{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T08:39:21Z","timestamp":1793263161561,"version":"4.5.0"},"reference-count":73,"publisher":"SAGE Publications","issue":"6","license":[{"start":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T00:00:00Z","timestamp":1780876800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T00:00:00Z","timestamp":1780876800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"name":"Children Miracle Network"},{"name":"Friedreich\u2019s Ataxia Research Alliance","id":[{"id":"https:\/\/ror.org\/04edgdk70","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neuromuscular Diseases"],"published-print":{"date-parts":[[2026,11]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      Friedreich\u2019s ataxia (FRDA), a common hereditary ataxia with multisystem involvement, is caused by a biallelic GAA trinucleotide repeat expansion in the\n                      <jats:italic toggle=\"yes\">FXN<\/jats:italic>\n                      gene, leading to reduced frataxin, mitochondrial dysfunction, and oxidative stress. This study evaluates cough effectiveness through voluntary peak cough flow (PCF).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>The objective was to assess airway clearance dysfunction in individuals with FRDA by examining PCF and its relationship with pulmonary and oral strength, disease severity, and genetic factors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      This cross-sectional study, part of the\n                      <jats:italic toggle=\"yes\">Biomarkers in Friedreich\u2019s Ataxia<\/jats:italic>\n                      observational study, involved 51 participants with molecularly proven FRDA undergoing respiratory testing, including spirometry, PCF, maximal respiratory pressures, sniff nasal inspiratory pressure, and lingual strength. Neurological impairment was assessed using the Modified Friedreich\u2019s Ataxia Rating Scale (mFARS) and Functional Staging for Ataxia.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Forty-seven participants completed PCF tests (31 female; mean age 22.60 years). Twenty-nine (61.7%) remained ambulant (mean mFARS 49.91). Seventeen (36.17%) had reduced PCF (&lt;270 L\/min). Lower PCF correlated with younger age, earlier disease onset, and decreased respiratory muscle strength. PCF showed strong associations with maximal inspiratory pressure (MIP), forced vital capacity (FVCpp), and maximal expiratory pressure (MEP). Multiple regression identified MIP and age of symptom onset as primary predictors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The study demonstrates respiratory dysfunction in patients with FRDA, and shows that disease severity and muscle weakness affect airway clearance. PCF is a more direct and clinically meaningful indicator of cough effectiveness than FVCpp. Comprehensive respiratory evaluations, including muscle-strength testing, can identify individuals who may benefit from targeted interventions to prevent complications.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/22143602261452334","type":"journal-article","created":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T09:21:51Z","timestamp":1780910511000},"page":"1518-1532","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Assessing airway clearance dysfunction in Friedreich\u2019s ataxia: A focus on peak cough flow"],"prefix":"10.1177","volume":"13","author":[{"given":"Barbara K.","family":"Smith","sequence":"first","affiliation":[{"name":"University of Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mackenzi A.","family":"Coker","sequence":"additional","affiliation":[{"name":"University of Florida"},{"name":"University of Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0394-7358","authenticated-orcid":false,"given":"Cristina","family":"Liberati","sequence":"additional","affiliation":[{"name":"University of Florida"},{"name":"University of Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Blake P.","family":"Meyer","sequence":"additional","affiliation":[{"name":"University of Florida"},{"name":"University of Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha","family":"Norman","sequence":"additional","affiliation":[{"name":"University of Florida"},{"name":"University of Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Ehrbar","sequence":"additional","affiliation":[{"name":"University of Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1800-4301","authenticated-orcid":false,"given":"Carmen","family":"Leon-Astudillo","sequence":"additional","affiliation":[{"name":"University of Florida"},{"name":"University of Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sub","family":"Subramony","sequence":"additional","affiliation":[{"name":"University of Florida"},{"name":"University of Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Corti","sequence":"additional","affiliation":[{"name":"University