{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T17:13:28Z","timestamp":1792084408933,"version":"4.4.0"},"reference-count":28,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,12,20]],"date-time":"2020-12-20T00:00:00Z","timestamp":1608422400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Arthroplasty in the spondyloarthropathy (SPOND) of alkaptonuria (AKU) in incompletely characterised. The aim was to improve the understanding of arthroplasty in AKU through a study of patients attending the National Alkaptonuria Centre (NAC). Eighty\u2010seven patients attended the NAC between 2007 and 2020. Seven only attended once. Fifty\u2010seven attended more than once and received nitisinone 2\u2009mg daily. Twenty\u2010three attended at least twice without receiving nitisinone. Assessments including questionnaire analysis eliciting details of arthroplasty and other surgical treatments for SPOND, <jats:sup>18<\/jats:sup>FPETCT and CT densitometry at the neck of hip and lumbar spine, as well as photographs of the eyes and ears were acquired from patients attending the National Alkaptonuria Centre (NAC) at baseline when 2\u2009mg nitisinone was commenced, and yearly thereafter. Photographs were scored to derive ochronosis scores. Blood and urine samples were collected for chemical analyses. The prevalence of arthroplasty was 36.8%, similar in males and females, occurring especially in the knees, hips and shoulders. Multiple arthroplasties were found in 29 patients (33.3%) in this cohort. Incident arthroplasty was 6.5% in the nitisinone group and 7.1% in the no\u2010nitisinone group. Incident arthroplasty was 11.3% in the group with baseline arthroplasty and 3.51% in the group without. A strong association of arthroplasty with SPOND (<jats:italic>R<\/jats:italic> = 0.5; <jats:italic>P<\/jats:italic>\u2009&lt;&lt;\u2009.0001) and ochronosis (<jats:italic>R<\/jats:italic> = 0.54; <jats:italic>P<\/jats:italic>\u2009&lt;\u2009.0001) was seen. Nitisinone had no significant effect on incident arthroplasty. Arthroplasty due to ochronosis and SPOND is common in AKU. Nitisinone decreased ochronosis but had no effect on arthroplasty in this cohort.<\/jats:p>","DOI":"10.1002\/jimd.12340","type":"journal-article","created":{"date-parts":[[2020,12,14]],"date-time":"2020-12-14T12:34:32Z","timestamp":1607949272000},"page":"656-665","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Characterising the arthroplasty in spondyloarthropathy in a large cohort of eighty\u2010seven patients with alkaptonuria"],"prefix":"10.1002","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4205-2269","authenticated-orcid":false,"given":"Lakshminarayan R.","family":"Ranganath","sequence":"first","affiliation":[{"name":"Department of Clinical Biochemistry &amp; Metabolic Medicine Royal Liverpool University Hospital  Liverpool UK"},{"name":"Department of Musculoskeletal Biology University of Liverpool  Liverpool UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James A.","family":"Gallagher","sequence":"additional","affiliation":[{"name":"Department of Musculoskeletal Biology University of Liverpool  Liverpool UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Davidson","sequence":"additional","affiliation":[{"name":"Department of Orthopaedic Surgery Royal Liverpool and Broadgreen NHS Trust  Liverpool UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sobhan","family":"Vinjamuri","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine, Division of Radiology Royal Liverpool University Hospital  Liverpool 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