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Development and disability

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A. L. Curran, H. E. Miller, R. J. McCarter, L. Hunt, A. Brownsdon, P. M. Sharples, and the Kids Head Injury Study Group. Frenchay Hospital, Bristol

Introduction: Traumatic brain injury (TBI) is a major cause of hospital admission in childhood yet few published data exist concerning health related quality of life (HRQL) after TBI or the factors which determine HRQL.

Aims: (1) To measure health related quality of life (HRQL) in TBI children admitted to hospital 1 and 6 months after injury; (2) to compare HRQL in TBI children to that in uninjured controls; and (3) to explore the factors related to HRQL.

Methods: Prospective cohort study. TBI was classed by Glasgow Coma Score (GCS) as severe (GCS 3–8), moderate (GCS 9–12), or mild (GCS 13–15). HRQL was assessed by Pediatric Quality of Life Scale (PedsQL). Cognitive response was assessed by the WISC–III. Psychological response was assessed by Birleson Depression Scale (BDS), Impact of Events Scale (IES), Revised Children’s Manifest Anxiety Scale (RCMAS), and Child Behaviour Checklist (CBCL). Maternal depression was assessed by Beck Depression Inventory II (BDI-II).

Results: 88 TBI subjects (51 severe/moderate; 37 mild) and 47 controls were recruited. At 1 and 6 months after TBI there was a significant difference for all domains of PedsQL between severe/moderate TBI and controls (physical p<0.001, p = 0.001; emotional p = 0.003, p = 0.003; school p<0.001, p<0.001; social p<0.001, p = 0.001) but not between mild TBI and controls. There was significant correlation between all domains of PedsQL and maternal depressive symptomatology 1 and 6 months after TBI (p<0.001; p = 0.002) and between all domains of PedsQL and CBCL (physical p = 0.001, p = 0.001; emotional p = 0.001, p<0.001; social p<0.001, p<0.001; school p<0.001, p<0.001). At 1 month there was a significant correlation between physical, social, and school domains and performance IQ (PIQ) (p = 0.005, p = 0.006, p = 0.001), between school domain and verbal IQ (VIQ) (p = 0.005), and between social domain and BDS (p = 0.002). At 6 months there was a significant correlation between school domain and PIQ (p = 0.04) and VIQ (p = 0.03), between physical, emotional, and school domains and BDS (p = 0.04; p = 0.002, p = 0.027), and between the social domain and the IES (p = 0.04).

Conclusion: Severe/moderate TBI produces a significant reduction in HRQL which persists for at least 6 months. HRQL is strongly related cognitive function at 1 month after TBI but psychological factors may be more important at 6 months.


Z. Bassi1, R. Watling2, A. M. Dalzell1, G. Lancaster3, L. Rosenbloom1. 1Royal Liverpool Childrens NHS trust Alder Hey, Liverpool, UK; 2Dietetics Department, Royal Liverpool Childrens NHS trust, Liverpool, UK; 3Centre for Medical Statistics and Health Evaluation, University of Liverpool, UK

Background: Feeding difficulty and undernutrition is common in children with neurodisability. Gastrostomy is a useful adjunct in feeding management. There are limited data on the nutrient intake in this patient group.

Aim: To evaluate quality (micronutrient) and quantity (macronutrient) of nutritional intake in children with neurodisability and to relate this to their growth, functional abilities, and route of feeding.

Method: A standardised three day diet diary was completed by carers of all new patients (n = 42) attending a multidisciplinary feeding clinic over six months. Nutrient intake was analysed using the Salford micro-diet system.

Results: 39 (92%) diet diaries were analysed (mean age 26 months). Feeding routes: oral (O)(n = 14), gastrostomy only (G)(n = 7), and combination of gastrostomy and oral (C)(n = 18). Median gross motor function classification system (GMFCS) levels: O:G:C 2:4:4. Median z scores (weight, height): O (−0.6,−1.22), G (−1.27,−3.75), and C (−2.35.−2.53). Mean percentage estimated average requirement (% EAR) intake for calories: O (78.64), G (65.33), and C (85.46). All other macronutrients were adequate to dietary reference values. The mean micronutrients percentage RNI for zinc (O:G:C 98%:146%:150%), vitamin D (O:G:C 58%:111%:111%), selenium (O:G:C 75%:139%:168%), and the intake for biotin, manganese, and panthothenate were lower in the orally fed group.

