UNICORNS: Uveitis in Childhood Prospective National Cohort Study

Study Overview
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Brief Summary
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Childhood uveitis (inflammation inside the eye) is an uncommon disorder that carries the risk of blindness. Inadequate treatment of active inflammation has been shown to be related to a poor outcome. There has been no population-based, prospective longitudinal study of all-cause childhood uveitis, with resultant limitations in the evidence base used to counsel affected families, balance treatment decisions, or plan further research. The aim of the study is to describe the characteristics of childhood-onset uveitis and describe outcomes. The investigators shall also aim to identify the socio-demographic, clinical, biological and treatment-related determinants of outcome. Early (1-2 years following diagnosis) outcomes will be described in the first instance: However, through the creation of a national inception cohort, the investigators shall enable longer-term studies of outcome for affected children and families. There will be no change to routine clinical care.

Detailed Description
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Background: Childhood uveitis descriptive term for a group of rare inflammatory eye disease, which are typically chronic, relapsing-remitting in nature, and of uncertain aetiology (idiopathic). Visual loss occurs due to structural damage caused by uncontrolled inflammation. Understanding of the determinants of long-term outcome is limited, particularly the predictors of therapeutic response or how to define disease control. Aims: To describe disease natural history and outcomes amongst a nationally representative group of children with non-infectious uveitis, describe the impact of disease course on quality of life for both child and family, and identify determinants of adverse visual, structural and developmental outcomes. Methods: UNICORNS is a prospective longitudinal multicentre cohort study of children newly diagnosed with uveitis about whom a core minimum clinical dataset will be collected. Participants and their families will also complete patient-reported outcome measures (PROMS) at study entry and annually from recruitment. PROMS collected at baseline are: Strength and Difficulties Questionnaire (SDQ): this is to screen the emotional and behavioural aspects of the participants' lives Child Health Utility 9D (CHU9D): generic preference based measure of health related quality of life Children's Sleep Habits Questionnaire (CHSQ): this evaluates incidence of behaviours linked with typical paediatric sleep difficulties 100-mm General Evaluation (GE): visual analogue score 100mm long used to capture overall perception of the burden of disease PROMS collected at year 1 and annually (subject to investigations of instrument redundancy) are: Child Health Utility 9D (CHU9D): generic preference based measure of health related quality of life 100-mm General Evaluation (GE): visual analogue score 100mm long used to capture overall perception of the burden of disease Paediatric Quality of life Score (PedsQL): a brief questionnaire used to evaluate health related quality of life in children Vision related quality of life metric - Children and Young People (VQoL_CYP): these instruments captures the functional and broader impacts of living with a visual disability The association of patient (child- and treatment- dependent) characteristics with outcome will be investigated using logistic and ordinal regression models which incorporate adjustment for within-child correspondence between eyes for those with bilateral disease and repeated outcomes measurement. Discussion: Through this population based, prospective longitudinal study of childhood uveitis, the investigators will describe the characteristics of childhood onset disease. Early (1-2 years following diagnosis) outcomes will be described in the first instance, and through the creation of a national inception cohort, longer term studies of outcome for affected children and families will be enabled.

Official Title
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Uveitis in Childhood Prospective National Cohort Study

Conditions
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Uveitis

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Children newly diagnosed with uveitis (within previous 6 months) Age < 18years Exclusion Criteria: Uveitis due to malignancy Uveitis due to ocular trauma (including iatrogenic, ie intraocular surgery) Uveitis due to confirmed ocular infection Children with developmental disorders or impairments which prevent their self-report of their function level or quality of life

Ages Eligible for Study
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Maximum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Childhood Uveitis<br>Children <18 years old newly diagnosed with non-infectious uveitis | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Incidence of ocular complications | New incident of sight threatening ocular complications, including glaucoma, cataract and macular oedema | Up to 3 years | 
| Pediatric Quality of Life Inventory Score | Quality of life (patient reported outcomes) using Pediatric Quality of Life Inventory (PedsQL) in which items are reversed scored and linearly transformed to a 0-100 scale, so that higher scores indicate better HRQOL (Health-Related Quality of Life) | Up to 3 years | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Corticosteroid exposure | Total prescribed topical and systemic corticosteroid burden | Up to 3 years | 
| Attainment of disease control | Number of children achieving absolute control defined as absence of inflammation Relative control defined as the absence of inflammation greater than 0.5 The lowest grade of inflammation, with the use of less than one drop of topical corticosteroid | Up to 3 years | 
| Health Utility | Child Health Utility Metric (CHU9D), with scores ranging from 0.33 (worst health state) to 1.00 (best health state) | Up to 3 years | 
| Change in Pediatric Quality of Life Inventory Score | Change in child reported quality of life using Pediatric Quality of Life Inventory (PedsQL) in which items are reversed scored and linearly transformed to a 0-100 scale, so that higher scores indicate better HRQOL (Health-Related Quality of Life), and a change of greater than 5 is clinically meaningful | Over one year from baseline to year one | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Vision, Quality of life, Child, Prospective Cohort