{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T09:04:55Z","timestamp":1784106295302,"version":"3.55.0"},"reference-count":18,"publisher":"Springer Science and Business Media LLC","issue":"10","license":[{"start":{"date-parts":[[2020,8,19]],"date-time":"2020-08-19T00:00:00Z","timestamp":1597795200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2020,8,19]],"date-time":"2020-08-19T00:00:00Z","timestamp":1597795200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Rheumatol Int"],"published-print":{"date-parts":[[2021,10]]},"DOI":"10.1007\/s00296-020-04688-0","type":"journal-article","created":{"date-parts":[[2020,8,19]],"date-time":"2020-08-19T05:38:37Z","timestamp":1597815517000},"page":"1875-1882","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Syndrome of progressive deforming non-inflammatory arthritis of childhood: two patients of camptodactyly-arthropathy-coxa vara-pericarditis syndrome"],"prefix":"10.1007","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9016-7722","authenticated-orcid":false,"given":"Nameirakpam","family":"Johnson","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6350-6920","authenticated-orcid":false,"given":"Himanshi","family":"Chaudhary","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5034-2007","authenticated-orcid":false,"given":"Rajni","family":"Kumrah","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9015-1704","authenticated-orcid":false,"given":"Rakesh Kumar","family":"Pilania","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6600-7094","authenticated-orcid":false,"given":"Yamini","family":"Sharma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4433-4355","authenticated-orcid":false,"given":"Avinash","family":"Sharma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2542-2691","authenticated-orcid":false,"given":"Amanpreet","family":"Kaur","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8959-9691","authenticated-orcid":false,"given":"Swetlana","family":"Mukherjee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2885-0424","authenticated-orcid":false,"given":"Nandita","family":"Kakkar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8490-5584","authenticated-orcid":false,"given":"Pandiarajan","family":"Vignesh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,8,19]]},"reference":[{"issue":"2","key":"4688_CR1","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1002\/humu.9399","volume":"27","author":"AM Alazami","year":"2006","unstructured":"Alazami AM, Al-Mayouf SM, Wyngaard C-A, Meyer B (2006) Novel PRG4 mutations underlie CACP in Saudi families. Hum Mutat 27(2):213","journal-title":"Hum Mutat"},{"issue":"2","key":"4688_CR2","doi-asserted-by":"publisher","first-page":"522","DOI":"10.2214\/ajr.185.2.01850522","volume":"185","author":"AC Offiah","year":"2005","unstructured":"Offiah AC, Woo P, Prieur A-M, Hasson N, Hall CM (2005) Camptodactyly-arthropathy-coxa vara-pericarditis syndrome versus juvenile idiopathic arthropathy. AJR Am J Roentgenol 185(2):522\u2013529","journal-title":"AJR Am J Roentgenol"},{"issue":"2","key":"4688_CR3","doi-asserted-by":"publisher","first-page":"230","DOI":"10.1002\/mgg3.364","volume":"6","author":"S Yilmaz","year":"2018","unstructured":"Yilmaz S, Uluda\u011f Alkaya D, Kasap\u00e7opur \u00d6, Barut K, Akdemir ES, Celen C et al (2018) Genotype-phenotype investigation of 35 patients from 11 unrelated families with camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome. Mol Genet Genomic Med 6(2):230\u2013248","journal-title":"Mol Genet Genomic Med"},{"issue":"3","key":"4688_CR4","first-page":"594","volume":"27","author":"GD Jay","year":"2000","unstructured":"Jay GD, Britt DE, Cha CJ (2000) Lubricin is a product of megakaryocyte stimulating factor gene expression by human synovial