Neurocognitive monitoring: Neurocognitive tests and MRI of the brain
Study summary
Biliary atresia is the most severe form of cholestatic liver disease. The children have high morbidity and mortality and get devastating pruritus and fatigue, failure to thrive, progressive hepatic failure and impaired neurodevelopment. The etiology is mostly unknown. More than half need a new liver from a living or deceased donor during childhood. However, correct timing of the transplantation is extremely difficult because of lack of consensus based on clinical assessment tools. All though the incidence is low, the cost of this disease is tremendous from both a clinical and human perspective. So far, protocolized neurodevelopment tests, genetic profiling, precise malnutrition evaluation based on clinical appearance, biochemical markers and brain MRI-scans, body composition, immunological function, level of physical activity and optimal time of transplantation in cholestatic children are unknown.
The aim is to determine risk factors for neurocognitive impairment in children suffering from severe cholestasis in order to determine optimal time for liver transplantation from a brain perspective.
In a prospective study, the investigators will investigate risk factors related to brain-, heart-, gut- and immunological function in the Danish cohort. This cohort consists of 75 children aged 0-18 years. In addition, 30 aged and gender matched healthy and 20 tetra fallot children will serve as control groups. The children will undergo extensive and advanced liver function evaluation, genetic profiling, nutrition and immunological status, neuro-imaging and neurocognitive evaluation at time of diagnose, 2 years of age, pre-school, pre-teenage, and teenage. In case of a liver transplantation, additional neuro-cognitive tests will be performed
Eligibility
Sex
ALL
Min age
0 Years
Max age
18 Years
Healthy volunteers
Accepted
Inclusion Criteria:
* Biliary atresia
* Tetralogy of Fallot
* Healthy controls
Exclusion Criteria:
\- Not able to participate in exams
Primary outcome measure(s)
MRI of the brain — Inclusion % of patients with anatomic anomalies on MRI of the brain
Neurocognitive status: Early movement repertoire (General movement) — Inclusion Neurocognitive test panel depending on age at inclusion
% of patients with of abnormal movement assessed using early movement repertoire (GM) if inclusion at diagnosis. Early movement repertoire is a measurement tool where abnormal movement is identified.
Neurocognitive status: Alberta Infant Motor Scale — Inclusion Neurocognitive test panel depending on age at inclusion:
Alberta Infant Motor Scale if inclusion at diagnosis
Percentile, highest is the best
Neurocognitive status: Bayley Scales of Development III — Inclusion Neurocognitive test panel depending on age at inclusion:
Bayley Scales of Development III if inclusion up to 2.5 years:
From 0-200, highest is best
Neurocognitive status: WIPPSI — Inclusion Neurocognitive test panel depending on age at inclusion:
WIPPSI if inclusion between 2.5-6 years:
Wechsler Preschool and Primary Scale of Intelligence, from 41 to 160, highest is best
Neurocognitive status: ABC Movement — Inclusion Neurocognitive test panel depending on age at inclusion:
ABC Movement if inclusion between 2.5-16 years
Movement Assessment Battery for Children, mean 10 SD 3, highest is best
Neurocognitive status: WISC-IV — Inclusion Neurocognitive test panel depending on age at inclusion:
WISC-IV if inclusion between 6-16 years
Wechsler Intelligence Scale for Children, 40 to 160, highest is best
Neurocognitive status: Auditory Verbal Learning Test/ToMaL — Inclusion Neurocognitive test panel depending on age at inclusion:
Auditory Verbal Learning Test/ToMaL if inclusion between 6-18 years Mean 10 SD 3, highest is best
Neurocognitive status: TEA-Ch — Inclusion Neurocognitive test panel depending on age at inclusion:
TEA-Ch if inclusion between 6-18 years
Test of Everyday Attention for Children, normalized to z-score, highest is best
Neurocognitive status: BADS-C — Inclusion Neurocognitive test panel depending on age at inclusion:
BADS-C if inclusion between 6-18 years Behavioural Assessment of the Dysexecutive Syndrome in Children, 0-24, mean 10 SD 3, highest is best
Neurocognitive status: Test of Visual Perceptual Skills — Inclusion Neurocognitive test panel depending on age at inclusion:
Test of Visual Perceptual Skills if inclusion between 6-18 years
Percentile, highest is best
Neurocognitive status: CANTAB — Inclusion Neurocognitive test panel depending on age at inclusion:
CANTAB if inclusion between 6-18 years Cambridge Neuropsychological Test Automated Battery
