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Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
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<a href="http://doi.org/10.1016/j.ejpn.2015.09.002" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ejpn.2015.09.002</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
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Development of global rating instruments for pediatric patients with ataxia telangiectasia
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European Journal of Paediatric Neurology
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2016
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Male; Child; Humans; Adolescent; Female; Child Preschool; Severity of Illness Index; Pediatrics/mt [Methods]; Ataxia Telangiectasia/di [Diagnosis]; Ataxia Telangiectasia/ep [Epidemiology]; tone and motor problems; ataxia telangiectasia; tool development; scale development; ICARS; SARA; Brief Ataxia Rating Scale
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Nissenkorn A; Borgohain R; Micheli R; Leuzzi V; Hegde A U; Mridula K R; Molinaro A; D'Agnano D; Yareeda S; Ben-Zeev B
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INTRODUCTION: Ataxia telangiectasia (AT) is a neurodegenerative disorder with cerebellar and extrapyramidal features. Interventional and epidemiological studies in AT should rely on specific scales which encompass the specific neurological features, as well the early progressive course and the subsequent plateau. The aim of this study was to build a scale of the CGI type (Clinical Global Impression) which is disease specific, as well as to check the feasibility of the ICARS scale for ataxia in this population. METHODS: We recruited 63 patients with ataxia, aged 10.76 +/- 3.2 years, followed at 6 international AT centers, 49 of them (77.8%) with classical AT. All patients were evaluated for ataxia with ICARS scale. In patients with AT, two CGI scales were scored, unstructured as structured for which separate anchors were provided. RESULTS: Mean ICARS score was 44.7 +/- 20.52, and it's severity positively correlated with age (Spearman correlation, r = 0.46, p < 0.01). Mean CGI score was 2 (moderately involved). There was a high correlation between the structured and unstructured CGIs (Spearman correlation, r = 0.87, p < 0.01). Both CGI scales showed positive correlation between severity and increasing age (Spearman correlation r = 0.59, p < 0.01 for structured CGI and r = 0.61, p < 0.01 for unstructured). DISCUSSION: We succeeded to build two CGI scales: structured and unstructured, which are disease specific for AT. The unstructured scale showed better connection to disease course; the sensitivity of the unstructured scale could be improved by adding anchors related to extrapyramidal features. In addition we showed that ataxia can be reliably measured in children with AT by using ICARS.Copyright © 2015 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.
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<a href="http://doi.org/10.1016/j.ejpn.2015.09.002" target="_blank" rel="noreferrer noopener">10.1016/j.ejpn.2015.09.002</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2016
Adolescent
ataxia telangiectasia
ataxia telangiectasia/di [Diagnosis]
Ataxia Telangiectasia/ep [Epidemiology]
Ben-Zeev B
Borgohain R
Brief Ataxia Rating Scale
Child
Child Preschool
D'Agnano D
European Journal of Paediatric Neurology
Female
Hegde A U
Humans
ICARS
Leuzzi V
Male
Micheli R
Molinaro A
Mridula K R
Nissenkorn A
Pediatrics/mt [methods]
SARA
scale development
Severity Of Illness Index
tone and motor problems
tool development
Yareeda S