Management of Corneal Clouding in Patients with Mucopolysaccharidosis
Mucopolysaccharidoses (MPS) are a rare group of lysosomal storage disorders characterized by the accumulation of incompletely degraded glycosaminoglycans (GAGs) in multiple organ systems including the eye. Visual loss occurs in MPS predominantly due to corneal clouding and retinopathy, but the scler...
| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-07-01
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| Series: | Journal of Clinical Medicine |
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| Online Access: | https://www.mdpi.com/2077-0383/10/15/3263 |
| _version_ | 1797525438206050304 |
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| author | Orlaith McGrath Leon Au Jane Ashworth |
| author_facet | Orlaith McGrath Leon Au Jane Ashworth |
| author_sort | Orlaith McGrath |
| collection | DOAJ |
| description | Mucopolysaccharidoses (MPS) are a rare group of lysosomal storage disorders characterized by the accumulation of incompletely degraded glycosaminoglycans (GAGs) in multiple organ systems including the eye. Visual loss occurs in MPS predominantly due to corneal clouding and retinopathy, but the sclera, trabecular meshwork and optic nerve may all be affected. Despite the success of therapies such as enzyme replacement therapy (ERT) and hematopoietic stem-cell transplantation (HSCT) in improving many of the systemic manifestations of MPS, their effect on corneal clouding is minimal. The only current definitive treatment for corneal clouding is corneal transplantation, usually in the form of a penetrating keratoplasty or a deep anterior lamellar keratoplasty. This article aims to provide an overview of corneal clouding, its current clinical and surgical management, and significant research progress. |
| first_indexed | 2024-03-10T09:13:53Z |
| format | Article |
| id | doaj.art-489d98f7db1d4857b6f32d163b1e2b08 |
| institution | Directory Open Access Journal |
| issn | 2077-0383 |
| language | English |
| last_indexed | 2024-03-10T09:13:53Z |
| publishDate | 2021-07-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Journal of Clinical Medicine |
| spelling | doaj.art-489d98f7db1d4857b6f32d163b1e2b082023-11-22T05:48:13ZengMDPI AGJournal of Clinical Medicine2077-03832021-07-011015326310.3390/jcm10153263Management of Corneal Clouding in Patients with MucopolysaccharidosisOrlaith McGrath0Leon Au1Jane Ashworth2Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Oxford Road, Manchester M13 9WL, UKManchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Oxford Road, Manchester M13 9WL, UKManchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Oxford Road, Manchester M13 9WL, UKMucopolysaccharidoses (MPS) are a rare group of lysosomal storage disorders characterized by the accumulation of incompletely degraded glycosaminoglycans (GAGs) in multiple organ systems including the eye. Visual loss occurs in MPS predominantly due to corneal clouding and retinopathy, but the sclera, trabecular meshwork and optic nerve may all be affected. Despite the success of therapies such as enzyme replacement therapy (ERT) and hematopoietic stem-cell transplantation (HSCT) in improving many of the systemic manifestations of MPS, their effect on corneal clouding is minimal. The only current definitive treatment for corneal clouding is corneal transplantation, usually in the form of a penetrating keratoplasty or a deep anterior lamellar keratoplasty. This article aims to provide an overview of corneal clouding, its current clinical and surgical management, and significant research progress.https://www.mdpi.com/2077-0383/10/15/3263Mucopolysaccharidosiscorneal cloudingpenetrating keratoplastydeep anterior lamellar keratoplasty |
| spellingShingle | Orlaith McGrath Leon Au Jane Ashworth Management of Corneal Clouding in Patients with Mucopolysaccharidosis Journal of Clinical Medicine Mucopolysaccharidosis corneal clouding penetrating keratoplasty deep anterior lamellar keratoplasty |
| title | Management of Corneal Clouding in Patients with Mucopolysaccharidosis |
| title_full | Management of Corneal Clouding in Patients with Mucopolysaccharidosis |
| title_fullStr | Management of Corneal Clouding in Patients with Mucopolysaccharidosis |
| title_full_unstemmed | Management of Corneal Clouding in Patients with Mucopolysaccharidosis |
| title_short | Management of Corneal Clouding in Patients with Mucopolysaccharidosis |
| title_sort | management of corneal clouding in patients with mucopolysaccharidosis |
| topic | Mucopolysaccharidosis corneal clouding penetrating keratoplasty deep anterior lamellar keratoplasty |
| url | https://www.mdpi.com/2077-0383/10/15/3263 |
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