Palliative Care Consultation is Associated with Decreased Rates of In-Hospital Mortality Among Patients Undergoing Major Amputation.

BACKGROUND: Despite advancements in medical care and surgical techniques, major amputation continues to be associated with risks for morbidity and mortality. Palliative care programs may help alleviate symptoms and align patients' goals and the care they receive with their treatment plan. Access to specialty palliative medicine among vascular surgery patients is limited. Here, we aim to describe utilization and impact of formal palliative care consultation for patients receiving major amputations. METHODS: This is a retrospective, secondary data analysis project examining the records of patients who received major amputations by the vascular surgery team between 2016 and 2021. Demographics, operative, and postoperative outcomes were recorded. The primary outcome variable was palliative care consultation during index admission (the admission in which the patient received their first major amputation). Secondary outcomes were in-hospital mortality and code status at the time of death, if death occurred during the index admission, location of death, and discharge destination. RESULTS: The cohort comprised of 292 patients (39% female, 53% Black, mean age 63), who received a lower extremity major amputation. Most patients (65%) underwent amputation for limb ischemia. One-year mortality after first major amputation was 29%. Average length of stay was 20 days. Thirty-five (12%) patients received a palliative care consultation during the hospitalization in which they received their first major amputation. On multivariable analysis, patients were more likely to receive a palliative care consult during their index admission if they had undergone a thorough knee amputation (OR = 2.89, P = 0.039) or acute limb ischemia (OR = 4.25, P = 0.005). A formal palliative care consult was associated with lower likelihood of in-hospital death and increased likelihood of discharge to hospice (OR = 0.248, P = 0.0167, OR = 1.283, P < 0.001).There were no statistically significant differences in the code status of patients who received a palliative care consultation. CONCLUSIONS: In a large academic medical center, palliative medicine consultation was associated with lower in-hospital mortality among patients with advanced vascular disease and major limb amputation. These data will hopefully stimulate much needed prospective research to develop and test tools to identify patients in need and derive evidence about the impact of palliative care services.

Dendrimer-N-acetyl-L-cysteine modulates monophagocytic response in adrenoleukodystrophy.

OBJECTIVE: X-linked adrenoleukodystrophy (ALD) is a neurodegenerative disorder due to mutations in the peroxisomal very long-chain fatty acyl-CoA transporter, ABCD1, with limited therapeutic options. ALD may manifest in a slowly progressive adrenomyeloneuropathy (AMN) phenotype, or switch to rapid inflammatory demyelinating cerebral disease (cALD), in which microglia have been shown to play a pathophysiological role. The aim of this study was to determine the role of patient phenotype in the immune response of ex vivo monophagocytic cells to stimulation, and to evaluate the efficacy of polyamidoamine dendrimer conjugated to the antioxidant precursor N-acetyl-cysteine (NAC) in modulating this immune response. METHODS: Human monophagocytic cells were derived from fresh whole blood, from healthy (n = 4), heterozygote carrier (n = 4), AMN (n = 7), and cALD (n = 4) patients. Cells were exposed to very long-chain fatty acids (VLCFAs; C24:0 and C26:0) and treated with dendrimer-NAC (D-NAC). RESULTS: Ex vivo exposure to VLCFAs significantly increased tumor necrosis factor α (TNFα) and glutamate secretion from cALD patient macrophages. Additionally, a significant reduction in total intracellular glutathione was observed in cALD patient cells. D-NAC treatment dose-dependently reduced TNFα and glutamate secretion and replenished total intracellular glutathione levels in cALD patient macrophages, more efficiently than NAC. Similarly, D-NAC treatment decreased glutamate secretion in AMN patient cells. INTERPRETATION: ALD phenotypes display unique inflammatory profiles in response to VLCFA stimulation, and therefore ex vivo monophagocytic cells may provide a novel test bed for therapeutic agents. Based on our findings, D-NAC may be a viable therapeutic strategy for the treatment of cALD. Ann Neurol 2018;84:452-462.

