Student-directed learning: A catalyst for academic achievement and self-determination for students with intellectual disability.

In this single-case experimental design study, eight adolescents with mild intellectual disability (ID) participated in a 3-month intervention with the Self-Determined Learning Model of Instruction. Findings indicate that student-directed learning may enable students with ID to increase their academic achievements, and the authors explore how this may lead to enhanced self-determination over time. Further data analysis suggests that student-directed learning first of all may have an impact at the level of the environment, such that teachers start to perceive their students with ID as capable agents who can take an active role in their own learning process. This change in teacher perception may lead to students getting more opportunities to practice and refine self-determination skills, which in turn may lead to increased capacity for self-determination. Findings from this study are uplifting, as even brief student-directed learning interventions may trigger positive effects on students' self-determination.

Adams-Oliver syndrome review of the literature: Refining the diagnostic phenotype.

The Adams-Oliver syndrome (AOS) is defined as aplasia cutis congenita (ACC) with transverse terminal limb defects (TTLD). Frequencies of associated anomalies are not well characterized. Six causative genes have been identified: ARHGAP31, DOCK6, EOGT, RBPJ, NOTCH1, and DLL4. We review 385 previously described individuals (139 non-familial and 246 familial probands and family members) and add clinical data on 13 previously unreported individuals with AOS. In addition to ACC and TTLD, the most commonly associated anomalies included a wide variety of central nervous system (CNS) anomalies and congenital heart defects each seen in 23%. CNS anomalies included structural anomalies, microcephaly, vascular defects, and vascular sequelae. CNS migration defects were common. Cutis marmorata telangiectasia congenita (CMTC) was found in 19% of the study population and other vascular anomalies were seen in 14%. Hemorrhage was listed as the cause of death for five of 25 deaths reported. A relatively large number of non-familial probands were reported to have hepatoportal sclerosis with portal hypertension and esophageal varices. Non-familial probands were more likely to have additional anomalies than were familial probands. The data reported herein provide a basis for refining the diagnostic features of AOS and suggest management recommendations for probands newly diagnosed with AOS. © 2017 Wiley Periodicals, Inc.

Consumer involvement in cancer research: example from a Cancer Network.

BACKGROUND: The involvement of consumers and the general public in improving cancer services is an important component of health services. However, consumer involvement in cancer research is relatively unexplored. The objective of this study was to explore different ways of involving consumers in cancer research in one regional network. METHODS: Thames Valley Cancer Network Consumer Research Partnership (CRP) group was formed in 2009. The group consists of consumers and professionals to help in promoting consumer involvement in Cancer Research in the Thames Valley. This study evaluated the project of consumer involvement in cancer research in the Thames Valley from March 2010 to March 2011. We used different indices to judge the level of consumer involvement: number of projects involving consumers through the group, types of projects, level of involvement (ranged from consultation on research documents to collaborating in preparing grant applications) and the methods of involving consumers in cancer research. RESULTS: Fifteen projects were submitted to the CRP group during the 12-month period studied. Of these, eight projects were clinical trials, three were qualitative research projects, two were patients' surveys and two were non-randomized interventional studies. Seven projects requested consumer involvement on patient information sheets for clinical trials. Of these seven applications, three also requested consumers' help in designing research questionnaires and another three requested that consumers should be involved in their project management group. In addition, four projects involved consumers in the proposal development phase and another four projects asked for advice on how to increase trial recruitment, conduct patient interviews or help with grant applications. CONCLUSIONS: The creation of the CRP and this audit of its activity have documented consumer involvement in cancer research in the Thames Valley. We have clearly shown that consumers can be involved in designing and managing cancer research projects.

Lesion segmentation from multimodal MRI using random forest following ischemic stroke.