of Florida"},{"name":"University of Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2026,6,8]]},"reference":[{"key":"e_1_3_6_2_2","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-014-0184-7"},{"key":"e_1_3_6_3_2","doi-asserted-by":"publisher","DOI":"10.1186\/2051-5960-1-26"},{"key":"e_1_3_6_4_2","doi-asserted-by":"publisher","DOI":"10.1097\/NEN.0b013e31827e5762"},{"key":"e_1_3_6_5_2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0116396"},{"key":"e_1_3_6_6_2","first-page":"554","article-title":"The Relationship Between Trinucleotide (GAA) Repeat Length and Clinical Features in Friedreich Ataxia","volume":"59","author":"Filla A","year":"1996","unstructured":"Filla A, Michele GD, Cavalcanti F, et al. The Relationship Between Trinucleotide (GAA) Repeat Length and Clinical Features in Friedreich Ataxia. Am J Hum Genet 1996; 59: 554\u2013560.","journal-title":"Am J Hum Genet"},{"key":"e_1_3_6_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jelekin.2019.102359"},{"key":"e_1_3_6_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00408-013-9540-y"},{"key":"e_1_3_6_9_2","first-page":"20220829","article-title":"Respiratory Function in Friedreich\u2019s Ataxia","volume":"9","author":"Vinante E","year":"2022","unstructured":"Vinante E, Colombo E, Paparella G, et al. Respiratory Function in Friedreich\u2019s Ataxia. Children (Basel) 2022; 9: 20220829. https:\/\/doi.org\/10.3390\/children9091319","journal-title":"Children (Basel)"},{"key":"e_1_3_6_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/bmb\/ldx034"},{"key":"e_1_3_6_11_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1460-6984.2011.00078.x"},{"key":"e_1_3_6_12_2","doi-asserted-by":"publisher","DOI":"10.1179\/136132803805576138"},{"key":"e_1_3_6_13_2","doi-asserted-by":"publisher","DOI":"10.1123\/mcj.15.3.376"},{"key":"e_1_3_6_14_2","doi-asserted-by":"publisher","DOI":"10.3109\/17549507.2011.529940"},{"key":"e_1_3_6_15_2","doi-asserted-by":"publisher","DOI":"10.1123\/mcj.14.4.490"},{"key":"e_1_3_6_16_2","doi-asserted-by":"publisher","DOI":"10.3109\/02699206.2010.511760"},{"key":"e_1_3_6_17_2","doi-asserted-by":"publisher","DOI":"10.1159\/000287207"},{"key":"e_1_3_6_18_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00455-017-9804-4"},{"key":"e_1_3_6_19_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100025579"},{"key":"e_1_3_6_20_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100119572"},{"key":"e_1_3_6_21_2","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100119560"},{"key":"e_1_3_6_22_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(16)30287-3"},{"key":"e_1_3_6_23_2","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.332"},{"key":"e_1_3_6_24_2","unstructured":"ClinicalTrials.gov. Clinical Outcome Measures in Friedreich's Ataxia. Identifier: NCT03090789. Updated October 2024. https:\/\/clinicaltrials.gov\/ct2\/show\/NCT03090789FCOMF-C"},{"key":"e_1_3_6_25_2","doi-asserted-by":"publisher","DOI":"10.1177\/0883073812448462"},{"key":"e_1_3_6_26_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.22912"},{"key":"e_1_3_6_27_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jns.2011.05.023"},{"key":"e_1_3_6_28_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.29687"},{"key":"e_1_3_6_29_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01214-2018"},{"key":"e_1_3_6_30_2","first-page":"1475","article-title":"Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options","volume":"11","author":"LoMauro A","year":"2015","unstructured":"LoMauro A, D'Angelo MG, Aliverti A. Assessment and management of respiratory function in patients with Duchenne muscular dystrophy: current and emerging options. Therapeutics and Clinical Risk Management 2015; 11: 1475\u20131488. https:\/\/doi.org\/10.2147\/TCRM.S55889","journal-title":"Therapeutics and Clinical Risk Management"},{"key":"e_1_3_6_31_2","doi-asserted-by":"publisher","DOI":"10.1097\/PHM.0b013e318174e4c7"},{"key":"e_1_3_6_32_2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00080312"},{"key":"e_1_3_6_33_2","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-017-0598-0"},{"key":"e_1_3_6_34_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26176"},{"key":"e_1_3_6_35_2","doi-asserted-by":"publisher","DOI":"10.1212\/NXG.0000000000000371"},{"key":"e_1_3_6_36_2","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000156802.15466.79"},{"key":"e_1_3_6_37_2","doi-asserted-by":"publisher","DOI":"10.1097\/00002060-200005000-00002"},{"key":"e_1_3_6_38_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.112.4.1024"},{"key":"e_1_3_6_39_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2023.03.011"},{"key":"e_1_3_6_40_2","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2018.00578"},{"key":"e_1_3_6_41_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201703-0507WS"},{"key":"e_1_3_6_42_2","doi-asserted-by":"publisher","DOI":"10.1080\/17482960601030188"},{"key":"e_1_3_6_43_2","first-page":"20230223","article-title":"Airway