session b: developmental pediatrics

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Session B: Developmental Pediatrics B:1 Prevalence of metabolic acidosis following enteroplasty in myelomeningocele RC AD AMS MD , W SNODGRASS MD, B VACHHA MD PHD Texas Scottish Rite Hospital for Children, Dallas; University of Texas Southwestern Medical Center, Dallas, Texas, USA Objective: Enteroplasty is a commonly performed surgical procedure for lower urinary tract reconstruction among per- sons with myelomeningocele (MMC). Current literature is mixed in regard to the risk for metabolic acidosis after enteroplasty in MMC. The object of this study was to describe the emergence of new onset metabolic acidosis in a group of children who had undergone ileal or colonic enteroplasty, serving as their own controls. Design: Retrospective cohort study with participants serving as their own controls for pre/post intervention analysis. Setting: Tertiary university-affiliated, interdisciplinary MMC program. Participants: All patients seen by the urology faculty partici- pating in the Texas Scottish Rite Hospital for Children Spina Bifida Program and who had undergone bladder augmenta- tion enteroplasty were included (n=113) for review. Exclusion criteria were: pre-surgical diagnosis of renal insufficiency; pre-existing metabolic acidosis consistent with renal tubular acidosis (pH<7.35; HCO 3 20); or augmentation procedures using gastric or ureteral tissue. Final analysis included 71 children with ileal or colonic enteroplasty who met inclu- sion/exclusion criteria. Method: Children in the Spina Bifida Program periodically and routinely receive laboratory evaluation of electrolytes; blood urea nitrogen; creatinine; blood count; and venous blood gases including pH, HCO 3 , and PCO 2 . Results of the (multiple) pre-surgical values and (multiple) post-surgical values were evaluated retrospectively. Primary outcome mea- sures were the comparative shifts in blood gases that would suggest the new onset of metabolic acidosis after enteroplas- ty. Changes in electrolytes and serum creatinine were sec- ondary outcome measures to identify potential markers for post-operative effects. With each child as his/her own con- trol, analysis included paired t-tests. Results: No statistically significant difference (p<0.05) was found when comparing pre- and post-bladder augmentation laboratory values including pH, HCO 3 , PCO 2 , and electrolytes. No child developed metabolic acidosis by the above criteria. Follow-up ranged from 1 to 138 months after enteroplasty (mean 46.8). Respiratory compensation was considered in the analysis, and no difference in PCO 2 before and following surgery was noted (p=0.65). Conclusions: No previous study has examined the matched paired results before and after development of metabolic aci- dosis among children with MMC undergoing ileal or colonic enteroplasty. The ‘negative’ statistical results in this controlled cohort are, nevertheless, of clinical importance. These results suggest that if a child with MMC develops metabolic acidosis after ileal or colonic enteroplasty, other clinical reasons in addi- tion to the effects of surgery warrant careful consideration. B:2 Factors associated with bullying of students with special needs K BORRUP MP A JD A , B DRAHEIM APRN B a Injury Prevention Center, Connecticut; b Special Kids’ Support Center at Connecticut Children’s Medical Center, Hartford, CT, USA Objectives: Bullying is pervasive and can have a negative impact on a child’s emotional and physical health. We investi- gated bullying in schools experienced by children with spe- cial needs to discern its prevalence based on the functional nature of a special need, mobility, and/or cognitive need. Design: Convenience sample survey of self-reported school experiences. Setting: Special Kids Support Center, and four special needs clinics. Participants: Two hundred and seventy-one participants of 1530 eligible school-aged special needs children. One hun- dred and fifty-nine child/parent dyads were recruited using a mailed survey to 1199 patients receiving services; and 112 child/parent dyads were recruited through a convenience sample of 331 patients visiting four special needs clinics. Method: There was no significant difference by age of child, or mobility/cognitive needs, between the completed mail and clinic surveys for the 271 participants. Survey data included: age, sex, special help required, diagnosis, and specific bullying behaviors. Data were analyzed using χ 2 analyses. Results: Children requiring special equipment for mobility were least likely to be teased, with 17% reporting being teased at least sometimes. Of children requiring no mobility assis- tance, 36% reported being teased at least sometimes. Of chil- dren with no mobility needs but with trouble learning, teasing and exclusion from play were reported at more than double the rate of children with no problems with learning (Table B:2). Conclusions: Children with special needs who require the use of equipment for mobility (i.e. wheelchair, braces, crutches) are less likely to be teased than other children with special needs who do not use such equipment. This study demon- strates that there are important differences in school-based bullying experiences for children based on the functional nature of their special need. Additional research should be Free Papers – Session B 7 Table B:2 Bullying experience (n [%]) by mobility needs and learning trouble (LT; n=271) Response Teased Excluded Yes help (n=90) 21 (17) 25 (27) No help (n=135) 49 (36) 51 (38) p 0.025 (ns) No help, No LT (n=65) 15 (23) 14 (22) No help, Yes LT (n=67) 33 (49) 37 (55) p 0.01 0.001 ‘Help’= assistance with mobility (braces, wheelchair, etc.).