Understanding structure-function relationships in the brain after stroke is reliant not only on the accurate anatomical delineation of the focal ischemic lesion, but also on previous infarcts, remote changes and the presence of white matter hyperintensities. The robust definition of primary stroke boundaries and secondary brain lesions will have significant impact on investigation of brain-behavior relationships and lesion volume correlations with clinical measures after stroke. Here we present an automated approach to identify chronic ischemic infarcts in addition to other white matter pathologies, that may be used to aid the development of post-stroke management strategies. Our approach uses Bayesian-Markov Random Field (MRF) classification to segment probable lesion volumes present on fluid attenuated inversion recovery (FLAIR) MRI. Thereafter, a random forest classification of the information from multimodal (T1-weighted, T2-weighted, FLAIR, and apparent diffusion coefficient (ADC)) MRI images and other context-aware features (within the probable lesion areas) was used to extract areas with high likelihood of being classified as lesions. The final segmentation of the lesion was obtained by thresholding the random forest probabilistic maps. The accuracy of the automated lesion delineation method was assessed in a total of 36 patients (24 male, 12 female, mean age: 64.57±14.23yrs) at 3months after stroke onset and compared with manually segmented lesion volumes by an expert. Accuracy assessment of the automated lesion identification method was performed using the commonly used evaluation metrics. The mean sensitivity of segmentation was measured to be 0.53±0.13 with a mean positive predictive value of 0.75±0.18. The mean lesion volume difference was observed to be 32.32%±21.643% with a high Pearson's correlation of r=0.76 (p<0.0001). The lesion overlap accuracy was measured in terms of Dice similarity coefficient with a mean of 0.60±0.12, while the contour accuracy was observed with a mean surface distance of 3.06mm±3.17mm. The results signify that our method was successful in identifying most of the lesion areas in FLAIR with a low false positive rate.

A national approach to improving adolescent and young adult (AYA) oncology psychosocial care: the development of AYA-specific psychosocial assessment and care tools.

OBJECTIVE: Age-based screening tools and assessment measures are crucial to the provision of best practice care for adolescent and young adult (AYA) cancer patients. Unfortunately, there are limited psychosocial tools developed for this age group and pediatric or adult measures are often distributed with the assumption that they are "close enough." We describe a collaborative Australian project that strives to improve what currently exists for the psychosocial assessment of and planning for the 15-25-year-old age group. METHOD: Using the National Comprehensive Cancer Network's Distress Thermometer and the HEADSS Assessment as a foundation, the tools were developed in consultation with Australia's leading AYA clinicians, AYAs currently undergoing medical treatment, and a group of AYA survivors through a process of discussion groups and clinical interviews. RESULTS: The result is the first available AYA-specific screening tool, care plan pro forma, and psychosocial assessment measure developed for use within the oncology sector. These new tools will assist clinicians working with this population group to support psychosocial coping during active treatment and promote healthy post-treatment survivorship. SIGNIFICANCE OF RESULTS: Over time, further validation procedures will add to the veracity of the measures. Until then, these measures represent the best available in Australia. They highlight a clinically recognized minimum standard of care that all young cancer patients, regardless of treatment location, have the right to receive.

Establishing a Causal Relationship between Interventions to Promote Self-Determination and Enhanced Student Self-Determination.

Promoting the self-determination of adolescents with disabilities has become best practice in secondary education and transition services, but to date there have been no studies establishing a causal relationship between efforts to promote self-determination and enhancement of the self-determination of youth with disabilities. This article reports a randomized trial, placebo control group study of 371 high school students receiving special education services under the categorical areas of mental retardation or learning disabilities. Students were randomly assigned to an intervention or control group (by high school campus), with students in the intervention condition receiving multiple instructional components to promote self-determination. Latent growth curve analysis showed that although all students in the study showed improved self-determination over the three years of the study, students in the intervention group showed significantly greater growth, though specific intra-individual variables impacted this growth. Implications for research and intervention are discussed.

Evaluation of a supportive care app for coordinating caring networks: an analysis of the first 19,000 users.

Background: Major illnesses such as cancer, and other traumatic life events, can lead to sudden increases in supportive care needs. This study aimed to describe engagement, acceptability and satisfaction with a supportive care networking app under real-world conditions. Methods: A total of 10,952 individuals used the app during the study period (2018-2022). The app is designed to enable "captains" to assemble a network of friends and family members to provide timely, and individually tailored, supportive care (including assistance with tasks such as taking children to school, cooking meals, grocery shopping, and transport to appointments). Engagement was determined from server data, whilst acceptability and satisfaction were captured using purposed-designed surveys. Results: Users were mostly female (76%) and aged between 30 and 49 years (61%). The most common reason for using the app was sudden illness (web: 81%; mobile: 64%). An average of 42 tasks were requested per network, with a 32% acceptance rate. Significantly more tasks were requested (web: 52.2 tasks per network; mobile: 31.7 tasks per network; p < 0.001) and accepted (web: 43.2%; mobile: 20.2%; p < 0.001) in the web app vs. the mobile app. Task requests in the web app most commonly related to food (43% of requested tasks), social (15% of requested tasks) and children (13% of requested tasks). The task acceptance rate differed by task categories (p < 0.001), with tasks relating to transport, medical appointments and children accepted at the highest rates (56%, 52% and 49%, respectively). Acceptability and satisfaction data suggested that the app was well received and overall, participants were satisfied with the app. Conclusion: Findings suggested that this support care networking app achieved widespread uptake for a wide variety of supportive care tasks. Future research focused on optimizing engagement with the mobile app and examining the effectiveness of the app for improving patient and hospital outcomes is warranted.

