A randomised controlled trial of a computerised intervention for children with social communication difficulties to support peer collaboration

An intervention aiming to support children with social communication difficulties was tested using a randomised controlled design. Children aged 5–6 years old (n = 32) were tested and selected for participation on the basis of their scores on the Test of Pragmatic Skills (TPS) and were then randomly assigned to the intervention arm or to the delayed intervention control group. Following previous research which suggested that computer technology may be particularly useful for this group of children, the intervention included a collaborative computer game which the children played with an adult. Subsequently, children's performance as they played the game with a classmate was observed. Micro-analytic observational methods were used to analyse the audio-recorded interaction of the children as they played. Pre- and post-intervention measures comprised the Test of Pragmatic Skills, children's performance on the computer game and verbal communication measures that the children used during the game.This evaluation of the intervention shows promise. At post-test, the children who had received the intervention, by comparison to the control group who had not, showed significant gains in their scores on the Test of Pragmatic Skills (p = .009, effect size r = −.42), a significant improvement in their performance on the computer game (p = .03, r = −.32) and significantly greater use of high-quality questioning during collaboration (p < .001, r = −.60). Furthermore, the children who received the intervention made significantly more positive statements about the game and about their partners (p = .02, r = −.34) suggesting that the intervention increased their confidence and enjoyment.     

Long-term outcome and tolerability of the ketogenic diet in drug-resistant childhood epilepsy—The Austrian experience

PurposeTo evaluate the long-term efficacy/tolerability of the ketogenic diet (KD) in paediatric drug-resistant epilepsies.MethodsData from children who were treated between 1999 and 2008 and had continuous follow-up of at least 6 months after initiation of the KD were analysed retrospectively. Response was defined as ≥50% seizure reduction. Treatment effects on EEG, developmental outcome and the “outcome-predictive” value of various clinical factors were also assessed.Results50 children (22 boys; mean age 4.5 years ± 3.55) were included. Mean follow-up was 3.93 ± 2.95. 50% of the patients were responders, 48% of them became seizure free. 50% were non-responders, 20% of them deteriorated. In responders, EEG background activity improved significantly (p = 0.014) and a significantly lower rate of epileptic discharges (p = 0.009) was seen after 6 months. In addition, neurological examination findings demonstrated significant developmental progress (p = 0.038).Favourable treatment outcome was associated with a shorter disease duration (p = 0.025) and generalised tonic clonic seizures (p = 0.059). No further significant outcome predictors were detected. However, response was 44% in patients with infantile spasms, 62.5% in those with Dravet syndrome and 50% in Lennox-Gastaut-syndrome.Side effects occurred in 28%, but discontinuation of the KD was not required in any case. They most often observed with concomitant topiramate (p = 0.001) and valproate (p = 0.046).ConclusionDespite the retrospective nature of the study and the inhomogeneous patient sample, we found good long-term effects of the KD on seizure frequency, EEG and neurological development.     

Effects of gastrocnemius fascia lengthening on gait pattern in children with cerebral palsy using the Gait Profile Score

The aim of the present study was to investigate the efficacy of the GPS regarding the quantification of changes in gait following the gastrocnemius fascia lengthening in children with CP. Nineteen children with CP were selected and evaluated in the preoperative period (PRE session) and approximately one year postoperatively (POST session; mean 13.1 ± 5.1 months) using 3D gait analysis and computing the GPS and GVSs. As the GPS represents the difference between the patient's data and the average from the reference dataset, the higher the value of GPS is, more compromised gait of the subject. A statistically significant improvement in mean GPS was found in the POST session (PRE: 13.38 ± 5°; POST: 10.26 ± 2.41°; p < 0.05), with an improvement close to 23%. Moreover, the GVSs demonstrated statistically significant improvements in ankle dorsi-plantarflexion (PRE: 22.20 ± 16.36°; POST: 11.50 ± 6.57°; p < 0.05) and pelvic rotation (PRE: 9.53 ± 3.87°; POST: 6.47 ± 2.98°; p < 0.05). A strong correlation (r = 0.75; p < 0.05) was found between the preoperative GPS and the percentage of GPS improvement. The results demonstrated that the gastrocnemius fascia lengthening produced a global gait pattern improvement, as showed by the GPS value, which decreased after surgery. Besides this, the GVS permitted to better evidence the joints more compromised by the pathology and their improvement due to the surgery, in this case not only the GVS of the ankle joint but also of the pelvis were characterized by higher GVS values.     