Examining the Transition from Child to Adult Care in Chronic Kidney Disease: An Open Exploratory Approach.

For youth with chronic kidney disease (CKD) and their families, shifting from pediatric to adult systems of renal care can be challenging. This study explored the transitional process experienced by youth with CKD and their families, including perceived facilitators and barriers to effective transition. Qualitative interviews were conducted with youth with CKD (n=28) and their parents (n=28). Ambiguity regarding healthcare provider roles within the adult system was frequently reported. Themes reflected parental challenge relinquishing care responsibility, synergistic and divergent expectations between youth and parents, tensions in youth self-care readiness, desired healthcare provider roles in transition preparedness, and system considerations in transition. A "learning stage" was recommended in which youth experienced the adult system while supported by known pediatric team members.

Epilepsy: Transition from pediatric to adult care. Recommendations of the Ontario epilepsy implementation task force.

The transition from a pediatric to adult health care system is challenging for many youths with epilepsy and their families. Recently, the Ministry of Health and Long-Term Care of the Province of Ontario, Canada, created a transition working group (TWG) to develop recommendations for the transition process for patients with epilepsy in the Province of Ontario. Herein we present an executive summary of this work. The TWG was composed of a multidisciplinary group of pediatric and adult epileptologists, psychiatrists, and family doctors from academia and from the community; neurologists from the community; nurses and social workers from pediatric and adult epilepsy programs; adolescent medicine physician specialists; a team of physicians, nurses, and social workers dedicated to patients with complex care needs; a lawyer; an occupational therapist; representatives from community epilepsy agencies; patients with epilepsy; parents of patients with epilepsy and severe intellectual disability; and project managers. Three main areas were addressed: (1) Diagnosis and Management of Seizures; 2) Mental Health and Psychosocial Needs; and 3) Financial, Community, and Legal Supports. Although there are no systematic studies on the outcomes of transition programs, the impressions of the TWG are as follows. Teenagers at risk of poor transition should be identified early. The care coordination between pediatric and adult neurologists and other specialists should begin before the actual transfer. The transition period is the ideal time to rethink the diagnosis and repeat diagnostic testing where indicated (particularly genetic testing, which now can uncover more etiologies than when patients were initially evaluated many years ago). Some screening tests should be repeated after the move to the adult system. The seven steps proposed herein may facilitate transition, thereby promoting uninterrupted and adequate care for youth with epilepsy leaving the pediatric system.

Expand your HEADS, follow the THRxEADS!

Adolescence can be a particularly challenging period for individuals with a chronic health condition or disability. We present a new mnemonic, THRxEADS (T for Transition, H for Home, Rx for Medication and Treatment, E for Education and Eating, A for Activities and Affect, D for Drugs and S for Sexuality), which can be used as a complement to the adolescent HEADS (Home-Education-Activities-Drugs-Sexuality) assessment. THRxEADS may serve as a clinical tool to explore key issues that are often not covered in subspecialty clinic visits such as transition, coping, adherence and understanding of illness, as they apply to youth with special health care needs. THRxEADS may be used as a vehicle to highlight successes and to promote resilience. It may also be used as an educational tool with medical trainees to allow a deeper understanding of the realities of adolescents with chronic health care needs. A short list of sample chronic illness and disability-specific questions is provided.

L'adolescence peut être une période particulièrement difficile chez les jeunes ayant une maladie chronique ou une incapacité. Les auteurs présentent un nouvel acronyme mnémonique, THRxEADS (T pour transition, H pour maison [home en anglais], Rx pour médicaments et traitement, E pour éducation et alimentation [eating en anglais], A pour activités et affect, D pour drogues et médicaments et S pour sexualité), qui peut compléter l'évaluation HEADS (Home [maison]­Éducation­Activités­Drogues et médicaments­Sexualité) chez les adolescents. L'évaluation THRxEADS peut servir d'outil clinique pour explorer de grands enjeux qui ne sont souvent pas abordés lors des rendez-vous en clinique surspécialisée, comme la transition, l'adaptation, la compliance et la compréhension de la maladie, dans la mesure où ils s'appliquent chez les jeunes ayant des besoins particuliers. L'évaluation THRxEADS peut souligner les réussites et promouvoir la résilience. Ce peut aussi être un outil de formation auprès des stagiaires en médecine, qui pourront mieux comprendre les réalités des adolescents ayant des besoins de santé chroniques. Une courte liste de questions sur les maladies chroniques et les incapacités est également proposée.