Parenting Styles and Practices in Enhancing Self-Determination of Children With Intellectual and Developmental Disabilities.

Parenting styles and practices are crucial in promoting the self-determination of children. The purpose of the current study was to investigate the role of parenting styles and practices in enhancing the self-determination of children with/without intellectual and developmental disabilities (IDD). The present study was carried out with a sample of 243 parents of children with/without IDD in Türkiye (Turkey). The results indicated that an authoritative parenting style and autonomy-supportive parenting practices positively affect the degree of child self-determination, whereas permissive and overprotective parenting practices may limit child opportunities in fostering self-determination. The study results also showed that urbanization, higher income, and higher education level of parents positively impacted the degree of child self-determination. Parents of typically developing children reported higher levels of overall self-determination for their typically developing children when compared with children with intellectual disability and autism spectrum disorder. On the other hand, parents of children with mild disabilities reported a higher level of self-determination than both children with moderate and severe disabilities. The results were discussed within the cultural context of the current sample.

The relationship between unmet needs and distress amongst young people with cancer.

PURPOSE: Most psychosocial research in cancer has been restricted to paediatric or older adult populations. This study aimed to explore psychological distress and unmet needs in adolescents and young adults (AYA) with cancer and identify predictors of distress among demographic and illness characteristics and supportive care needs. METHODS: Fifty-three patients between 16 and 30 years completed a cross-sectional survey, administered shortly after presentation to an AYA oncology service and within 4 months of diagnosis. Measures included the Beck Depression Inventory-Fast Screen (BDI-FS), State-Trait Anxiety Inventory-State Form (STAI-S) and the Supportive Care Needs Survey. Level of distress-related sypmtomatology in this population was based on previous work, whereby a cut-off score of 4 or greater was used for the BDI-FS, and one standard deviation above the sample population mean was used for the STAI-S. RESULTS: Prevalence of distress (25%) was lower than that found previously in AYA with cancer. Physical and daily living needs were the most frequently unmet needs overall, followed by psychological needs, health system and information needs and care and support needs. Lastly, being pre-treatment predicted increased depression and state anxiety, while having treatment post-surgery predicted reduced state anxiety. After controlling for treatment status, however, the main predictors of depression and state anxiety were physical and daily living needs and health system and information needs, respectively. CONCLUSIONS: Lower levels of distress and unmet psychological needs were related to the few participants (17%) in this study who were pre-treatment, when distress was most likely. However, physical needs and information needs, which are almost inevitable throughout treatment and beyond, were more important predictors of distress. Further exploration must consider the psychosocial difficulties underlying this association and the needs of AYA at transitions between critical periods in their cancer journey (i.e., upon diagnosis, during treatment, etc.).

Impact of the Self-Determined Learning Model of Instruction on Self-Determination: A Randomized-Trial Control Group Study.

Promoting self-determination has become best practice in special education. There remains, however, a paucity of causal evidence for interventions to promote self-determination. We conducted a group-randomized, modified equivalent control group design study of the efficacy of the Self-Determined Learning Model of Instruction to promote self-determination. Data on self-determination using multiple measures was collected with 312 high school students with cognitive disabilities in both a control and treatment group. We examined the relationship between the SDLMI and self-determination using structural equation modeling. After determining strong measurement invariance for each latent construct, we found significant differences in latent means across measurement occasions and differential effects attributable to the SDLMI. This was true across disability category, though there was variance across disability populations.

Effect of Intervention with the Self-Determined Learning Model of Instruction on Access and Goal Attainment.

Promoting student self-determination has been identified as best practice in special education and transition services and as a means to promote access to the general education curriculum for students with disabilities. There have been, however, limited evaluations of the effects of interventions to promote self-determination on outcomes related to access to the general education curriculum. This article reports finding from a cluster or group-randomized trial control group study examining the impact of intervention using the Self-Determined Learning Model of Instruction on student academic and transition goal attainment and on access to the general education curriculum for students with intellectual disability and learning disabilities Findings support the efficacy of the model for both goal attainment and access to the general education curriculum, though students varied in the patterns of goal attainment as a function of type of disability.

