Functional vision and quality of life in children with microphthalmia/anophthalmia/coloboma-a cross-sectional study.

PURPOSE: To determine the child's and parental perception of functional visual ability (FVA), vision-related and health-related quality of life (VR-QoL, HR-QoL) in children with microphthalmia/anophthalmia/coloboma (MAC). METHODS: Between June 25, 2014, and June 3, 2015, we carried out a cross-sectional observational study at Moorfields Eye Hospital, London, UK, enrolling 45 children 2-16 years of age with MAC attending our clinics, and their parents. To assess FVA, VR-QoL, and HR-QoL we asked participants to complete three validated tools, the Cardiff Visual Ability Questionnaire for Children (CVAQC), the Impact of Vision Impairment for Children (IVI-C) instrument, and the PedsQL V 4.0. The main outcome measures were the FVA, VR-QoL, and HR-QoL scores, reported by children and parents. RESULTS: In children with MAC, FVA is moderately reduced, with a median CVAQC score of -1.4 (IQR, -2.4 to 0.4; range, -3.0 [higher FVA] to +2.8 [lower FVA]). VR-QoL and HR-QoL are greatly reduced, with an IVI-C median score of 63 (IQR, 52-66; normal VR-QoL, 96), a median self-reported PedsQL score of 77 (IQR, 71-90; normal HR-QoL, 100) and parental score of 79 (IQR, 61-93), and a family impact score of 81 (67-93). Psychosocial well-being scores are lower than physical well-being scores. Parents and children have a different perception of the impact of the condition on the child's HR-QoL. CONCLUSIONS: MAC has a significant impact on a child's FVA and QoL, similar to that described by children with acute lymphoblastic leukaemia and chronic systemic conditions. Children and families may benefit from psychosocial support.

Quality of Life and Functional Vision in Children with Glaucoma.

PURPOSE: To evaluate the effect of glaucoma on functional vision and on vision-related (VR) and health-related (HR) quality of life (QoL) in children up to 16 years of age. DESIGN: Cross-sectional observational study. PARTICIPANTS: One hundred nineteen children 2 to 16 years of age (mean age, 9.4 years; standard deviation [SD], 4.56 years) with glaucoma and their parents. METHODS: Completion of 3 validated instruments for children to assess (1) functional visual ability (FVA) with the Cardiff Visual Ability Questionnaire for Children (CVAQC), (2) VR QoL with the Impact of Vision Impairment for Children (IVI-C), and (3) HR QoL with the Pediatric Quality of Life Inventory (PedsQL) version 4.0. MAIN OUTCOME MEASURES: Cardiff Visual Ability Questionnaire for Children, IVI-C, and PedsQL scores. RESULTS: Scores for FVA, VR QoL, and HR QoL were reduced in children with glaucoma: median CVAQC score, -1.24 (interquartile range [IQR], -2.2 to -0.11; range, -3.00 higher visual ability to +2.80 lower visual ability); mean IVI-C score, 67.3 (SD, 14.4; normal VR QoL, 96); median PedsQL self-report, 78.8 (IQR, 67.4-90.2); parent report, 71.2 (IQR, 55.7-85.8); and family impact score, 74.3 (IQR, 56.9-88.5; normal HR QoL, 100). Psychosocial subscores were lower than physical subscores on the PedsQL. Older children reported less impairment on CVAQC, IVI-C, and PedsQL than younger children. Parents reported greater impact on their child's HR QoL than children reported themselves. CONCLUSIONS: Glaucoma and its management have a marked impact on a child's FVA and QoL. Children with glaucoma report HR QoL scores similar to those described by children with severe congenital cardiac defects, who have undergone liver transplants, or who have acute lymphoblastic leukemia.

Novel molecular insights into the function and the antioxidative stress response of a Peroxiredoxin Q protein from cyanobacteria.