Impact of Adolescent Gender Dysphoria on Treatment Uptake in an Obesity Management Program.

A 13-year-old biological female patient presented with gender dysphoria while receiving treatment for obesity. Body distress and a desired masculine phenotype motivated his engagement with various therapies. We describe body image concerns in an obese adolescent with gender dysphoria to highlight the importance of assessing gender in adolescents.

A cluster randomized trial of a transition intervention for adolescents with congenital heart disease: rationale and design of the CHAPTER 2 study.

BACKGROUND: The population of adolescents and young adults with congenital heart disease (CHD) is growing exponentially. These survivors are at risk of late cardiac complications and require lifelong cardiology care. However, there is a paucity of data on how to prepare adolescents to assume responsibility for their health and function within the adult health care system. Evidence-based transition strategies are required. METHODS: The Congenital Heart Adolescents Participating in Transition Evaluation Research (CHAPTER 2) Study is a two-site cluster randomized clinical trial designed to evaluate the efficacy of a nurse-led transition intervention for 16-17 year olds with moderate or complex CHD. The primary endpoint is excess time to adult CHD care, defined as the time interval between the final pediatric cardiology appointment and the first adult CHD appointment, minus the recommended time interval between these appointments. Secondary endpoints include the MyHeart score (CHD knowledge), Transition Readiness Assessment Questionnaire score, and need for catheter or surgical re-intervention. Participants are enrolled in clusters based on week of attendance in the pediatric cardiology clinic. The intervention consists of two one-hour individualized sessions between a cardiology nurse and study participant. Session One focuses on knowledge of the participant's CHD, review of their cardiac anatomy and prior interventions, and potential late cardiac complications. Session Two focuses on self-management and communication skills through review and discussion of videos and role-play. The study will recruit 120 participants. DISCUSSION: Many adolescents and young adults experience a gap in care predisposing them to late cardiac complications. The CHAPTER 2 Study will investigate the impact of a nurse-led transition intervention among adolescents with CHD. Fidelity of the intervention is a major focus and priority. This study will build on our experience by (i) enrolling at two tertiary care programs, (ii) including a self-management intervention component, and (iii) evaluating the impact of the intervention on time to ACHD care, a clinically relevant outcome. The results of this study will inform pediatric cardiology programs, patients and policy makers in judging whether a structured intervention program provides clinically meaningful outcomes for adolescents and young adults living with CHD. TRIAL REGISTRATION: ClinicalTrials.gov ID NCT01723332.

Experience with genetic counseling: the adolescent perspective.

Adolescence is a complex period of development that involves creating a sense of identity, autonomy, relationships and values. This stage of adjustment can be complicated by having a genetic condition. Genetic counseling can play an important role in providing information and support to this patient population; however, resources and guidelines are currently limited. In order to appropriately establish genetic counseling approaches and resource development, we investigated the experiences and perspectives of adolescents with a genetic condition with respect to their genetic counseling interactions. Using a qualitative exploratory approach, eleven semi-structured interviews were conducted with adolescents diagnosed with a genetic condition who received genetic counseling between the ages of 12 and 18 years at The Hospital for Sick Children. Transcripts were analyzed thematically using qualitative content analysis, from which three major interrelated themes emerged: 1) understanding the genetic counselor's role; 2) increasing perceived personal control; and 3) adolescent-specific factors influencing adaptation to one's condition. Additionally, a list of suggested tools and strategies for genetic counseling practice were elucidated. Our findings can contribute to the development of an adolescent-focused framework to enhance emerging genetic counseling approaches for this patient population, and can also facilitate the transition process from pediatric to adult care within patient and family-centered contexts.

