Defects and Disabilities of Thalidomide Children

The range of defects and disabilities in thalidomide children is very much wider than is generally realized. The defects of 154 children are described and classified. Their disabilities range from incapacitating to negligible.     

Quantitative Assessment of Cortical Auditory-tactile Processing in Children with Disabilities

Objective and easy measurement of sensory processing is extremely difficult in nonverbal or vulnerable pediatric patients. We developed a new methodology to quantitatively assess children''s cortical processing of light touch, speech sounds and the multisensory processing of the 2 stimuli, without requiring active subject participation or causing children discomfort. To accomplish this we developed a dual channel, time and strength calibrated air puff stimulator that allows both tactile stimulation and sham control. We combined this with the use of event-related potential methodology to allow for high temporal resolution of signals from the primary and secondary somatosensory cortices as well as higher order processing. This methodology also allowed us to measure a multisensory response to auditory-tactile stimulation.     

Measuring Coping in Parents of Children with Disabilities: A Rasch Model Approach

Background

Parents of a child with disability must cope with greater demands than those living with a healthy child. Coping refers to a person’s cognitive or behavioral efforts to manage the demands of a stressful situation. The Coping Health Inventory for Parents (CHIP) is a well-recognized measure of coping among parents of chronically ill children and assesses different coping patterns using its three subscales. The purpose of this study was to provide further insights into the psychometric properties of the CHIP subscales in a sample of parents of children with disabilities.

Methods

In this cross-sectional study, 220 parents (mean age, 33.4 years; 85% mothers) caring for a child with disability enrolled in special schools as well as in mainstream schools completed the 45-item CHIP. Rasch analysis was applied to the CHIP data and the psychometric performance of each of the three subscales was tested. Subscale revision was performed in the context of Rasch analysis statistics.

Results

Response categories were not used as intended, necessitating combining categories, thereby reducing the number from 4 to 3. The subscale – ‘maintaining social support’ satisfied all the Rasch model expectations. Four item misfit the Rasch model in the subscale –maintaining family integration’, but their deletion resulted in a 15-item scale with items that fit the Rasch model well. The remaining subscale – ‘understanding the healthcare situation’ lacked adequate measurement precision (<2.0 logits).

Conclusions

The current Rasch analyses add to the evidence of measurement properties of the CHIP and show that the two of its subscales (one original and the other revised) have good psychometric properties and work well to measure coping patterns in parents of children with disabilities. However the third subscale is limited by its inadequate measurement precision and requires more items.     

Activity and Participation Characteristics of Adults with Learning Disabilities - A Systematic Review

Background

‘Learning disabilities’ (LD) refer to a wide group of neurological disorders caused by deficits in the central nervous system which influence the individual''s ability to maintain-, process or convey information to others in an efficient way. A worldwide discussion about the definitions of LD continues while a conceptual framework for studying the diverse life outcomes of adults with LD is still missing.

Objective

The aim was to review the literature on the activity and participation of adults with LD based on the International Classification of Functioning, Disability and Health (ICF) concepts.

Methods

“PsychInfo”, “Eric” and “PubMed” were searched for relevant literature according to the guidelines of Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). After a three-stage process, 62 articles relevant for domains of activity and participation of adults with LD were included in the review.

Results

Thirty-two articles focused on the domain of major life areas of education, work and employment and twelve articles focused on the domain of learning and applying knowledge. Limitations in activity and participation of the population with LD in these domains are recognized and discussed. Eighteen additional articles demonstrated that adults with LD confront difficulties in various life domains (e.g., communication, interpersonal interactions, mobility, and domestic life), however literature concerning these domains is scarce.

Conclusions

The ICF can be useful for further exploration of activity and participation characteristics of adults with LD in various life domains. Such exploration is required in order to gain a wider perspective of their functional characteristics and daily needs.     

