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Identifying Children with HEreditary Coagulation disorders (iCHEC): A protocol for a prospective cohort study

Lookup NU author(s): Dr Tina Biss

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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).


Abstract

© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted. Introduction It is challenging to obtain a reliable bleeding history in children who are referred for a suspected inherited bleeding disorder. Bleeding symptoms may be subtle as children face fewer haemostatic challenges compared with adults. In order to standardise bleeding histories, questionnaires have been developed, called bleeding assessment tools (BATs). Although it has been shown that high bleeding scores are associated with the presence of a mucocutaneous bleeding disorder, these BATs lack sensitivity, efficiency and flexibility in the paediatric setting. We developed a new BAT (the iCHEC (identifying Children with HEreditary Coagulation disorders) BAT) to improve on these characteristics. We aim to evaluate the diagnostic accuracy of the iCHEC BAT as a screening tool for children who are suspected for having a bleeding disorder. Methods and analysis This is a prospective cohort study. Children (age 0-18 years) suspected for a bleeding disorder who present at tertiary haematology clinics, and/or their parents/guardians, will be asked to complete the iCHEC BAT. Sensitivity was increased by inclusion of paediatric-specific bleeding symptoms and novel qualitative questions per bleeding symptom. Efficiency was improved by developing a self-administered (online) version of the questionnaire. Flexibility for changes in the bleeding phenotype of developing children was improved by including questions that define when the bleeding symptoms occurred in the past. The diagnostic accuracy of the specific bleeding items will be evaluated by receiver operator characteristic curves, using classification based on the results from laboratory assessment as the reference standard. Analysis of the discriminative power of individual bleeding symptoms will be assessed. Ethics and dissemination The study has been approved by the medical ethics committees of all participating centres in the Netherlands, Canada and the UK. All paediatric subjects and/or their parents/guardians will provide written informed consent. Study results will be submitted for publication in peer-reviewed journals.


Publication metadata

Author(s): Stokhuijzen E, Rand ML, Cnossen MH, Biss TT, James PD, Suijker MH, Peters M, Van Der Lee JH, Peters B, Meijer AB, Blanchette VS, Fijnvandraat K

Publication type: Review

Publication status: Published

Journal: BMJ Open

Year: 2018

Volume: 8

Issue: 5

Online publication date: 03/05/2018

Acceptance date: 22/03/2018

ISSN (electronic): 2044-6055

Publisher: BMJ Publishing Group

URL: https://doi.org/10.1136/bmjopen-2017-020686

DOI: 10.1136/bmjopen-2017-020686


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