Spatio-temporal gait characteristics in children with Tourette syndrome: A preliminary study

Earlier studies had suggested that variability of stride length in gait is a pathological sign of basal ganglia disease. Some evidence implicates the involvement of the basal ganglia and related thalamocortical circuitry in Tourette syndrome (TS). To date, the gait of subjects with TS has only discussed in case reports. This investigation compared the spatial and temporal gait characteristics of a sample of children with TS (N = 8) with those of healthy controls (HC; N = 8). All children were instructed to walk under two speed conditions: “preferred” and “fastest.” Gait parameters were measured using an electronic walkway. Spatial and temporal gait parameters were compared using a two-way (group) × (conditions) repeated measures ANOVA. The preliminary results suggested that similar to HC children, children with TS were capable of regulating temporal characteristics of gait based on walking speed. They also exhibited subtle gait anomalies such as irregular step length, as evidenced by significant differences in step length differential (p = 0.003), detectable despite the small sample size. These findings warrant further investigation into the gait control of children with TS.     

Factors associated with survival for patients with glioblastoma with poor pre-operative functional status

Patients with glioblastoma (GB) are known to have poor prognoses, and among these patients, those with poor neurological function have an even poorer prognosis. Consequently, aggressive surgeries and adjuvant therapies are often withheld because of this dismal outlook. The effects of aggressive therapies in this small subset of patients remain unknown. The goal of this study was to evaluate outcomes and factors associated with survival for poor functioning patients who underwent aggressive resection of their GB. Adult patients who underwent surgical resection of an intracranial primary GB at an academic tertiary-care institution between 1997 and 2007 were retrospectively reviewed. Patients with a Karnofsky Performance Scale (KPS) score of ?60 were included. A total of 100 patients with primary GB met the inclusion criteria. The average age (±standard deviation) and KPS score of this cohort were 54 ± 15 years and 53 ± 12, respectively. No patient (0%) experienced perioperative mortality, and 0 (0%), 10 (10%), and 3 (3%) of patients incurred a new or increasing language, motor, and visual deficit, respectively. At last follow-up, 88 (88%) patients died with a median survival of 6.6 months. The factors associated with improved survival were age <65 year (p = 0.005), tumor size >2 cm (p = 0.01), radical tumor resection (p = 0.01), and temozolomide (p = 0.001). This study identifies a subset of patients with poor functional status who may benefit from aggressive surgical resection.     

Outcomes and cost-effectiveness of gamma knife radiosurgery and whole brain radiotherapy for multiple metastatic brain tumors