Long-Term Effectiveness of Dolphin-Assisted Therapy for Children with Severe Disabilities

ABSTRACT

Long-term effectiveness of dolphin assisted therapy, as practiced by Dolphin Human Therapy, was analyzed via a 15 item closed form, ratio scale parent questionnaire (n=71). Children with severe disabilities of many etiologies, from eight countries, received either one or two weeks of therapy in the multidisciplinary, behavior modification program. Results on three clinical issues were analyzed. First, children maintained or improved skills acquired in therapy about 50% of the time even after 12 months away from therapy. Second, no difference in long-term effects occurred as a function of differences in three categories (genetic, brain damage, unknown causes) of etiology (ANOVA, F_(2,39)=2.79, p>0.05). Third, two weeks of therapy produced significantly better long-term results than did one week of therapy (t=3.105, df=28, p<0.01).     

Effectiveness of Short-Term Dolphin-Assisted Therapy for Children with Severe Disabilities

ABSTRACT

The effectiveness of two week dolphin-assisted therapy was compared to the effectiveness of six month conventional physical and speech-language therapy. Data were analyzed using a multiple baseline single subject across settings design, for 47 children with severe disabilities (20 females, 27 males), of multiple etiologies. Children were placed in a physical therapy group (n = 17, mean age = 6 years, 8 months) and a speech language group (n = 30, mean age = 6 years 5 months). Standardized charting procedures were used to measure acquisition of independent motor and speech-language skills. Use of t tests for nonindependent samples indicates that relative to conventional long-term therapy, dolphin-assisted therapy, as practiced by Dolphin Human Therapy, achieves positive results more quickly and is also more cost effective.     

Social Effects of a Dog's Presence on Children with Disabilities

ABSTRACT

Productive and positive interactions between dogs and humans have been documented in studies using dogs trained as companion animals and as assistants for people with disabilities. In this study, the effects of the presence of a dog on social interactions between three 5–9-year-old children with developmental disabilities and their teacher at an elementary school were analyzed. A single-case experimental design with repeated measures and with replicated effects across participants was employed to assess changes in interactions from baseline to an intervention condition. During baseline, interactions were assessed in the social environment of a room adjacent to the classroom, which had a toy dog and other play materials, during time with the teacher. The experimental change introduced sequentially and systematically across the participants was the additional presence of an obedience-trained dog, a German Shepherd/Labrador Retriever cross. Interactions between the children and their teacher were examined during morning sessions using reliable direct observation interval recording procedures. All participants demonstrated an increase in overall positive initiated behaviors (verbal and non-verbal) toward both the teacher and the dog. The children also showed an overall decrease in negative initiated behaviors. In addition, observational ratings showed positive generalization of improved social responsiveness by the children in their classroom following the completion of the experimental sessions. This study supports the position that children with developmental disabilities benefit from the use of skilled dogs as teaching assistants and therapeutic adjuncts.     

Participation of Children in Medical Decision-Making: Challenges and Potential Solutions

Participation in healthcare decision-making is considered to be an important right of minors, and is highlighted in both international legislation and public policies. However, despite the legal recognition of children’s rights to participation, and also the benefits that children experience by their involvement, there is evidence that legislation is not always translated into healthcare practice. There are a number of factors that may impact on the ability of the child to be involved in decisions regarding their medical care. Some of these factors relate to the child, including their capacity to be actively involved in these decisions. Others relate to the family situation, sociocultural context, or the underlying beliefs and practices of the healthcare provider involved. In spite of these challenges to including children in decisions regarding their clinical care, we argue that it is an important factor in their treatment. The extent to which children should participate in this process should be determined on a case-by-case basis, taking all of the potential barriers into account.     

Behavior of Children with Learning Disabilities Interacting With a Therapy Dog

ABSTRACT

Therapy using animals is now a widespread phenomenon, but some authorities have argued that there is insufficient evidence either to justify or to guide animal-assisted therapy. This reflects a paucity of adequate quantitative methodology for investigations in this area. The little research that has been carried out suggests that animal presence may increase general social behavior. Our study aimed to develop a methodology to investigate the effects of a dog on the behavior of children with severe learning disabilities. A repeated measures design was used to observe eight children with Down's syndrome in controlled sessions, under the direction of an adult. Each session consisted of seven minutes of interaction with a real dog, and seven minutes with an imitation dog. Differences in visual attending, and verbal and non-verbal initiation and response behaviors indicated that the real dog provided a more sustained focus for positive and co-operative interactions with itself and the adult, than did the imitation dog.     

