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      Trying to see, failing to focus: near visual impairment in Down syndrome

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      Scientific Reports
      Nature Publishing Group

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          Abstract

          The majority of individuals with Down syndrome (DS) do not exhibit accurate accommodation, with the aetiology of this deficit unknown. This study examines the mechanism underlying hypoaccommodation in DS by simultaneously investigating the ‘near triad’ – accommodation, vergence and pupillary response. An objective photorefraction system measured accommodation, pupil size and gaze position (vergence) under binocular conditions while participants viewed an animated movie at 50, 33, 25 and 20 cm. Participants were aged 6–16 years (DS = 41, controls = 76). Measures were obtained from 59% of participants with DS and 99% of controls. Accommodative response was significantly less in DS (p < 0.001) and greater accommodative deficits were associated with worsening visual acuity (p = 0.02). Vergence responses were as accurate in DS as in controls (p = 0.90). Habitual pupil diameter did not differ between groups (p = 0.24) but reduced significantly with increasing accommodative demand in both participants with and without DS (p < 0.0001). This study is the first to report simultaneous binocular measurement of the near triad in DS demonstrating that hypoaccommodation is linked to poor visual acuity. Vergence responses were accurate indicating that hypoaccommodation cannot be dismissed as a failure to visually engage with near targets, but rather is a consequence of underlying neurological or physiological deficits.

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          Most cited references41

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          Health care concerns and guidelines for adults with Down syndrome.

          Down syndrome (DS) is the most common cause of mental retardation in North America, yet little information is available on the natural history of DS in adults. We report on significant medical problems of adults with DS (DS adults) residing in a British Columbia provincial residential center, Woodlands, over the 12-year period from 1981 through 1992. Prospective, yearly health care reviews on 38 DS adults are summarized according to age. Group 1 consists of 18 middle-aged DS adults less than 50 years old, and group 2 comprises 20 elderly DS adults 50 years and older. Significant health problems in all DS adults include untreated congenital heart anomalies (15. 8%), acquired cardiac disease (15.8%), pulmonary hypertension (7.8%), recurrent respiratory infections/aspiration leading to chronic pulmonary interstitial changes (30%), complications from presenile dementia/Alzheimer-type disease (42%), adult-onset epilepsy (36.8%), osteoarthritic degeneration of the spine (31.6%), osteoporosis with resultant fractures of the long bones (55%) or vertebral bodies (30%), and untreated atlantooccipital instability (7.9%). Acquired sensory deficits are significant problems including loss of vision due to early onset of adult cataracts (50%), recurrent keratitis (21%) or keratoconus (15.8%), and significant hearing loss (25%). Behavioral problems (50%), loss of cognitive abilities, and onset of symptoms of Alzheimer disease (group 1: 5.5%; group 2: 75%) pose ongoing challenges for care. In conclusion, the quality of life for adults with DS can be improved by routine, systematic health care screening to identify treatable diseases that may be missed because of poor communication or confusion due to Alzheimer disease. Copyright 1999 Wiley-Liss, Inc.
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            Validation of the PowerRefractor for measuring human infant refraction.

            Eccentric photorefraction provides an opportunity to gather rapid and remote estimates of refraction and gaze position from infants. The technique has the potential for extensive use in vision screenings and studies of visual development. The goal of this study was to assess the refraction calibration of the PowerRefractor (Multichannel Systems) for use with uncyclopleged infants. The defocus measurements from the instrument were compared with the results of simultaneous retinoscopy in one analysis and with known amounts of defocus induced with trial lenses in another. Data were collected from infants 1 to 6 months of age and adults. The PowerRefractor typically read < 1 D of myopia when the retinoscopy reflex was judged to be neutral at the same working distance in both infants and adults. The slopes of both infant and adult validation functions (trial lens power vs. measurement of induced defocus) were close to 1 over a 4D range. The infant slopes were significantly greater than those of the adults, however. The results suggest that the instrument is capable of detecting large amounts of defocus but needs individual calibration for detailed studies of accommodative accuracy and absolute levels of defocus, as has been recommended previously for adult subjects.
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              Strabismus and binocular function in children with Down syndrome. A population-based, longitudinal study.

              We have performed a population-based, longitudinal study on strabismus in children with Down syndrome. The aims of the study were to examine the frequency and type of strabismus, the age at onset, and the binocular potential. An unselected population of 60 children with Down syndrome born 1988-1999 was followed with repeated examinations. Mean follow-up time was 55+/-23 months (range 24--115). The alignment of the eyes was examined using Hirschberg corneal reflex test and cover test for near fixation. To evaluate binocular function, Titmus House Fly Test and Lang's stereo test were used. Twenty-five patients (42%) had strabismus (21 esotropias, two exodeviations and two vertical deviations). Only one case of infantile esotropia was found, the other esotropias were acquired forms. The mean age at "onset" (e.g. when strabismus was first noticed) was 54+/-35 months. In the acquired esotropia group (n=20), 15 (75%) were associated with hypermetropia (mean spherical equivalent +4.3+/-1.7 D). Seventeen of the strabismic patients had an accommodation weakness. Eleven of the strabismus patients gave a clearly positive response to one or both stereotests. The majority of the Down syndrome children with strabismus have an acquired esotropia and hence a potential for binocularity. Hypermetropia and accommodation weakness are probably important factors in esotropia in Down syndrome patients.
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                Author and article information

                Journal
                Sci Rep
                Sci Rep
                Scientific Reports
                Nature Publishing Group
                2045-2322
                05 February 2016
                2016
                : 6
                : 20444
                Affiliations
                [1 ]School of Biomedical Sciences, University of Ulster , Cromore Road, Coleraine, Northern Ireland, BT52 1SA.
                Author notes
                Article
                srep20444
                10.1038/srep20444
                4742775
                26847360
                b08b0388-9398-4871-ba6b-5e34f56a5a1e
                Copyright © 2016, Macmillan Publishers Limited

                This work is licensed under a Creative Commons Attribution 4.0 International License. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in the credit line; if the material is not included under the Creative Commons license, users will need to obtain permission from the license holder to reproduce the material. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

                History
                : 02 October 2015
                : 04 January 2016
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