Conclusion: The level of physical impairment (GMFCS) has an impact on nutritional status and feeding ability. There was significant deficit in the energy intake irrespective of the route of feeding. Micronutrient deficiencies were variable with a trend towards lower intake in the orally fed group. This could relate to the use of nutrient dense feeds or use of low micronutrient content foods.


A. O’Hare, W. Cohen, A. Hodson, J. Boyle, T. Durani, E. McCartney, M. Mattey, L. Naftalin, J. Watson. Department of Child, Life and Health, University of Edinburgh, University of Strathclyde

Aims: To study the effect of exposure to FFW games on the language levels of children with receptive specific language impairment through the design of a randomised controlled trial with blind assessment of outcome.

Methods: Seventy seven children between the ages of 6 and 10 years, with specific receptive language impairment (SLI), participated in a randomised controlled trial of Fast ForWord Language™ (FFW), a computer based intervention using acoustically modified speech. All children maintained their regular speech and language therapy and school regime throughout the trial. Standardised measures of receptive and expressive language were used to assess performance at baseline and to measure outcome at 9 weeks and 6 months. Children were allocated to one of three groups. Group A (n = 23) received the FFW intervention as a home based therapy for 6 weeks. Group B (n = 27) received commercially available computer based activities designed to promote language in order to control for computer games exposure. Group C (n =  27) received no additional study intervention.

Results: Multivariate analysis of variance (MANOVA) was used to determine the effects of group, data collection point (baseline v 9 week v 6 months) and the group by data collection point interaction which, in the light of the equivalence of the pre-treatment scores, provides a measure of any post-baseline between group differences resulting from intervention. There were no significant main effects of group for the primary outcome measures of receptive language scores (F(2, 71) 0.007, p 0.993), expressive language scores (F(2, 70) 0.207, p 0.814), or total language scores (F(2, 70) 0.261, p 0.771) on the CELF 3UK. Similarly, there were no significant main effects of group for the other secondary outcome measures (p>0.085).

Conclusions: Groups made significant gains in language levels but there was no additional effect for either computer intervention. The data failed to provide support for the temporal processing deficit account of receptive SLI.


J. R. Parr1, G. Baird2, A. Le Couteur3, M. Rutter1, A. J. Bailey1, and The International Molecular Genetic Study of Autism Consortium (IMGSAC). 1Centre for Social, Genetic and Developmental Psychiatry, London, UK; 2Newcomen Centre, Guy’s Hospital, UK; 3Department of Child and Adolescent Psychiatry, University of Newcastle, UK

Aims and Methods: Some individuals with autism lose skills in the first few years of life, most commonly language, and an environmental aetiology has been suggested. In order to evaluate the characteristics of regression in a multiplex sample, IMGSAC recruited 486 individuals with a pervasive developmental disorder from 239 multiplex families. Language regression was identified using data from the Autism Diagnostic Interview (ADI), and ADI domain scores, Vineland Adaptive Behaviour Scale domain scores, performance, and verbal IQ were compared according to regression status.

Results: The incidence of language regression using strict criteria (loss of 5 or more words used with meaning) was 15%, rising to 24% when loss of less complex language was included. Individuals who lost meaningful language acquired first words earlier (21 months) than non-regressors (34 months) (p<0.001). Concordance for language regression occurred in 15 pairs; 85 pairs were discordant (not significant). Cases who regressed had significantly higher mean ADI scores, lower mean Vineland domain scores and lower mean IQ scores than controls. Some 16% of individuals who lost language had not regained their previous skills and these individuals had significantly lower Vineland Domain scores and IQ scores than individuals who lost and then regained language.

Conclusions: The incidence of language regression in this multiplex sample broadly in line with the rate in autistic singletons and autistic regression most commonly affected only one individual in an affected pair. The findings suggest that regression is an intrinsic but emergent feature of genetic susceptibility and indexes more severe phenotypic expression.


K. Robertshaw, L. Watson, T. Parkin, R. E. Morton. Derbyshire Children’s Hospital, Derby

Introduction: Large doses of botulinum are often needed to treat children with cerebral palsy (CP). This may cause side effects by systemic spread, for which it would be useful to have an objective test. We have previously investigated simple tests of autonomic dysfunction after treatment with botulinum toxin A (BT-A) which were unhelpful. This study investigated changes in overnight cardiac variability with BT-A treatment.

Method: 24 hour ECGs were performed on 10 control subjects on two occasions 14–21 days apart and analysed for three measures of cardiac variability, over the full 24 hours and for a shortened 6 hour overnight period. To reflect the variability of these measures over time, results were expressed as the second measurement divided by the first; the autonomic ratio (AR). The most acceptable measure of cardiac variability was determined and used to calculate AR for the rest of the study. Thirty six subjects were investigated with 6 hour overnight ECGs before and 14–21 days after treatment with BT-A (Dysport, 17–40 units/kg) to determine the AR, that is the change cardiac variability after the injections. These results were compared with the controls and correlated with the amount of toxin given.