Neurocognitive status: The Beery Visuo-Motor Integration test — Inclusion Neurocognitive test panel depending on age at inclusion:
The Beery Visuo-Motor Integration test if inclusion between 6-18 years Mean of 100 and standard deviation of 15, highest is best
Neurocognitive status: WAIS IV — Inclusion Neurocognitive test panel depending on age at inclusion:
WAIS IV if inclusion from 16 to 18 years Wechsler Adult Intelligence Scale, 40-160, highest is best
Neurocognitive status: Kiddie-sads — Inclusion Neurocognitive test panel depending on age at inclusion:
Kiddie-sads if included between 2-18 years Kiddie Schedule for Affective Disorders and Schizophrenia, 0-61, lowest is best
Neurocognitive status: BRIEF 1 — Inclusion Neurocognitive test panel depending on age at inclusion:
BRIEF 1 if inclusion up to 6 years Behaviour Rating Inventory of Executive Function, percentile, lowest is best
Neurocognitive status: BRIEF 2 — Inclusion Neurocognitive test panel depending on age at inclusion:
BRIEF 2 if inclusion between 6-18 years Behaviour Rating Inventory of Executive Function, percentile, lowest is best
Neurocognitive status: CBCL — Inclusion Neurocognitive test panel depending on age at inclusion:
CBCL if included between 2-18 years Child Behavior Checklist, percentile, lowest is best
Neurocognitive status: ADHD screening — Inclusion Neurocognitive test panel depending on age at inclusion:
ADHD screening if included between 2-18 years Lowest is best, 0-78
Neurocognitive status: SRS-2 — Inclusion Neurocognitive test panel depending on age at inclusion:
SRS-2 screening if included between 6-18 years
Social Responsiveness Scale, 32-114. lowest is best
Neurocognitive status: Vineland — Inclusion Neurocognitive test panel depending on age at inclusion:
Vineland screening if included between 6-18 years Vineland Adaptive Behavior Scales, 20 to 160, highest is best
MRI of the brain — 1 year % of patients with anatomic anomalies on MRI of the brain
Neurocognitive status: Alberta Infant Motor Scale — 1 year Alberta Infant Motor Scale Percentile, highest is the best
Neurocognitive status: Bayley Scales of Development III — 1 year Bayley Scales of Development III
From 0-200, highest is best
Neurocognitive status: BRIEF 1 — 1 year BRIEF 1 Behaviour Rating Inventory of Executive Function, percentile, lowest is best
Neurocognitive status: CBCL — 1 year CBCL Child Behavior Checklist, percentile, lowest is best
Neurocognitive status: Kiddie-Sads — 1 year Kiddie-Sads Kiddie Schedule for Affective Disorders and Schizophrenia, 0-61, lowest is best
MRI of the brain — 2 years % of patients with anatomic anomalies on MRI of the brain
Neurocognitive status: Bayley Scales of Development III — 2 years Bayley Scales of Development III From 0-200, highest is best
Neurocognitive status: BRIEF 1 — 2 years BRIEF 1 Behaviour Rating Inventory of Executive Function, percentile, lowest is best
Neurocognitive status: CBCL — 2 years CBCL Child Behavior Checklist, percentile, lowest is best
Neurocognitive status: Kiddie-Sads — 2 years Kiddie-Sads Kiddie Schedule for Affective Disorders and Schizophrenia, 0-61, lowest is best
Neurocognitive status: ADHD — 2 years ADHD Lowest is best, 0-78
Neurocognitive status: Vineland — 2 years Vineland Adaptive Behavior Scales, 20 to 160, highest is best
MRI of the brain — 6 years % of patients with anatomic anomalies on MRI of the brain
Neurocognitive: Movement ABC — 6 years Neurocognitive test panel depending on age Movement Assessment Battery for Children, mean 10 SD 3, highest is best
Neurocognitive status: WISC-IV — 6 years WISC-IV
Wechsler Intelligence Scale for Children, 40 to 160, highest is best
Neurocognitive status: Auditory Verbal Learning Test/ToMaL — 6 years Auditory Verbal Learning Test/ToMaL Mean 10 SD 3, highest is best
Neurocognitive status: TEA-Ch — 6 years TEA-Ch Test of Everyday Attention for Children, normalized to z-score, highest is best
Neurocognitive status: BADS-C — 6 years BADS-C Behavioural Assessment of the Dysexecutive Syndrome in Children, 0-24, mean 10 SD 3, highest is best
Neurocognitive status: Test of Visual Perceptual Skills — 6 years Test of Visual Perceptual Skills Percentile, highest is best
Neurocognitive status: The Beery Visuo-Motor Integration test — 6 years The Beery Visuo-Motor Integration test Mean of 100 and standard deviation of 15, highest is best
Neurocognitive status: CANTAB — 6 years CANTAB Cambridge Neuropsychological Test Automated Battery
Neurocognitive status: BRIEF 2 — 6 years BRIEF 2 Behaviour Rating Inventory of Executive Function, percentile, lowest is best
Neurocognitive status: ADHD screening — 6 years ADHD screening Lowest is best, 0-78
