{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T21:52:25Z","timestamp":1777153945067,"version":"3.51.4"},"reference-count":29,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2018,5,23]],"date-time":"2018-05-23T00:00:00Z","timestamp":1527033600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100004047","name":"Karolinska Institutet","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004047","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004348","name":"Stockholms L\u00e4ns Landsting","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004348","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2018,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>This prospective study assessed the long\u2010term ocular and visual outcomes of children with mucopolysaccharidoses type I Hurler syndrome (<jats:styled-content style=\"fixed-case\">MPS IH<\/jats:styled-content>) who were treated with haematopoietic stem cell transplants (<jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Clinical ophthalmological assessments were performed on eight patients at the St Erik Eye Hospital, Huddinge, Stockholm, Sweden, from 2001\u20132018: The median age at diagnosis and <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content> were 12.2 (range 5.0\u201316.4) and 16.7 (8.0\u201320.4)\u00a0months. The last eye examination was at a median of 13.4 (6.3\u201319.0)\u00a0years and follow\u2010up lasted a median of 12.0 (5.0\u201317.4)\u00a0years.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Poor visual acuity, poor night vision and, or, photophobia were reported by six children. The best corrected visual acuity at the last visit was a median of 0.4 and 0.5 in the right and left eye and had declined significantly in two patients. Corneal opacities had increased despite <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content> in five patients. High hyperopia, at a median of +6 Dioptres, occurred in all patients and stiff corneas in all four patients that were measured. The patients\u2019 corrected intraocular pressures were normal. Retinal degeneration was identified in two patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Despite <jats:styled-content style=\"fixed-case\">HSCT<\/jats:styled-content>, the long\u2010term follow\u2010up of patients with <jats:styled-content style=\"fixed-case\">MPS IH<\/jats:styled-content> showed reduced visual acuity due to corneal opacities or retinal degeneration.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.14368","type":"journal-article","created":{"date-parts":[[2018,4,23]],"date-time":"2018-04-23T15:33:14Z","timestamp":1524497594000},"page":"1995-2003","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Children with mucopolysaccharidosis risk progressive visual dysfunction despite haematopoietic stem cell transplants"],"prefix":"10.1111","volume":"107","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4165-0731","authenticated-orcid":false,"given":"Kristina","family":"Te\u00e4r Fahnehjelm","sequence":"first","affiliation":[{"name":"Department of Clinical Neuroscience Karolinska Institutet Stockholm Sweden"},{"name":"Department of Paediatric Ophthalmology and Strabismus St Erik Eye Hospital Stockholm Sweden"},{"name":"Department of Clinical Neuroscience Sahlgrenska Academy Gothenburg University Gothenburg Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Monica","family":"Olsson","sequence":"additional","affiliation":[{"name":"Department of Clinical Neuroscience Karolinska Institutet Stockholm Sweden"},{"name":"Department of Paediatric Ophthalmology and Strabismus St Erik Eye Hospital Stockholm Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Enping","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Clinical 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