{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,18]],"date-time":"2025-11-18T11:30:12Z","timestamp":1763465412690,"version":"3.45.0"},"reference-count":0,"publisher":"AVES YAYINCILIK A.\u015e.","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["AOTT"],"abstract":"<jats:p>Abstract\n\nMucopolysaccharidosis IVA (MPS IVA: Morquio A syndrome) is a lysosomal storage disorder caused by a deficiency of N-acetylgalactosamine-6-sulfate sulfatase. Patients with MPS IVA appear healthy at birth. Morquio-specific radiographic changes can be observed prior to clinical signs and symptoms. Patients are usually affected by a severe joint degeneration from the 2nd or 3rd decade. Hyperlaxity of the joints is prominent due to the excess of intermediate metabolites. We report a patient with inherited dwarfism, in which a proximal soft tissue realignment procedure was performed to treat chronic patellar dislocation.\n\n\u00d6zet\n\nMukopolisakkaridoz IVA (MPS IVA: Morquio sendromu) N-asetilgalaktozamin-6-s\u00fclfat s\u00fclfataz enzim eksikli\u011finden kaynaklanan bir lizozomal depo hastal\u0131\u011f\u0131d\u0131r. MPS IVA hastalar\u0131 do\u011fumda sa\u011fl\u0131kl\u0131 g\u00f6r\u00fcn\u00fcrler. Bu sendroma \u00f6zg\u00fcn radyolojik de\u011fi\u015fiklikler klinik belirti ve bulgulardan \u00f6nce g\u00f6zlenirler. Hastalar\u0131n \u00e7o\u011funda, ikinci veya \u00fc\u00e7\u00fcnc\u00fc on y\u0131lda, ilerlemi\u015f eklem dejenerasyonu ortaya \u00e7\u0131kar. Ara metabolitlerin artmas\u0131na ba\u011fl\u0131 olarak eklem hiperlaksitesi belirgindir. Bu \u00e7al\u0131\u015fmada, proksimal yumu\u015fak doku dizilim cerrahisi yap\u0131larak tedavi edilen kronik patella \u00e7\u0131k\u0131kl\u0131, kal\u0131tsal c\u00fcceli\u011fi olan bir hasta sunulmu\u015ftur.<\/jats:p>","DOI":"10.3944\/aott.2011.2446","type":"journal-article","created":{"date-parts":[[2012,1,24]],"date-time":"2012-01-24T06:02:26Z","timestamp":1327384946000},"page":"466-469","source":"Crossref","is-referenced-by-count":3,"title":["Proximal realignment surgery for unilateral chronic patella dislocation in Morquio syndrome: a case report"],"prefix":"10.5152","volume":"45","author":[{"given":"Ali","family":"Bulent Baz","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Serdar","family":"Akalin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hasan","family":"Arik","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ali","family":"Ergun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"3111","published-online":{"date-parts":[[2020,2,7]]},"container-title":["Acta Orthopaedica et Traumatologica Turcica"],"original-title":[],"link":[{"URL":"https:\/\/aott.org.tr\/index.php\/pub\/article\/download\/2544\/2500","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,18]],"date-time":"2025-11-18T11:27:38Z","timestamp":1763465258000},"score":1,"resource":{"primary":{"URL":"https:\/\/aott.org.tr\/index.php\/pub\/article\/view\/2544"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,2,7]]},"references-count":0,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2011,1,6]]}},"URL":"https:\/\/doi.org\/10.3944\/aott.2011.2446","relation":{},"ISSN":["2589-1294","1017-995X"],"issn-type":[{"type":"electronic","value":"2589-1294"},{"type":"print","value":"1017-995X"}],"subject":[],"published":{"date-parts":[[2020,2,7]]}}}