (hazard ratio [HR] 1.51, 95% CI 1.08–2.12, P = 0.02), history of arrhythmic disorder (HR 2.93, 95% CI 1.34–6.39, P = 0.007), and diabetes mellitus (HR 2.31, 95% CI 1.05–5.10, P = 0.04) were independent predictors of survival. GAA repeat lengths did not improve the survival model. A risk score built on the previously described factors plus the presence of left ventricular systolic dysfunction at echocardiography enabled identification of four trajectories to prognosticate up to 10‐year survival (log‐rank test P < 0.001).
Conclusions
Arrhythmias, progressive neurological disability, and diabetes mellitus influence the overall survival in FA. We built a survival prognostic score which identifies patients meriting closer surveillance and who may benefit from early invasive cardiac monitoring and therapy. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.</abstract><cop>Hoboken, USA</cop><pub>John Wiley & Sons, Inc</pub><pmid>38140802</pmid><doi>10.1002/mds.29687</doi><tpages>9</tpages><orcidid>https://orcid.org/0000-0002-1808-2134</orcidid><orcidid>https://orcid.org/0000-0003-1657-7368</orcidid><orcidid>https://orcid.org/0000-0003-0217-8630</orcidid><orcidid>https://orcid.org/0000-0002-9730-9228</orcidid><orcidid>https://orcid.org/0000-0003-2405-3564</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0885-3185 |
| ispartof | Movement disorders, 2024-03, Vol.39 (3), p.510-518 |
| issn | 0885-3185 1531-8257 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_2905528901 |
| source | Wiley-Blackwell Read & Publish Collection |
| subjects | Ataxia cardiomyopathy Diabetes Diabetes mellitus disability stage Echocardiography Friedreich's ataxia Heart Life tables Medical prognosis Movement disorders Neurological complications Survival |
| title | Predictors of Survival in Friedreich's Ataxia: A Prospective Cohort Study |
| url | http://sfxeu10.hosted.exlibrisgroup.com/loughborough?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-22T11%3A45%3A18IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Predictors%20of%20Survival%20in%20Friedreich's%20Ataxia:%20A%20Prospective%20Cohort%20Study&rft.jtitle=Movement%20disorders&rft.au=Indelicato,%20Elisabetta&rft.aucorp=European%20Friedreich's%20Ataxia%20Consortium%20for%20Translational%20Studies%20(EFACTS)&rft.date=2024-03&rft.volume=39&rft.issue=3&rft.spage=510&rft.epage=518&rft.pages=510-518&rft.issn=0885-3185&rft.eissn=1531-8257&rft_id=info:doi/10.1002/mds.29687&rft_dat=%3Cproquest_cross%3E2973885584%3C/proquest_cross%3E%3Cgrp_id%3Ecdi_FETCH-LOGICAL-c3887-7253815e0715cfe39766e84ad6a447d55849b4ca2df5d5af3740f604159b4e653%3C/grp_id%3E%3Coa%3E%3C/oa%3E%3Curl%3E%3C/url%3E&rft_id=info:oai/&rft_pqid=2973885584&rft_id=info:pmid/38140802&rfr_iscdi=true |