Reach Out and Read is Feasible and Effective for Adolescent Mothers: A Pilot Study.

OBJECTIVES: The Reach Out and Read program (ROaR) is associated with increased parent-child book reading and improved language development in children. Though children of adolescent parents may have an elevated risk of language delay, ROaR has never been specifically studied among adolescent-headed families. This pilot evaluated the feasibility and effectiveness of ROaR among adolescent mothers and their children. METHODS: This randomized controlled pilot followed thirty adolescent mothers with children aged 6-20 months in a teen-tot clinic in downtown Toronto. At each of three consecutive well child checkups, intervention families received a new children's book, reading-related anticipatory guidance customized to the mother's developmental stage, counselling from a librarian, and a public library card. Control families received routine care. At baseline and study completion, all mothers completed a survey on family reading patterns and the Beck Depression Inventory-Revised (BDI-IA). RESULTS: Though regression models were not statistically significant, bivariate analyses at study completion revealed that intervention mothers were significantly more likely than controls to report reading as one of the child's favorite activities (29 vs 0 %) and had significantly lower maternal depression scores (7.0 vs 12.5; ≥10 = clinically significant depression). Trends for all other variables, including time spent reading together and maternal enjoyment of reading, were also in the direction of benefit. This program was implemented at minimal cost and adopted permanently following study completion. CONCLUSIONS: This feasible and developmentally appropriate intervention shows promise in promoting shared book reading and reducing maternal depression within adolescent-headed families, warranting investigation with larger trials.

Thanks for asking: Adolescent attitudes and preferences regarding the use of chaperones during physical examinations.

BACKGROUND: There is no uniformity as to how and when chaperones should be used for general and intimate (genitalia and/or breasts) physical examinations of adolescents. OBJECTIVE: To explore adolescents' attitudes and preferences regarding the use of medical chaperones during physical examinations. METHODS: The present analysis was a cross-sectional descriptive study performed as part of a quality improvement project in the Adolescent Medicine Clinics at The Hospital for Sick Children (Toronto, Ontario) between January 1 and April 30, 2011. Adolescents 13 to 18 years of age completed an anonymous 10-item, self-administered questionnaire regarding their thoughts on chaperones during physical examinations. Demographic and descriptive data were collected. RESULTS: A total of 127 adolescents participated in the present study. The mean (± SD) age was 16.3±1.5 years and the majority (93.7%) were female. More than one-half (61%) of female adolescents had previous experience with an intimate examination; however, a chaperone was present only 36% of the time. Seventy percent of female adolescents wanted the choice of a chaperone for a general examination compared with 61% for an intimate examination. Among female adolescents with past chaperone experience, 78% wanted the choice of a chaperone for subsequent intimate examinations, compared with 55% among those with no previous chaperone experience. Only 21% believed they would ask for a chaperone if one were not offered. CONCLUSIONS: Although there was variation in adolescents' attitudes and preferences regarding the use of chaperones, many females indicated a desire to discuss the option of a chaperone for all types of examinations.