We aimed to analyze the outcomes and cost-effectiveness of gamma knife radiosurgery (GKRS) and whole brain radiotherapy (WBRT) for multiple metastatic brain tumors. Over a period of 5 years, 156 patients with multiple metastatic brain tumors were enrolled and freely assigned by the referring doctors to either gamma knife radiosurgery (GKRS, Group A, n = 56), or to whole brain radiotherapy (WBRT, Group B, n = 100). The follow-up time was set at 1200 days (3.3 years) post-treatment. The number of tumors, patient age, extent of systemic disease and Karnofsky performance scale (KPS) score, were recorded and recursive partitioning analysis used. The outcomes analyzed were: mortality, survival time, neurological complications, post-treatment KPS score, quality-adjusted life years (QALY), and cost-effectiveness. A paired t-test was used for statistical analysis. Mortality rates for patients receiving GKRS and WBRT were 81.1% and 93.0%, respectively (p = 0.05). The mortality rate was lower for GKRS (74.4%) than for WBRT (97.1%) in patients with initial KPS ? 70 (p = 0.02). The mortality rate was also significantly lower for GKRS (78.9%) than WBRT (95.5%) in patients with 2–5 tumors (p < 0.05). Post-treatment KPS score (mean ± standard deviation [s.d.] was higher for patients receiving GKRS (73.8 ± 13.2) than for those receiving WBRT (45.5 ± 26.0), p < 0.01. The median survival time for GKRS and WBRT was 9.5 months and 8.3 months, respectively, p = 0.72. The mean (± s.d.) QALY was 0.76 ± 0.23 for GKRS and 0.59 ± 0.18 for WBRT, respectively (p < 0.05). The cost-effectiveness per unit of QALY was better for the GKRS treatment (US$10,381/QALY) than in the WBRT treatment (US$17,622/QALY), p < 0.05. The cost-effectiveness per KPS score was also higher for the GKRS treatment (US$139/KPS score) than for WBRT (US$229/KPS score), p < 0.01. Thus, the mortality rate for multiple metastatic brain tumors treated by GKRS is significantly better with a good initial KPS score and when the tumor number is 2–5. GKRS results in a better post-treatment KPS score, QALY, and higher cost-effectiveness than WBRT for treating multiple metastatic brain tumors.     

Auditory event-related potentials at preschool age in children born very preterm

ObjectiveTo assess auditory event-related potentials at preschool age in children born very preterm (VP, 27.4 ± 1.9 gestational weeks, n = 70) with a high risk of cognitive dysfunction.MethodsWe used an oddball paradigm consisting of a standard tone randomly replaced by one of three infrequent deviants (differing in frequency, sound direction or duration).ResultsThe P1 and N2 latencies were inversely correlated to age (50–63 months) both in VP (r = −0.451, p < 0.001, and r = −0.305, p = 0.01, respectively) and term born controls (TC; n = 15). VP children had smaller P1 than near-term (n = 12) or TC (1.70 ± 0.17 μV vs 2.68 ± 0.41 and 2.92 ± 0.43, respectively; p < 0.05). Mismatch negativity response did not differ between groups.ConclusionsOur data suggest a fast maturation of P1 and N2 responses with fast decrease in P1 and N2 latencies around the age of 5 years. Mismatch negativity response does not seem to be a robust measure for defining abnormalities in VP children.SignificanceIn ERP studies in preschool children, even small, non-significant group differences in age at recording should be corrected for. Very preterm born children at preschool age have aERP patterns as earlier described in full-term born children with cognitive deficits.     

Stability and change of cognitive attributes in children with uneven/delayed cognitive development from preschool through childhood

As part of an ongoing clinical service program for children with developmental delay in an Asian developing country, we analyzed the cognitive attributes of 362 Taiwanese children (average age 48.5 ± 12.9 month-old) with uneven/delayed cognitive development as they were assessed repeatedly with average duration of 39.7 ± 22.6 months from preschool through early childhood. The objectives were to determine the stability and related factors in cognitive scores of these 362 children belonging to three diagnostic subgroups: 181 children with non-autistic mental retardation (MR), 95 children with autism spectrum disorder (ASD) and 64 children with mixed type developmental language disorder (DLD); and to contribute to the accumulation of data on cognitive outcome in preschool children with developmental delay. Analysis revealed that mean initial cognitive score (IQ1) was 64.9 ± 16.9 while mean cognitive measure at follow-up (IQ2) was 72.2 ± 19.7. Whole group analysis showed the correlation between IQ1 and IQ2 was moderate (r = 0.73, p < 0.001). Analysis by a general linear model showed only male gender (β = 4.95, p = 0.02, C.I. = 0.8–9.1) and IQ1 (β = 0.79, p < 0.001, C.I. = 0.68–0.90) to be significant predictors of IQ2. There were differences among three groups in IQ1 (p < 0.001), IQ2 (p < 0.001) and IQ change (p < 0.001). Correlation coefficients of IQ1 and IQ2 were 0.6 for ASD group, 0.7 for MR group and 0.4 for DLD group respectively. The greatest proportion of children remained within the same cognitive range for both assessment points, however, it is noted that a substantial minority of children changed IQ ranges drastically from preschool through early childhood. Our results suggest that measurements of cognitive function at preschool age for children with developmental delay were valid in the context of a developing country, and the observed change in cognitive scores during follow-up emphasized the need to interpret the initial results of cognitive tests with caution.     

