Rural and Racial/Ethnic Differences in Children Receiving Early Intervention Services.

A review of the literature shows that racial and ethnic minority children (eg, African American, Asian, and Hispanic) received diagnoses for developmental concerns later in life compared with their age-matched white counterparts. Research has also documented disparities in access to and receipt of health care services among children with developmental concerns as compared with children with other disabilities. OBJECTIVES: We examined health care providers' (HCPs') responses to parents' developmental concerns about their children. We looked at the association with race, ethnicity, gender, rurality, and time to diagnosis. METHODS: All data were secondary and derived from the Centers for Disease Control and Prevention's Survey of Pathways to Diagnosis and Services. Participants consisted of 1321 parents of children who had received early intervention services as reported by respondents' data collected in 2011. RESULTS: From a nationally representative sample of families receiving early intervention services, 76% were white, 10% were African American, 3% were Asian, 5% were Native American, and 9% were Hispanic. Families who were Hispanic were more likely to have received only a delaying response from HCPs. The average time to a developmental delay diagnosis was 5 months longer for families who received a delaying HCP response. CONCLUSIONS: Families who were Hispanic or who were from rural areas were most likely to receive a delayed HCP response; for parents who received a delayed HCP response, a developmental delay diagnosis took 5 months longer than for families from the other groups listed.

Attitudes Toward Inclusion and Benefits Perceived by Families in Schools with Students with Autism Spectrum Disorders.

The aim of this study was to find out about attitudes toward inclusion and benefits perceived by families with children enrolled in schools attended by students with ASD at different educational stages (from kindergarten to high school). 323 families of classmates of students with ASD from different educational stages of 16 mainstream schools participated. The analysis of the attitudes, perceived benefits, relationship with the teacher, and relationship with the school was carried out through questionnaires. The results show positive attitudes toward the inclusive education of students with ASD in all families, but especially among families of children with SEN. All the families identified the benefit of inclusion. Attitudes are related to collaboration with the school and satisfaction with teachers.

Rasch Analysis of Authentic Evaluation of Young Children's Functioning in Classroom Routines.

This study evaluated the functioning of children in early childhood education classroom routines, using the 3M Functioning in Preschool Routines Scale. A total of 366 children aged 36 to 70 months and 22 teachers from six early childhood education centers in Spain participated in the study. The authors used the Rasch model to determine the item fit and the difficulty of the items in relation to children's ability levels in this age range. The Rasch Differential Item Functioning (DIF) analysis by child age groups showed that the item difficulty differed according to the children's age and according to their levels of competence. The results of this study supported the reliability and validity of the 3M scale for assessing children's functioning in preschool classroom routines. A few items, however, were identified as needing to be reworded and more difficult items needed to be added to increase the scale difficulty level to match the performance of children with higher ability levels. The authors introduced the new and reworded items based on the results of this study and the corresponding ICF codes per item. Moreover, the authors indicate how to use the ICF Performance Qualifiers in relation to the 3M scale response categories for developing a functioning profile for the child.

The Routines-Based Model Internationally Implemented.

Professionals from 10 countries are implementing practices from the Routines-Based Model, which has three main components: needs assessment and intervention planning, a consultative approach, and a method for running classrooms. Its hallmark practices are the Routines-Based Interview, support-based visits with families, and a focus on child engagement. Implementers were interested in actual practices for putting philosophy and theory into action in their systems and cultures. We describe implementation challenges and successes and conclude that (a) models have to be adaptable, (b) some principles and practices are indeed universal, (c) we can shape excellent practices for international use, and (d) leadership is vital.

Muscle ultrasound in the assessment of suspected neuromuscular disease in childhood.

One hundred paediatric, muscle ultrasound examinations performed in the evaluation of suspected neuromuscular disease were reviewed. The results were related to the presence or absence of neuromuscular disease in each child assessed. The group comprised 66 males and 34 females, age range 2 months to 16 years (mean 5.3 years). Scans were graded I-IV, according to muscle echogenicity, using Heckmatt's criteria. Thirty-two children had a final diagnosis of neuromuscular disease. The sensitivity of ultrasound in detecting neuromuscular disease was 78% with 91% specificity. The test was more reliable in the sub-group of > 3 years with a sensitivity of 81% and specificity of 96%. There was a significant difference in disease status, (with and without neuromuscular disease), between children with a normal, grade I, scan and those with an abnormal, grade II, III, IV, image (chi-square, P < 0.001, 95% confidence limits 0.54-0.86). Muscle ultrasound is a specific and sensitive investigation for suspected neuromuscular disease in children.

