{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,30]],"date-time":"2026-05-30T00:22:22Z","timestamp":1780100542142,"version":"3.54.0"},"reference-count":10,"publisher":"Texas Heart Institute Journal","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2017,12]]},"abstract":"<jats:p>Morquio A syndrome (mucopolysaccharidosis IV type A), an autosomal recessive lysosomal storage disorder caused by a defective <jats:italic>N<\/jats:italic>-acetylgalactosamine 6-sulfatase gene, leads to lysosomal accumulation of keratan sulfate and chondroitin 6-sulfate. This accumulation affects multiple systems and causes notable cardiovascular manifestations, such as thickening of the left-sided valves, ventricular hypertrophy, and intimal stenosis of the coronary arteries. There have been few reports of vasculopathy in this population. We present the case of a 58-year-old woman with Morquio A syndrome who was found to have aortic dilation on a routine screening echocardiogram. Magnetic resonance images revealed multiple tortuous, dilated arteries in her head, neck, and abdomen. The diffuse vasculopathy seen in this patient should prompt further study to determine whether this is an underreported phenomenon of clinical significance or an unusual finding in this rare disorder.<\/jats:p>","DOI":"10.14503\/thij-16-6121","type":"journal-article","created":{"date-parts":[[2017,12,19]],"date-time":"2017-12-19T15:54:31Z","timestamp":1513698871000},"page":"420-423","source":"Crossref","is-referenced-by-count":5,"title":["Widespread Vasculopathy in a Patient with Morquio A Syndrome"],"prefix":"10.14503","volume":"44","author":[{"given":"Adam W.","family":"Powell","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael D.","family":"Taylor","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T. Andrew","family":"Burrow","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert J.","family":"Hopkin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos E.","family":"Prada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John L.","family":"Jefferies","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"5628","reference":[{"issue":"1","key":"i1526-6702-44-6-420-b1","series-title":"Am J Med Genet A","first-page":"11","article-title":"International guidelines for the management and treatment of Morquio A syndrome","volume":"167A","year":"2015","unstructured":"Hendriksz\n              CJ\n            , BergerKI, GiuglianiR, HarmatzP, KampmannC, MackenzieWG, \n          et al. International guidelines for the management and treatment of Morquio A syndrome. Am J Med Genet A2015;167A(1):11\u201325."},{"issue":"1","key":"i1526-6702-44-6-420-b2","series-title":"Br Heart J","first-page":"113","article-title":"Mucopolysaccharidosis type IV as a cause of mitral stenosis in an adult","volume":"46","year":"1981","unstructured":"Ireland\n              MA\n            , RowlandsDB.\n          Mucopolysaccharidosis type IV as a cause of mitral stenosis in an adult. Br Heart J1981;46(1):113\u20135."},{"issue":"7","key":"i1526-6702-44-6-420-b3","series-title":"Arch Dis Child","first-page":"746","article-title":"Echocardiographic abnormalities in type IV mucopolysaccharidosis","volume":"65","year":"1990","unstructured":"John\n              RM\n            , HunterD, SwantonRH.\n          Echocardiographic abnormalities in type IV mucopolysaccharidosis. Arch Dis Child1990;65(7):746\u20139."},{"issue":"9","key":"i1526-6702-44-6-420-b4","series-title":"PLoS One","first-page":"e0162612","article-title":"Heart and cardiovascular involvement in patients with mucopolysaccharidosis type IVA (Morquio-A syndrome)","volume":"11","year":"2016","unstructured":"Kampmann\n              C\n            , Abu-TairT, GokceS, LampeC, ReinkeJ, MengelE, \n          et al. Heart and cardiovascular involvement in patients with mucopolysaccharidosis type IVA (Morquio-A syndrome). PLoS One2016;11(9):e0162612."