{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T04:46:45Z","timestamp":1773118005091,"version":"3.50.1"},"reference-count":21,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,3,7]],"date-time":"2024-03-07T00:00:00Z","timestamp":1709769600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,3,7]],"date-time":"2024-03-07T00:00:00Z","timestamp":1709769600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Orphanet J Rare Dis"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>This study aimed to assess medication adherence and demographic, clinical, and psychopathological parameters such as quality of life, depression, and anxiety levels that can affect pediatrics with Wilson\u2019s Disease (WD).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>A prospective cohort study was conducted at an outpatient clinic in Turkey among pediatric patients (2 to 18 years) with WD between November 2022 and April 2023. The Medication Adherence Report Scale (MARS-5) as a subjective and Medication Possession Ratio (MPR) as an objective assessment were scored. Physical, genetic and biochemical parameters, the Pediatric Quality of Life Inventory (PedsQL) for both parents and patients, Childhood Depression Inventory, State Trait Anxiety Inventory were also administered.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>A total of 30 pediatric outpatients who were prescribed D-penicillamine (<jats:italic>n<\/jats:italic>\u2009=\u200927) or trientine (<jats:italic>n<\/jats:italic>\u2009=\u20093) as chelators and zinc (<jats:italic>n<\/jats:italic>\u2009=\u200929) and pyridoxine (<jats:italic>n<\/jats:italic>\u2009=\u200919) as supplements were included. Proteinuria (<jats:italic>n<\/jats:italic>\u2009=\u20093), skin rash (<jats:italic>n<\/jats:italic>\u2009=\u20092), and gastrointestinal upset (<jats:italic>n<\/jats:italic>\u2009=\u20092) were observed. When the correlation between MARS-5 and duration of follow-up was examined, a significant negative correlation was found (<jats:italic>p<\/jats:italic>\u2009=\u20090.014). According to MPRs, non-adherence rates (missed doses\u2009\u2265\u200920%) were 29.6%, 17.2% and 5.3% for D-penicillamine, zinc and pyridoxine, respectively. PedsQL scores were higher than those of parents, with a positive correlation between them (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). Also, there was a significant positive correlation between PedsQL and State Anxiety Inventory (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). Comparing the change in urinary copper levels between different levels of treatment knowledge, significant differences were observed between high- and low levels (<jats:italic>p<\/jats:italic>\u2009=\u20090.043).<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Overall, nonadherence rates were 23.3% based on MARS-5 and 5.3\u201329.6% based on MPR. It is essential to consider factors such as the duration of follow-up, biochemical parameters, treatment knowledge, quality of life and anxiety as potential influencers of medication adherence.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13023-024-03113-0","type":"journal-article","created":{"date-parts":[[2024,3,7]],"date-time":"2024-03-07T19:01:54Z","timestamp":1709838114000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Adherence to medical treatment for Wilson\u2019s disease in children and adolescents: a cohort study from Turkey"],"prefix":"10.1186","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2067-8663","authenticated-orcid":false,"given":"Mehmet Akif","family":"G\u00f6kta\u015f","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2280-8727","authenticated-orcid":false,"given":"Nadir","family":"Yalcin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,3,7]]},"reference":[{"issue":"1","key":"3113_CR1","doi-asserted-by":"publisher","first-page":"21","DOI":"10.4103\/sjg.sjg_501_21","volume":"28","author":"A Chanpong","year":"2022","unstructured":"Chanpong A, Dhawan A. Wilson disease in children and young adults - state of the art. Saudi J Gastroenterol. 2022;28(1):21\u201331.","journal-title":"Saudi J Gastroenterol"},{"key":"3113_CR2","doi-asserted-by":"publisher","first-page":"141","DOI":"10.1016\/B978-0-444-63625-6.00012-4","volume":"142","author":"EA Roberts","year":"2017","unstructured":"Roberts EA, Socha P. Wilson disease in children. Handb Clin Neurol. 