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Session B: DevelopmentalPediatrics

B:1Prevalence of metabolic acidosis followingenteroplasty in myelomeningoceleR C ADAMS MD, W SNODGRASS MD, B VACHHA MD PHD

Texas Scottish Rite Hospital for Children, Dallas; University

of Texas Southwestern Medical Center, Dallas, Texas, USA

Objective: Enteroplasty is a commonly performed surgicalprocedure for lower urinary tract reconstruction among per-sons with myelomeningocele (MMC). Current literature ismixed in regard to the risk for metabolic acidosis afterenteroplasty in MMC. The object of this study was to describethe emergence of new onset metabolic acidosis in a group ofchildren who had undergone ileal or colonic enteroplasty,serving as their own controls. Design: Retrospective cohort study with participants serving astheir own controls for pre/post intervention analysis. Setting: Tertiary university-affiliated, interdisciplinary MMCprogram.Participants: All patients seen by the urology faculty partici-pating in the Texas Scottish Rite Hospital for Children SpinaBifida Program and who had undergone bladder augmenta-tion enteroplasty were included (n=113) for review. Exclusioncriteria were: pre-surgical diagnosis of renal insufficiency;pre-existing metabolic acidosis consistent with renal tubularacidosis (pH<7.35; HCO3≤20); or augmentation proceduresusing gastric or ureteral tissue. Final analysis included 71children with ileal or colonic enteroplasty who met inclu-sion/exclusion criteria. Method: Children in the Spina Bifida Program periodicallyand routinely receive laboratory evaluation of electrolytes;blood urea nitrogen; creatinine; blood count; and venousblood gases including pH, HCO3, and PCO2. Results of the(multiple) pre-surgical values and (multiple) post-surgicalvalues were evaluated retrospectively. Primary outcome mea-sures were the comparative shifts in blood gases that wouldsuggest the new onset of metabolic acidosis after enteroplas-ty. Changes in electrolytes and serum creatinine were sec-ondary outcome measures to identify potential markers forpost-operative effects. With each child as his/her own con-trol, analysis included paired t-tests.Results: No statistically significant difference (p<0.05) wasfound when comparing pre- and post-bladder augmentationlaboratory values including pH, HCO3, PCO2, and electrolytes.No child developed metabolic acidosis by the above criteria.Follow-up ranged from 1 to 138 months after enteroplasty(mean 46.8). Respiratory compensation was considered inthe analysis, and no difference in PCO2 before and followingsurgery was noted (p=0.65). Conclusions: No previous study has examined the matchedpaired results before and after development of metabolic aci-dosis among children with MMC undergoing ileal or colonicenteroplasty. The ‘negative’ statistical results in this controlledcohort are, nevertheless, of clinical importance. These resultssuggest that if a child with MMC develops metabolic acidosis

after ileal or colonic enteroplasty, other clinical reasons in addi-tion to the effects of surgery warrant careful consideration.