The Peroxiredoxin Q (PrxQ) proteins are thiol-based peroxidases that are important for maintaining redox homeostasis in several organisms. Activity of PrxQs is mediated by two cysteines, peroxidatic (Cp) and resolving (Cr), in association with a reducing partner. A PrxQ, Alr3183, from the cyanobacterium, Anabaena PCC 7120, was characterized in this study. Alr3183, which required thioredoxin A (TrxA) for peroxidase activity, was an intramolecular disulfide bond-containing monomeric protein. However, Alr3183 lacking Cp (Alr3183C46S) or Cr (Alr3183C51S) formed intermolecular disulfide linkages and was dimeric. Alr3183C46S was completely inactive, while Alr3183C51S required higher concentration of TrxA for peroxidase activity. Surface plasmon resonance analysis showed that unlike Alr3183 or Alr3183C46S, Alr3183C51S bound rather poorly to TrxA. Also, compared to the oxidized protein, the DTT-treated (reduced) Alr3183 displayed decreased interaction with TrxA. In vivo, Alr3183 was found to be induced in response to γ-radiation. On exposure to H2O2, Anabaena strain over-expressing Alr3183 showed reduced formation of ROS, intact photosynthetic pigments and consequently better survival than the wild-type, whereas overproduction of Alr3183C46S did not provide any protection. Significantly, this study (1) reveals the importance of Cr for interaction with thioredoxins and (2) demonstrates that over-expression of PrxQs can protect cyanobacteria from oxidative stresses.

Childhood amblyopia: current management and new trends.

INTRODUCTION OR BACKGROUND: With a prevalence of 2-5%, amblyopia is the most common vision deficit in children in the UK and the second most common cause of functional low vision in children in low-income countries. SOURCES OF DATA: Pubmed, Cochrane library and clinical trial registries (clinicaltrials.gov, ISRCTN, UKCRN portfolio database). AREAS OF AGREEMENT: Screening and treatment at the age of 4-5 years are cost efficient and clinically effective. Optical treatment (glasses) alone can improve visual acuity, with residual amblyopia treated by part-time occlusion or pharmacological blurring of the better-seeing eye. Treatment after the end of the conventional 'critical period' can improve vision, but in strabismic amblyopia carries a low risk of double vision. AREAS OF CONTROVERSY: It is not clear whether earlier vision screening would be cost efficient and associated with better outcomes. Optimization of treatment by individualized patching regimes or early start of occlusion, and novel binocular treatment approaches may enhance adherence to treatment, provide better outcomes and shorten treatment duration. GROWING POINTS: Binocular treatments for amblyopia. AREAS TIMELY FOR DEVELOPING RESEARCH: Impact of amblyopia on education and quality of life; optimal screening timing and tests; optimal administration of conventional treatments; development of child-friendly, effective and safe binocular treatments.

Delivering clinical trials and observational studies in child eye health: a nationwide survey in the United Kingdom.

PURPOSE: Evidence-based medicine requires primary results from randomized controlled trials and high-quality observational studies; however, in pediatric medicine it can be difficult to enroll sufficient numbers of children to reach sample sizes required for statistical significance. The experience of clinicians and researchers with the practicalities of recruitment has not been explored, but could potentially inform best practice and lead to the development of successful research networks. Perceived barriers and facilitators to recruitment in child eye health in the United Kingdom, where adoption of trials and studies onto a central database has created a register of high quality projects, were investigated. METHODS: Studies and trials in child eye health from the United Kingdom Clinical Research Network portfolio database were identified and the named researchers contacted. Based on a validated tool, an electronic survey was created to ask about recruitment experience to identify facilitators and barriers. RESULTS: There were 46 trials and studies recruiting children, and 51 completed questionnaires from researchers were received. Children's eye research activity is mainly based at tertiary referral centers. Although most studies recruit to target, many researchers report difficulties. Availability of dedicated research teams in clinics and good communication between research team, clinical team, and families are strong facilitators. Administrative difficulties and the burden of research to clinical teams and families are strong barriers. CONCLUSIONS: Support for investigators in the form of dedicated research and administrative staff and good communication enhance recruitment to studies in child eye health.