Factor V Leiden, prothrombin 20210G-->A and the MTHFR C677T mutations in childhood stroke.

Ischaemic stroke is a rare occurrence in children and in a proportion of cases the aetiology remains unknown. We have investigated the role of thrombophilia in the aetiology of this condition. Of 50 cases identified at two centres, 37 were available for detailed haematological analysis. No cases were identified with deficiencies of antithrombin, protein C or protein S. One case had elevated IgG anticardiolipin antibodies at low titre. The prevalence of the prothrombin 20210 G-->A mutation, factor V Leiden (FVL) mutation and the C677T mutation in the MTHFR gene was compared in cases to that observed in random unselected cord blood controls. The odds ratio for stroke was not significantly increased in carriers of the prothrombin mutation (OR 1.2; 95% CI 0.1-10.7), FVL (OR 2.5; 95% CI 0.5-13.5), or the C677T mutation (OR 1.7; 95% CI 0.6-4.5). Our findings suggest that thrombophilia may not play a significant role in the aetiology of stroke in children, although a large prospective study is required to investigate this area further.

Somatosensory evoked potentials in the detection of spinal cord ischemia in aortic coarctation repair.

Cortical somatosensory evoked potential (SEP) monitoring was used in 15 patients 2 to 50 years old undergoing repair of aortic coarctation to detect the onset of spinal cord ischemia during the cross-clamp period. Three different response patterns were observed. In 8 patients (53%), the SEP remained unchanged throughout the cross-clamping. This was designated a type 1 response. Six patients (40%) showed a gradual deterioration in the SEP after 15 minutes of cross-clamping (type 2 response). All SEPs returned to normal levels within 5 minutes of release of the clamp. One patient (7%) demonstrated a decline in SEP commencing prior to the application of the cross-clamp when an intercostal vessel was controlled with slings. The SEP completely disappeared within 5 minutes of cross-clamping, but after 19 minutes the repair was completed and the SEP returned within 3 minutes of reperfusion (type 3 response). No patient sustained neurological sequelae of repair. We believe that SEP monitoring offers the potential to identify the patient at risk of developing spinal cord ischemia intraoperatively before irreversible damage occurs. However, it is susceptible to deep halothane anesthesia, which abolishes all cortical responses and requires expert monitoring.

Growth factors in human ovarian follicle fluid and growth factor receptors in granulosa-luteal cells.

The levels of oestradiol (E2), progesterone (P4), transforming growth factor alpha (TGF alpha), transforming growth factor beta 2 (TGF beta 2), insulin-like growth factor I (IGF-I), platelet-derived growth factor AB (PDGF-AB) and epidermal growth factor (EGF) were measured in follicular fluids obtained from patients undergoing ovarian stimulation as part of an in vitro fertilisation program. Each of the substances was detected in all of the fluid samples tested, except TGF alpha (which was detected in 90% of samples tested), PDGF-AB (70%) and EGF (2%). Comparisons were made between each of these factors, follicular maturity, successful oocyte recovery and the outcome of fertilisation and embryo transfer. No statistically significant correlations were found. The presence of receptors for EGF, IGF-I and PDGF in extracts from granulosa-luteal cells isolated from follicular fluids was detected by means of Western blotting. The co-localisation of these growth factors and their receptors within the ovarian follicle suggests a likely role in control of follicular development.

Monitoring response to bronchodilator therapy in asthma in childhood.

Recently there has been increased interest in the role of bronchodilators in the maintenance therapy of asthma in childhood. We report the use of slow-release aminophylline (Phyllocontin Continus tablets) in childhood asthma. Ten patients, all of whom were receiving disodium cromoglycate (DSG), were given a single dose of Phyllocontin tablets (12.5 mg/kg) and observed over a period of 8 hours. Measurements of peak flow rate (PFR) and forced expiratory volume in one second (FEV1) showed an increase of at least 30% at 3 to 5 hours and the improvement continued to 8 hours. No effect was noted on heart rate. Ten patients were assessed over a 4-week period on DSG then over two 4-week periods on Phyllocontin tablets and placebo on a double-blind crossover basis. The dosage of Phyllocontin tablets used was 12.5 mg/kg twice daily. Using twice daily PFR recordings there was no significant difference between the two periods on DSG and Phyllocontin tablets. The reduction in PFR during the period on placebo was significant, however, and more markedly so between the period of placebo and the period on Phyllocontin tablets. We conclude that Phyllocontin tablets in these patients compared favourably with DSG in the maintenance therapy of asthma in childhood. The results obtained after single dose administration also suggest that a proportion of children with asthma may benefit from combined treatment with DSG and regular bronchodilators.