B:2Factors associated with bullying of studentswith special needsK BORRUP MPA JDA, B DRAHEIM APRNB

aInjury Prevention Center, Connecticut; bSpecial Kids’

Support Center at Connecticut Children’s Medical Center,

Hartford, CT, USA

Objectives: Bullying is pervasive and can have a negativeimpact on a child’s emotional and physical health. We investi-gated bullying in schools experienced by children with spe-cial needs to discern its prevalence based on the functionalnature of a special need, mobility, and/or cognitive need.Design: Convenience sample survey of self-reported schoolexperiences.Setting: Special Kids Support Center, and four special needsclinics.Participants: Two hundred and seventy-one participants of1530 eligible school-aged special needs children. One hun-dred and fifty-nine child/parent dyads were recruited using amailed survey to 1199 patients receiving services; and 112child/parent dyads were recruited through a conveniencesample of 331 patients visiting four special needs clinics. Method: There was no significant difference by age of child,or mobility/cognitive needs, between the completed mail andclinic surveys for the 271 participants. Survey data included:age, sex, special help required, diagnosis, and specific bullyingbehaviors. Data were analyzed using χ2 analyses.Results: Children requiring special equipment for mobilitywere least likely to be teased, with 17% reporting being teasedat least sometimes. Of children requiring no mobility assis-tance, 36% reported being teased at least sometimes. Of chil-dren with no mobility needs but with trouble learning, teasingand exclusion from play were reported at more than doublethe rate of children with no problems with learning (Table B:2).Conclusions: Children with special needs who require theuse of equipment for mobility (i.e. wheelchair, braces, crutches)are less likely to be teased than other children with specialneeds who do not use such equipment. This study demon-strates that there are important differences in school-basedbullying experiences for children based on the functionalnature of their special need. Additional research should be

Free Papers – Session B 7

Table B:2 Bullying experience (n [%]) by mobility needs andlearning trouble (LT; n=271)

Response Teased Excluded

Yes help (n=90) 21 (17) 25 (27)No help (n=135) 49 (36) 51 (38) p ≤0.025 (ns)

No help, No LT (n=65) 15 (23) 14 (22)No help, Yes LT (n=67) 33 (49) 37 (55)p ≤0.01 ≤0.001

‘Help’= assistance with mobility (braces, wheelchair, etc.).

undertaken using a larger study design to look at condition-specific effects.

B:3Increased neurodevelopmental delays inpreterm multiples with birthweight less than1500gJ A MICHALEC MD, C RITTENBERG MD, M EBELING MD, M M MACIAS MD, T C HULSEY MSPH SCD, L D KATIKANENI MD

Medical University of South Carolina, Charleston, SC, USA

Background: Multiple gestations are known to be at increasedrisk for neurodevelopmental delay (NDD). Perinatal factorsthat contribute to these delays have yet to be determined.Design: Retrospective analysis of a computerized databasefor neonatal intensive care unit stay and from a high-riskdevelopmental follow-up clinic over a 9-year period.Setting: Tertiary care medical center.Participants: Seven hundred and thirty-three infants, birth-weight <1500g.Method: Participants included 170 twins/triplets (23.19%)and 563 singletons (76.81%). Pertinent perinatal variableswere compared between the groups. Neurodevelopment wasassessed at 12 months adjusted age in visual-motor problemsolving, language, and gross motor domains using the CaputeScales and gross motor assessment. Developmental quotient<80 in one or more areas was considered NDD.Results: Groups were compared using χ2, non-parametricanalysis, and regression analysis. Specific variables separatingmultiples from singletons were identified; p<0.05 was consid-ered significant (Table B:3). Multiples had increased NDD withadjusted developmental quotient (ADQ) <80 in one or moreareas (30.61% vs 22.7%; p=0.037). In infants with ADQ <80 inone or more areas, there was no difference in the Ponderalindex between the multiples and singletons. Monochorionicmultiples had increased incidence of NDD compared withdichorionic multiples (92.3% vs 69.6%; p=0.03). There was nodifference in birthweight or gestational age between mono-and didichorionic multiples. Regression analysis showed that

chorionicity and bacterial sepsis were significant. Conclusion: Twins/triplets with birthweight <1500g have anincreased rate of NDD at 12 months compared with single-tons, in spite of a significant decrease in perinatal risk factors.Among the multiples, monochorionic neonates had inc-reased incidence of NDD compared with dichorionic neo-nates, even though birthweights and gestational ages weresimilar. Parental counseling and early intervention mayimprove the NDD of these infants.