Severity of depressive symptomatology and functional impairment in children and adolescents with temporal lobe epilepsy

ObjectiveDepression is a frequent psychiatric disorder in children with temporal lobe epilepsy (TLE). However, severity of depressive symptoms (DS) is frequently neglected in these patients. This study aimed to determine severity of DS and global functioning by using quantitative measures and to establish their correlation with patients’ demographics and clinical variables.Methods31 children (mean age of 11.8 ± 2.3 years) with TLE were assessed with K-SADS-PL for axis I DSM-IV diagnosis. Severity of DS was measured by Children Depression Rating Scale-Revised – CDRS-R. Global functional impairment was evaluated with Child Global Assessment Scale-CGAS.Results25 patients (56% boys; 12 ± 2.3 years) had current DS, moderate or severe in 84% according to CDRS-R T-Score. Severity of DS was not correlated with age (p = 0.377), gender (p = 0.132), seizure control (p = 0.936), age of onset (p = 0.731), duration of epilepsy (p = 0.602) and the presence of hippocampal sclerosis (p = 0.614). Patients had moderate to major functional impairment measured by CGAS (48.7 ± 8.8), being adolescents more impaired than children (p = 0.03). Impairment of global functioning was not associated with epilepsy variables (p > 0.05).ConclusionChildren with TLE had moderate to severe DS early in the course of their disease with a relevant impact on their global functional activities, especially considering adolescents. Epilepsy severity seems not to be correlated to the severity of DS, contradicting the idea of a cause–consequence relationship. More systematic research is needed to better understand the association of depressive disorders in children and adolescents with TLE.     

Sleep and cardiometabolic function in obese adolescent girls with polycystic ovary syndrome

ObjectiveTo compare the polysomnography findings and cardiometabolic function among adolescent girls with polycystic ovary syndrome (PCOS) and matched female and male controls.MethodRetrospective chart review of electronic medical records of 28 girls with PCOS (age: 16.8 ± 1.9 years, body mass index (BMI) Z-score 2.4 ± 0.4), 28 control females (age: 17.1 ± 1.8, BMI Z-score 2.4 ± 0.3) and 28 control males (age: 16.6 ± 1.6, BMI Z-score 2.5 ± 0.5) in a tertiary care centre.ResultsThe prevalence of obstructive sleep apnoea (OSA) was higher in girls with PCOS compared to control females (16/28 (57%) vs. 4/28(14.3%), p < 0.01); however, it was comparable to that of the control males (16/28(57%) vs. 21/28(75%), p = 0.4). Girls with PCOS had a significantly higher prevalence of insulin resistance compared to control females and control males (20/28 (71.4%) vs. 9/22 (41.0%) (p = 0.04) vs. 8/23 (34.8%) (p = 0.01). Among girls with PCOS, those with OSA had significantly higher proportions of metabolic syndrome (MetS) (9/16 (56.3%) vs. 1/12 (8.3%) p = 0.03), higher insulin resistance (14/16 (87.5%) vs. 6/12 (50%), p = 0.04), elevated daytime systolic blood pressure (128.4 ± 12.8 vs. 115.6 ± 11.4, p < 0.01), lower high-density lipoprotein (HDL) (38.6 ± 8.7 vs. 49 ± 10.9, p = 0.01) and elevated triglycerides (TG) (149.7 ± 87.7 vs. 93.3 ± 25.8, p = 0.03) compared to those without OSA.ConclusionsWe report a higher prevalence of OSA and metabolic dysfunction in a selected group of obese girls with PCOS referred with sleep-related complaints compared to BMI-matched control girls without PCOS. We also report higher prevalence of cardiometabolic dysfunction in girls with PCOS and OSA compared to girls with PCOS without OSA.     