2017;142:141\u201356.","journal-title":"Handb Clin Neurol"},{"issue":"1","key":"3113_CR3","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1016\/j.jceh.2022.11.015","volume":"13","author":"B Jagadisan","year":"2023","unstructured":"Jagadisan B, Dhawan A. Adherence to Medication in Children with Liver Disease in India, the First Report - Every Journey starts with a first step! J Clin Exp Hepatol. 2023;13(1):3\u20136.","journal-title":"J Clin Exp Hepatol"},{"issue":"5","key":"3113_CR4","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1080\/09540261.2017.1311845","volume":"29","author":"G Mura","year":"2017","unstructured":"Mura G, Zimbrean PC, Demelia L, Carta MG. Psychiatric comorbidity in Wilson\u2019s disease. Int Rev Psychiatry. 2017;29(5):445\u201362.","journal-title":"Int Rev Psychiatry"},{"issue":"2","key":"3113_CR5","first-page":"141","volume":"34","author":"N Zelikovsky","year":"2008","unstructured":"Zelikovsky N, Schast AP. Eliciting accurate reports of adherence in a clinical interview: development of the medical adherence measure. Pediatr Nurs. 2008;34(2):141\u20136.","journal-title":"Pediatr Nurs"},{"issue":"7","key":"3113_CR6","doi-asserted-by":"publisher","first-page":"e69060","DOI":"10.1371\/journal.pone.0069060","volume":"8","author":"TL Vasylyeva","year":"2013","unstructured":"Vasylyeva TL, Singh R, Sheehan C, Chennasamudram SP, Hernandez AP. Self-reported adherence to medications in a pediatric renal clinic: psychological aspects. PLoS ONE. 2013;8(7):e69060.","journal-title":"PLoS ONE"},{"issue":"6","key":"3113_CR7","doi-asserted-by":"publisher","first-page":"2089","DOI":"10.1002\/hep.22261","volume":"47","author":"EA Roberts","year":"2008","unstructured":"Roberts EA, Schilsky ML. American Association for Study of Liver D: diagnosis and treatment of Wilson disease: an update. Hepatology. 2008;47(6):2089\u2013111.","journal-title":"Hepatology"},{"issue":"10","key":"3113_CR8","doi-asserted-by":"publisher","first-page":"1756","DOI":"10.2105\/AJPH.97.10.1756","volume":"97","author":"AB Hollingshead","year":"2007","unstructured":"Hollingshead AB, Redlich FC. Social class and mental illness: a community study. 1958. Am J Public Health. 2007;97(10):1756\u20137.","journal-title":"Am J Public Health"},{"issue":"1","key":"3113_CR9","doi-asserted-by":"publisher","first-page":"23","DOI":"10.4306\/pi.2015.12.1.23","volume":"12","author":"YR Bang","year":"2015","unstructured":"Bang YR, Park JH, Kim SH. Cut-off scores of the children\u2019s Depression Inventory for Screening and Rating Severity in Korean adolescents. Psychiatry Investig. 2015;12(1):23\u20138.","journal-title":"Psychiatry Investig"},{"issue":"1","key":"3113_CR10","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1007\/s10880-019-09631-5","volume":"27","author":"LM Shain","year":"2020","unstructured":"Shain LM, Pao M, Tipton MV, Bedoya SZ, Kang SJ, Horowitz LM, Wiener L. Comparing parent and child self-report measures of the state-trait anxiety inventory in children and adolescents with a Chronic Health Condition. J Clin Psychol Med Settings. 2020;27(1):173\u201381.","journal-title":"J Clin Psychol Med Settings"},{"issue":"11","key":"3113_CR11","doi-asserted-by":"publisher","first-page":"3107","DOI":"10.1007\/s11136-019-02237-2","volume":"28","author":"WH Emons","year":"2019","unstructured":"Emons WH, Habibovic M, Pedersen SS. Prevalence of anxiety in patients with an implantable cardioverter defibrillator: measurement equivalence of the HADS-A and the STAI-S. Qual Life Res. 2019;28(11):3107\u201316.","journal-title":"Qual Life Res"},{"issue":"1","key":"3113_CR12","doi-asserted-by":"publisher","first-page":"52","DOI":"10.26650\/IUITFD.413637","volume":"82","author":"T\u015e Esin","year":"2019","unstructured":"Esin T\u015e, Hanife SB, Dil\u015fad S. The validity and reliability study of the Turkish adaptation of medical adherence report scale. J Istanbul Fac Med. 2019;82(1):52\u201361.","journal-title":"J Istanbul Fac Med"},{"issue":"1","key":"3113_CR13","doi-asserted-by":"publisher","first-page":"23","DOI":"10.1186\/2047-9158-1-23","volume":"1","author":"QY Dong","year":"2012","unstructured":"Dong QY, Wu ZY. Advance in the pathogenesis and treatment of Wilson disease. Transl Neurodegener. 