B:4School-readiness and functional disabilityafter preterm birth and respiratory distresssyndrome AI PATRIANAKOS MDA, ME MSALL MDA,C, JD MARKS PHD MDA, D HUO PHDB, MD SCHREIBER MDA

aDepartment of Pediatrics; bDepartment of Health Sciences,

University of Chicago; cSection of Developmental

Pediatrics, Comer and LaRabida Children’s Hospitals,

Chicago, IL, USA

Background: It is not known what factors adversely affectschool-readiness in recent cohorts of high-risk, preterm chil-dren surviving respiratory distress syndrome. Objective: To assess risk factors for delayed school-readinessin children who survived very low and extremely low birth-weight status. Design: Prospective follow-up study.Participants: Preterm infants enrolled in a previous study forthe management of respiratory distress syndrome.1

Method: At a mean age of 5 years 8 months [SD 1y] theparticipants underwent standardized neurodevelopmentalassessments and socioeconomic status (SES) classification(Hollingshead Index of Social Position). School-readiness wasdetermined using neurodevelopmental assessments of basicconcepts (Bracken School-Readiness), perceptual skills (BeeryVisual-Motor Integration Test), receptive vocabulary (PeabodyPicture Vocabulary Test, 3rd edn), daily living functional skills(WeeFIM), and need for special education services requiringmodified classroom curricula and rehabilitation supports.Proportional odds models were used to identify those risk fac-tors predicting delays in school-readiness. Results: Of the children examined (n=135; 81% of survivors),birthweight was 1016g [SD 391] with a gestational age of27.5 weeks [SD 2.6]. Ninety-one children (68%) were school-ready and 114 (86%) had normal functional skills (WeeFIM>79%). Self-care limitations were present in 18% and mobilitylimitations in 8.3%. Male sex (odds ration [OR] 2.19; 95% confi-dence interval [CI] 1.15–4.21; p=0.02), and African-Americanrace (OR 2.29; CI 1.08–4.867; p=0.03) increased the risk fordelays in school-readiness as did bronchopulmonary dysplasia(OR 2.53; CI 1.32–4.85; p=0.005) and severe intraventricularhemorrhage (IVH) or periventricular leukomalacia (PVL; OR2.61; CI 1.15–5.94; p=0.02). However, the most significantpredictors for school-readiness delays were low SES (OR 3.42;CI 1.64–7.14; p=0.001) and being in foster care (OR 4.25; CI1.14–15.8; p=0.03). Each 100g increase in birthweightdecreased the risk for delays in school-readiness (OR 0.90 per100g increments; CI 0.81–0.99; p=0.03). Conclusion: Although many neonatal intensive care gradu-

8 AACPDM Abstracts 2007

Table B:3 Significant differences between multiple andsingleton groups

Variable Multiples Singletons p

Number followed, n (%) 170 (23.19) 533 (76.81) –NDD, % 30.6 22.7 0.03Birthweight, g 1100 1000 0.027Gestational age, wks 29 28 0.009Apgar at 1min 5.5 5 0.03Apgar at 5min 8 7 0.04Birth head circumference, cm 27.0 26.0 <0.0001African-American, % 43.5 60.57 <0.001Bacterial sepsis, % 15.8 19.4 0.05Fungal sepsis, % 4.12 9.06 0.0365Pre-eclampsia, % 17.65 34.46 <0.0001Maternal hypertension, % 5.88 11.9 0.0249Cesarean-section rate 80.59 62.52 <0.0001

NDD, neurodevelopmental delay.

ates achieve school-readiness at an appropriate age, nearlyone-third does not. Treatment targeted to reduce the inci-dence of BPD and severe IVH/PVL will probably improve thisoutcome. In addition, post-discharge resources must be tar-geted towards impoverished and vulnerable children andinclude supports for optimizing developmental and adap-tive skills.