HISTORIQUE: Il n'y a pas d'uniformité quant à la manière et au moment de faire appel à un chaperon lors des examens généraux et intimes (organes génitaux ou seins) des adolescents. OBJECTIF: Explorer les attitudes et les préférences des adolescents à l'égard du recours à des chaperons médicaux pendant l'examen physique. MÉTHODOLOGIE: La présente analyse descriptive transversale fait partie d'un projet d'amélioration de la qualité aux cliniques de médecine des adolescents du Hospital for Sick Children de Toronto, en Ontario, entre le janvier et le 30 avril 2011. Des adolescents de 13 à 18 ans ont rempli eux-mêmes un questionnaire anonyme de dix questions sur leurs perceptions à l'égard de la présence d'un chaperon pendant les examens physiques. Les données démographiques et descriptives ont été colligées. RÉSULTATS: Au total, 127 adolescents ont participé à la présente étude. Ils avaient un âge moyen (± ÉT) de 16,3±1,5 ans, et la majorité (93,7 %) étaient des filles. Plus de la moitié (61 %) des adolescents avaient déjà subi un examen physique, mais un chaperon était présent dans seulement 36 % des cas. Pourtant, 70 % des adolescentes auraient voulu se faire proposer la présence d'un chaperon lors de l'examen général, par rapport à 61 % lors d'un examen intime. Chez les adolescentes qui avaient déjà été accompagnées d'un chaperon, 78 % voulaient qu'on leur propose la présence d'un chaperon lors des prochains examens physiques, par rapport à 55 % de celles qui n'avaient jamais été accompagnées d'un chaperon. Seulement 21 % pensaient demander la présence d'un chaperon si on ne la leur proposait pas. CONCLUSIONS: Même si les adolescents avaient des attitudes et des préférences variées à l'égard de la présence d'un chaperon, de nombreuses filles ont exprimé le souhait qu'on leur propose la présence d'un chaperon lors de tous les types d'examens.

Transition to adult care in pediatric solid-organ transplant: development of a practice guideline.

BACKGROUND: Transition to adult-centered care is becoming an increasingly important area of practice in pediatric organ transplant. Standardized, best-practice guidelines are needed to assist transplant practitioners in providing optimal transitional care for this population of patients. OBJECTIVE: To describe the development and implementation of a practice guideline for the transitional care of pediatric transplant recipients. METHODS: A quality improvement project was undertaken in a pediatric multiorgan transplant program setting. Strategies employed included (1) creation of an interdisciplinary working group, (2) survey of transition-related practices and learning needs of transplant practitioners, (3) review of the literature and existing transition-related materials, and (4) creation of transition guidelines. RESULTS: An interdisciplinary survey of transplant practitioners at our institution identified practice strengths related to transitional care and learning needs. Review of relevant literature and other materials revealed limited but emerging research related to the transition of pediatric transplant recipients from pediatric to adult care. Existing transition tools were examined and applicable items identified. A practice guideline for use with pediatric transplant recipients transitioning to adult care was developed. Strategies to educate staff about the guideline and promote ongoing guideline use were implemented. CONCLUSION: Preparing pediatric transplant recipients and their families for transition to adult-centered care is an emerging challenge for transplant teams. These guidelines provide practitioners with a developmentally sensitive overview of important transition-related domains and strategies directed toward patients and their caregivers, who may experience the process of transition differently. Dissemination of the pediatric transplant transition guideline will make transition information more widely available to transplant practitioners.

A qualitative study on the experiences of young adults with eating disorders transferring from pediatric to adult care.

The study's objective was to identify systemic facilitators and barriers of transferring young adults (ages 17-21) with eating disorders from pediatric to adult health and mental health services. Qualitative interviews were conducted and three themes emerged: (a) difficulties navigating care during the transfer period; (b) challenges achieving and maintaining recovery due to systemic barriers after the transfer of care;and (c) recommendations for facilitating the transfer between systems of care. From the perspective of young adults with eating disorders our study shows that the transition to adult care services may be improved with increased coordination, communication, and collaborative partnerships between pediatric and adult providers.

Development of a smartphone app for adolescents with lupus: a collaborative meeting-based methodology inclusive of a wide range of stakeholders.

