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.

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.

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.

Mitochondrial aminoacyl-tRNA synthetase disorders: an emerging group of developmental disorders of myelination.

BACKGROUND: The mitochondrial aminoacyl-tRNA synthetase proteins (mt-aaRSs) are a group of nuclear-encoded enzymes that facilitate conjugation of each of the 20 amino acids to its cognate tRNA molecule. Mitochondrial diseases are a large, clinically heterogeneous group of disorders with diverse etiologies, ages of onset, and involved organ systems. Diseases related to mt-aaRS mutations are associated with specific syndromes that affect the central nervous system and produce highly characteristic MRI patterns, prototypically the DARS2, EARS, and AARS2 leukodystrophies, which are caused by mutations in mitochondrial aspartyl-tRNA synthetase, mitochondria glutamate tRNA synthetase, and mitochondrial alanyl-tRNA synthetase, respectively. BODY: The disease patterns emerging for these leukodystrophies are distinct in terms of the age of onset, nature of disease progression, and predominance of involved white matter tracts. In DARS2 and EARS2 disorders, earlier disease onset is typically correlated with more significant brain abnormalities, rapid neurological decline, and greater disability. In AARS2 leukodystrophy cases reported thus far, there is nearly invariable progression to severe disability and atrophy of involved brain regions, often within a decade. Although most mutations are compound heterozygous inherited in an autosomal recessive fashion, homozygous variants are found in each disorder and demonstrate high phenotypic variability. Affected siblings manifest disease on a wide spectrum. CONCLUSION: The syndromic nature and selective vulnerability of white matter tracts in these disorders suggests there may be a shared mechanism of mitochondrial dysfunction to target for study. There is evidence that the clinical variability and white matter tract specificity of each mt-aaRS leukodystrophy depend on both canonical and non-canonical effects of the mutations on the process of mitochondrial translation. Furthermore, different sensitivities to the mt-aaRS mutations have been observed based on cell type. Most mutations result in at least partial retention of mt-aaRS enzyme function with varied effects on the mitochondrial respiratory chain complexes. In EARS2 and AARS2 cells, this appears to result in cumulative impairment of respiration. Mt-aaRS mutations may also affect alternative biochemical pathways such as the integrated stress response, a homeostatic program in eukaryotic cells that typically confers cytoprotection, but can lead to cell death when abnormally activated in response to pathologic states. Systematic review of this group of disorders and further exploration of disease mechanisms in disease models and neural cells are warranted.

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.

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.

Can the Mindful Awareness and Resilience Skills for Adolescents (MARS-A) Program Be Provided Online? Voices from the Youth.

Mindfulness-based interventions (MBIs) have been shown to improve health and well-being in adolescents with chronic illnesses. Because they are most often delivered in person in a group setting, there are several barriers that limit access to MBIs for youth with limited mobility or who cannot access in-person MBIs in their communities. The objective of this study was to determine if eHealth is a viable platform to increase accessibility to MBIs for teens with chronic illnesses. This study reports the qualitative results of a mixed method randomized trial describing the experience of the Mindful Awareness and Resilience Skills for Adolescents (MARS-A) program, an eight-week MBI, delivered either in person or via eHealth. Participants were adolescents between the ages of 13 and 18 with a chronic illness recruited at a tertiary pediatric hospital in Toronto, Canada. Individual semi-structured post-participation audio-video interviews were conducted by a research assistant. A multiple-pass inductive process was used to review interview transcripts and interpret emergent themes from the participants' lived experiences. Fifteen participants (8 online and 7 in person) completed post-participation interviews. Four distinct themes emerged from participants in both groups: Creation of a safe space, fostering peer support and connection, integration of mindfulness skills into daily life, and improved well-being through the application of mindfulness. Direct quotations representative of those four themes are reported. Results from this study suggest that eHealth delivery of an adapted MBI for adolescents with chronic illnesses may be an acceptable and feasible mode of delivery for MBIs in this population. EHealth should be considered in future studies of MBIs for adolescents with chronic illnesses as a promising avenue to increase access to MBIs for youth who might not be able to access in-person programs.

Transition Intervention for Adolescents With Congenital Heart Disease.