Acknowledgements: Supported in part by a grant from the AmericanAcademy of Pediatrics for residency research (Dr Patrianakos) and aninvestigator initiated research grant from INO Therapeutics (DrSchreiber).

Reference1. Schreiber M, Gin-Mestan K, Marks J, Huo D, Lee G, Srisuparp P.

(2003) Inhaled nitric oxide in premature infants with therespiratory distress syndrome. NEJM 349: 2099–2107.

B:5Predicting abnormal motor outcome in verypreterm infants at 12 months: the role of qualitative MRI and general movementassessmentsA SPITTLEA,B,E, R BOYDA,B,D, T E INDERA, LW DOYLEA,B,C,E

aMurdoch Childrens Research Institute; bDepartment of

Paediatrics; cDepartment of Obstetrics and Gynaecology

University of Melbourne; dSchool of Physiotherapy, La

Trobe University; eRoyal Women’s Hospital, Melbourne,

Australia

Background: General Movements (GMs) assessments arereported to be more valid than neurological examinationand cranial ultrasound in predicting the long-term outcomeof preterm infants. However, advances in neonatal magneticresonance imaging (MRI) have also improved prediction ofoutcome. Objective: To compare qualitative MRI of brain structure atterm equivalent age and GMs assessments at 1 and 3 months’corrected age in predicting motor outcome at 1 year correct-ed age in very preterm infants. Design: Prospective cohort study.Setting: Two neonatal intensive care units.Participants: Preterm infants born at <30 weeks’ gestationwithout major congenital anomalies.Method: Infants had MRI at term equivalent age (1.5-Tesla GEscanner). White matter abnormality (WMA) was scored as nil-minimal or moderate-severe by a neonatalogist. Standardizedvideotaped recordings of GMs were obtained at 1 and 3months postterm. At 1 month GMs of a writhing characterwere classified as normal or abnormal (poor repertoire,cramped synchronized, or chaotic). At 3 months the GMschanged character to fidgety GMs, classified as normal orabnormal. Motor outcome was assessed at 1 year correctedage using the Alberta Infant Motor Scale (AIMS) with a score≤5th centile classified as abnormal, and the Neuro-SensoryMotor Development Assessment (NSMDA) classified neuro-motor development as normal, minimal, or abnormal. Allassessors were blinded to the results of other assessments.Correlation between GMs, WMA, and the 1-year outcomes(AIMS and NSMDA) were assessed using Spearman’s rankcorrelation coefficient (r).Results: To date, 25 infants have completed the study. At 12months the AIMS categorized 11 (44%) as abnormal; and the

NSMDA categorized 11 (44%) with minimal motor problemsand five (20%) with abnormal motor development. AbnormalAIMS scores were related to the presence of moderate to severeWMA at term and abnormal GMs at 1 month, whereas minimaland abnormal motor problems on NSDMA were associatedwith WMA and GMs at both 1 and 3 months (Table B:5).Conclusion:White matter abnormalities on MRI and GMs assess-ments are both predictive of motor outcome at 12 months.Functional examinations should have an important role inpredicting long-term motor outcome.

B:6Efficacy of applied behavioural interventionfor preschool children with autism oncognitive, behavioural, and languageoutcomes: a systematic review andmeta-analysisM SPRECKLEY PTA, R BOYD PHD MSC PTA,B,C