fibroblasts. J Rheumatol 27(3):594\u2013600","journal-title":"J Rheumatol"},{"issue":"3","key":"4688_CR5","doi-asserted-by":"publisher","first-page":"436","DOI":"10.1002\/art.1780290321","volume":"29","author":"G Bulutlar","year":"1986","unstructured":"Bulutlar G, Yazici H, Ozdo\u011fan H, Schreuder I (1986) A familial syndrome of pericarditis, arthritis, camptodactyly, and coxa vara. Arthritis Rheum 29(3):436\u2013438","journal-title":"Arthritis Rheum"},{"issue":"4","key":"4688_CR6","doi-asserted-by":"publisher","first-page":"730","DOI":"10.1002\/1529-0131(199804)41:4<730::AID-ART22>3.0.CO;2-Y","volume":"41","author":"SA Bahabri","year":"1998","unstructured":"Bahabri SA, Suwairi WM, Laxer RM, Polinkovsky A, Dalaan AA, Warman ML (1998) The camptodactyly-arthropathy-coxa vara-pericarditis syndrome: clinical features and genetic mapping to human chromosome 1. Arthritis Rheum 41(4):730\u2013735","journal-title":"Arthritis Rheum"},{"issue":"3","key":"4688_CR7","doi-asserted-by":"publisher","first-page":"319","DOI":"10.1038\/15496","volume":"23","author":"J Marcelino","year":"1999","unstructured":"Marcelino J, Carpten JD, Suwairi WM, Gutierrez OM, Schwartz S, Robbins C et al (1999) CACP, encoding a secreted proteoglycan, is mutated in camptodactyly-arthropathy-coxa vara-pericarditis syndrome. Nat Genet 23(3):319\u2013322","journal-title":"Nat Genet"},{"issue":"1","key":"4688_CR8","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.ijpam.2018.01.004","volume":"5","author":"SM Al-Mayouf","year":"2018","unstructured":"Al-Mayouf SM (2018) Noninflammatory disorders mimic juvenile idiopathic arthritis. Int J Pediatr Adolesc Med 5(1):1\u20134","journal-title":"Int J Pediatr Adolesc Med"},{"issue":"2","key":"4688_CR9","doi-asserted-by":"publisher","first-page":"292","DOI":"10.1016\/j.semarthrit.2012.11.004","volume":"43","author":"I Albuhairan","year":"2013","unstructured":"Albuhairan I, Al-Mayouf SM (2013) Camptodactyly-arthropathy-coxa vara-pericarditis syndrome in Saudi Arabia: clinical and molecular genetic findings in 22 patients. Semin Arthritis Rheum 43(2):292\u2013296","journal-title":"Semin Arthritis Rheum"},{"issue":"2","key":"4688_CR10","first-page":"221","volume":"140","author":"RS Nandagopalan","year":"2014","unstructured":"Nandagopalan RS, Phadke SR, Dalal AB, Ranganath P (2014) Novel mutations in PRG4 gene in two Indian families with camptodactyly-arthropathy- coxa vara- pericarditis (CACP) syndrome. Indian J Med Res 140(2):221\u2013226","journal-title":"Indian J Med Res"},{"issue":"6","key":"4688_CR11","first-page":"684","volume":"56","author":"M Ta\u015far","year":"2014","unstructured":"Ta\u015far M, Eyileten Z, Kas\u0131mzade F, U\u00e7ar T, Kendirli T, Uysalel A (2014) Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome. Turk J Pediatr 56(6):684\u2013686","journal-title":"Turk J Pediatr"},{"issue":"2","key":"4688_CR12","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1016\/j.arcmed.2011.04.006","volume":"42","author":"S Basit","year":"2011","unstructured":"Basit S, Iqbal Z, Umicevic-Mirkov M, Kamran Ul-Hassan Naqvi S, Coenen M, Ansar M et al (2011) A novel deletion mutation in proteoglycan-4 underlies camptodactyly-arthropathy-coxa-vara-pericarditis syndrome in a consanguineous pakistani family. Arch Med Res 42(2):110\u2013114","journal-title":"Arch Med Res"},{"issue":"7","key":"4688_CR13","doi-asserted-by":"publisher","first-page":"553","DOI":"10.1002\/bdra.23031","volume":"94","author":"NA Akawi","year":"2012","unstructured":"Akawi NA, Ali BR, Al-Gazali L (2012) A novel mutation in PRG4 gene underlying camptodactyly-arthropathy-coxa vara-pericarditis syndrome with the possible expansion of the phenotype to include congenital cataract. Birth Defects Res A Clin Mol Teratol 94(7):553\u2013556","journal-title":"Birth