BACKGROUND: There is little evidence regarding the efficacy of interventions to prepare adolescents with congenital heart disease (CHD) to enter adult care. OBJECTIVES: The goal of this study was to evaluate the impact of a nurse-led transition intervention on lapses between pediatric and adult care. METHODS: A cluster randomized clinical trial was conducted of a nurse-led transition intervention for 16- to 17-year-olds with moderate or complex CHD versus usual care. The intervention group received two 1-h individualized sessions targeting CHD education and self-management skills. The primary outcome was excess time to adult CHD care, defined as the interval between the final pediatric and first adult cardiology appointments, minus the recommended time interval, analyzed by using Cox proportional hazards regression accounting for clustering. Secondary outcomes included scores on the MyHeart CHD knowledge survey and the Transition Readiness Assessment Questionnaire. RESULTS: A total of 121 participants were randomized to receive the intervention (n = 58) or usual care (n = 63). At the recommended time of first adult appointment (excess time = 0), intervention participants were 1.8 times more likely to have their appointment within 1 month (95% confidence interval: 1.1 to 2.9; Cox regression, p = 0.018). This hazard increased with time; at an excess time of 6 months, intervention participants were 3.0 times more likely to have an appointment within 1 month (95% confidence interval: 1.1 to 8.3). The intervention group had higher scores at 1, 6, 12, and 18 months on the MyHeart knowledge survey (mixed models, p < 0.001) and the Transition Readiness Assessment Questionnaire self-management index (mixed models, p = 0.032). CONCLUSIONS: A nurse-led intervention reduced the likelihood of a delay in adult CHD care and improved CHD knowledge and self-management skills. (Congenital Heart Adolescents Participating in Transition Evaluation Research [CHAPTER 2]; NCT01723332).

In-Person Versus eHealth Mindfulness-Based Intervention for Adolescents With Chronic Illness: Protocol for a Randomized Controlled Trial.

BACKGROUND: Eight-week mindfulness-based interventions (MBIs) have a beneficial impact on mental health and well-being in adolescents with chronic health conditions. Usually delivered in person in a group setting, these programs are difficult to access for teens with disabilities or who do not have in-person MBIs available in their communities. OBJECTIVE: This paper outlines the rationale, development, and design of a randomized controlled trial comparing the effects of an MBI delivered in person or via eHealth in adolescents with a chronic illness. Quantitative outcomes will include mindfulness skills acquisition (primary outcome), effects of the MBI on self-reported mood, anxiety, self-esteem, illness perception, and physiological stress (via salivary cortisol), and qualitative outcomes will include individual practice, participant appreciation, and adaptation of the MBI for eHealth. METHODS: This is a randomized noninferiority mixed methods study comparing 2 MBI arms: in-person and eHealth. Participants are eligible to participate if they are aged 13 to 18 years, have a diagnosis of chronic medical condition, live close enough to the recruitment hospital to participate in the in-person arm of the study, and are currently followed by a health care provider. Each participant will receive an adapted 8-week MBI delivered either in person at a tertiary pediatric hospital or via a secure audio-visual platform allowing group interactions in real time. Groups will be facilitated by 2 experienced mindfulness providers. Quantitative and qualitative data will be collected through standardized research questionnaires administered via a secure, youth-friendly online platform and through semistructured interviews, participant log books, facilitator log books, and salivary cortisol analysis. Qualitative data will be analyzed using a grounded theory model. RESULTS: Data collection is currently underway. Data analysis, manuscript writing, and additional publications are expected to be completed in the winter and spring of 2018. CONCLUSIONS: Based on previous results from in-person trials conducted in adolescents and eHealth trials conducted in adults, we anticipate that both modes of delivery will significantly improve mindfulness skills acquisition, mood, anxiety, self-esteem, illness perception, and stress and that the magnitude of the effects will be correlated to the level of home practice. We predict that participants in both arms will show similar levels of home practice and that both modes of delivery will have high levels of feasibility and acceptability. If successful, this study could provide evidence for the use of eHealth in the delivery of 8-week MBIs in clinical adolescent populations, potentially increasing availability to MBIs for a large group of youth with mobility issues or living away from large urban centers. TRIAL REGISTRATION: ClinicalTrials.org NCT03067207; https://clinicaltrials.gov/ct2/show/NCT03067207 (archived by WebCite at http://www.webcitation.org/6v4ZK8RBH).

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.

Improving Adolescent Sexual and Reproductive Health: A Systematic Review of Potential Interventions.

Adolescents have special sexual and reproductive health needs (whether or not they are sexually active or married). This review assesses the impact of interventions to improve adolescent sexual and reproductive health (including the interventions to prevent female genital mutilation/cutting [FGM/C]) and to prevent intimate violence. Our review findings suggest that sexual and reproductive health education, counseling, and contraceptive provision are effective in increasing sexual knowledge, contraceptive use, and decreasing adolescent pregnancy. Among interventions to prevent FGM/C, community mobilization and female empowerment strategies have the potential to raise awareness of the adverse health consequences of FGM/C and reduce its prevalence; however, there is a need to conduct methodologically rigorous intervention evaluations. There was limited and inconclusive evidence for the effectiveness of interventions to prevent intimate partner violence. Further studies with rigorous designs, longer term follow-up, and standardized and validated measurement instruments are required to maximize comparability of results. Future efforts should be directed toward scaling-up evidence-based interventions to improve adolescent sexual and reproductive health in low- and middle-income countries, sustain the impacts over time, and ensure equitable outcomes.

The iPeer2Peer Program: a pilot randomized controlled trial in adolescents with Juvenile Idiopathic Arthritis.