aThe Royal Children’s Hospital; bMurdoch Childrens

Research Institute; cLaTrobe University, Melbourne,

Australia

Objectives: To assess the effect of applied behavioural inter-vention (ABI) for preschool children with autism spectrumdisorder (ASD) on cognitive, behaviour, and language out-comes compared with standard care. Design: Systematic review and meta-analysis of publishedrandomized trials (RCTs).Setting: Tertiary and community centres.Participants: Three hundred and thirty-nine preschool-agedchildren diagnosed with ASD participated in ABI trials. ABI isa method of teaching appropriate behaviours by breakingtasks down into small discrete steps and using intensivetraining in a systematic way (discrete trial training). Inclusioncriteria were randomized control or comparative trialswhere ABI interventions were delivered to the parents/care-giver and/or child. Exclusion criteria were children withphysical, hearing, and/or vision impairment and pharmaco-logical interventionsMethod: Search strategies followed the guidelines of theDevelopmental, Psychosocial and Learning Problems CochraneReview group. Fifty-nine studies were extracted from thedatabases. Of these, 13 studies met the inclusion criteria. Forqualitative analysis the Physiotherapy Evidence Database(PEDro) Scale of quality assessment was performed by twoindependent raters. Data on the primary outcome cognitive

Free Papers – Session B 9

Table B:5 General movements (GMs) classification and whitematter abnormality (WMA)

Assessment 12mo Spearman’s r p

WMA at term AIMS 0.45 0.023NSMDA 0.55 0.004

GMs at 1mo AIMS 0.49 0.009NSMDA 0.60 0.001

GMs at 3mo AIMS 0.37 0.058NSMDA 0.56 0.003

AIMS, Alberta Infant Motor Scale; NSMDA, Neuro-Sensory MotorDevelopment Assessment.

development (Bayley Scale of Infant Development 2nd edn),behavioural outcomes (Vineland Adaptive Behaviour Scales),and language (Reynell Developmental Language Scale) wereextracted and compared by meta-analysis calculating fixedstandardized mean differences and 95% confidence intervalsusing the Rev Man 4.2 program. Results: Thirteen studies met the inclusion criteria (n=339).Five of these RCTs had strong methodological quality, scor-ing >6 on the PEDro scale (n=139) and were used in themeta-analysis (Table B:6). All the trials were comparativestudies receiving standard care rather than controlled trials,thus explaining why some comparison groups had betteroutcomes than the experimental groups. There is significantvariability with regard to intensity (range 4–39h/wk); dura-tion (3mo–4y); method of delivery (parent vs therapist medi-ated), and setting (home and preschool). Conclusions: ABI is time consuming, of high intensity and,therefore, costly. For children with such significant disabilitythis intervention does not yet offer any conclusive evidenceof additional benefit to cognitive, behavioural, and languageoutcomes over standard care.

B:7Cognitive profile in young Icelandic childrenwith cerebral palsyS SIGURDARDOTTIR MDA, A EIRIKSDOTTIR PSYCHOLOGISTA, E GUNNARSDOTTIR PSYCHOLOGISTA, M MEINTEMA PHYSICAL

THERAPISTA, U ARNADOTTIR PHYSICAL THERAPISTA, T VIK MDB

aState Diagnostic and Counseling Centre, Kopavogur,

Iceland; bNorwegian University of Science and Technology,

Trondheim, Norway

Background: Cognitive impairment is often associated withcerebral palsy (CP). In Iceland, children with CP have a multidis-ciplinary assessment at 4 to 7 years of age at one central insti-tution, The State Diagnostic and Counseling Center (SDCC). Objective: To describe the cognitive profile in a completenational cohort of children with CP.Method: Children with congenital CP, born from 1985 to 2000and evaluated at 4 to 7 years of age were identified in theSDCC’s database. When possible IQ was assessed using theWechsler Preschool and Primary Scales of Intelligence (WPPSI)or a Developmental Index (DI; months of performance/-months of age ×100) was obtained by applying various instru-ments, including the Bayley Scales of Infant Development-II(BSID-II) and the Reynell-Zinkin Developmental Scales (RZ).Results: One hundred and twenty-seven children (67 females,60 males) were tested at a mean age of 5 years 5 months (range