Results: The best measure of cardiac variability for a 6 hour recording in controls and used to calculate AR was the standard deviation score (SD). The mean AR for those treated with BT-A for controls was 1.14 (95% CI 1.04 to 1.24), and treated subjects was 0.85 (95% CI 0.76 to 0.94) (p = 0.0002). The correlation coefficient for total dose BT-A/kg and AR was −0.28. Clinical side effects were present in one child in the form of constipation, who received 37units/kg BT-A. This was reflected by an AR of 0.33, the lowest recorded.

Conclusion: Autonomic side effects of treatment with BT-A can be detected with an overnight 6 hour record of cardiac variability. This provides a measure of systemic spread of BT-A, which may be useful for monitoring treatment with high doses.


S. Warriner1, M. T. Gibson1, V. Ramesh2. 1Child Development Centre, Royal Victoria Infirmary; 2Newcastle General Hospital, Newcastle upon Tyne

Background: Hypoxic ischaemic encephalopathy (HIE) is the most common neurological problem in term newborns and cerebral palsy the commonest adverse neurological outcome. Types of cerebral palsy syndromes in following term perinatal HIE are well described and include dyskinetic and bilateral spastic syndromes. Such disorders have wide ranging implications for the patient and family as well as medicolegally.

Presentation: We present three children of primary school age (two female and one male). Following moderate or severe HIE at term they have presented with postnatal seizures, learning difficulties, and visual impairments, in particular a defect of inferior visual fields. All have shown a fall off in head circumference to below the 2nd centile and none of the children have clinical signs of cerebral palsy. All children have had moderate or severe perinatal HIE following delivery at term. Magnetic resonance imaging was not performed neonatally but later studies have shown features compatible with hypoxia and ischaemia in the region of the posterior circulation without any basal ganglia involvement.

Conclusions: These three children highlight a non motor clinical syndrome characterised by learning difficulties, epilepsy, and visual disorders following perinatal HIE at term. These observations have important implications for counselling families and for the ongoing assessment and educational advice for the children. They may also have a medicolegal significance.


J. Murphy1, M. Philip1, A. Walsh2, D. McShane3, M. O’Regan4, E. Roche1, H. M. C. V. Hoey1. 1Department of Paediatrics, University of Dublin, Trinity College and the National Children’s Hospital, AMNCH, Tallaght, Dublin 24; 2Department of Audiology, The National Children’s Hospital, AMNCH, Tallaght, Dublin 24; 3ENT Department, The National Children’s Hospital, AMNCH, Tallaght, Dublin 24; 4Department of Statistics, University of Dublin, Trinity College, Dublin 2

Hearing impairment is one of the most prevalent disabilities in children and adolescents with Down’s syndrome (DS) and is challenging to identify and manage. Current hearing screening may be unreliable in these children due to limited cooperation and ability to respond at an appropriately early age as a result of associated intellectual disability. Early identification of hearing impairment and effective management is essential to prevent unnecessary secondary handicap.

Aims: This study was undertaken to assess: 1) the feasibility of screening hearing function using three screening tools; otoscopy to detect outer ear abnormalities, tympanometry for middle ear function, and distortion product oto acoustic emissions (DPOAEs) for cochlear (inner ear) function; 2) the prevalence of impaired hearing in children and adolescents with DS in the Eastern Health Board; and 3) the age when hearing first screened.

Methods: A cross sectional study of 394 children and adolescents (217 boys and 177 girls) aged from 3 months to 18.9 years (mean age 9.3 years) was undertaken. Clinical examination was performed and demographic data were recorded by questionnaire. Hearing screening was performed using otoscopy, tympanometry, and DPOAEs. This screening was conducted in 11 venues or at the child’s home as preferred.

Results: This study found 75% of children had hearing abnormalities. These included abnormal findings in the outer ear on otoscopy (64.4%), in the middle ear by tympanometry (58%), and in the inner ear by DPOAE (10.7%). Parents reported that children had their first hearing screening at the median age of 1.5 years (range 2 months to 14.5 years).

Conclusion: The use of this combined three tier screening for assessing hearing function is feasible, rapid, and non-invasive, which was acceptable and well tolerated by this group. Hearing screening in this manner could be performed from infancy.

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