Examining Individual and Instruction-Related Predictors of the Self-Determination of Students with Disabilities: Multiple Regression Analyses.

This study examined individual and instructional predictors of the self-determination of students with disabilities, as measured by The Arc's Self-Determination Scale and the AIR Self-Determination Scale, Student version. The general findings indicated that instructional, knowledge and dispositional factors predicted students' self-determination over personal predictor variables. In particular, self-efficacy and outcome expectancy scores, student-directed transition planning instruction, and students' pre-intervention transition planning knowledge predicted higher students' self-determination.

Parental habitus in promoting self-determination of children with/without intellectual and developmental disabilities in Türkiye.

BACKGROUND: Enhancing the self-determination of children with intellectual and developmental disabilities (IDD) is a prominent factor in their daily, community, school, or post-school outcomes. Parental practices play a crucial role in promoting self-determination of children with IDD. Families worldwide engage in parenting practices determined by each family's beliefs and values filtered through cultural experiences related to the place of origin, social structure, and living area. AIMS: This study investigated the impact of parental habitus as structured within social and cultural capital on family ratings of child self-determination in two distinct regions of Turkey (Türkiye). Our assumption is that the gap in terms of social, economic, and cultural capital between different districts of the same country affects parental habitus in fostering their children's self-determination. METHOD: Researchers collected information from 232 family members regarding the degree of their children's self-determination in two different geographic areas of Türkiye. We used the American Institutes for Research (AIR) Self-Determination Scale - Parent Form (AIR-SDS-PF questionnaire and a socio-demographic form to collect data. We employed the univariate analysis (two-way ANOVA) to identify the main and interactional effect among variables. RESULTS: Parental habitus depending on where families live, socioeconomic level, and child's disability status was influential in promoting self-determination for their children with IDD and counterparts. CONCLUSIONS: Regional or micro-cultural differences impacting parental dispositions should be considered in developing or planning self-determination interventions for children with/without IDD in the same country.

The Community of Practice for Supporting Families of Persons With Intellectual and Developmental Disabilities.

It is important to continuously support families to improve the lives of people with intellectual and developmental disabilities and their family members. Using a life course approach to address strengths and needs of families, a National Community of Practice, infused with the Charting the LifeCourse framework, focused on systems change to improve policy and practices to enhance the lives of families. A qualitative analysis used the Value Creation framework to evaluate both process and product outcomes of 16 state communities of practice as to changes in knowledge and practices. Results emphasize the relative effectiveness of aspects of policy and overarching practices designed to support people with disabilities and their families in participating states.

Connections of the dorsomedial visual area: pathways for early integration of dorsal and ventral streams in extrastriate cortex.

The dorsomedial area (DM), a subdivision of extrastriate cortex characterized by heavy myelination and relative emphasis on peripheral vision, remains the least understood of the main targets of striate cortex (V1) projections in primates. Here we placed retrograde tracer injections encompassing the full extent of this area in marmoset monkeys, and performed quantitative analyses of the numerical strengths and laminar patterns of its afferent connections. We found that feedforward projections from V1 and from the second visual area (V2) account for over half of the inputs to DM, and that the vast majority of the remaining connections come from other topographically organized visual cortices. Extrastriate projections to DM originate in approximately equal proportions from adjacent medial occipitoparietal areas, from the superior temporal motion-sensitive complex centered on the middle temporal area (MT), and from ventral stream-associated areas. Feedback from the posterior parietal cortex and other association areas accounts for <10% of the connections. These results do not support the hypothesis that DM is specifically associated with a medial subcircuit of the dorsal stream, important for visuomotor integration. Instead, they suggest an early-stage visual-processing node capable of contributing across cortical streams, much as V1 and V2 do. Thus, although DM may be important for providing visual inputs for guided body movements (which often depend on information contained in peripheral vision), this area is also likely to participate in other functions that require integration across wide expanses of visual space, such as perception of self-motion and contour completion.

Rapid determination of C4-acylcarnitine and C5-acylcarnitine isomers in plasma and dried blood spots by UPLC-MS/MS as a second tier test following flow-injection MS/MS acylcarnitine profile analysis.

