Search

Reliable and valid cognitive outcome measures, including examiner-administered and computer-facilitated assessments of processing speed and reaction time, are necessary for future clinical trials that include individuals with Down syndrome (DS). The current study evaluated the score distributions and psychometric properties of four examiner-administered and three computerized processing speed and reaction time measures. Participants included 97 individuals with DS, aged 6 to 17 (M = 12.6, SD = 3.3). Two examiner-administered measures (Differential Ability Scales-II Rapid Naming and Cat/dog Stroop Congruent) met most predetermined psychometric criteria. Other assessments demonstrated good test-retest reliability and had negligible practice effects but lacked adequate feasibility. Recommendations for using processing speed and reaction time assessments in research and suggestions for modifications of measures are discussed.

Approximately one in every 700 babies in the United States is born with Down syndrome, or 0.14%. Children with Down syndrome have cognitive impairment and congenital malformations necessitating frequent occurrences of general anesthesia and surgery. The thoughtful perioperative care of children with Down syndrome is relevant and acutely complex for the pediatric anesthesiologist. Behavior, sedation, hypotonia, upper airway obstruction, venous access, and bradycardia are omnipresent concerns apart from the surgical pathology. Down syndrome is also associated with autonomic nervous system dysfunction, a comorbidity that is overlooked in discussions of perioperative care and is described thus far in adults. Autonomic nervous system function or dysfunction may explain the phenotypical features of the perioperative challenges listed above. For this reason, understanding the development and measurement of autonomic nervous system function is important for the pediatric anesthesiologist. Definition and quantification of sympathetic and parasympathetic function will be reviewed.

OBJECTIVE: This study aimed to evaluate ADHD symptom patterns among children with Down syndrome with or without ADHD as well as typically developing children with ADHD. METHOD: Parents and teachers rated symptoms of inattention, hyperactivity, and general behavioral concerns for 22 children with Down syndrome and comorbid diagnoses of ADHD (DS+ADHD), 66 gender- and age-matched children with Down syndrome with no diagnosis of ADHD (DS-ADHD), and 66 gender- and age-matched typically developing children with ADHD (TD+ADHD). Children with Down syndrome were recruited from the community. Typically developing children with ADHD were recruited from a speciality clinic evaluating for ADHD. RESULTS: Parents tended to report higher scores of inattention and hyperactivity for typically developing children with ADHD compared to children with Down syndrome and no ADHD. Although mean ADHD symptom summary scores were not significantly different in DS+ADHD and DS-ADHD, specific parent report items (e.g., distractibility and being “on the go”) did tend to differentiate these groups. In contrast, teachers tended to report higher inattention and hyperactivity scores for DS+ADHD compared to both DS-ADHD and TD+ADHD. Specific teacher-reported items tending to differentiate DS+ADHD and DS-ADHD included difficulties following through on tasks, avoiding tasks, leaving one’s seat, and excessive talking. CONCLUSION: Variability in response patterns between parent- and teacher-reports for children with and without Down syndrome highlight the need to evaluate ADHD symptoms across environments. Our findings also suggest specific items that may particularly helpful in distinguishing children with Down syndrome who do and do not have ADHD, although replication is needed.

Background There is a critical need for the psychometric evaluation of outcome measures to be used in clinical trials targeting cognition in Down syndrome (DS). This study examines a specific cognitive skill that is of particular importance in DS, working memory, and the psychometric properties of a set of standardised measurements to assess working memory in individuals with DS. Methods Ninety children and adolescents ages 6 to 18 years old with DS were assessed on a selection of verbal and visuospatial working memory subtests of standardised clinical assessments at two time points to examine feasibility, distributional qualities, test-retest reliability and convergent validity against a priori criteria. Caregivers also completed an adaptive behaviour questionnaire to address working memory subtests' associations with broader developmental functioning. Results The Stanford Binet-5 Verbal Working Memory, Differential Ability Scales-2 Recognition of Pictures, Stanford Binet-5 Nonverbal Working Memory and Wechsler Intelligence Scale for Children-5 Picture Span measures met the most psychometric criteria overall across the full age and IQ range of the study. Although Differential Ability Scales-2 Recall of Sequential Order and Differential Ability Scales-2 Recall of Digits Backward met the fewest a priori criteria, follow-up analyses suggested greater feasibility in specific age and IQ ranges. Conclusions Several working memory measures appear to be psychometrically sound and appropriate for use in clinical trials for children with DS, especially when focusing on raw scores. However, floor effects on standard scores and feasibility of some measures were problematic. Guidelines for use of the working memory subtests with this population are provided.

