Findings in the anterior segment on ultrasound biomicroscopy in Maroteaux-Lamy syndrome

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dc.contributor.authorCasanova, FHC
dc.contributor.authorAdan, CBD
dc.contributor.authorAllemann, N.
dc.contributor.authorFreitas, D. de
dc.contributor.institutionUniversidade Federal de São Paulo (UNIFESP)
dc.date.accessioned2016-01-24T12:31:21Z
dc.date.available2016-01-24T12:31:21Z
dc.date.issued2001-04-01
dc.description.abstractPurpose. Maroteaux-Lamy syndrome is one of the mucopolysaccharidoses caused by enzyme deficiency (arylsulfatase B) that leads to incomplete degradation and storage of dermatan sulfate. We report a case of mucopolysaccharidosis type VI (MPS VI; Maroteaux-Lamy syndrome) with corneal involvement and introduce ultrasound biomicroscopy (UBM) as an examination with which to follow disease progression in relation to deposition in cornea, angle, and iris, Methods. We describe a Ii-year-old boy with a clinical and laboratorial diagnosis of MPS VI who developed increasing bilateral corneal opacification and decreased visual acuity. He underwent two seriate UBM (50-MHz transducer) evaluations. Results. UBM examination showed diffuse and homogeneous stromal hyper-reflective deposit in both eyes and an increase in peripheral corneal thickness throughout time. Conclusion. High-frequency ultrasound documentation of corneal deposit and anterior segment involvement in a patient with Maroteaux-Lamy syndrome is unique, and follow-up revealed thickening of the corneal periphery, which may be related to the progression of the disease (continuous mucopolysaccharide deposits in corneal stroma). UBM was used to locate and document the deposit, as well as to accompany the deposit's evolution, characterizing corneal changes and angle structure involvement.en
dc.description.affiliationUniversidade Federal de São Paulo, Dept Ophthalmol, Paulista Sch Med, BR-04023062 São Paulo, Brazil
dc.description.affiliationUnifespUniversidade Federal de São Paulo, Dept Ophthalmol, Paulista Sch Med, BR-04023062 São Paulo, Brazil
dc.description.sourceWeb of Science
dc.format.extent333-338
dc.identifierhttp://dx.doi.org/10.1097/00003226-200104000-00019
dc.identifier.citationCornea. Philadelphia: Lippincott Williams & Wilkins, v. 20, n. 3, p. 333-338, 2001.
dc.identifier.doi10.1097/00003226-200104000-00019
dc.identifier.issn0277-3740
dc.identifier.urihttp://repositorio.unifesp.br/handle/11600/26523
dc.identifier.wosWOS:000167643000019
dc.language.isoeng
dc.publisherLippincott Williams & Wilkins
dc.relation.ispartofCornea
dc.rightsinfo:eu-repo/semantics/restrictedAccess
dc.subjectMaroteaux-Lamy syndromeen
dc.subjectmucopolysaccharidosesen
dc.subjectultrasound biomicroscopyen
dc.titleFindings in the anterior segment on ultrasound biomicroscopy in Maroteaux-Lamy syndromeen
dc.typeinfo:eu-repo/semantics/article
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