Traditional challenges of creating a medical app include hearing the voices of various stakeholders as a collective rather than in a consultative process that is sequential. This report describes the development of a mobile (smartphone) app for adolescents with lupus as well as the process that was used to overcome the challenge described above. The development of the smartphone app addressed optimal ways to incorporate information about 1) lupus, including the effects of both the disease and the medications used to treat it; 2) how life choices can affect lupus patients' condition; and 3) ways to increase self-management and communication. The collaborative concept-generating and requirements-gathering methodology was used during a two-day workshop with a range of stakeholders (ages 16 - 59 years) that focused on leveraging user-centered design methods to generate guidance to mobile app developers. The app development process conducted during the workshop included the following steps: 1) recruiting a goal-focused collaborative group, 2) defining app objectives, 3) evaluating potential needs of users, 4) brainstorming app features and use-case modeling, 5) reviewing existing app features and prototypes, 6) refining functionalities, 7) writing user narratives, 8) visualizing navigation and feature design, and 9) identifying content. The use of creative devices such as drawing interfaces fostered fun, engagement, and sustained energy, and the use of a brainstorming technique leveraged methods that ensured an inclusive process so that even participants who were shy, quiet, or easily intimidated by "professionals" felt confident to contribute. In addition to a name change for the app, project outcomes included the selection of the following app features: symptom tracking; appointment and medication reminders; a social media component; a medical summary; easy navigation; informational content; gamification; and personalization (options for customization).

A systematic review of transition readiness and transfer satisfaction measures for adolescents with chronic illness.

BACKGROUND: The transition from pediatric to adult health care can be challenging for adolescents with chronic illnesses. As a result, many adolescents are unable to transfer to adult health care successfully. Adequate measurement of transition readiness and transfer satisfaction with disease management is necessary in order to determine areas to target for intervention towards improving transfer outcomes. OBJECTIVES: This study aims to systematically review and critically appraise research on transition readiness and transfer satisfaction measures for adolescents with chronic illnesses as well as to assess the psychometric quality of these measures. METHODS: Electronic searches were conducted in MEDLINE, EMBASE, CINAHL, PsychINFO, ERIC, and ISI Web of Knowledge for transition readiness and transfer satisfaction measures for adolescents with chronic health conditions. Two reviewers independently selected articles for review and assessed methodological quality. RESULTS: In all, eight readiness and six satisfaction measures met the inclusion criteria, for a total of 14 studies, which were included in the final analysis. None of these measures have well-established evidence of reliability and validity. Most of the measures were developed ad hoc by the study investigators, with minimal to no evidence of reliability and/or validity using the Cohen criteria and COSMIN checklist. CONCLUSION: This research indicates a major gap in our knowledge of transitional care in this population, because there is currently no well-validated questionnaire that measures readiness for transfer to adult health care. Future research must focus on the development of well-validated transition readiness questionnaires, the validation of existing measures, and reaching consensus on outcomes of successful transfer.

Anorexia nervosa in a patient with congenital adrenal hyperplasia.

Life stressors and hormonal dysregulation in the hypothalamic-pituitary-adrenal (HPA) axis are common factors that play a role in both anorexia nervosa (AN) and congenital adrenal hyperplasia (CAH). The complexity of combined CAH and AN on the HPA axis has not, to our knowledge, been previously illustrated. We present a report of an adolescent with CAH who developed AN, requiring hospitalized management. We use this case to review the pathophysiology of CAH and AN as it relates to the stress response and hormonal dysregulation within the HPA axis. We then highlight the interplay between these conditions, and provide support to the theory that AN acts as a chronic stressor in patients with CAH, leading to HPA axis hyperactivity and elevated levels of androgen precursors. It is prudent for the medical team to monitor and modify CAH steroid dosing during the acute phase of AN, weaning appropriately with AN treatment success.

Predictive Factors for Mortality Following Major Lower Extremity Amputation.

BACKGROUND: Despite advances in techniques and care, major amputation bears a high risk for mortality. Previously identified factors associated with increased risk of mortality include amputation level, renal function, and pre-operative white cell count. METHODS: A single center retrospective chart review was conducted identifying patients who had undergone a major amputation. Chi-squared, t-testing, and Cox proportional hazard modeling were performed examining death at 6 months and 12 months. RESULTS: Factors associated with an increased risk of six-month mortality include age (OR 1.01-1.05, P < .001), sex (OR 1.08-3.24, P < .01), minority race (OR 1.18-18.19, P < .01), chronic kidney disease (OR 1.40-6.06, P < .001), and use of pressors at the induction of anesthesia for index amputation (OR 2.09-7.85, P < .000). Factors associated with increased risk of 12 month mortality were similar. DISCUSSION: Patients undergoing major amputation continue to suffer high mortality. Those patients who received their amputation under physiologically stressful conditions were more likely to die within 6 months. Reliably predicting six-month mortality can assist surgeons and patients in making appropriate care decisions.