Children who are deaf and hard of hearing (DHH) often demonstrate pragmatic language difficulties which can impact academic and social outcomes. This randomized control trial for DHH children, ages 3–12 years, explored the Technology-Assisted Language Intervention (TALI), incorporating augmentative and alternative communication technology (AAC) into traditional speech/language therapy, compared to treatment-as-usual (TAU) to determine impact on pragmatics. Pragmatic outcome measures included parent reported Pragmatics Profile of the CELF-5 (for children age ≥5 years) and CELF-P Descriptive Pragmatics Profile (for children

OBJECTIVE Literacy skill development in deaf or hard of hearing (DHH) children is essential for success in school and beyond. Our objective was to evaluate the association between age of early intervention (EI) enrollment for DHH children and emergent literacy in preschool. METHODS This was a population-based study that leveraged state public health and education data on all children identified with hearing loss. The sample included children born between 2008 and 2014 enrolled in EI for hearing loss who received preschool supports (years 2011-2014) through the Ohio Department of Education. The Get it! Got it! Go!, measuring emergent literacy domains of picture naming, rhyming, and alliteration, was administered during preschool in fall and spring. Exposure was enrollment into EI before age 6 months (early) versus at/after 6 months (later). Propensity score matching and mixed model analyses were used to examine associations between EI enrollment and outcomes over time. Model least square means with 95% confidence intervals (CIs) were reported. RESULTS One hundred two successful matches were made for 256 preschoolers. Children enrolled in EI early had significantly higher mean scores (mean [95% CI]) over time for emerging literacy domains of picture naming (2.42 [0.47-4.37]), rhyming (1.2 [0.35-2.06]), and alliteration (0.61 [0.15-1.07]) compared with later enrolled children. Children enrolled early had significantly higher emergent literacy scores at entry, although literacy development was similar between groups. CONCLUSION Children enrolled in EI before age 6 months had consistently higher scores in emergent literacy components over time compared with children enrolled at/after age 6 months.

PURPOSE: There is a gap in knowledge regarding the use of emergency services by pediatric spina bifida patients. The goal of this study was to describe Emergency Department utilization patterns in this population. METHODS: Through a retrospective observational study, patients with spina bifida who visited the emergency department during a four-year period were identified; medical and demographic information was obtained though the Centers for Disease Control National Spina Bifida Patient Registry. Chief complaints and final diagnoses of visits were classified and related to medical needs of spina bifida to determine the appropriate care level. RESULTS: Among 303 children within the registry, 161 patients (53%) accounted for 579 visits. 70% of visits were for spina bifida-related complaints. Approximately half (51.7%) had a shunt-related chief complaint, although final diagnosis was largely unrelated to the shunt. Admission rate was 39%, higher than institutional baseline, and largely represented by genitourinary (GU) complaints. CONCLUSION: Pediatric patients with spina bifida presenting to a single center emergency department were most likely to present with shunt and urinary concerns; these patients were most likely to be admitted. This potentially suggests that improving outpatient care for bladder management may decrease emergency department use among this population.

We quantified the intensity of early intervention (EI) services allocated to 1262 children who were deaf or hard of hearing (DHH) within a state program and identified factors associated with intervention intensity. Child specific data were collected on children born between 2008 and 2014. Data from Individualized Family Service Plans of children enrolled in Part C EI programming were evaluated for the type and duration of services during their EI enrollment. Associations between EI intensity and child/family variables were examined. Median age of EI enrollment was 5.3 months. The most frequently received services included primary service coordination, specialized DHH service, special instruction, language therapy, and family training; 60% of children received 4 or more different EI services. The median service intensity was 138.1 min per month across all EI years. The factors associated with higher EI intensity included severe hearing loss, bilateral hearing loss and presence of a disability. Children enrolled in EI at later ages received higher intensity of specialized DHH services, suggesting a need to “catch up” due to late acquisition of services. Evaluating EI service intensity broadens our understanding of effective components of state-based programs that support the developmental needs of children who are DHH.

