Central corneal thickness and its relationship to intraocular pressure in mucopolysaccararidoses-1 following bone marrow transplantation. 2008

Paul Connell, and Kathryn McCreery, and Aoife Doyle, and Fiona Darcy, and Anne O'Meara, and Donal Brosnahan
Our Lady's Hospital for Sick Children, Ophthalmic Department, Crumlin, Dublin, Republic of Ireland.

OBJECTIVE To examine the ocular findings in mucopolysaccaridoses-1 (MPS-1) and the relationship between intraocular pressure (IOP) and central corneal thickness following bone marrow transplantation. METHODS METHODS clinical practice. METHODS 23 subjects with MPS-1 following bone marrow transplantation were examined. METHODS age, sex, visual acuity, presence of strabismus, refractive error, fundus examination, intraocular pressure, and central corneal thickness were assessed for each individual. METHODS correlation of central corneal thickness with intraocular pressure. Only the right eye was used in correlations. RESULTS Forty-six eyes of 23 subjects were examined. All subjects had been treated with successful bone marrow transplantation with mean follow-up of 8.5 years. Ages ranged from 17 months to 19 years (SD 5.03). Of the subjects, 60.8% were female; 85.5% were hyperopic; 8.6% had best-corrected visual acuity of >/=6/12; 34.5% had best-corrected visual acuity of <6/12 and >6/36 with 56.9% <6/36; 30% had strabismus. IOP ranged from 13 to 41 mm Hg with four children on antiglaucoma therapy. Central corneal thickness ranged from 484 microm to 705 microm and was moderately correlated with measured IOP in the right eye (r = 0.56). Corneal opacification moderately correlated with central corneal thickness (r = 0.57). Seventy percent had a normal disk evaluation; 30% had abnormal disk cupping. CONCLUSIONS This is the first large case series of MPS-1 subjects demonstrating a correlation between measured IOP and central corneal thickness. All subjects had corneal opacification moderately correlating with corneal pachymetry.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007429 Intraocular Pressure The pressure of the fluids in the eye. Ocular Tension,Intraocular Pressures,Ocular Tensions,Pressure, Intraocular,Pressures, Intraocular,Tension, Ocular,Tensions, Ocular
D008059 Mucopolysaccharidosis I Systemic lysosomal storage disease caused by a deficiency of alpha-L-iduronidase (IDURONIDASE) and characterized by progressive physical deterioration with urinary excretion of DERMATAN SULFATE and HEPARAN SULFATE. There are three recognized phenotypes representing a spectrum of clinical severity from severe to mild: Hurler syndrome, Hurler-Scheie syndrome and Scheie syndrome (formerly mucopolysaccharidosis V). Symptoms may include DWARFISM; hepatosplenomegaly; thick, coarse facial features with low nasal bridge; corneal clouding; cardiac complications; and noisy breathing. Hurler's Syndrome,Hurler-Scheie Syndrome,Lipochondrodystrophy,Mucopolysaccharidosis V,Pfaundler-Hurler Syndrome,Scheie's Syndrome,Gargoylism,Gargoylism, Hurler Syndrome,Hurler Disease,Hurler Syndrome,Hurler's Disease,Mucopolysaccharidosis 1,Mucopolysaccharidosis 5,Mucopolysaccharidosis I-S,Mucopolysaccharidosis Type I,Mucopolysaccharidosis Type Ih,Mucopolysaccharidosis Type Ih S,Mucopolysaccharidosis Type Is,Scheie Syndrome,alpha-L-Iduronidase Deficiency,Disease, Hurler's,Gargoylisms,Hurler Scheie Syndrome,Hurler Syndrome Gargoylism,Lipochondrodystrophies,Mucopolysaccharidosis I S,Mucopolysaccharidosis Is,Mucopolysaccharidosis Type Ihs,Syndrome, Hurler's,Syndrome, Scheie's,Type Ih, Mucopolysaccharidosis,Type Ihs, Mucopolysaccharidosis,alpha L Iduronidase Deficiency,alpha-L-Iduronidase Deficiencies
D008297 Male Males
D010786 Photoreceptor Cells Specialized cells that detect and transduce light. They are classified into two types based on their light reception structure, the ciliary photoreceptors and the rhabdomeric photoreceptors with MICROVILLI. Ciliary photoreceptor cells use OPSINS that activate a PHOSPHODIESTERASE phosphodiesterase cascade. Rhabdomeric photoreceptor cells use opsins that activate a PHOSPHOLIPASE C cascade. Ciliary Photoreceptor Cells,Ciliary Photoreceptors,Rhabdomeric Photoreceptor Cells,Rhabdomeric Photoreceptors,Cell, Ciliary Photoreceptor,Cell, Photoreceptor,Cell, Rhabdomeric Photoreceptor,Cells, Ciliary Photoreceptor,Cells, Photoreceptor,Cells, Rhabdomeric Photoreceptor,Ciliary Photoreceptor,Ciliary Photoreceptor Cell,Photoreceptor Cell,Photoreceptor Cell, Ciliary,Photoreceptor Cell, Rhabdomeric,Photoreceptor Cells, Ciliary,Photoreceptor Cells, Rhabdomeric,Photoreceptor, Ciliary,Photoreceptor, Rhabdomeric,Photoreceptors, Ciliary,Photoreceptors, Rhabdomeric,Rhabdomeric Photoreceptor,Rhabdomeric Photoreceptor Cell
D011184 Postoperative Period The period following a surgical operation. Period, Postoperative,Periods, Postoperative,Postoperative Periods
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003315 Cornea The transparent anterior portion of the fibrous coat of the eye consisting of five layers: stratified squamous CORNEAL EPITHELIUM; BOWMAN MEMBRANE; CORNEAL STROMA; DESCEMET MEMBRANE; and mesenchymal CORNEAL ENDOTHELIUM. It serves as the first refracting medium of the eye. It is structurally continuous with the SCLERA, avascular, receiving its nourishment by permeation through spaces between the lamellae, and is innervated by the ophthalmic division of the TRIGEMINAL NERVE via the ciliary nerves and those of the surrounding conjunctiva which together form plexuses. (Cline et al., Dictionary of Visual Science, 4th ed) Corneas

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