4y–6y 6mo). The WPPSI test was applied in 91 (71.7%) chil-dren. The remaining children were tested using BSID-II(n=17), RZ (n=4), or various combinations of tests (n=15).Children tested with the WPPSI had a mean (SD) Verbal IQ of89 (20), Performance IQ of 78 (20.4,) and a Full-scale IQ of 83(20). Children with hemiplegia had the highest IQ scores(mean total 88 [SD 18]) while children with dyskinetic CP hadthe lowest scores (mean total 60 [SD 20]; p=0.003). Childrenwith bilateral spastic CP had higher Verbal than Performancescores (mean difference: 15 points for diplegia and 22 pointsfor quadriplegia), whereas children with other subtypes didnot have major differences between the two subscores. Twentychildren had an IQ above 100 and 43 had total IQ score above85 (diplegic: 23; hemiplegic: 17; dyskinetic, ataxic, and quadri-plegic: 1 each). A significant inverse correlation was found bet-ween motor function assessed with the Gross Motor FunctionClassification System and IQ (Spearman’s r: –0.44; p<0.01).Mean DI among children without IQ scores was 39 (range3–90), and 79% had a DI below 70 (74% of these children haddyskinetic or quadriplegic forms of CP). Conclusion:Approximately one-third of children with CP, main-ly children with hemiplegia and diplegia, have no intellectualdeficits (IQ scores >85). Cognitive abilities are significantlyassociated with type and severity of motor impairment.

B:8Causes of progressive intellectual andneurological deterioration in UK children:findings of a prospective national study afteralmost 10 years of surveillanceC M VERITY FRCPCHA, L STELLITANOA, A M WINSTONE RGN/RMA, A NICOLL FRCPCHB, R G WILL FRCPC

aAddenbrookes Hospital, Cambridge; bCommunicable

Disease Surveillance Centre, Colindale, London; cNational

Creutzfeldt-Jakob Disease Surveillance Unit, Edinburgh, UK

Objective: To report on children with diagnosed neurode-generative disease in our national study of progressive intel-lectual and neurological deterioration (PIND).Design: Prospective population-based epidemiological study.Method: A monthly surveillance card is sent to all UK consul-tant paediatricians by the British Paediatric Surveillance Unit(BPSU) of the Royal College of Paediatrics and Child Health(RCPCH). Since May 1997 we have used the BPSU surveillancesystem to identify UK children with PIND. Clinical informa-tion about notified cases is obtained by telephone question-naire or hospital visit. The anonymized cases are classified bythe PIND Study Expert Group of paediatric neurologists.

10 AACPDM Abstracts 2007

Table B:6 Meta-analysis of cognitive, behaviour, and language outcomes

Outcome Age range Measured outcomes ABI/control Heterogeneity, MD 95% confidence p

(mo) across five studies (n) % interval

Cognitive 33–94 3 41/35 31.6 0.36 –0.10 to 0.83 0.12Behaviour 33–83 3 40/36 0 0.23 –0.23 to 0.68 0.33Expressive language 33–94 4 55/49 32.4 0.27 –0.12 to 0.66 0.18Receptive language 33–94 4 55/49 5.6 0.21 –0.18 to 0.60 0.29

ABI, applied behavioural intervention; MD, mean difference.

Results: By January 2007, UK paediatricians had identified2176 children who were thought to meet the criteria forPIND. Among them were six with probable or definite variantCreutzfeldt-Jakob Disease (vCJD). There were 933 children withPIND and other confirmed diagnoses and in these childrenthere were 114 known neurodegenerative conditions, whichillustrates the complexity of classifying children with PIND.

In the diagnosed cases, the six most common groups were:neuronal ceroid lipofuscinoses (NCL; n=114), mitochondrialcytopathies (n=111), mucopolysaccharidoses (MPS; n=92),gangliosidoses (n=84), adrenoleukodystrophy (n=57), andRett syndrome (n=51).

Within the groups the most common diagnoses were asfollows. NCL group: late infantile (n=58), juvenile (n=38),infantile (n=17); MPS group: type IIIa (Sanfilippo n=61);mitochondrial cytopathy group: respiratory chain abnormal-ities (n=33); Leigh syndrome (n=24), pyruvate dehydroge-nase deficiency (n=6); neuropathy, ataxia, and retinitis pig-mentosa (n=6); Alpers (n=6); gangliosidosis group: GM2(n=62), GM1 (n=22). As expected the distribution of disor-ders varied according to age.Conclusions: This long-term surveillance study has providedunique epidemiological data about the distribution of neu-rodegenerative diseases in the UK child population. It is reas-suring that we have identified only six children with vCJDafter almost 10 years of surveillance, but there is still concernthat more cases will appear because there have now beenfour adult cases of vCJD associated with blood transfusion.