Sleep problems have a bi-directional impact on the daytime performance of children, parental well-being, and overall family functioning in the general population. Children with Down syndrome (DS) are at a high risk of sleep problems, yet the relationship between sleep problems, adaptive functioning, and family stress in children with DS is not well documented. We examined the relationship between sleep (i.e., duration and quality) and child and parent/family functioning. Sixty-six children with DS wore an actigraph for a week to assess their sleep duration and sleep efficiency. Their parents completed ratings on child sleep duration and parasomnias, child adaptive functioning, parental depression and sleep, and family stress. The parents’ reports of their children’s sleep duration were associated with parental depressive symptoms. The parents’ reports of their children’s restless sleep behaviors were associated with poorer performances in child-compliant/calm behaviors, worse parental sleep, and negative parental feelings and sibling relationships. The findings from actigraph measures of the children’s sleep demonstrated that greater sleep efficiency was associated with greater child adaptive functioning and fewer parental depressive symptoms. The study findings provide preliminary evidence that sleep problems are related to child adaptive functioning, parental functioning, and family stress in children with DS.

OBJECTIVES. To compare the percentage and mean age of children with Down syndrome (DS) who underwent polysomnography (PSG) to evaluate for obstructive sleep apnea (OSA) before and after the introduction of the American Academy of Pediatrics guidelines recommending universal screening by age 4 years. STUDY DESIGN. Retrospective cohort study. SETTING. Single tertiary pediatric hospital. METHODS. This study is a review of patients with DS seen in a subspecialty clinic. Children born preguidelines (2000-2006) were compared with children born postguidelines (2007-2012) regarding percentage receiving PSG, age at first PSG, and rate of OSA. RESULTS. We included 766 children with DS; 306 (40%) were born preguidelines. Overall, 61% (n = 467) underwent PSG, with a mean ± SD age of 4.2 ± 2.9 years at first PSG; 341 (44.5%) underwent first PSG by age 4 years. The rate of OSA (obstructive index ≥1 event/hour) among children undergoing first PSG was 78.2%. No difference was seen in the percentage receiving PSG preguidelines (63.4%) versus postguidelines (59.4%, P = .26). The mean age at the time of first PSG was 5.3 ± 3.5 years preguidelines versus 3.4 ± 2.0 years postguidelines (P < .0001). Children in the postguidelines cohort were more likely to undergo first PSG during the ages of 1 through 4 years (67.4% vs 52.1%, P < .0001). There was no difference in rates of OSA between the pre- and postguidelines cohorts (79.8% vs 75.9%, P = .32). CONCLUSIONS. Nearly two-thirds of children with DS (61%) underwent PSG overall, with a significant shift toward completion of PSG at an earlier age after the introduction of the American Academy of Pediatrics guidelines for universal screening for OSA.

OBJECTIVES: To determine if neurodevelopmental deficits in children with single-ventricle physiology change with age and early developmental scores predict 6-year outcomes. METHODS: In the Single Ventricle Reconstruction Trial, Bayley Scales of Infant Development, Second Edition, were administered at 14 months of age, and parents completed the Behavior Assessment System for Children, Second Edition (BASC-2) annually from the ages of 2 to 6 years. Scores were classified as average, at risk, or impaired. We calculated sensitivities, specificities, and positive and negative predictive values of earlier tests on 6-year outcomes. RESULTS: Of 291 eligible participants, 244 (84%) completed the BASC-2 at 6 years; more Single Ventricle Reconstruction participants than expected on the basis of normative data scored at risk or impaired on the BASC-2 Adaptive Skills Index at that evaluation (28.7% vs 15.9%; P < .001). Children with Adaptive Skills Composite scores

