{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T19:38:13Z","timestamp":1786649893678,"version":"build-2736575974"},"reference-count":18,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"12","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:sec>\n                    <jats:title>Purpose:<\/jats:title>\n                    <jats:p>Our study aimed to evaluate the utility of the anterior segment morphometry for objectively assessing anterior segment architectural changes of corneal clouding in the mucopolysaccharidoses (MPS) cohort and to investigate whether these measurements correlate with the slit-lamp findings on the cornea and early diagnosis of glaucoma.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>This retrospective study involved 70 eyes of 35 children with cloudy cornea due to MPS variants. Anterior segment architectural alterations were measured using anterior segment imaging and biometry in MPS children and compared with controls.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Mean age of the cohort at the time of assessment was 7.9 \u00b1 4.5 years. Males constituted two-thirds of the cohort. Variants of MPS with cloudy cornea were as follows: Type I (62%), Type IV (11%), and Type VI (22%). Morphometric measurements were available in 22 eyes of 11 MPS children and an age-matched healthy control group. There were significant differences between MPS cohort and controls in refraction in Diopters (5.03 \u00b1 0.39 and 0.01 \u00b1 0.04;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt; 0.0001), axial length (AXL) in mm (21.39 \u00b1 0.28 and 23.04 \u00b1 0.28;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      = 0.0002), average keratometry in Diopters (40.67 \u00b1 0.44 and 42.83 \u00b1 0.44;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt; 0.0001), anterior chamber depth (ACD) in mm (2.92 \u00b1 0.07 and 3.65 \u00b1 0.07;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt; 0.0001), and intraocular pressure (IOP) in mmHg (25.2 \u00b1 2.0 and 14.1 \u00b1 2.3;\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      = 0.0003). Secondary glaucoma was observed in 28% of the MPS cohort.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>The anterior segment morphometry in the cloudy cornea due to MPS provides an objective measurement of anterior segment architectural changes, thus diagnosing early-onset secondary glaucoma. These findings highlight that cloudy cornea due to MPS variants merits close monitoring throughout life.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/ijo.ijo_1300_22","type":"journal-article","created":{"date-parts":[[2022,12,6]],"date-time":"2022-12-06T08:53:31Z","timestamp":1670316811000},"page":"4180-4185","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":2,"title":["Objectively measuring anterior segment alterations in the eyes of mucopolysaccharidoses: Its utility in early diagnosis of glaucoma"],"prefix":"10.4103","volume":"70","author":[{"given":"Divya Sree Ramya","family":"Achanta","sequence":"first","affiliation":[{"name":"Centre for Rare Eye Diseases and Ocular Genetics, L V Prasad Eye Institute, Hyderabad, India"},{"name":"Jasti V Ramanamma Children\u2019s Eye Care Center, L V Prasad Eye Institute, Hyderabad, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashik","family":"Mohamed","sequence":"additional","affiliation":[{"name":"Centre for Rare Eye Diseases and Ocular Genetics, L V Prasad Eye Institute, Hyderabad, India"},{"name":"Ophthalmic Biophysics, L V Prasad Eye Institute, Hyderabad, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sunita","family":"Chaurasia","sequence":"additional","affiliation":[{"name":"Centre for Rare Eye Diseases and Ocular Genetics, L V Prasad Eye Institute, Hyderabad, India"},{"name":"Jasti V Ramanamma Children\u2019s Eye Care Center, L V Prasad Eye Institute, Hyderabad, India"},{"name":"The Cornea Institute, L V Prasad Eye Institute, Hyderabad, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sirisha","family":"Senthil","sequence":"additional","affiliation":[{"name":"VST Glaucoma Center, L V Prasad Eye Institute, Hyderabad, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anil Kumar","family":"Mandal","sequence":"additional","affiliation":[{"name":"VST Glaucoma Center, L V Prasad Eye Institute, Hyderabad, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brijesh","family":"Takkar","sequence":"additional","affiliation":[{"name":"Centre for Rare Eye Diseases and Ocular Genetics, L V Prasad Eye Institute, Hyderabad, India"},{"name":"Srimati Kanuri Santamma Centre for Vitreoretinal Diseases, L V Prasad Eye Institute, Hyderabad, India"},{"name":"Indian Health Outcomes, Public Health & Economics Research Centre, L V Prasad Eye Institute, Hyderabad, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dilip Kumar","family":"Mishra","sequence":"additional","affiliation":[{"name":"Ophthalmic Pathology Laboratory, L V Prasad Eye Institute, Hyderabad, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deepak Paul","family":"Edward","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology and Visual Sciences and Pathology, University of Illinois College of Medicine, Chicago, IL, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muralidhar","family":"Ramappa","sequence":"additional","affiliation":[{"name":"Centre for Rare Eye Diseases and Ocular Genetics, L V Prasad Eye Institute, Hyderabad, India"},{"name":"Jasti V Ramanamma Children\u2019s Eye Care Center, L V Prasad Eye Institute, Hyderabad, India"},{"name":"The Cornea Institute, L V Prasad Eye