BACKGROUND: Flow-injection MS/MS methods for elevated acylcarnitines are routinely performed in most newborn screening and biochemical genetics laboratories; however this technique cannot distinguish between isobaric compounds; therefore, chromatographic separation is required to quantitate isomers for differential diagnosis of some inborn errors of metabolism. METHODS: A UPLC-MS/MS method has been developed for the simultaneous quantitation of isobutyrylcarnitine and butyrylcarnitine, and a second UPLC-MS/MS method for the quantitation of isovalerylcarnitine, (S) and (R) 2-methylbutyrylcarnitine, pivaloylcarnitine and valerylcarnitine. Plasma and dried blood spots samples are extracted with methanol and derivatized with butanolic HCl. Deuterium labeled internal standards are used for quantitation. Separation is obtained using a methanol/water gradient with a C18 BEH, 1x100mm, 1.7microm UPLC column, at 60 degrees C; run time is less than 10min. The isomers are detected with a Quattro Premier triple quadrupole, with electrospray ionization in positive ion mode. RESULTS: Intra-day precision in plasma and dried blood spots ranged from 1.4% to 14% and accuracy from 88% to 114% respectively for butyrylcarnitine and isobutyrylcarnitine. Precision for the isomers of C5-acylcarnitine ranged from 1.3% to 15% and accuracy 87% to 119%, respectively in plasma or dried blood spots. Inter-day precision was within 20% at each concentration of isobutyrylcarnitine and butyrylcarnitine. Precision for 2-methylbutyrylcarnitine and isovalerylcarnitine at concentrations above the normal range was within 24%. CONCLUSIONS: Two diagnostic tests based on the separation of C4-acylcarnitine and C5-acylcarnitine isomers by UPLC-MS/MS provide fast differential diagnosis of SCAD deficiency versus IBCD deficiency and IVA versus 2-MBCD deficiency. The separation of C5-acylcarnitines can reveal false elevation due to pivalic acid-containing antibiotics. Abnormal newborn screen results due to pivalate-generating prodrug antibiotics of maternal origin were confirmed. This separation of isomers can resolve multiple diagnostic challenges in both newborn screening and in cases with ambiguous metabolic test results.

Functional MRI interactions between dysplastic nodules and overlying cortex in periventricular nodular heterotopia.

Periventricular nodular heterotopia (PVNH) is a malformation of cortical development associated with epilepsy. It is unclear whether the epileptogenic focus is the nodule, overlying cortex, or both. We performed electroencephalography (EEG)-functional magnetic resonance imaging (fMRI) in a patient with bilateral PVNH, capturing 45 "left temporal" epileptiform discharges. The relative time at which fMRI-involved regions became active was assessed. Additionally, nodule-cortex interactions were explored using fMRI functional connectivity. There was EEG-fMRI activity in specific periventricular nodules and overlying cortex in the left temporoparietal region. In both nodules and cortex, the peak BOLD response to epileptiform events occurred earlier than expected from standard fMRI hemodynamic modeling. Functional connectivity showed nodule-cortex interactions to be strong in this region, even when the influence of fMRI activity fluctuations due to spiking was removed. Nonepileptogenic, contralateral nodules did not show connectivity with overlying cortex. EEG-fMRI and functional connectivity can help identify which of the multiple abnormal regions are epileptogenic in PVNH.

The psychometric validation of the Sheehan Disability Scale (SDS) in patients with bipolar disorder.

Bipolar disorder (BD) adversely affects daily activities/functioning. The Sheehan Disability Scale (SDS) assesses disability in work/school activities, family relationships, and social functioning, and it evaluates the functional impact of psychiatric disorders. BD outpatients from 21 U.S. sites completed a battery of validated instruments (including the SDS) three times over 8-12 weeks. Instrument reliability (internal consistency, test-retest), validity (construct, convergent validity, known groups) and responsiveness were measured. There were missing data for the SDS in 2% of the 225 subjects with BD. One factor explained 82% of the variance. All SDS items had rotated factor loadings on the first factor >0.90, confirming the appropriateness of the SDS total score. Item-scale correlations surpassed 0.40. There was excellent internal consistency reliability for the SDS total score (Cronbach's alpha=0.89). Test-retest reliability was acceptable for the SDS total score (intraclass correlation coefficient=0.73). Correlations with other instruments demonstrate convergent and divergent validity. The SDS total and item scores significantly discriminated between (self-rated) overall health status, clinician-rated functional status, and clinician-rated depression, evidencing known group validity. The SDS demonstrated ability to detect change over time. The SDS is a valid, reliable measure of disability and is responsive to change over time when used in subjects with BD.