OBJECTIVES. To compare the demographic and clinical characteristics of children with Down syndrome who did and did not receive polysomnography to evaluate for obstructive sleep apnea after publication of the American Academy of Pediatrics’ guidelines recommending universal screening by age 4 years. STUDY DESIGN. Retrospective cohort study. SETTING. Single tertiary pediatric hospital. METHODS. Review was conducted of children with Down syndrome born between 2007 and 2012. Children who obtained polysomnography were compared with children who did not, regarding demographic data, socioeconomic status, and comorbidities. RESULTS. We included 460 children with Down syndrome; 273 (59.3%) received at least 1 polysomnogram, with a median age of 3.6 years (range, 0.1–8.9 years). There was no difference in the distribution of sex, insurance status, or socioeconomic status between children who received polysomnography and those who did not. There was a significant difference in race distribution (P = .0004) and distance from home to the medical center (P < .0001) between groups. Among multiple medical comorbidities, only children with a history of hypothyroidism (P = .003) or pulmonary aspiration (P = .01) were significantly more likely to have obtained polysomnography. CONCLUSIONS. Overall, 60% of children with Down syndrome obtained a polysomnogram. There was no difference between groups by payer status or socioeconomic status. A significant difference in race distribution was noted. Proximity to the medical center and increased medical need appear to be associated with increased likelihood of obtaining a polysomnogram. This study illustrates the need for improvement initiatives to increase the proportion of patients receiving guideline-based screening.

CHARGE syndrome (OMIM 214800) is a condition characterized by multisystem involvement with CHD7 pathogenic mutations leading to disease in the majority of patients. Discovery of the molecular cause of CHARGE unmasked a larger phenotypic spectrum than was previously appreciated. Within our interdisciplinary CHARGE syndrome program, we sought to characterize our CHD7-positive CHARGE cohort without coloboma or choanal atresia, highlighting complications and outcomes. We describe 18 individuals with CHD7-confirmed diagnosis from 15 families. The most sensitive finding in the cohort was temporal bone malformations, present in 13/15 individuals. Individuals had an average of 1.6 major features and 3.3 minor features defined by the Blake et al. guidelines. Despite lack of major features or major malformations, the majority of individuals continued to have difficulties with pneumonia, aspiration, secretion management and motility issues that greatly impacted their lives. Our findings illustrate the need for molecular testing and timely recognition given that the major co-morbidities are frequently experienced by patients with the mildest clinical spectrum of CHARGE syndrome.

What constitutes a ‘quality’ college intern? Interning is an integral part of the student experience at George Washington University. Although the benefits of interning are well known, we know less about the qualities and skills that make a desirable intern. In this research, we examine what internship sponsors desire in a George Washington University political science student intern. We analyze quantitative and qualitative sponsor evaluations of the student intern’s performance looking at dependability, work ethic, attendance, usefulness to the organization, and level of initiative. The analysis is done by the intern’s gender and by the organizational category. Through this research, we offer a greater understanding of the role of internships to undergraduate education, as well as provide insight into interns’ performance while completing their internships.

The type 2 lysine methyltransferases KMT2C and KMT2D are large, enzymatically active scaffold proteins that form the core of nuclear regulatory structures known as KMT2C/D COMPASS complexes (complex of proteins associating with Set1). These evolutionarily conserved proteins regulate DNA promoter and enhancer elements, modulating the activity of diverse cell types critical for embryonic morphogenesis, central nervous system development, and post-natal survival. KMT2C/D COMPASS complexes and their binding partners enhance active gene expression of specific loci via the targeted modification of histone-3 tail residues, in general promoting active euchromatic conformations. Over the last 20 years, mutations in five key COMPASS complex genes have been linked to three human congenital syndromes: Kabuki syndrome (type 1 [KMT2D] and 2 [KDM6A]), Rubinstein-Taybi syndrome (type 1 [CBP] and 2 [EP300]), and Kleefstra syndrome type 2 (KMT2C). Here, we review the composition and biochemical function of the KMT2 complexes. The specific cellular and embryonic roles of the KMT2C/D COMPASS complex are highlight with a focus on clinically relevant mechanisms sensitive to haploinsufficiency. The phenotypic similarities and differences between the members of this new family of disorders are outlined and emerging therapeutic strategies are detailed.

