{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,16]],"date-time":"2026-03-16T20:00:39Z","timestamp":1773691239345,"version":"3.50.1"},"reference-count":21,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2019,7,1]],"date-time":"2019-07-01T00:00:00Z","timestamp":1561939200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Rare Diseases Foundation of British Columbia"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J of Med Genetics Pt C"],"published-print":{"date-parts":[[2019,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Individuals with Arthrogryposis Multiplex Congenita (AMC) are born with multiple joint contractures in multiple body areas, typically manifested as clubfeet, extended or flexed knees and\/or elbows, and internal shoulder rotation, and clasped hands. They require multiple surgeries as children, but there is little data that reports their aging and future quality of life (QOL). This study describes the relationship between AMC\u2010related surgically\u2010managed joints in childhood and adulthood, and QOL as adults. Participants (<jats:italic>n<\/jats:italic> = 83) from 14 countries completed an online questionnaire followed by a telephone\/Skype interview as adults. Data points collected regarding total number of surgeries, affected joints, country of origin, sex, age, and SF\u201036's Physical Capacity Score (PCS) for QOL were analyzed using a beta regression model to explore which factors may potentially influence adult QOL. The average number of surgeries per participant was 9.8, with at least 50% performed during childhood. 78, 45, and 31% of participants had foot, knee, and hip surgeries, respectively. The model demonstrated that knee and\/or shoulder surgeries were more likely to have a negative correlation with PCS; elbow surgery, however, showed a positive correlation, as elbow function may impact independent function. However, future expansion of this data set to a longitudinal registry would provide better ongoing surgery\u2010specific data.<\/jats:p>","DOI":"10.1002\/ajmg.c.31720","type":"journal-article","created":{"date-parts":[[2019,7,1]],"date-time":"2019-07-01T15:34:40Z","timestamp":1561995280000},"page":"469-473","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["The relationship between joint surgery and quality of life in adults with arthrogryposis: An international study"],"prefix":"10.1002","volume":"181","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8901-2301","authenticated-orcid":false,"given":"Bonita","family":"Sawatzky","sequence":"first","affiliation":[{"name":"Faculty of Medicine University of British Columbia  Vancouver British Columbia Canada"},{"name":"Department of Orthopaedics University of British Columbia  Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Talon","family":"Jones","sequence":"additional","affiliation":[{"name":"Faculty of Medicine University of British Columbia  Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rebecca","family":"Miller","sequence":"additional","affiliation":[{"name":"Faculty of Medicine University of British Columbia  Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hirmand","family":"Noureai","sequence":"additional","affiliation":[{"name":"Faculty of Medicine University of British Columbia  Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,7]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.5435\/00124635-200211000-00006"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/BPO.0b013e318070cc76"},{"key":"e_1_2_8_4_1","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1097\/00003086-198504000-00016","article-title":"Arthrogryposis multiplex Congenita: A long\u2010term follow\u2010up study","volume":"194","author":"Carlson W.","year":"1985","journal-title":"Clinical Orthopaedics and Related Research"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000005418"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11832-009-0187-4"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.306.6890.1440"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/01202412-199707000-00002"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejmg.2014.03.008"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.36938"},{"issue":"3","key":"e_1_2_8_11_1","first-page":"65","article-title":"Canadian normative data for the SF\u201036 health survey","volume":"163","author":"Hopman W.","year":"2000","journal-title":"Canadian Medical Association Journal"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/BPO.0b013e3181a6bf77"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rehab.2018.05.1319"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.5114\/aoms.2016.57578"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11832-016-0712-1"},{"key":"e_1_2_8_16_1","first-page":"1","article-title":"Outcomes at 2\u2010year minimun follow\u2010up of shoulder, elbow and wrist surgery","volume":"3","author":"Miller R.","year":"2017","journal-title":"Journal of Clinical Experimental Orthopedics"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.38169"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1080\/09638288.2017.1357211"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11999-008-0685-6"},{"issue":"1","key":"e_1_2_8_20_1","doi-asserted-by":"crossref","first-page":"56","DOI":"10.2106\/JBJS.16.00304","article-title":"Reorientional proximal femoral osteotomies for arthrogrypotic hip contractures","volume":"99","author":"Van Bosse H. J.","year":"2017","journal-title":"Journal of Bone and Joint Surgery"},{"key":"e_1_2_8_21_1","first-page":"75","volume-title":"Regression methods in biostatistics: Linear, logistic, survival, and repeated measures models","author":"Vittinghoff E.","year":"2005"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/01241398-200205000-00018"}],"container-title":["American Journal of Medical Genetics Part C: Seminars in Medical Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.c.31720","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.c.31720","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ajmg.c.31720","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.c.31720","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,10]],"date-time":"2023-09-10T03:25:28Z","timestamp":1694316328000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.c.31720"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,7]]},"references-count":21,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2019,9]]}},"alternative-id":["10.1002\/ajmg.c.31720"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.c.31720","archive":["Portico"],"relation":{},"ISSN":["1552-4868","1552-4876"],"issn-type":[{"value":"1552-4868","type":"print"},{"value":"1552-4876","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,7]]},"assertion":[{"value":"2019-02-27","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-06-17","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-07-01","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}