clearance in patients with neuromuscular disease","volume":"47","author":"Gipsman AI","year":"2023","unstructured":"Gipsman AI, Lapinel NC, Mayer OH. Airway clearance in patients with neuromuscular disease. Paediatr Respir Rev 2023; 47: 20230223. https:\/\/doi.org\/10.1016\/j.prrv.2023.02.002","journal-title":"Paediatr Respir Rev"},{"key":"e_1_3_6_44_2","doi-asserted-by":"publisher","DOI":"10.3233\/JND-221575"},{"key":"e_1_3_6_45_2","doi-asserted-by":"publisher","DOI":"10.1044\/2022_JSLHR-21-00619"},{"key":"e_1_3_6_46_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.10-0342"},{"key":"e_1_3_6_47_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.08-1122"},{"key":"e_1_3_6_48_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00455-015-9687-1"},{"key":"e_1_3_6_49_2","doi-asserted-by":"publisher","DOI":"10.1044\/jshr.3404.768"},{"key":"e_1_3_6_50_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-013-7208-4"},{"key":"e_1_3_6_51_2","first-page":"20230910","article-title":"Propensity matched comparison of omaveloxolone treatment to Friedreich ataxia natural history data","volume":"11","author":"Lynch DR","year":"2023","unstructured":"Lynch DR, Goldsberry A, Rummey C, et al. Propensity matched comparison of omaveloxolone treatment to Friedreich ataxia natural history data. Ann Clin Transl Neurol 2023; 11: 20230910. https:\/\/doi.org\/10.1002\/acn3.51897","journal-title":"Ann Clin Transl Neurol"},{"key":"e_1_3_6_52_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00455-022-10499-1"},{"key":"e_1_3_6_53_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00408-018-0171-1"},{"key":"e_1_3_6_54_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.resp.2013.08.009"},{"key":"e_1_3_6_55_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.pmr.2008.06.001"},{"key":"e_1_3_6_56_2","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2011.213157"},{"key":"e_1_3_6_57_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00455-022-10503-8"},{"key":"e_1_3_6_58_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2015.09.003"},{"key":"e_1_3_6_59_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000206830"},{"key":"e_1_3_6_60_2","doi-asserted-by":"publisher","DOI":"10.3233\/JPD-202223"},{"key":"e_1_3_6_61_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.physio.2025.101842"},{"key":"e_1_3_6_62_2","doi-asserted-by":"publisher","DOI":"10.1097\/PEP.0000000000000648"},{"key":"e_1_3_6_63_2","doi-asserted-by":"publisher","DOI":"10.1183\/16000617.0065-2022"},{"key":"e_1_3_6_64_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.08-1389"},{"key":"e_1_3_6_65_2","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2015.00284"},{"key":"e_1_3_6_66_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00408-018-0133-7"},{"key":"e_1_3_6_67_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200307-885ST"},{"key":"e_1_3_6_68_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2006.02.003"},{"key":"e_1_3_6_69_2","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201808-506OC"},{"key":"e_1_3_6_70_2","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.10217"},{"key":"e_1_3_6_71_2","doi-asserted-by":"publisher","DOI":"10.4187\/respcare.05460"},{"key":"e_1_3_6_72_2","first-page":"20230511","article-title":"Cut-Off Value of Voluntary Peak Cough Flow in Patients with Parkinson\u2019s Disease and Its Association with Severe Dysphagia: A Retrospective Pilot Study","volume":"59","author":"Lee KW","year":"2023","unstructured":"Lee KW, Kim SB, Lee JH, et al. Cut-Off Value of Voluntary Peak Cough Flow in Patients with Parkinson\u2019s Disease and Its Association with Severe Dysphagia: A Retrospective Pilot Study. Medicina (Kaunas) 2023; 59: 20230511. https:\/\/doi.org\/10.3390\/medicina59050921","journal-title":"Medicina (Kaunas)"},{"key":"e_1_3_6_73_2","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26320"},{"key":"e_1_3_6_74_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.118.5.1390"}],"container-title":["Journal of Neuromuscular Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/22143602261452334","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/22143602261452334","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/22143602261452334","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T08:30:49Z","timestamp":1793262649000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/22143602261452334"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6,8]]},"references-count":73,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2026,11]]}},"alternative-id":["10.1177\/22143602261452334"],"URL":"https:\/\/doi.org\/10.1177\/22143602261452334","relation":{},"ISSN":["2214-3599","2214-3602"],"issn-type":[{"value":"2214-3599","type":"print"},{"value":"2214-3602","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6,8]]}}}