The type 2 lysine methyltransferases KMT2C and KMT2D are large, enzymatically active scaffold proteins that form the core of nuclear regulatory structures known as KMT2C/D COMPASS complexes (complex of proteins associating with Set1). These evolutionarily conserved proteins regulate DNA promoter and enhancer elements, modulating the activity of diverse cell types critical for embryonic morphogenesis, central nervous system development, and post-natal survival. KMT2C/D COMPASS complexes and their binding partners enhance active gene expression of specific loci via the targeted modification of histone-3 tail residues, in general promoting active euchromatic conformations. Over the last 20 years, mutations in five key COMPASS complex genes have been linked to three human congenital syndromes: Kabuki syndrome (type 1 [KMT2D] and 2 [KDM6A]), Rubinstein-Taybi syndrome (type 1 [CBP] and 2 [EP300]), and Kleefstra syndrome type 2 (KMT2C). Here, we review the composition and biochemical function of the KMT2 complexes. The specific cellular and embryonic roles of the KMT2C/D COMPASS complex are highlight with a focus on clinically relevant mechanisms sensitive to haploinsufficiency. The phenotypic similarities and differences between the members of this new family of disorders are outlined and emerging therapeutic strategies are detailed.

BACKGROUND: Children who are deaf or hard of hearing (D/HH) have improved language outcomes when enrolled in early intervention (EI) before the age of 6 months. Little is understood about the long-term impact of EI on outcomes of kindergarten readiness (K-readiness). The study objective was to evaluate the impact of EI before the age of 6 months (early) versus after 6 months (later) on K-readiness in children who are D/HH. METHODS: In this study, we leveraged data from the Ohio Early Hearing Detection and Intervention Data Linkage Project, which linked records of 1746 infants identified with permanent hearing loss born from 2008 to 2014 across 3 Ohio state agencies; 417 had kindergarten records. The Kindergarten Readiness Assessment was used to identify children as ready for kindergarten; 385 had Kindergarten Readiness Assessment scores available. Multiple logistic regression was used to investigate the relationship between K-readiness and early EI entry while controlling for confounders (eg, hearing loss severity and disability status). RESULTS: Children who were D/HH and entered EI early (n = 222; 57.7% of the cohort) were more likely to demonstrate K-readiness compared with children who entered EI later (33.8% vs 20.9%; P = .005). Children who entered early had similar levels of K-readiness as all Ohio students (39.9%). After controlling for confounders, children who entered EI early were more likely to be ready for kindergarten compared with children who entered later (odds ratio: 2.02; 95% confidence interval 1.18–3.45). CONCLUSIONS: These findings support the sustained effects of early EI services on early educational outcomes among children who are D/HH. EI entry before the age of 6 months may establish healthy trajectories of early childhood development, reducing the risk for later academic struggles.

(1) To examine language performance in the context of cognitive abilities in young children who are deaf or hard-of-hearing and (2) to identify factors associated with having a language underperformance, defined as a gap between the language standard score and the nonverbal IQ (NVIQ) standard score.Children 6 to 82 months of age with bilateral hearing loss were enrolled. Language performance was defined as a ratio of language skills relative to cognitive abilities with language underperformance defined as a ratio of language scores to NVIQ0.85.Among 149 children, approximately half had hearing loss that was clinically classified as mild or moderate, and over one-third received a cochlear implant. Participants had a mean NVIQ in the average range (95.4 [20.3]). Receptive language scores were significantly lower than their NVIQ by 10.6 points (p.0001). Among children with NVIQs 80 to 100, 62.5% had receptive scores85 and 50% had a language underperformance (ratio0.85). Among children with NIVQs100, 21.1% had receptive scores85 with 42% having a language underperformance. Children with language underperformance (n = 61, 41.5%) were more likely to have more severe levels of hearing loss, lower socioeconomic status, and be nonwhite.Many children early identified with hearing loss continue to demonstrate language underperformance, defined using their cognitive potential. Language deficits have a cascading effect on social functioning in children who are deaf or hard-of-hearing. This study highlights the need to understand a child's cognitive potential to adequately address language needs in existing intervention models.

