12 April 2017
Recruiting Patients for Newborn Screening Research Study
Who are we looking for?
Patients: Researchers at Sheffield Children's
NHS Foundation Trust are currently recruiting children and adult
patients with the following Newborn Screening (NBS) disorders:
- Phenylketonuria (PKU)
- Medium-Chain Acyl-CoA Dehydrogenase
Deficiency (MCADD)
- Maple Syrup Urine Disease (MSUD)
- Isovaleric Acidaemia (IVA)
- Glutaric Aciduria Type 1 (GA1)
- Homocystinuria (pyridoxine
unresponsive; HCU)
The study would like to recruit patients from the following
Hospitals:
- Birmingham Children's Hospital
- Central Manchester University Hospitals
- Sheffield Children's Hospital
- Guy's and St Thomas'
- Great Ormond Street Hospital
- Nottingham Queen Medical Centre
- Leicester Royal Infirmary
- Sheffield Northern General Hospital
- University College London Hospitals
- Bristol Royal Hospital for Children
At the patient's hospital, participants will be asked to provide
a blood sample. DNA from this sample will be used for genetic
sequencing of specific genes associated with these inherited
disorders. Participants will need to consent to sharing of medical
record information such as symptoms, treatments and test results
including genetics. All information collected will be stored
securely in a confidential database. Hospital visits with the
research nurse should last approximately thirty minutes and
refreshments will be provided. It is recommended that children are
accompanied by a parent, carer or relative during the hospital
visit. Participants may withdraw from the study at any point.
Why are we doing this?
NBS is used worldwide to identify babies affected with
rare, often fatal, but treatable disorders. Analysis of dried blood
spot (DBS) samples taken shortly after birth provides
cost-effective testing in a short time frame. Infants found to have
a specific condition will receive rapid medical attention, but
predicting disease severity and appropriate management in a child
without symptoms can be challenging.
The research team will use Next Generation DNA Sequencing (NGS)
to analyse genetic changes associated with NBS disorders. Clinical
details including patient symptoms, blood chemicals and genetic
mutations will be entered into a database. Understanding better the
relationship between the genetics and the clinical symptoms in
these diseases, will help provide improved and personalised
treatment to affected babies, children and adults. This project
will establish whether NGS can be used as an initial or follow-up
test for NBS to improve the clinical utility of existing UK NBS
programmes.
This research study is led by Dr Ann Dalton and Professor Anne
Goodeve at Sheffield Children's NHS Foundation Trust. This research
is supported by an award from the Health Innovation Challenge Fund
(HICF-R9-518), a parallel funding partnership between the
Department of Health and the Wellcome Trust.
This research has been approved by the West Midlands - South
Birmingham Research Ethics Committee
Interested Participants:
If you are interested in taking part or if you have any queries
about the study, please contact the Project Manager Clare.Gladding@sch.nhs.uk.
Thank you in advance for your help.
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