2012;1(1):23.","journal-title":"Transl Neurodegener"},{"key":"3113_CR14","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1186\/1750-1172-9-41","volume":"9","author":"G Ranucci","year":"2014","unstructured":"Ranucci G, Di Dato F, Spagnuolo MI, Vajro P, Iorio R. Zinc monotherapy is effective in Wilson\u2019s disease patients with mild liver disease diagnosed in childhood: a retrospective study. Orphanet J Rare Dis. 2014;9:41.","journal-title":"Orphanet J Rare Dis"},{"issue":"1","key":"3113_CR15","doi-asserted-by":"publisher","first-page":"22","DOI":"10.1016\/j.jceh.2022.10.006","volume":"13","author":"A Suchismita","year":"2023","unstructured":"Suchismita A, Ashritha A, Sood V, Lal BB, Khanna R, Kumar G, Alam S. Study of adherence to Medication in Pediatric Liver diseases (SAMPLD Study) in Indian children. J Clin Exp Hepatol. 2023;13(1):22\u201330.","journal-title":"J Clin Exp Hepatol"},{"issue":"9","key":"3113_CR16","doi-asserted-by":"publisher","first-page":"101978","DOI":"10.1016\/j.clinre.2022.101978","volume":"46","author":"O Guillaud","year":"2022","unstructured":"Guillaud O, Woimant F, Couchonnal E, Dumortier J, Laurencin C, Lion-Francois L, Belmalih A, Bost M, Morvan E, Oussedik-Djebrani N, et al. Maintenance therapy simplification using a single daily dose: a preliminary real-life feasibility study in patients with Wilson disease. Clin Res Hepatol Gastroenterol. 2022;46(9):101978.","journal-title":"Clin Res Hepatol Gastroenterol"},{"issue":"5","key":"3113_CR17","doi-asserted-by":"publisher","first-page":"1433","DOI":"10.1007\/s10620-014-3495-6","volume":"60","author":"A Ala","year":"2015","unstructured":"Ala A, Aliu E, Schilsky ML. Prospective pilot study of a single daily dosage of trientine for the treatment of Wilson disease. Dig Dis Sci. 2015;60(5):1433\u20139.","journal-title":"Dig Dis Sci"},{"issue":"1","key":"3113_CR18","doi-asserted-by":"publisher","first-page":"278","DOI":"10.1186\/s12883-019-1502-4","volume":"19","author":"W Maselbas","year":"2019","unstructured":"Maselbas W, Czlonkowska A, Litwin T, Niewada M. Persistence with treatment for Wilson disease: a retrospective study. BMC Neurol. 2019;19(1):278.","journal-title":"BMC Neurol"},{"issue":"6","key":"3113_CR19","doi-asserted-by":"publisher","first-page":"1481","DOI":"10.1002\/jimd.12430","volume":"44","author":"E Jacquelet","year":"2021","unstructured":"Jacquelet E, Poujois A, Pheulpin MC, Demain A, Tinant N, Gastellier N, Woimant F. Adherence to treatment, a challenge even in treatable metabolic rare diseases: a cross sectional study of Wilson\u2019s disease. J Inherit Metab Dis. 2021;44(6):1481\u20138.","journal-title":"J Inherit Metab Dis"},{"issue":"3","key":"3113_CR20","doi-asserted-by":"publisher","first-page":"260","DOI":"10.1016\/S0028-3843(14)60040-2","volume":"44","author":"W Maselbas","year":"2010","unstructured":"Maselbas W, Chabik G, Czlonkowska A. Persistence with treatment in patients with Wilson disease. Neurol Neurochir Pol. 2010;44(3):260\u20133.","journal-title":"Neurol Neurochir Pol"},{"issue":"5","key":"3113_CR21","doi-asserted-by":"publisher","first-page":"596","DOI":"10.1097\/MPG.0b013e3180467715","volume":"44","author":"R Arnon","year":"2007","unstructured":"Arnon R, Calderon JF, Schilsky M, Emre S, Shneider BL. Wilson disease in children: serum aminotransferases and urinary copper on triethylene tetramine dihydrochloride (trientine) treatment. J Pediatr Gastroenterol Nutr. 2007;44(5):596\u2013602.","journal-title":"J Pediatr Gastroenterol Nutr"}],"container-title":["Orphanet Journal of Rare Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-024-03113-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13023-024-03113-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13023-024-03113-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,3,10]],"date-time":"2024-03-10T23:02:22Z","timestamp":1710111742000},"score":1,"resource":{"primary":{"URL":"https:\/\/ojrd.biomedcentral.com\/articles\/10.1186\/s13023-024-03113-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,3,7]]},"references-count":21,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["3113"],"URL":"https:\/\/doi.org\/10.1186\/s13023-024-03113-0","relation":{},"ISSN":["1750-1172"],"issn-type":[{"value":"1750-1172","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,3,7]]},"assertion":[{"value":"11 September 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 March 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 March 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The study was conducted according to the guidelines of the Declaration of Helsinki, and approved by the Ethics Committee of Hacettepe University. Informed consent was obtained from all subjects involved in the study.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"105"}}