Mental Health of Parents as Caregivers of Children with Disabilities: Based on Japanese Nationwide Survey

The number of children with disability is increasing gradually in Japan. Previous researches in other countries have reported that parents as caregivers (CGs) of children with disability have mental health problems, but the actual situation has not been examined nationwide in Japan so far. The aim of this study was to evaluate the association between mental health of CGs who had children with disability and characteristics of children, CGs, and household based on the nation-wide survey. This study utilized data from 2010 Comprehensive Survey of the Living Conditions, and defined children with disability aged 6 to 17. Individual data of children and CGs were linked, and 549 pairs of them were extracted. The Japanese version of Kessler 6 (K6) was used to assess mental health status of caregiver, scored 5 and over represented to general psychological distress. Logistic regression was used to evaluate the associations of interest. The almost half (44.4%) of CGs had psychological distress (k6 score; 5 +) in nationwide, and 8.9% of CGs might have serious mental illness (K6 score; 13+). After adjusting covariates of child, CG, and household factors, CG having a current symptom (OR, 95% CI: 3.26, 1.97–5.39), CG''s activity restriction (OR, 95% CI: 2.95, 1.38–6.32), low social support (OR, 95%CI: 9.31, 1.85–46.8), three generation family (OR, 95% CI: 0.49, 0.26–0.92), and lower 25% tile group of monthly household expenditure (OR, 95% CI:1.92, 1.05–3.54), were significantly associated with psychological distress of CGs. This study encourages health care providers to pay more attentions toward parent''s mental health, especially for in case of having low social support, and lower income family. Further research should examine the detailed information of child''s disease and disability, medical service use, and quality and quantity of social support in nationwide to straighten the system for supporting services of both children with disabilities and their CGs.     

Gene Mutation Analysis in 253 Chinese Children with Unexplained Epilepsy and Intellectual/Developmental Disabilities

Objective

Epilepsy and intellectual/developmental disabilities (ID/DD) have a high rate of co-occurrence. Here, we investigated gene mutations in Chinese children with unexplained epilepsy and ID/DD.

Methods

We used targeted next-generation sequencing to detect mutations within 300 genes related to epilepsy and ID/DD in 253 Chinese children with unexplained epilepsy and ID/DD. A series of filtering criteria was used to find the possible pathogenic variations. Validation and parental origin analyses were performed by Sanger sequencing. We reviewed the phenotypes of patients with each mutated gene.

Results

We identified 32 novel and 16 reported mutations within 24 genes in 46 patients. The detection rate was 18% (46/253) in the whole group and 26% (17/65) in the early-onset (before three months after birth) epilepsy group. To our knowledge, we are the first to report KCNAB1 is a disease-causing gene of epilepsy by identifying a novel de novo mutation (c.1062dupCA p.Leu355HisfsTer5) within this gene in one patient with early infantile epileptic encephalopathy (EIEE). Patients with an SCN1A mutation accounted for the largest proportion, 17% (8/46). A total of 38% (9/24) of the mutated genes re-occurred at least 2 times and 63% (15/24) occurred only one time. Ion channel genes are the most common (8/24) and genes related to synapse are the next most common to occur (5/24).

Significance

We have established genetic diagnosis for 46 patients of our cohort. Early-onset epilepsy had the highest detection rate. KCNAB1 mutation was first identified in EIEE patient. We expanded the phenotype and mutation spectrum of the genes we identified. The mutated genes in this cohort are mostly isolated. This suggests that epilepsy and ID/DD phenotypes occur as a consequence of brain dysfunction caused by a highly diverse population of mutated genes. Ion channel genes and genes related to synapse were more common mutated in this patient cohort.     