Assessment of cardiorespiratory and neuromotor fitness in children with developmental coordination disorder

The decreased participation in physical activity by children with probable developmental coordination disorder (pDCD) has raised concerns about their aerobic fitness and lung function levels. The purpose of the present study was to examine assessment of cardiorespiratory and neuromotor fitness, using laboratory-based tests during an incremental treadmill protocol in healthy children with and without pDCD. Twenty sex children ages 6–9 years took part in this study. Motor coordination was assessed using the Movement Assessment Battery for Children (MABC). All participants performed a cardiopulmonary exercise test (CPET) on a cycle ergometer. Pulmonary function was assessed by spirometric measurements (forced vital capacity: FVC, forced expiratory volume in 1 s: FEV₁) and walking distance (6MWD) was assessed using the 6-min walking test. The children with pDCD had lower VO_2 max than children without pDCD (p < 0.01). Moreover, FVC and FEV₁ were significantly higher in children without pDCD than in children with the disorder (p < 0.05, p < 0.01 respectively). Likewise, children with pDCD had poorer performance on the 6MWD than children without pDCD (p < 0.01). A significant correlation between the absolute value for FEV₁ and 6MWD (r = 0.637, p < 0.05) in pDCD group was observed. We found a significant correlation between VO_2 max and MABC score (r = −0.612, p < .001) and between VO_2 max and 6MWD (r = 0.502, p < .001) for all children. Moreover, a significant correlation between VO_2 max and FEV₁ (r = 0.668, p < .05) was found in children with pDCD. Overall, the reduced aerobic capacity of DCD was associated with decreased of lung function, as well as an alteration of peripheral muscle responses.     

Afferent control of walking: Are there distinct deficits associated to loss of fibres of different diameter?

ObjectivesTo compare the gait pattern in patients affected by different types of neuropathy.MethodsWe recruited healthy subjects (HS, n = 38), patients with Charcot–Marie–Tooth disease type 1A (CMT1A) (n = 10) and patients with diabetic neuropathy (DNP) (n = 12). Neuropathy impairment score and neuropathy score were assessed. Body sway during quiet stance, and spatio-temporal gait parameters were recorded.ResultsMost patients had reduced or absent tendon-tap reflexes. Strength of foot dorsiflexor muscles (p < 0.05) and conduction velocity (CV) of leg nerves (p < 0.0001) were more impaired in CMT1A than DNP, whereas joint-position sense was more affected (p < 0.05) in DNP. Body sway while standing was larger in DNP compared to CMT1A and HS (p < 0.01 and p < 0.0001 respectively). During gait, the distribution of foot sole contact pressure was abnormal in CMT1A (p < 0.05) but not in DNP. Velocity and step length were decreased, and foot yaw angle at foot flat increased, in DNP with respect to CMT1A and HS (both variables, p < 0.001). Gait velocity and step length were decreased (p < 0.005) also in CMT1A, but to a smaller extent than in DNP, so that the difference between patient groups was significant (p < 0.0005). Duration of the double support was protracted in DNP compared to CMT1A and HS (p < 0.0005). For DNP only, velocity of gait and duration of single support were correlated (p < 0.05) both to sway path and lower limb muscle strength.ConclusionsChanges in both body sway and stance phase of gait were larger in DNP than CMT1A, indicating more impaired static and dynamic control of balance when neuropathy affects the small in addition to the large afferent fibres. Diminished somatosensory input from the smaller fibres rather than muscle weakness or foot deformity plays a critical role in the modulation of the support phase of gait.SignificanceThe analysis of balance and gait in patients with neuropathy can offer a tool for understanding the nature and functional impact of the neuropathy and should be included in their functional evaluation.     