},{"issue":"4","key":"i1526-6702-44-6-420-b5","series-title":"J Vasc Surg","first-page":"704","article-title":"Mucopolysaccharidosis presenting as pediatric multiple aortic aneurysm: first reported case","volume":"26","year":"1997","unstructured":"Engle\n              J\n            , SafiHJ, AbbassiO, IliopoulosDC, DorsayD, CartwrightJJr, WeilbaecherD.\n          Mucopolysaccharidosis presenting as pediatric multiple aortic aneurysm: first reported case. J Vasc Surg1997;26(4):704\u201310."},{"key":"i1526-6702-44-6-420-b6","series-title":"JIMD Rep","first-page":"59","article-title":"Mortality in patients with Morquio syndrome A","volume":"15","year":"2015","unstructured":"Lavery\n              C\n            , HendrikszC.\n          Mortality in patients with Morquio syndrome A. JIMD Rep2015;15:59\u201366."},{"issue":"1","key":"i1526-6702-44-6-420-b7","series-title":"Virchows Arch A Pathol Anat Histol","first-page":"1","article-title":"Coronary intimal sclerosis in Morquio's syndrome","volume":"379","year":"1978","unstructured":"Factor\n              SM\n            , BiempicaL, GoldfischerS.\n          Coronary intimal sclerosis in Morquio's syndrome. Virchows Arch A Pathol Anat Histol1978;379(1):1\u201310."},{"issue":"2","key":"i1526-6702-44-6-420-b8","series-title":"Mol Genet Metab","first-page":"150","article-title":"Obstructive airway in Morquio A syndrome, the past, the present and the future","volume":"117","year":"2016","unstructured":"Tomatsu\n              S\n            , AverillLW, SawamotoK, MackenzieWG, BoberMB, PizarroC, \n          et al. Obstructive airway in Morquio A syndrome, the past, the present and the future. Mol Genet Metab2016;117(2):150\u20136."},{"issue":"3","key":"i1526-6702-44-6-420-b9","series-title":"Mol Genet Metab","first-page":"301","article-title":"Pathogenesis of Morquio A syndrome: an autopsied case reveals systemic storage disorder","volume":"109","year":"2013","unstructured":"Yasuda\n              E\n            , FushimiK, SuzukiY, ShimizuK, TakamiT, ZustinJ, \n          et al. Pathogenesis of Morquio A syndrome: an autopsied case reveals systemic storage disorder. Mol Genet Metab2013;109(3):301\u201311."},{"issue":"2","key":"i1526-6702-44-6-420-b10","series-title":"Am J Med Genet A","first-page":"134","article-title":"Three new families with arterial tortuosity syndrome","volume":"131","year":"2004","unstructured":"Wessels\n              MW\n            , Catsman-BerrevoetsCE, ManciniGM, BreuningMH, HoogeboomJJ, StroinkH, \n          et al. Three new families with arterial tortuosity syndrome. Am J Med Genet A2004;131(2):134\u201343."}],"container-title":["Texas Heart Institute Journal"],"original-title":[],"link":[{"URL":"https:\/\/thij.kglmeridian.com\/view\/journals\/thij\/44\/6\/article-p420.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/meridian.allenpress.com\/thij\/article-pdf\/44\/6\/420\/1967194\/thij-16-6121.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thij.kglmeridian.com\/downloadpdf\/journals\/thij\/44\/6\/article-p420.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thij.kglmeridian.com\/downloadpdf\/journals\/thij\/44\/6\/article-p420.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,7]],"date-time":"2024-11-07T15:34:36Z","timestamp":1730993676000},"score":1,"resource":{"primary":{"URL":"https:\/\/thij.kglmeridian.com\/view\/journals\/thij\/44\/6\/article-p420.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,12]]},"references-count":10,"journal-issue":{"issue":"6"},"URL":"https:\/\/doi.org\/10.14503\/thij-16-6121","relation":{},"ISSN":["0730-2347","1526-6702"],"issn-type":[{"value":"0730-2347","type":"print"},{"value":"1526-6702","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,12]]}}}