Risk of Tuberculosis in Children with Juvenile Idiopathic Arthritis: A Nationwide Population-Based Study in Taiwan

Objective

We aimed to determine the risk of tuberculosis in children with juvenile idiopathic arthritis (JIA) in Taiwan.

Methods

We used the Taiwan National Health Insurance Research Database (NHIRD) to conduct a nested case-control study. We identified a JIA cohort and matched each JIA child with non-JIA children for comparison. Methotrexate (MTX), tumor necrosis factor (TNF) inhibitor administration, and new tuberculosis cases were determined during our study period. To compare tuberculosis (TB) risk among our study groups, Cox proportional regression models were used to determine adjusted hazard ratios (aHRs).

Results

We identified 1495 children with JIA and 11592 non-JIA children. Majority (68.7%) children with JIA had not received MTX or TNF inhibitors; 23.9% used MTX without TNF inhibitors, and 7.4% received TNF inhibitors, irrespective of MTX administration. In total, 43 children developed tuberculosis. The overall tuberculosis infection rate for children with JIA was two times higher than that for non-JIA children. Compared with non-JIA children, children with JIA who used MTX without TNF inhibitors revealed a significantly increased of tuberculosis infection rate (aHR = 4.67; 95% CI: 1.65–13.17; P = 0.004). Children with JIA who either received TNF inhibitors or never used MTX and TNF inhibitors revealed a tuberculosis infection rate comparable to that of non-JIA children.

Conclusions

Analysis of nationwide data of Taiwan suggested that children with JIA were at higher risk of tuberculosis compared with those without JIA.     

Independence between Rhythms of Temperature and Progesterone in Mares

We investigated the ultradian and diurnal body temperature and plasma progesterone concentration rhythms and the temporal relationship between them in mares during the midluteal phase. Data were obtained from 6 mares at 3-h intervals for 3 consecutive days. Cosinor analysis showed that body temperature acrophases occurred in the evening, whereas the progesterone acrophases occurred in the early morning (3 mares) and at night (3 mares). The spectral analysis of the temperature data showed periods of 24 hr for all mares and of 8 hr for 1 mare. In contrast, the periods observed for plasma progesterone concentration were quite divergent (24 and 12 hr). The small variation in the rhythmic parameters of body temperature suggests that its oscillator system is stable. No phase relationship was detected between the rhythmometric parameters of body temperature and plasma progesterone concentration, suggesting that the physiological mechanisms of control of the 24 hr and ultradian rhythm of the two variables are not coupled during the midluteal phase.     

Independence between Rhythms of Temperature and Progesterone in Mares

We investigated the ultradian and diurnal body temperature and plasma progesterone concentration rhythms and the temporal relationship between them in mares during the midluteal phase. Data were obtained from 6 mares at 3-h intervals for 3 consecutive days. Cosinor analysis showed that body temperature acrophases occurred in the evening, whereas the progesterone acrophases occurred in the early morning (3 mares) and at night (3 mares). The spectral analysis of the temperature data showed periods of 24 hr for all mares and of 8 hr for 1 mare. In contrast, the periods observed for plasma progesterone concentration were quite divergent (24 and 12 hr). The small variation in the rhythmic parameters of body temperature suggests that its oscillator system is stable. No phase relationship was detected between the rhythmometric parameters of body temperature and plasma progesterone concentration, suggesting that the physiological mechanisms of control of the 24 hr and ultradian rhythm of the two variables are not coupled during the midluteal phase.     