Cross sectional area reference values for sonography of peripheral nerves and brachial plexus

ObjectiveUltrasound measurements of the cross sectional area (CSA) variability have been recently introduced to quantify pathological changes in peripheral nerves (PN).MethodsReference values from 75 healthy subjects and their correlation to age, height, weight and sex are reported.ResultsThe mean values in PN were: (1) intranerve CSA-variability: median 1.05 (SD ± 0.13), ulnar 1.53 (SD ± 0.51), fibular 1.33 (SD ± 0.37), tibial 1.39 (SD ± 0.39), (2) internerve CSA-variability 1.76 (SD ± 0.37), (3) intraplexus CSA-variability 1.52 (SD ± 0.37), (4) side-to-side difference ratio of the CSA-variability: median 1.21 (SD ± 0.04), ulnar 1.2 (SD ± 0.25), fibular 1.19 (SD ± 0.23), tibial 1.28 (SD ± 0.24) and brachial plexus 1.19 (SD ± 0.23). CSA did not correlate with height in PN, but correlated with weight in the ulnar nerve [Guyon’s canal, r = 0.411, p = 0.0237, elbow r = 0.409, p = 0.0248]. Significant changes between sex were found only in the ulnar (Guyon’s canal, p = 0.0265), fibular (popliteal fossa, p = 0.0336) and sural nerve (p = 0.048). CSA decreased with age in the median (axilla, p = 0.0236), and radial nerve (spiral groove, p = 0.0037) and increased in the tibial nerve (ankle, p < 0.0001).ConclusionsThe CSA reference values reported seem to correlate at certain sites with age, weight and sex but not with height.SignificanceThe new CSA variability measures may be helpful in investigating pathologies of the PN.     

Sensorimotor function in preschool-aged children with expressive language disorder

AimThe aim of the study was to evaluate functional motor performance and haptic object recognition in 5-year-old children with mild expressive language disorder (ELD) in comparison with age- and gender-matched healthy children.MethodsThe subjects were classified by speech-language pathologist using The Reynell Developmental Language Scales III and Boehm Test of Basic Concepts: Preschool as children with mild ELD (n = 29, incl. 23 boys and 6 girls) and children with typical language development as controls (n = 29, incl. 23 boys and 6 girls). The children were examined for manual dexterity, ball skills, static and dynamic balance by Movement-ABC, haptic object recognition (HOR), hand-grip strength (HGS) and vertical jumping performance.ResultsChildren with mild ELD demonstrated significantly higher scores (i.e., inferior performance) in all subtests of M-ABC (all p values <0.05), in haptic object recognition (p < 0.01) and vertical jumping height (p < 0.05) compared to controls. However, no statistically significant differences (p > 0.05) emerged from HGS. Boys with mild ELD demonstrated higher results in impairment score (p < 0.001), ball skills (p < 0.01) and balance (p < 0.01) of M-ABC, as well as in HOR (p < 0.05). Girls with mild ELD showed higher impairment score (p < 0.05) with lower percentile (p < 0.05) in M-ABC, indicating inferior motor performance, and lower HGS for the non-dominant hand (p < 0.05). Seven out of 29 (24.1%) children with mild ELD had definite or borderline motor difficulties, while only one child in control group (3.4%) demonstrated borderline motor difficulties.ConclusionsChildren with mild expressive language disorder do not perform as well as controls in tests of functional motor skills, but their results in tests demanding maximal muscle force generation are in level with typically developing children. Boys and girls with mild ELD demonstrated higher impairment scores in M-ABC, indicating the need to follow their overall development more closely.     

Use of the Gait Profile Score for the evaluation of patients with joint hypermobility syndrome/Ehlers–Danlos syndrome hypermobility type

