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Children with mucop...
Children with mucopolysaccharidosis risk progressive visual dysfunction despite haematopoietic stem cell transplants
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- Teär Fahnehjelm, Kristina (författare)
- Karolinska Institutet,Gothenburg University,Göteborgs universitet,Institutionen för neurovetenskap och fysiologi, sektionen för klinisk neurovetenskap,Institute of Neuroscience and Physiology, Department of Clinical Neuroscience
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- Olsson, M. (författare)
- Karolinska Institutet
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- Chen, E. P. (författare)
- Karolinska Institutet
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Hengstler, J. (författare)
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- Naess, K. (författare)
- Karolinska Institutet
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- Winiarski, J. (författare)
- Karolinska Institutet
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(creator_code:org_t)
- 2018-05-23
- 2018
- Engelska.
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Ingår i: Acta Paediatrica. - : Wiley. - 0803-5253 .- 1651-2227. ; 107:11, s. 1995-2003
- Relaterad länk:
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https://gup.ub.gu.se...
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https://doi.org/10.1...
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http://kipublication...
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Abstract
Ämnesord
Stäng
- Aim: This prospective study assessed the long-term ocular and visual outcomes of children with mucopolysaccharidoses type I Hurler syndrome (MPS IH) who were treated with haematopoietic stem cell transplants (HSCT). Methods: Clinical ophthalmological assessments were performed on eight patients at the St Erik Eye Hospital, Huddinge, Stockholm, Sweden, from 2001-2018: The median age at diagnosis and HSCT were 12.2 (range 5.0-16.4) and 16.7 (8.0-20.4) months. The last eye examination was at a median of 13.4 (6.3-19.0) years and follow-up lasted a median of 12.0 (5.0-17.4) years. Results: 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 HSCT 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' corrected intraocular pressures were normal. Retinal degeneration was identified in two patients. Conclusion: Despite HSCT, the long-term follow-up of patients with MPS IH showed reduced visual acuity due to corneal opacities or retinal degeneration.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Pediatrik (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Pediatrics (hsv//eng)
Nyckelord
- Corneal opacity
- Hematopoietic stem cell transplant
- Mucopolysaccharidosis
- Retinal degeneration
- optical coherence tomography
- mucopoly-saccharidoses
- intraocular-pressure
- ocular findings
- vi
- guidelines
- keratoplasty
- glaucoma
- eye
- Pediatrics
Publikations- och innehållstyp
- ref (ämneskategori)
- art (ämneskategori)
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