Principal Investigators

    Dr Christopher Verity

    Institution

    Cambridge University Hospitals NHS Foundation Trust

    Contact information of lead PI

    Country

    United Kingdom

    Title of project or programme

    To Undertake Prospective Multisource Surveillance for all Cases of Progressive Intellectual and Neurological Deterioration Occurring in Children in the UK

    Source of funding information

    NIHR (PRP projects (RDD: June 1993-July 2007))

    Total sum awarded (Euro)

    € 3,232,238

    Start date of award

    01/04/1997

    Total duration of award in years

    20.0

    The project/programme is most relevant to:

    Prion disease

    Keywords

    Research Abstract

    The proposal will undertake prospective multi-source surveillances through the
    Surveillance Unit of Paediatrics and Child Health (the ‘British Paediatric
    Surveillance Unit’), liaising with the National Creutzfeldt-Jakob Surveillance Unit
    (CJDSU), for all cases of progressive intellectual and neurological deterioration
    (PIND) occuring in children under the age of 16 in the United Kingdom. The
    incidence of PIND and, as far as possible, its causes will be determined. Further
    investigation will be encouraged on those cases without a readily identifiable cause to detect children suffering from any form of CJD, should they occur.

    The programme will commence through the production of an initial surveillance
    case definition for PIND by an Expert Neurological Advisory Group. This will be
    circulated to all paediatricians via the Surveillance Unit of the Royal College of
    Paediatrics and Child Health (the ‘British Paediatric Surveillance Unit’).
    Paediatricians will make returns of cases and will be sent an “initial contact form”. Following this, if a reported case meets the surveillance case definition and does not have a confirmed or likely diagnosis, the case will be followed up by a telephone interview or visit from the research nurse to the clinician. The research paediatric neurologist may need to contact the local clinician to discuss
    investigations of individual cases of PIND. Any cases identified as possible CJD
    will be referred to the CJDSU, which will follow them up according to currently
    agreed CJDSU protocols. The Expert Neurological Advisory Group will agree the
    case definition and will meet quarterly to discuss all the notified cases of PIND and allocate them to appropriate diagnostic groups.

    The surveillance will be overseen by a Steering Committee that includes the
    participating institutes and representation from the Department of Health. This
    Committee will also review overall progress and agree release of data.

    The contract will run for 5 years but a review after 18 months will be held to give
    an early warning of a need to extend the surveillance beyond this time. Annual
    reports will be provided to the Department of Health.

    Aims
    HISTORIC PROJECT – Entire abstract transferred from the previous database into the [Scientific summary background] field of this database.

    Plan of Investigation
    HISTORIC PROJECT – Entire abstract transferred from the previous database into the [Scientific summary background] field of this database.

    Potential Impact
    HISTORIC PROJECT – Entire abstract transferred from the previous database into the [Scientific summary background] field of this database.

    Policy Relevance
    By observing the epidemiology and causes of Progressive Intellectual and
    Neurological Deterioration in Childhood (PIND), the proposal addresses three important issues for the Department of Health:

    to determine whether CJD (either sporadic, new variant or other forms)exists in children in the UK

    to improve diagnosis and management of CJD in children, should it occur, by heightening the awareness of doctors to these diseases and by using common protocols to detect them

    to provide a mechanism to monitor CJD numbers in children and to determine risk factors which may lead to CJD

    Lay Summary

    Further information available at:

Types: Investments > €500k
Member States: United Kingdom
Diseases: Prion disease
Years: 2016
Database Categories: N/A
Database Tags: N/A

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