{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T01:36:04Z","timestamp":1760060164964,"version":"build-2065373602"},"reference-count":38,"publisher":"MDPI AG","issue":"16","license":[{"start":{"date-parts":[[2025,8,14]],"date-time":"2025-08-14T00:00:00Z","timestamp":1755129600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100001422","name":"American Society of Hematology","doi-asserted-by":"publisher","award":["ASH Global Research Award 2019"],"award-info":[{"award-number":["ASH Global Research Award 2019"]}],"id":[{"id":"10.13039\/100001422","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Objectives: Sickle Cell Disease (SCD) has a significant impact on the musculoskeletal system. The use of the Oxford Hip (OHS) and Shoulder score (OSS) as patient-reported outcome measures (PROMs) revealed a high rate of long-term impairment in joints with a pre-existing diagnosis of avascular necrosis (AVN). With this study, we aimed at detecting dysfunction in joints apparently not affected by AVN. Methods: This is a subgroup analysis of a previous core study assessing the OHS and OSS in 47 SCD patients with a pre-existing diagnosis of AVN. For this study, only patients with a pre-existing diagnosis of isolated AVN (only hip or only shoulder) were selected, and the OHS or OSS was measured in previously unaffected joints. Results: Among 37 patients with isolated AVN, 19 (51%) patients presented abnormal scores in the apparently unaffected joint; 16 (50%) patients with pre-existing isolated hip AVN had an abnormal OSS; and 9 (56%) had moderate to severe shoulder impairment. All patients with pre-existing isolated shoulder AVN had an abnormal OHS, with severe hip impairment in two out of three. As per clinical practice, patients with an abnormal score were prescribed joint magnetic resonance imaging (MRI) and\/or X-rays. Only 10 out 19 (53%) performed imaging studies and all showed signs of AVN. Conclusions: Abnormal OHS and OSS values indicated a high rate of joint dysfunction in sites apparently not affected by AVN. The routine use of these PROMs should be applied to all SCD adults and subjects with a pathological score should have priority access to diagnostic radiological tests.<\/jats:p>","DOI":"10.3390\/jcm14165744","type":"journal-article","created":{"date-parts":[[2025,8,14]],"date-time":"2025-08-14T15:44:21Z","timestamp":1755186261000},"page":"5744","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Oxford Hip and Shoulder Scores as Potential Tools for the Early Detection of Avascular Necrosis in Apparently Unaffected Sites in Sickle Cell Disease: Results from a Prospective Cohort Study"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4740-2421","authenticated-orcid":false,"given":"Maddalena","family":"Casale","sequence":"first","affiliation":[{"name":"Department of the Woman, The Child and General and Specialized Surgery, University of Campania Luigi Vanvitelli, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8560-721X","authenticated-orcid":false,"given":"Giuseppe","family":"Toro","sequence":"additional","affiliation":[{"name":"Department of Medical and Surgical Specialties and Dentistry, University of Campania Luigi Vanvitelli, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"Porcelli","sequence":"additional","affiliation":[{"name":"Department of the Woman, The Child and General and Specialized Surgery, University of Campania Luigi Vanvitelli, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vincenzo","family":"Spadola","sequence":"additional","affiliation":[{"name":"Thalassemia Centre, Hospital \u201cGiovanni Paolo II\u201d, 97100 Ragusa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosamaria","family":"Rosso","sequence":"additional","affiliation":[{"name":"Thalassemia Unit, AOU Policlinico G.Rodolico-San Marco, 95123 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Bulla","sequence":"additional","affiliation":[{"name":"Thalassemia Unit, AOU Policlinico G.Rodolico-San Marco, 95123 Catania, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandra","family":"Quota","sequence":"additional","affiliation":[{"name":"Thalassemia Centre, Hospital Vittorio Emanuele, 93012 Gela, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-7247-1139","authenticated-orcid":false,"given":"Concetta Simona","family":"Perrotta","sequence":"additional","affiliation":[{"name":"Thalassemia Centre, Hospital Vittorio Emanuele, 93012 Gela, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saveria","family":"Campisi","sequence":"additional","affiliation":[{"name":"Thalassemia Unit, Hospital \u201cUmberto Primo\u201d, 96100 Siracusa, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Arcioni","sequence":"additional","affiliation":[{"name":"Pediatric Oncology-Hematology, Hospital of Perugia, 06122 Perugia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Maddalena","family":"Marrapodi","sequence":"additional","affiliation":[{"name":"Department of the Woman, The Child and General and Specialized Surgery, University of Campania Luigi Vanvitelli, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6149-6845","authenticated-orcid":false,"given":"Silverio","family":"Perrotta","sequence":"additional","affiliation":[{"name":"Department of the Woman, The Child and General and Specialized Surgery, University of Campania Luigi Vanvitelli, 80138 Naples, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,8,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"e633","DOI":"10.1016\/S2352-3026(23)00096-0","article-title":"Defining global strategies to improve outcomes in sickle cell disease: A Lancet Haematology Commission","volume":"10","author":"Piel","year":"2023","journal-title":"Lancet Haematol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1186\/s13023-019-1099-0","article-title":"Current challenges in the management of patients with sickle cell disease\u2014A report of the Italian experience","volume":"14","author":"Russo","year":"2019","journal-title":"Orphanet J. 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