Fortunately, there are relatively few children with PIND,but there are many underlying causes for PIND and the infor-mation from our large study should be very useful when pae-diatricians are considering the differential diagnosis in childrenwith worsening neurological symptoms and signs.

Acknowledgements: Many thanks to all the UK paediatricians whoreport cases to the PIND Study, to the members of the PIND ExpertGroup, to the British Paediatric Surveillance Unit of the RCPCH, andto the Department of Health for funding the study.

Session C: Orthopaedics

C:1Proximal femoral geometry in cerebral palsy:a population-based cross-sectional studyJ ROBIN MBBSA,B,C, F DOBSON PHDA,B,C, R BAKER PHDA, P SELBER

MDA, H K GRAHAM MDA,B

aHugh Williamson Gait Laboratory, Royal Children’s

Hospital; bThe University of Melbourne, cThe Murdoch

Childrens Research Institute, Melbourne, Australia

Background: Proximal femoral deformity in the transverse(increased femoral neck anteversion [FNA]) and coronal planes(increased neck shaft angle [NSA] or ‘coxa valga’) are commonin children with cerebral palsy (CP), contributing to hip instabili-ty and ambulation difficulties. The prevalence and significanceof these deformities have not been studied in a population-based cohort to our knowledge. Hip instability was exam-ined in these deformities via migration percentage (MP).Design: Large, population-based cross-sectional study.Participants: Children with a confirmed diagnosis of CPborn within a 3-year period were identified from a statewideregister with high levels of ascertainment and accuracy.Inclusion criteria were: FNA measurement by experiencedphysical therapists using a reliable, standardized clinicaltechnique (trochanteric palpation test [TPAT]), good qualityanteroposterior pelvis x-ray, and well documented motortype, topographical distribution, and Gross Motor FunctionClassification System (GMFCS) level.Method: Each child’s unique identifying number was used toobtain clinical (movement disorder, topographical distribu-tion, GMFCS levels, hip rotation range, and FNA) and radio-logical data (NSA and MP) held in either a motion analysislaboratory or hip surveillance database. NSA was measuredfrom an anteroposterior pelvis x-ray with the hips internallyrotated by the FNA amount suggested clinically or during flu-oroscopic screening of the hip with a guide wire in the centreof the femoral neck.Linear regression analyses were performed for FNA, NSA,and MP according to GMFCS.Results: Two hundred and ninety-two children were eligiblefor the study; results are presented in Table C:1. Mean FNAwas increased in all GMFCS levels (p<0.001). The lowestmeasurements were at GMFCS Levels I and II; GMFCS LevelsIII, IV, and V were uniformly high (p<0.001). Neck shaftangle was found to increase sequentially from GMFCS LevelsI to V (p<0.001).

Free Papers – Session C 11

Table C:1 Mean scores (95% confidence interval) of FNA, NSA, and MP by GMFCS level

GMFCS level n Age, y:m (range, y) FNA, degs NSA, degs MP (%)

I 86 10:0 (5–15) 30.4 (27.2–33.2) 135.9 (131.0–137.3) 8.1 (7.2–9.2)II 55 8:2 (5–13) 35.5 (32.3–38.7) 141.0 (134.0–145.7) 13 (10.3–16.0)III 41 8:1 (3–15) 40.5 (37.6–43.1) 148.8 (141.8–154.5) 25 (21.1–28.7)IV 50 8:6 (5–11) 40.1 (37.6–43.1) 155.0 (148.2–160.7) 36.8 (32.2–41.9)V 60 8:4 (4–14) 40.5 (37.6–43.1) 163.0 (156.2–168.0) 46.2 (41.3–52.0)

GMFCS, Gross Motor Function Classification System; FNA, femoral neck anteversion; NSA, neck shaft angle; MP, migration percentage.