Despite the clinical significance of balanced chromosomal abnormalities (BCAs), their characterization has largely been restricted to cytogenetic resolution. We explored the landscape of BCAs at nucleotide resolution in 273 subjects with a spectrum of congenital anomalies. Whole-genome sequencing revised 93% of karyotypes and demonstrated complexity that was cryptic to karyotyping in 21% of BCAs, highlighting the limitations of conventional cytogenetic approaches. At least 33.9% of BCAs resulted in gene disruption that likely contributed to the developmental phenotype, 5.2% were associated with pathogenic genomic imbalances, and 7.3% disrupted topologically associated domains (TADs) encompassing known syndromic loci. Remarkably, BCA breakpoints in eight subjects altered a single TAD encompassing MEF2C, a known driver of 5q14.3 microdeletion syndrome, resulting in decreased MEF2C expression. We propose that sequence-level resolution dramatically improves prediction of clinical outcomes for balanced rearrangements and provides insight into new pathogenic mechanisms, such as altered regulation due to changes in chromosome topology. info:eu-repo/semantics/publishedVersion

Objective. To examine the association of intracranial radiographic abnormalities and developmental measures with outcomes in children with congenital symptomatic cytomegalovirus (CMV) and cochlear implants (CI). Design/Methods. It was a retrospective review of 15 children implanted from 2004 to 2010. Preimplant nonverbal intelligence quotient/developmental quotient (IQ/DQ) and head circumference (HC) were obtained. Computed tomography and magnetic resonance imaging of the brain and post-CI audiometry and language assessments were reviewed. Results. Eleven children (73%) had cognitive delay. Most had >1 developmental disability. Median IQ/DQ was 65 (23–90). All had imaging abnormalities. Most imaging abnormalities were in parietal (60%) and temporal (60%) lobes. Children with HC < 5th percentile had poorer median post-CI PTA (38 dB versus 27 dB, ). Periventricular calcifications were associated with lower receptive (, ) and expressive (, ) language. Because IQ/DQ was associated with periventricular calcifications (, ) and small HC (, ), their relationships with language appear partially driven by IQ/DQ. Conclusions. The location of brain abnormalities appears to correlate with worse outcomes after CI. These findings may allow for more accurate counseling of parents regarding anticipated postimplantation performance.

BACKGROUND: Newborn hearing screening has a high participation rate of ∼97% of infants nationally, but a high lost to follow-up of ∼32% limits the effectiveness of the program. This study tested an intervention of targeted outpatient rescreening of infants through collaboration with the Women, Infants, and Children (WIC) program to improve follow-up rates for newborn hearing screen referrals. METHODS: Controlled intervention study of WIC-eligible infants who referred on newborn hearing screens at target hospitals. Hearing rescreens were performed by using screening auditory brainstem response testing by trained research assistants, coordinated with the infant’s WIC appointment. Loss to follow-up rates and age at follow-up were compared with non-WIC infants tracked via the Ohio Department of Health during the same time periods at the same hospitals and at nonintervention hospitals. RESULTS: During a 2-year period, there were 1493 hearing screen referrals at 6 hospitals in the Cincinnati region recorded by the Ohio Department of Health. Of these, 260 WIC-eligible infants were referred to the study. Among WIC-eligible intervention infants, the lost to follow-up rate over 2 years was 9.6%, compared with 28.7% for nonintervention infants in the same hospitals and 18.1% for nonintervention hospitals. The average age of hearing confirmation for the WIC intervention group was 34.8 days, compared with 63.6 days in non-WIC infants. One-third of mothers reported barriers to follow-up. CONCLUSIONS: Collaborating with WIC to provide targeted follow-up for newborn hearing screening improved loss to follow-up rates, decreased the age at hearing confirmation by 1 month, and addressed reported care barriers.