A new challenge for pediatric audiology: Public Law 99-457.

When fully implemented, the most recent amendments to the Education of the Handicapped Act will have a substantial impact on expanding services to infants and toddlers with handicapping conditions and their families. Audiologists in many settings will be called upon to participate in the implementation of this new mandate. It is imperative that audiologists be well informed about Public Law 99-457 as state and local policies are being developed and implemented. This article reviews the new law with emphasis on its implications for young hearing-impaired children, their families, and the audiologists who serve them.

Non-progressive mental retardation and peripheral neuropathy in a mother and her son.

The association of facial dysmorphism, moderate mental retardation and peripheral neuropathy was observed in a mother and her son. The son also has pyramidal tract involvement in the lower limbs. Although exactly the same association has not been described previously, it seems probable that it results from variable expression of a dominant gene defect.

Parents' reactions and recommendations after diagnosis and hearing aid fitting.

This study was designed to explore parent reactions to the early stages of audiologic assessment and intervention. A total of 213 parents whose children were under the age of 6 years returned a mail survey. Respondents from 45 states participated. Parents were asked to (1) report the approximate age of diagnosis and hearing aid fitting; (2) comment on reasons for any delays encountered from diagnosis to fitting; and (3) respond to questions concerning their reactions to the initial fitting of amplification. The median age of identification was earlier than some previous investigations; however, substantial delays occurred between diagnosis and hearing aid fitting. Reasons for delay included the need for further audiologic evaluation, problems obtaining return appointments, illness of the child, and difficulties obtaining adequate earmolds. Parent reactions to hearing aids, once fitted, included concerns about appearance and questions about maintenance and use, but attitudes regarding hearing aids and their perceived benefits improved over time.

The impact of checklist-based training on teachers' use of the zone defense schedule.

We assessed the impact of checklist-based training on teaching teams' use of the zone defense schedule. Three teaching teams (lead teacher plus 2 assistant teachers) in an inclusive early childhood program participated. A multiple baseline design across teams was used to determine whether accurate implementation of the zone defense schedule increased when checklist-based training was provided. All teaching teams reached the preestablished criterion, implementing a minimum of 80% of checklist items accurately for 3 consecutive observations.

Fatal and near-fatal encephalopathy with hyponatraemia in two siblings with fluticasone-induced adrenal suppression.

AIM: To document previously unreported acute effects of adrenal insufficiency. METHODS: We describe two siblings who presented acutely with hyponatraemia and cerebral oedema following prolonged treatment with high dose inhaled fluticasone. RESULTS: A girl aged 5.5 years presented with vomiting, headache, visual impairment and seizures. She was hyponatraemic but not hypoglycaemic. Her conscious level continued to deteriorate and she died, post mortem examination showing small adrenal glands and cerebral oedema. Four weeks later her 7-year-old brother presented with similar symptoms. Assessment showed hyponatraemia with cerebral oedema. His illness responded to intensive care. A diagnosis of adrenal insufficiency was made retrospectively in both cases. The siblings had been receiving Fluticasone propionate (FP) in doses of up to 2000 microg/day for several years. CONCLUSION: We believe that the hyponatraemia and cerebral oedema was related to cortisol deficiency, leading to impaired excretion of water. We emphasize the need for careful cerebral monitoring in acute adrenal insufficiency presenting with impaired consciousness.

Investigation of global developmental delay.

The investigation of global developmental delay in preschool children varies between centres and between paediatricians. Following a literature search and review of the evidence base, guidelines were developed to assist in the assessment and management of such children presenting to secondary level services. Evidence supporting the use of genetic and biochemical investigations on a screening basis was found, but there was no evidence to support the use of metabolic investigations or neuroimaging in the absence of other positive findings on history or examination. Detailed history and examination are paramount in the assessment of children with global developmental delay. Investigations can be a useful adjunct in determining aetiology. Evidence based guidelines have been developed to assist doctors in the selection of appropriate investigations for this group of children.