{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T20:31:08Z","timestamp":1787776268973,"version":"build-2784847793"},"reference-count":5,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>An autosomal recessively inherited noninflammatory arthropathy known as camptodactyly, arthropathy, coxa vara, and pericarditis (CACP) syndrome was discovered in 1999. It is distinguished by synoviocyte hyperplasia and subcapsular fibrosis of the synovial capsule, which results in a shortage of lubricin production. The resulting lack of joint lubrication induces increased mechanical stress, causing progressive deformities that become evident with weight-bearing and heightened joint activity. Animal models with a lubricin gene knock-out display similar traits, underscoring the impact of mechanical stress on disrupting type II collagen on the articular surface. The gradual development of pericarditis and constriction often results in misdiagnosis as juvenile rheumatoid arthritis with cardiac involvement, but the defining feature remains the noninflammatory nature of the disease. Early recognition is pivotal, as interventions such as pericardiectomy and recombinant human lubricin hold promise for altering the disease\u2019s natural course. In our familial case of CACP, two siblings exhibited distinct phenotypic variations \u2013 one with fibrosis-dominant features and pericardial constriction and the other displaying synovial hyperplasia without pericardial involvement.<\/jats:p>","DOI":"10.4103\/apc.apc_18_24","type":"journal-article","created":{"date-parts":[[2024,10,2]],"date-time":"2024-10-02T05:00:13Z","timestamp":1727845213000},"page":"221-223","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":2,"title":["Jammed joints and constricted heart: The science of tribology and missing lubricin. A case report on camptodactyly-arthropathy-coxa vara-pericarditis syndrome"],"prefix":"10.4103","volume":"17","author":[{"given":"Krishnan Ganapathy","family":"Subramaniam","sequence":"first","affiliation":[{"name":"Department of Cardiothoracic and Vascular Surgery, Sri Padmavathi Children Heart Centre, Tirupati, Andhra Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satish","family":"Mohanty","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Sri Padmavathi Children Heart Centre, Tirupati, Andhra Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dhruva","family":"Sharma","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic and Vascular Surgery, SMS Medical College and Attached Hospitals, Jaipur, Rajasthan, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Komal","family":"Tamildasan","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic and Vascular Surgery, Sri Padmavathi Children Heart Centre, Tirupati, Andhra Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Narahari Srinath","family":"Reddy","sequence":"additional","affiliation":[{"name":"Department of Paediatric Cardiology, Sri Padmavathi Children Heart Centre, Tirupati, Andhra Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2024,10,1]]},"reference":[{"key":"R1-20241204","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1038\/15496","article-title":"CACP, encoding a secreted proteoglycan, is mutated in camptodactyly-arthropathy-coxa vara-pericarditis syndrome","volume":"23","author":"Marcelino","year":"1999","journal-title":"Nat Genet"},{"key":"R2-20241204","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1002\/art.33337","article-title":"Cyclic loading increases friction and changes cartilage surface integrity in lubricin-mutant mouse knees","volume":"64","author":"Drewniak","year":"2012","journal-title":"Arthritis Rheum"},{"key":"R3-20241204","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1016\/j.semarthrit.2012.11.004","article-title":"Camptodactyly-arthropathy-coxavara-pericarditis syndrome in Saudi Arabia: Clinical and molecular genetic findings in 22 patients","volume":"43","author":"Albuhairan","year":"2013","journal-title":"Semin Arthritis Rheum"},{"key":"R4-20241204","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.matbio.2014.08.008","article-title":"The biology of lubricin: Near frictionless joint motion","volume":"39","author":"Jay","year":"2014","journal-title":"Matrix Biol"},{"key":"R5-20241204","doi-asserted-by":"crossref","first-page":"1598","DOI":"10.1016\/j.jtcvs.2018.03.170","article-title":"Human pericardial proteoglycan 4 (lubricin): Implications for postcardiotomy intrathoracic adhesion formation","volume":"156","author":"Park","year":"2018","journal-title":"J Thorac Cardiovasc Surg"}],"container-title":["Annals of Pediatric Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_18_24","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T20:02:22Z","timestamp":1787774542000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_18_24"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5]]},"references-count":5,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2024]]}},"URL":"https:\/\/doi.org\/10.4103\/apc.apc_18_24","relation":{},"ISSN":["0974-2069","0974-5149"],"issn-type":[{"value":"0974-2069","type":"print"},{"value":"0974-5149","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,5]]},"assertion":[{"value":"2024-02-02","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-05-16","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}