Neurocognitive status: SRS-2 — 6 years SRS-2 Social Responsiveness Scale, 32-114. lowest is best
Neurocognitive status: Kiddie-Sads — 6 years Kiddie-Sads Kiddie Schedule for Affective Disorders and Schizophrenia, 0-61, lowest is best
Neurocognitive status: CBCL — 6 years CBCL Child Behavior Checklist, percentile, lowest is best
Neurocognitive status: Vineland — 6 years Vineland Vineland Adaptive Behavior Scales, 20 to 160, highest is best
MRI of the brain — 11 years % of patients with anatomic anomalies on MRI of the brain
Neurocognitive status: Movement ABC — 11 years Neurocognitive test panel depending on age at inclusion:
ABC Movement if inclusion between 2.5-16 years
Movement Assessment Battery for Children, mean 10 SD 3, highest is best
Neurocognitive status: WISC-IV — 11 years WISC-IV
Wechsler Intelligence Scale for Children, 40 to 160, highest is best
Neurocognitive status: Auditory Verbal Learning Test/ToMaL — 11 years Auditory Verbal Learning Test/ToMaL Mean 10 SD 3, highest is best
Neurocognitive status:TEA-Ch — 11 years TEA-Ch Test of Everyday Attention for Children, normalized to z-score, highest is best
Neurocognitive status: BADS-C — 11 years BADS-C Behavioural Assessment of the Dysexecutive Syndrome in Children, 0-24, mean 10 SD 3, highest is best
Neurocognitive status: Test of Visual Perceptual Skills — 11 years Test of Visual Perceptual Skills Percentile, highest is best
Neurocognitive status: The Beery Visuo-Motor Integration test — 11 years The Beery Visuo-Motor Integration test Mean of 100 and standard deviation of 15, highest is best
Neurocognitive status: CANTAB — 11 years CANTAB Cambridge Neuropsychological Test Automated Battery
Neurocognitive status: BRIEF 2 — 11 years BRIEF 2 Behaviour Rating Inventory of Executive Function, percentile, lowest is best
Neurocognitive status: ADHD screening — 11 years ADHD screening Lowest is best, 0-78
Neurocognitive status: SRS-2 — 11 years SRS-2 Social Responsiveness Scale, 32-114. lowest is best
Neurocognitive status: Kiddie-Sads — 11 years Kiddie-Sads Kiddie Schedule for Affective Disorders and Schizophrenia, 0-61, lowest is best
Neurocognitive status: CBCL — 11 years CBCL Child Behavior Checklist, percentile, lowest is best
Neurocognitive status: Vineland — 11 years Vineland Adaptive Behavior Scales, 20 to 160, highest is best
MRI of the brain — 16 years % of patients with anatomic anomalies on MRI of the brain
Neurocognitive status: Movement ABC — 16 years Neurocognitive test panel depending on age at inclusion:
ABC Movement if inclusion between 2.5-16 years
Movement Assessment Battery for Children, mean 10 SD 3, highest is best
Neurocognitive status: WISC-IV — 16 years WISC-IV Wechsler Adult Intelligence Scale, 40-160, highest is best
Neurocognitive status: Auditory Verbal Learning Test/ToMaL — 16 years Auditory Verbal Learning Test/ToMaL Mean 10 SD 3, highest is best
Neurocognitive status: TEA-Ch — 16 years TEA-Ch Test of Everyday Attention for Children, normalized to z-score, highest is best
Neurocognitive status: BADS-C — 16 years BADS-C Behavioural Assessment of the Dysexecutive Syndrome in Children, 0-24, mean 10 SD 3, highest is best
Neurocognitive status: Test of Visual Perceptual Skills — 16 years Test of Visual Perceptual Skills Percentile, highest is best
Neurocognitive status: The Beery Visuo-Motor Integration test — 16 years The Beery Visuo-Motor Integration test Mean of 100 and standard deviation of 15, highest is best
Neurocognitive status: CANTAB — 16 years CANTAB Cambridge Neuropsychological Test Automated Battery
Neurocognitive status: BRIEF 2 — 16 years BRIEF 2 Behaviour Rating Inventory of Executive Function, percentile, lowest is best
Neurocognitive status: ADHD screening — 16 years ADHD screening Lowest is best, 0-78
Neurocognitive status: SRS-2 — 16 years SRS-2 Social Responsiveness Scale, 32-114. lowest is best
Neurocognitive status: Kiddie-Sads — 16 years Kiddie-Sads Kiddie Schedule for Affective Disorders and Schizophrenia, 0-61, lowest is best
Neurocognitive status: CBCL — 16 years CBCL Child Behavior Checklist, percentile, lowest is best
Neurocognitive status: Vineland — 16 years Vineland Adaptive Behavior Scales, 20 to 160, highest is best
Trial sites (1)
Facility
City
Region
Status
Rigshospitalet
Copenhagen
Denmark
Recruiting
More Copenhagen University Hospital, Rigshospitalet trials in Denmark
This page summarises publicly available registry data for informational purposes — not medical advice. Eligibility is determined by each study team; patients should discuss participation with their clinician.
We use cookies to analyse site traffic and improve your experience. With your consent, we may also use cookies for advertising. You can change your choice at any time.