Gait analysis (GA) is widely used for clinical evaluations in various pathological states, both in children and in adults, such as in patients with joint hypermobility syndrome/Ehlers–Danlos syndrome hypermobility type (JHS/EDS-HT). Otherwise, GA produces a large volume of data and there is the clinical need to provide also a quantitative measure of the patient's overall gait. Starting from this aim some global indexes were proposed by literature as a summary measure of the patient's gait, such as the Gait Profile Score (GPS). While validity of the GPS was demonstrated for the evaluation of the functional limitation of children with Cerebral Palsy, no studies have been conducted in patients JHS/EDS-HT. The aim of our study was therefore to investigate the effectiveness of the GPS in the quantification of functional limitation of patients with JHS/EDS-HT. Twenty-one adult (age: 36.1 ± 12.7 years) individuals with JHS/EDS-HT were evaluated using GA and from GA data the GPS was computed. The results evidenced that the GPS value of patients was 8.9 ± 2.6, statistically different from 4.6 ± 0.9 displayed by the control group. In particular, all values of Gait Variable Scores (GVS) which compose the GPS were higher if compared to controls, with the exception of Pelvic Tilt and Foot Progression. The correlations between GPS/GVS and Lower Extremity Functional Scale (LEFS) showed significant relationship between GPS and the item 11 (“Walking 2 blocks”) (ρ = ?0.56; p < 0.05) and 12 (“Walking a mile”) of LEFS (ρ = ?0.76; p < 0.05). Our results showed that GPS and GVS seem to be appropriate outcome measures for the evaluation of the functional limitation during gait of patients with JHS/EDS-HT.     

Anthropometric measures of Spanish children with autism spectrum disorder

We aimed to compare body mass index (BMI) and healthy eating index (HEI) in children with autism spectrum disorder (ASD, n = 105) and typically developing (TD, n = 495) children. They were aged 6–9 years, lived in Valencia (Spain) and came from similar cultural and socio-economic backgrounds. In this case–control study, the weight, height and BMI were measured for both groups. Three-day food records were used to assess dietary intake. Although the differences between children with ASD and TD children in raw BMI (p = 0.44), BMI z-score (p = 0.37), HEI (p = 0.43) and total energy intake (p = 0.86) were not significant, children with ASD and the boys subgroup were shorter (p = 0.01), but not the girls subgroup, compared to TD children of the same gender. Using the controls values as a reference, the BMI distribution in children with ASD became distorted, with values below the 5th percentile (11% vs. 4%, p = 0.03) and above the 95th percentile (8% vs. 5%, p = 0.04). The gender- and age-adjusted odds ratios for being underweight in the groups of all children and boys with ASD were 3.03 and 2.39, respectively, vs. TD children. Our data suggest that routine monitoring of children with ASD should include anthropometric measurements and assessment of their dietary habits.     

Uric acid excretion in North American and Southeast European children with obstructive sleep apnea

Background and objectivesResponses to nocturnal hypoxemia accompanying sleep-disordered breathing (SDB) may vary in different populations. Aims of this study were to (1) assess whether severity of SDB is related to uric acid excretion in North American and Southeast European children and (2) evaluate the interaction between nocturnal hypoxemia and country of children’s origin in uric acid excretion.MethodsConsecutive US and Greek children with snoring who were referred for polysomnography were recruited. Uric acid excretion expressed as uric acid-to-creatinine concentrations ratio in a morning urine specimen was the primary outcome measure.ResultsOne hundred and twenty-six US children (6.8 ± 0.7 years old) and 123 Greek children (6.4 ± 2.5 years old) were recruited. Forty-three US and 53 Greek participants had moderate-to-severe nocturnal hypoxemia (SpO₂ nadir <90%). Obstructive apnea-hypopnea index and SpO₂ nadir were related to uric acid excretion in Greek (but not US) children after adjustment by age, gender and body mass index z-score (p < 0.05). There was a significant interaction between severity of hypoxemia and country of children’s origin in uric acid excretion after adjustment by age, gender and body mass index z-score (p = 0.036). Greek children with moderate-to-severe hypoxemia had higher uric acid excretion (0.85 ± 0.35) than those with mild/no hypoxemia (0.69 ± 0.25) (p = 0.005). US children with moderate-to-severe hypoxemia (0.41 ± 0.20) did not differ in uric acid excretion from those with mild/no hypoxemia (0.42 ± 0.22) (p = 0.823).ConclusionsUric acid excretion differs in children with SDB and different ethnic backgrounds or environmental exposures.     