Body image and eating attitudes and behaviors among adolescent heart and lung transplant recipients: a brief report.

BACKGROUND: Adolescents with chronic illnesses are at increased risk for body image and eating disorders; however, this has not been investigated in solid organ transplant recipients. Adolescent transplant recipients are a vulnerable cohort because of the sustained follow-up and immune-suppressing therapies, which often include steroids and may lead to weight gain and cosmetic changes. Consequences of body dissatisfaction such as disordered behaviors have not been well studied in transplant recipients. PURPOSE: To examine body image, eating attitudes, and behaviors among 28 adolescent thoracic transplant recipients. METHODS: Adolescent (11-18 years old) heart and lung transplant recipients a minimum of 3 months after transplant provided informed written consent and completed a standardized questionnaire package about eating attitudes and behaviors; body image and drive for thinness; actual, perceived, and desired weight; and medical and anthropometric information (eg, body mass index) during regular transplant clinics. RESULTS: Of 25 heart and 3 lung transplant recipients (54% female; median age, 14.5 years; median, 1.6 years after transplant), 37% perceived their current weight as too high or low. Moreover, 81% were dissatisfied with their current weight (38% wanted to lose and 44% wanted to gain weight), yet few engaged in disordered behaviors. CONCLUSIONS: Despite high levels of self-reported body dissatisfaction, low rates of disordered behaviors were observed. Weight dissatisfaction was high (81%) but bidirectional (to lose or to gain weight). Future assessment of disordered eating behaviors should include insidious activities such as medication nonadherence, in addition to traditional weight-control behaviors such as binge eating, strict dieting, or assiduous exercise. Further research will delineate the impact of body dissatisfaction and eating behaviors and outcomes on long-term transplant survivors, older adolescent cohorts, and other recipients of solid organ transplants.

Wearable sensors detect impaired gait and coordination in LBSL during remote assessments.

BACKGROUND: Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) is a rare leukodystrophy with motor impairment due to biallelic mutations in DARS2, which encodes mitochondrial aspartyl tRNA synthetase. Progressive ataxia is the primary feature. OBJECTIVE: The study objective is to determine the feasibility of remotely collecting quantitative gait and balance measures in LBSL. METHODS: The study design uses wearable accelerometers and the scale for the assessment and rating of ataxia (SARA) scale to assess gait and postural sway in LBSL and control participants' homes through video conferencing. RESULTS: Lateral step variability (LSV), which indicates stride variability, and elevation of the step at mid-swing are increased for LBSL patients during brief walking tests. During stance with the eyes closed, LBSL participants show rapid accelerations and decelerations of body movement covering a large sway area and path. Both the LSV and sway area during stance with the feet together and eyes closed correlate strongly with the SARA. CONCLUSIONS: Wearable accelerometers are valid and sensitive for detecting ataxia in LBSL patients during remote assessments. The finding of large increases in the sway area during stance with the eyes closed is intriguing since dorsal column dysfunction is universally seen in LBSL. This approach can be applied to related rare diseases that feature ataxia.

Tools for addressing systems issues in transition.

Moving from pediatric to adult healthcare is a time of stress and opportunity for adolescents with special healthcare needs (ASHCN) and their families. With over 90% of children with special healthcare needs surviving into young adulthood, there is an increasing imperative to actively engage youth in preparing for the adult system (Betz and Smith 2011; Pai and Schwartz 2011). The goal of transition care is to provide young people with a coordinated, uninterrupted and developmentally appropriate transfer to adult healthcare (Kaufman and Pinzon 2007). This is often complicated by the complex medical, social and psychological tasks and requirements that adolescents experience as they navigate their healthcare and their lives as young, developing people.