Defects Res A Clin Mol Teratol"},{"issue":"2","key":"4688_CR14","doi-asserted-by":"publisher","first-page":"197","DOI":"10.1038\/ejhg.2013.123","volume":"22","author":"S Ciullini Mannurita","year":"2014","unstructured":"Ciullini Mannurita S, Vignoli M, Bianchi L, Kondi A, Gerloni V, Breda L et al (2014) CACP syndrome: identification of five novel mutations and of the first case of UPD in the largest European cohort. Eur J Hum Genet 22(2):197\u2013201","journal-title":"Eur J Hum Genet"},{"issue":"5","key":"4688_CR15","first-page":"1081","volume":"30","author":"A El-Garf","year":"2003","unstructured":"El-Garf A, Mahmoud G, Gheith R, Abd El-Aaty G, Abd El-Aaty H (2003) Camptodactyly, arthropathy, coxa vara, and pericarditis syndrome among Egyptians. J Rheumatol 30(5):1081\u20131086","journal-title":"J Rheumatol"},{"issue":"1","key":"4688_CR16","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1186\/s12969-016-0093-5","volume":"14","author":"B Peters","year":"2016","unstructured":"Peters B, Schuurs-Hoeijmakers JH, Fuijkschot J, Reimer A, van der Flier M, Lugtenberg D et al (2016) Protein-losing enteropathy in camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome. Pediatr Rheumatol Online J 14(1):32","journal-title":"Pediatr Rheumatol Online J"},{"issue":"2","key":"4688_CR17","doi-asserted-by":"publisher","first-page":"S284","DOI":"10.1016\/j.ihj.2016.03.020","volume":"68","author":"DV Patil","year":"2016","unstructured":"Patil DV, Phadke MS, Pahwa JS, Dalal AB (2016) Brothers with constrictive pericarditis - A novel mutation in a rare disease. Indian Heart J 68(2):S284\u2013S287","journal-title":"Indian Heart J"},{"issue":"1","key":"4688_CR18","doi-asserted-by":"publisher","first-page":"33","DOI":"10.4274\/mirt.29484","volume":"26","author":"SM Al-Mayouf","year":"2017","unstructured":"Al-Mayouf SM, Almutairi N, Alismail K (2017) The efficacy of yttrium-90 radiosynovectomy in patients with Camptodactyly-Arthropathy-Coxa vara-pericarditis syndrome. Mol Imaging Radionucl Ther 26(1):33\u201337","journal-title":"Mol Imaging Radionucl Ther"}],"container-title":["Rheumatology International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00296-020-04688-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00296-020-04688-0\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00296-020-04688-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,8,27]],"date-time":"2021-08-27T00:28:45Z","timestamp":1630024125000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00296-020-04688-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,8,19]]},"references-count":18,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2021,10]]}},"alternative-id":["4688"],"URL":"https:\/\/doi.org\/10.1007\/s00296-020-04688-0","relation":{},"ISSN":["0172-8172","1437-160X"],"issn-type":[{"value":"0172-8172","type":"print"},{"value":"1437-160X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,8,19]]},"assertion":[{"value":"24 April 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"14 August 2020","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"19 August 2020","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"All authors declare that he\/she has no potential conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"All procedures performed in studies involving human participants were in accordance with the ethical standards of the Institutional and National Research Committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"Written informed consent was obtained from parents of children included in this study for publication as case report.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"All authors have given final approval for publication.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}}]}}