Prenatal exposure to the organophosphate pesticide chlorpyrifos and childhood tremor

BackgroundThe organophosphate insecticide chlorpyrifos (CPF), widely used for agricultural purposes, has been linked to neurodevelopmental deficits. Possible motor effects at low to moderate levels of exposure have not been evaluated.MethodsPrenatal exposure to CPF was measured in umbilical cord blood in a sample of 263 inner-city minority children, who were followed prospectively. At approximately 11 years of age (mean age 10.9 ± 0.85 years, range = 9.0–13.9), during a neuropsychological assessment, children were asked to draw Archimedes spirals. These were rated by a senior neurologist specializing in movement disorders who was blind to CPF exposure level.ResultsCompared to all other children, those with prenatal CPF exposure in the upper quartile range (n = 43) were more likely to exhibit mild or mild to moderate tremor (≥1) in either arm (p = 0.03), both arms (p = 0.02), the dominant arm (p = 0.01), and the non-dominant arm (p = 0.055). Logistic regression analyses showed significant CPF effects on tremor in both arms, either arm, the dominant arm (p-values <0.05), and the non-dominant arm (p = 0.06), after adjustment for sex, age at testing, ethnicity, and medication.ConclusionPrenatal CPF exposure is associated with tremor in middle childhood, which may be a sign of the insecticide's effects on nervous system function.     

Motor and cognitive assessment of infants and young boys with Duchenne Muscular Dystrophy: results from the Muscular Dystrophy Association DMD Clinical Research Network

Therapeutic trials in Duchenne Muscular Dystrophy (DMD) exclude young boys because traditional outcome measures rely on cooperation. The Bayley III Scales of Infant and Toddler Development (Bayley III) have been validated in developing children and those with developmental disorders but have not been studied in DMD. Expanded Hammersmith Functional Motor Scale (HFMSE) and North Star Ambulatory Assessment (NSAA) may also be useful in this young DMD population. Clinical evaluators from the MDA-DMD Clinical Research Network were trained in these assessment tools. Infants and boys with DMD (n = 24; 1.9 ± 0.7 years) were assessed. The mean Bayley III motor composite score was low (82.8 ± 8; p ? .0001) (normal = 100 ± 15). Mean gross motor and fine motor function scaled scores were low (both p ? .0001). The mean cognitive comprehensive (p = .0002), receptive language (p ? .0001), and expressive language (p = .0001) were also low compared to normal children. Age was negatively associated with Bayley III gross motor (r = ?0.44; p = .02) but not with fine motor, cognitive, or language scores. HFMSE (n = 23) showed a mean score of 31 ± 13. NSAA (n = 18 boys; 2.2 ± 0.4 years) showed a mean score of 12 ± 5. Outcome assessments of young boys with DMD are feasible and in this multicenter study were best demonstrated using the Bayley III.     

Children diagnosed with autism spectrum disorder before or after the age of 6 years

This study compared children with early and late diagnosis of autism spectrum disorder (ASD). All children in four consecutive birth cohorts in Iceland diagnosed with ASD were divided into two groups based on their age at initial ASD diagnosis: 58 children were diagnosed before age 6 (group 1) and 41 children after age 6 (group 2). Children in group 1 were more likely to receive a diagnosis of childhood autism (p ≤ 0.001), their average IQ/DQ was lower (p < 0.001), verbal status was lower (p < 0.001), and a history of autistic regression was more common (p < 0.01) than in group 2. Half of the children in group 2 had received other developmental diagnoses prior to the ASD diagnosis, but this applied to only a few of the children in group 1 (p < 0.001). There was no difference between the groups with regard to autistic symptoms as measured by the Autism Diagnostic Interview-Revised (p = 0.224), frequency of associated medical conditions (p = 0.640), age of first parental concern (p = 0.244), and age of first autistic symptoms on hindsight (p = 0.540). The majority of parents (76.2%) had developmental concerns before age 3, and with hindsight 83.3% thought that autistic symptoms had been present before age 2.