Hearing loss (HL) is the most prevalent sensory defect affecting 1 in 500 neonates. Genetic factors are involved in half of the cases. The extreme heterogeneity of HL makes it difficult to analyze and determine the accurate genetic causes of the impairment. Up to now, 10 genes, namely, GJB2, GJB6, SLC26A4, TECTA, PJVK, Col11A2, Myo15A, TMC1, RDX and microRNA (miR-183), have been studied in an Iranian population. The prevalence of HL in Iran was estimated to be 2-3 times higher than that in other parts of the world. Here, the most common bases of congenital nonsyndromic hearing loss (NSHL) are discussed. We reviewed GJB2, GJB6 (large deletion), TECTA, SLC26A4 and PEJVK mutations, and studied their frequencies and distributions in different ethnic groups in 1934, 500, 121, 80 and 34 unrelated families throughout Iran, respectively. GJB2 mutation was the most common factor causing NSHL, with a mean frequency of 18.17% in the Iranian population. The importance of Iran's geographical location in the migration pathway from west to east through the silk route was also highlighted. SLC26A4 and TECTA mutations were the second and third main reasons of HL and accounted for up to 10 and 4% of prelingual HL in Iran, respectively. Mutations in GJB2, SLC26, TECTA and PJVK genes have an important role in HL in Iran and a screening test should be generated for better intervention and diagnosis programs.

Understanding a care coordination framework, its functions, and its effects on children and families is critical for patients and families themselves, as well as for pediatricians, pediatric medical subspecialists/surgical specialists, and anyone providing services to children and families. Care coordination is an essential element of a transformed American health care delivery system that emphasizes optimal quality and cost outcomes, addresses family-centered care, and calls for partnership across various settings and communities. High-quality, cost-effective health care requires that the delivery system include elements for the provision of services supporting the coordination of care across settings and professionals. This requirement of supporting coordination of care is generally true for health systems providing care for all children and youth but especially for those with special health care needs. At the foundation of an efficient and effective system of care delivery is the patient-/family-centered medical home. From its inception, the medical home has had care coordination as a core element. In general, optimal outcomes for children and youth, especially those with special health care needs, require interfacing among multiple care systems and individuals, including the following: medical, social, and behavioral professionals; the educational system; payers; medical equipment providers; home care agencies; advocacy groups; needed supportive therapies/services; and families. Coordination of care across settings permits an integration of services that is centered on the comprehensive needs of the patient and family, leading to decreased health care costs, reduction in fragmented care, and improvement in the patient/family experience of care.

Little is known about children who are deaf or hard of hearing (D/HH) with a coexisting autism spectrum disorder (ASD). The objective of our study was to understand the needs of children who are D/HH with coexisting ASD. We posed questions for group discussion about diagnostic process, impact of dual diagnosis on communication, and helpful resources. Four parents of three children participated. Challenges in the diagnostic process included the challenges in the appropriateness of the evaluation tools and the limited expertise of the professionals performing the evaluations. Broad-based special educational settings were perceived as helpful. Families described a range of broad-based communication strategies (spoken, sign, and written language, augmentative communication approaches). Families prioritized a focus on behavior and day-to-day functioning over academic performance. Families recognized the lack of professionals who understand ASD and deafness but have found the internet and technology as a mechanism to connect to information and families with similar needs.

The ISA S84 and IEC 1508/1511 standards, along with the AIChE CCPS Guidelines on safety instrumented (interlock) systems are performance oriented, not prescriptive. They do not tell people what logic system to use, what field device configuration to use, or how often to test a system. They merely list the performance requirements for the system. In other words, the greater the level of risk of the process, the greater the performance needed of the safety system. These standards, along with OSHA PSM requirements, state companies need to “determine and document that equipment is designed, maintained, inspected, tested and operating in a safe manner”. Engineering tools are currently available which can model (i.e. determine and document) the performance of different types of systems. When one quantifies the performance of the overall system, from sensor to final element, one quickly comes to the conclusion that the valves represent the “weak link” in most of today’s systems. The typical failure mode of discrete shutoff valves is being stuck. The only way to test for such a condition is to stroke the valve, but closing the valve completely and stopping production is not desirable. One does not need, however, to fully stroke the valve in order to test its functionality. If one were able to partially stroke the valve (in a simple, reliable, and secure manner), on-line, without stopping production, a dramatic improvement in safety can result. When one quantifies the safety impact of this test method, the result is typically an improvement by one order of magnitude.