Institute, Hyderabad, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2022,11,30]]},"reference":[{"key":"R1-20251006","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.survophthal.2005.11.007","article-title":"Mucopolysaccharidoses and the eye","volume":"51","author":"Ashworth","year":"2006","journal-title":"Surv Ophthalmol"},{"key":"R2-20251006","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1038\/sj.eye.6701921","article-title":"The ocular features of the mucopolysaccharidoses","volume":"20","author":"Ashworth","year":"2006","journal-title":"Eye (Lond)"},{"key":"R3-20251006","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1007\/s10545-005-0112-z","article-title":"Cumulative incidence rates of the mucopolysaccharidoses in Germany","volume":"28","author":"Baehner","year":"2005","journal-title":"J Inherit Metab Dis"},{"key":"R4-20251006","doi-asserted-by":"crossref","first-page":"1577","DOI":"10.1111\/j.1651-2227.2008.00965.x","article-title":"Mucopolysaccharidoses in the Scandinavian countries:Incidence and prevalence","volume":"97","author":"Malm","year":"2008","journal-title":"Acta Paediatr"},{"key":"R5-20251006","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1007\/s00431-007-0635-4","article-title":"Mucopolysaccharidosis type II (Hunter syndrome):A clinical review and recommendations for treatment in the era of enzyme replacement therapy","volume":"167","author":"Wraith","year":"2008","journal-title":"Eur J Pediatr"},{"key":"R6-20251006","doi-asserted-by":"crossref","first-page":"1929","DOI":"10.1056\/NEJMoa2106596","article-title":"Hematopoietic stem- and progenitor-cell gene therapy for Hurler syndrome","volume":"385","author":"Gentner","year":"2021","journal-title":"N Engl J Med"},{"key":"R7-20251006","doi-asserted-by":"crossref","first-page":"1467","DOI":"10.3390\/jcm8091467","article-title":"Ophthalmological findings in mucopolysaccharidoses","volume":"8","author":"Tomatsu","year":"2019","journal-title":"J Clin Med"},{"key":"R8-20251006","doi-asserted-by":"crossref","first-page":"e111","DOI":"10.1111\/aos.12607","article-title":"Assessment and diagnosis of suspected glaucoma in patients with mucopolysaccharidosis","volume":"93","author":"Ashworth","year":"2015","journal-title":"Acta Ophthalmol"},{"key":"R9-20251006","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1016\/j.ajo.2020.01.007","article-title":"Anterior chamber angles in different types of mucopolysaccharidoses","volume":"212","author":"Zhang","year":"2020","journal-title":"Am J Ophthalmol"},{"key":"R10-20251006","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1001\/archopht.1985.01050060045020","article-title":"Refractive development of the human eye","volume":"103","author":"Gordon","year":"1985","journal-title":"Arch Ophthalmol"},{"key":"R11-20251006","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1111\/j.1755-3768.2011.02280.x","article-title":"Clinical guidelines for diagnosing and managing ocular manifestations in children with mucopolysaccharidosis","volume":"90","author":"Fahnehjelm","year":"2012","journal-title":"Acta Ophthalmol"},{"key":"R12-20251006","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1111\/j.1755-3768.2010.01934.x","article-title":"Ocular axial length and corneal refraction in children with mucopolysaccharidosis (MPS I-Hurler)","volume":"90","author":"Fahnehjelm","year":"2012","journal-title":"Acta Ophthalmol"},{"key":"R13-20251006","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1038\/eye.1999.16","article-title":"The development and maintenance of emmetropia","volume":"13","author":"Brown","year":"1999","journal-title":"Eye (Lond)"},{"key":"R14-20251006","doi-asserted-by":"crossref","first-page":"744","DOI":"10.1016\/j.ajo.2004.06.030","article-title":"Corneal thickness in children","volume":"138","author":"Hussein","year":"2004","journal-title":"Am J Ophthalmol"},{"key":"R15-20251006","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1038\/eye.2013.281","article-title":"Anterior segment OCT imaging in mucopolysaccharidoses type I, II, and VI","volume":"28","author":"Ahmed","year":"2014","journal-title":"Eye (Lond)"},{"key":"R16-20251006","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1186\/s13023-021-01935-w","article-title":"Glaucoma in mucopolysaccharidoses","volume":"16","author":"Kong","year":"2021","journal-title":"Orphanet J Rare Dis"},{"key":"R17-20251006","doi-asserted-by":"crossref","DOI":"10.1136\/bcr-2013-202730","article-title":"Deep anterior lamellar keratoplasty in case of Hurler-Scheie syndrome","author":"Sati","year":"2014","journal-title":"BMJ Case Rep 2014"},{"key":"R18-20251006","doi-asserted-by":"crossref","first-page":"570","DOI":"10.1016\/j.ajo.2012.09.029","article-title":"Deep anterior lamellar keratoplasty in children","volume":"155","author":"Ashar","year":"2013","journal-title":"Am J Ophthalmol"}],"container-title":["Indian Journal of Ophthalmology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/ijo.IJO_1300_22","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T18:59:35Z","timestamp":1786647575000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/ijo.IJO_1300_22"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,30]]},"references-count":18,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2022]]}},"URL":"https:\/\/doi.org\/10.4103\/ijo.ijo_1300_22","relation":{},"ISSN":["0301-4738","1998-3689"],"issn-type":[{"value":"0301-4738","type":"print"},{"value":"1998-3689","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,11,30]]},"assertion":[{"value":"2022-05-25","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-08-30","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}