{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,8]],"date-time":"2026-06-08T23:40:22Z","timestamp":1780962022496,"version":"3.54.1"},"reference-count":18,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2010,3,9]],"date-time":"2010-03-09T00:00:00Z","timestamp":1268092800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2010,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The aims of the study were to assess the effectiveness of enzyme replacement therapy (ERT) with laronidase on the range of motion (ROM) of upper extremities and influence on activities of daily living (ADLs) of patients with mucopolysaccharidosis type I (MPS I). The ROM of 17 patients with MPS I was followed from the first year of life until the introduction of ERT and after 52\u2013208 weeks of treatment. In all patients (group 1, <jats:italic>n<\/jats:italic>\u2009=\u200910), passive ROM was assessed. In patients with Hurler\/Scheie or Scheie phenotype (group 2, <jats:italic>n<\/jats:italic>\u2009=\u20097) both passive and active ROM, as well as daily life activities, were evaluated. Passive and active ROM was measured by a goniometer, while a health assessment questionnaire was used to assess activities of daily living. The data since the first months of life until the beginning of treatment were obtained by retrospective review of patients\u2019 charts. Restriction in ROM of the upper extremities of patients with MPS I was observed from the first year of life. These limitations intensified and became more severe with the patients\u2019 age, making patients\u2019 self\u2010care more difficult or even impossible. Introduction of ERT led to slower progression of symptoms, especially in the passive range of motion in all patients. Additionally, patients with normal mental development, or only slightly delayed (group 2), who underwent active physical rehabilitation (including mobilisation of nerve system, passive techniques for joint mobility, active gymnastics for muscle power, as well as massage and the training of families for therapy at home) showed improvement in active movement followed by enhanced self\u2010care.<\/jats:p>","DOI":"10.1007\/s10545-010-9059-9","type":"journal-article","created":{"date-parts":[[2010,3,8]],"date-time":"2010-03-08T14:54:35Z","timestamp":1268060075000},"page":"151-157","source":"Crossref","is-referenced-by-count":47,"title":["Efficacy of recombinant human \u03b1\u2010<scp>L<\/scp>\u2010iduronidase (laronidase) on restricted range of motion of upper extremities in mucopolysaccharidosis type I patients"],"prefix":"10.1002","volume":"33","author":[{"given":"Anna","family":"Tylki\u2010Szymanska","sequence":"first","affiliation":[{"name":"Department of Metabolic Diseases, Endocrinology and Diabetology The Children's Memorial Health Institute Al. Dzieci Polskich 20 Warsaw 04730 Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jolanta","family":"Marucha","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases, Endocrinology and Diabetology The Children's Memorial Health Institute Al. Dzieci Polskich 20 Warsaw 04730 Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agnieszka","family":"Jurecka","sequence":"additional","affiliation":[{"name":"Department of Metabolic Diseases, Endocrinology and Diabetology The Children's Memorial Health Institute Al. Dzieci Polskich 20 Warsaw 04730 Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malgorzata","family":"Syczewska","sequence":"additional","affiliation":[{"name":"Department of Pediatric Physical Therapy The Children's Memorial Health Institute Warsaw Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Czartoryska","sequence":"additional","affiliation":[{"name":"Department of Genetics Institute of Psychiatry and Neurology Warsaw Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,3,9]]},"reference":[{"key":"e_1_2_6_2_1","volume-title":"Klinische Tests an Knochen Geleken und Muskeln, vol. 4","author":"Backup K","year":"1995"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2007-3847"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-006-0490-x"},{"key":"e_1_2_6_5_1","first-page":"147","article-title":"The globe system: an unambiguous description of shoulder positions in daily life movements","volume":"40","author":"Doorenbosh CA","year":"2003","journal-title":"J Rehabil Res Dev"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1047-9651(18)30047-0"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)64961-9"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200101183440304"},{"key":"e_1_2_6_9_1","first-page":"3421","volume-title":"The metabolic and molecular bases of inherited disease","author":"Neufeld EF","year":"2001"},{"key":"e_1_2_6_10_1","first-page":"216","volume-title":"Abstract book, 9th International Symposium on Mucopolysaccharidosis and Related Diseases","author":"Norato DYJ","year":"2006"},{"key":"e_1_2_6_11_1","first-page":"146","volume-title":"Physical rehabilitation: assessment and treatment","author":"O'Sullivan SB","year":"1994"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.bor.0000147283.40529.13"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2006.08.007"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/labinvest.3780345"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2008.070564"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.2001.tb00249.x"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2004.01.046"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2006-2156"},{"key":"e_1_2_6_19_1","first-page":"239","article-title":"Kinezyterapia","volume":"68","author":"Zembaty A","year":"2002","journal-title":"Krak\u00f3w"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-010-9059-9","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1007\/s10545-010-9059-9","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-010-9059-9","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T22:47:08Z","timestamp":1694818028000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-010-9059-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,3,9]]},"references-count":18,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2010,4]]}},"alternative-id":["10.1007\/s10545-010-9059-9"],"URL":"https:\/\/doi.org\/10.1007\/s10545-010-9059-9","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,3,9]]}}}