Atypical Kawasaki Disease, Kawasaki Disease
Conditions
Keywords
Kawasaki Disease
Brief summary
Kawasaki disease (KD) is an acute self-limited vasculitis of infancy and early childhood. Most patients recover without sequelae although the inflammatory process causes permanent damage to the coronary arteries in 20-25% of untreated children. An infectious aetiology is suspected, but the causative agent has not been identified. The investigators aim to identify the genes underlying both susceptibility to Kawasaki disease, and the development of coronary artery aneurysms.
Detailed description
The problem to be addressed; Kawasaki disease is now the most common cause of acquired heart disease in children in Japan and North America. Kawasaki disease arises when genetically predisposed children encounter an as yet unidentified infectious agent which may cause only mild illness or no illness at all in children without the genetic predisposition. Other children may suffer permanent damage to the coronary arteries. Identification of the genes involved will help to improve understanding of the disease, and the development of better treatments. Objective The investigators aim to identify the genes underlying both susceptibility to Kawasaki disease, and the development of coronary artery aneurysms. Design The study will recruit nuclear families (affected child and their biological parents) through participating NHS hospitals or through the records of the UK Kawasaki Support Group. Study size 400 affected children and both biological parents (i.e. 1200 participants) Procedures Informed consent using age appropriate patient/parent/guardian information sheets will be taken from parents (or from children aged 16 and over), assent will be taken from the child under the age of 16 (if appropriate). Children recruited during the acute illness; routine clinical and laboratory data and research samples (blood, urine, throat swab). Children recruited retrospectively; study questionnaire (completed by parents), saliva samples.Saliva samples from parents
Interventions
None listed
Sponsors
Guy's and St Thomas' NHS Foundation Trust
University Hospitals Bristol and Weston NHS Foundation Trust
Great Ormond Street Hospital for Children NHS Foundation Trust
UK Kawasaki Support Group
Imperial College Healthcare NHS Trust
University Hospitals of North Midlands NHS Trust
Burton Hospitals NHS Foundation Trust
York Teaching Hospitals NHS Foundation Trust
Shrewsbury and Telford Hospitals NHS Trust
East Suffolk and North Essex NHS Foundation Trust
New Cross Hospital
Darlington Memorial Hospital
Torbay Hospital
Peterborough City Hospital
University Hospitals Coventry and Warwickshire NHS Trust
University Hospital of North Tees
Cambridge University Hospitals NHS Foundation Trust
Royal Cornwall Hospitals Trust
Birmingham Children's Hospital
University Hospital Birmingham NHS Foundation Trust
Wye Valley NHS Trust
South Tees Hospitals NHS Foundation Trust
Leeds General Infirmary
Bradford Royal Infirmary
University Hospitals of Morecambe Bay NHS Trust
Calderdale and Huddersfield NHS Foundation Trust
St. Richard's Hospital
Pinderfields General Hospital
Worthing Hospital
Airedale General Hospital
Sheffield Children's NHS Foundation Trust
Northwick Park Hospital
West Middlesex Hospital
Royal Albert Edward Infirmary
Macclesfield District General Hospital
Royal Oldham Hospital
Stepping Hill Hospital
North Manchester General Hospital
Royal Bolton Hospital NHS Foundation Trust
Kingston Hospital NHS Trust
Tameside Hospital NHS Foundation Trust
East Surrey Hospital
St. George's Hospital, London
Royal Alexandra Children's Hospital
Imperial College London
Study design
Observational model
COHORT
Time perspective
OTHER
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
Inclusion criteria
* Affected children will be recruited if the treating clinician has made a diagnosis of possible Kawasaki disease (even if they do not fulfil the criteria below for Kawasaki disease). The current standard diagnostic criteria for KD (Circulation 2001 103 335-336 doi: 10.1161/01.CIR 103.2.335) are: The presence of fever for at least five days plus four of the following criteria: 1. Changes in the peripheral extremities Acute: erythema and oedema of hands and feet Convalescent: membranous desquamation of fingertips 2. Polymorphous exanthema 3. Bilateral painless bulbar conjunctival injection without exudate 4. Changes in lips and oral cavity: erythema and cracking of lips, strawberry tongue, diffuse injection of oral and pharyngeal mucosae 5. Cervical lymphadenopathy (\>1.5cm diameter), usually unilateral Patients meeting not all of these criteria may meet the criteria for atypical Kawasaki disease, ie. if they have fever and two or three of the above criteria and elevation of CRP or echocardiographic evidence of coronary artery dilatation. Parents of affected child must be biological parents.
Exclusion criteria
* children who do not have a diagnosis of possible Kawasaki disease
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Susceptibility of coronary artery aneurysms for Kawasaki patients | end date; 30 December 2022 | Identification of genes that are associated with susceptibility of coronary artery aneurysms |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Disease severity for Kawasaki patients | end date; 30 December 2022 | Identification of genes that are associated with disease severity |
Countries
United Kingdom
Outcome results
None listed