Prevalence and Correlates of Elopement in a Nationally Representative Sample of Children with Developmental Disabilities in the United States

Despite increased awareness and concern about children with developmental disabilities wandering away from adult supervision, there is a paucity of research about elopement. This is the first study to examine and report the prevalence and correlates of elopement in a nationally representative sample of school-age children in the United States with an autism spectrum disorder (ASD) and/or cognitive impairment. Data were obtained from the CDC''s "Pathways" Survey, a follow-up telephone survey of the parents of 4,032 children with a developmental condition. 3,518 children that had ASD, intellectual disability (ID), and/or developmental delay (DD) at the time of survey administration were included for analysis. Children were divided into three condition groups: ASD-only; ID/DD-only; ASD+ID/DD. Logistic regression analyses were used to compare the prevalence of elopement and rates of preventive measure use (barriers and/or electronic devices) across condition groups, and to examine the clinical and demographic correlates of elopement. T-tests were also performed to compare scores on the Children''s Social Behavior Questionnaire (CSBQ) between wanderers and non-wanderers. Overall, 26.7% of children had reportedly eloped within the previous year, most commonly from public places. Children with ASD-only and ASD+ID/DD were more likely to have eloped than those with ID/DD-only. Across all groups, wanderers scored higher than non-wanderers on five out of six CSBQ subscales; they were more likely not to realize when there is danger, to have difficulty distinguishing between strangers and familiar people, to show sudden mood changes, to over-react to everything/everyone, to get angry quickly, to get lost easily, and to panic in new situations or if change occurs. Even after controlling for elopement history, parents of children in the ASD+ID/DD group were more likely than those in the other condition groups to report using physical or electronic measures to prevent wandering.     

Dolphin-Assisted Therapy with Parental Involvement for Children with Severe Disabilities: Further Evidence for a Family-Centered Theory for Effectiveness

Dolphin-assisted therapy has frequently been criticized for a lack of empirical results and theoretical foundation. In this study, results from an innovative dolphin-assisted therapy program for children with disabilities are presented. The therapeutic program is based on a sophisticated framework and developed with reference to previous findings about special education interventions. Parental involvement was integrated purposefully into the dolphin–child interaction to initiate an improvement in parent–child interactions. The effectiveness of this approach was supported in a controlled pretest-posttest design with matched samples. Pre-testing was conducted 4 weeks before dolphin-assisted therapy commenced, and post-testing occurred 4 weeks and 6 months after it finished. In total, 47 children with disabilities (Down syndrome, physical or mental retardation) and their parents and pedagogic-therapeutic staff participated in the study. The results of the parental questionnaires showed stable, positive changes in children's communicative abilities and social-emotional behavior and in parental quality of life, with mainly large effect sizes. These results exceed previously published effects from animal-assisted therapy and likewise of dolphin-assisted therapy. Data derived from staff questionnaires showed a moderate therapeutic effect on one of the three factors tested in relation to children's communicative abilities, but not on the social-emotional behavior of the child. Possible explanations for these findings are discussed.     

Frequency of Chromosomally-Integrated Human Herpesvirus 6 in Children with Acute Lymphoblastic Leukemia

Introduction

Human herpesvirus 6 (HHV-6) is a ubiquitous pathogen infecting nearly 100% of the human population. Of these individuals, between 0.2% and 1% of them carry chromosomally-integrated HHV-6 (ciHHV-6). The biological consequences of chromosomal integration by HHV-6 remain unknown.

Objective

To determine and compare the frequency of ciHHV-6 in children with acute lymphoblastic leukemia to healthy blood donors.

Methodology

A total of 293 DNA samples from children with pre-B (n = 255), pre-pre-B (n = 4), pre-T (n = 26) and undetermined (n = 8) leukemia were analyzed for ciHHV-6 by quantitative TaqMan PCR (QPCR) using HHV-6 specific primers and probe. As control, DNA samples from 288 healthy individuals were used. Primers and probe specific to the cellular GAPDH gene were used to estimate integrity and DNA content.

Results

Out of 293 DNA samples from the leukemic cohort, 287 contained amplifiable DNA. Of these, only 1 (0.35%) contained ciHHV-6. Variant typing indicates that the ci-HHV-6 corresponds to variant A. None of the 288 DNA samples from healthy individuals contained ciHHV-6.

Conclusion

The frequency of ciHHV-6 in children with acute lymphoblastic leukemia is similar (p = 0.5) to that of healthy individuals. These results suggest that acute lymphoblastic leukemia does not originate as a consequence to integration of HHV-6 within the chromosomes.