BACKGROUND: Adolescents with Juvenile Idiopathic Arthritis (JIA) are at risk for physical, emotional, social and role challenges that negatively impact quality of life. Peer mentoring has been shown to improve positive health behaviours in adolescents with chronic disease while simultaneously providing social support. The objectives of this paper are to examine the feasibility and acceptability of an online peer mentoring program (iPeer2Peer Program) for adolescents with JIA. METHODS: The iPeer2Peer program was examined using a waitlist pilot randomized control trial (RCT). Participants were randomly allocated to the intervention or wait-list control group via a secure, web-based randomization service. Health care providers and investigators were blinded to participant group allocation. Trained peer mentors (16-25 years; successfully managing their JIA) were matched to participants (12-18 years; diagnosed with JIA) randomized to the intervention group to provide peer support and education for effective self-management of JIA. Participant-mentor pairings connected ten times over 8 weeks using Skype video calls. Primary outcomes focused on implementation (i.e. measures of feasibility and acceptability). Secondary outcomes focused on effectiveness (i.e. measures of self-management, self-efficacy, pain, social support and quality of life). RESULTS: Thirty adolescents (mean age 14.3 ± 1.7 years, 97 % female) completed the RCT (intervention n = 16, control n = 14). PRIMARY OUTCOMES: One third (32 %) of adolescents approached agreed to participate, completed baseline measures and were randomized. Half of pairings completed ten calls within 8 weeks. Average call length was twice the required amount with call lengths of 44.72 ± 15.76 min. Participants reported satisfaction with the program and all reported that they would recommend it to their peers. Participants' mean engagement level with the program was 8.53/10 (range = 7-10). SECONDARY OUTCOMES: Participants who completed the iPeer2Peer Program demonstrated improvements in their perceived ability to manage JIA (p < 0.04), compared to controls. No adverse events were reported. CONCLUSION: The iPeer2Peer Program is a promising intervention that improves acceptability of self-management and peer support treatments for adolescents with JIA. By using the Internet to connect mentors to adolescents with JIA it may also improve accessibility to these resources. Findings will be used to adapt the program and refine the methodology for a full-scale RCT. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT01986400 . Registered November 11, 2013.

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.

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.

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.

Sexuality and Relationships in Young People With Spina Bifida and Their Partners.

PURPOSE: Research focusing on sexuality in those living with disabilities, such as spina bifida (SB), has not specifically addressed adolescents and has been largely quantitative in design. Our study qualitatively explored how young people with SB think about and discuss sexuality with their sexual and romantic partners in the context of their disability. METHODS: Participants aged 16-25 years were recruited using purposive sampling from a large urban pediatric rehabilitation center in Toronto, Canada, as well as through a large Spina Bifida and Hydrocephalus Association. Semistructured interviews were conducted in person or by telephone. Inductive coding and descriptive thematic analysis were conducted on verbatim transcripts. RESULTS: There were mixed views on the importance of disclosing their condition to partners. While some participants strongly believed that their disability was important to share with partners, others worried that potential partners would focus on the disability rather than the person. Participants reported challenges about the timing of disclosure, lack of confidence in their abilities to express their sexual needs, and fears of rejection. After disclosure, however, participants often experienced increased confidence in themselves and their relationships. Participants identified a lack of SB-specific sexual education and a desire to learn more from their health care providers. CONCLUSIONS: The findings underscore the importance of empowering young people to become more confident talking about their disability, especially in the context of sexual and romantic relationships. Being able to discuss their abilities, needs and desires could potentially facilitate the development of healthy relationships during their transition to adulthood.

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.

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.

Benefits of a transfer clinic in adolescent and young adult kidney transplant patients.

BACKGROUND: Adolescent and young adult kidney transplant recipients have worse graft outcomes than older and younger age groups. Difficulties in the process of transition, defined as the purposeful, planned movement of adolescents with chronic health conditions from child to adult-centered health care systems, may contribute to this. Improving the process of transition may improve adherence post-transfer to adult care services. OBJECTIVE: The purpose of this study is to investigate whether a kidney transplant transfer clinic for adolescent and young adult kidney transplant recipients transitioning from pediatric to adult care improves adherence post-transfer. METHODS: We developed a joint kidney transplant transfer clinic between a pediatric kidney transplant program, adult kidney transplant program, and adolescent medicine at two academic health centers. The transfer clinic facilitated communication between the adult and pediatric transplant teams, a face-to-face meeting of the patient with the adult team, and a meeting with the adolescent medicine physician. We compared the outcomes of 16 kidney transplant recipients transferred before the clinic was established with 16 patients who attended the clinic. The primary outcome was a composite measure of non-adherence. Non-adherence was defined as either self-reported medication non-adherence or displaying two of the following three characteristics: non-attendance at clinic, non-attendance for blood work appointments, or undetectable calcineurin inhibitor levels within 1 year post-transfer. RESULTS: The two groups were similar at baseline, with non-adherence identified in 43.75 % of patients. Non-adherent behavior in the year post-transfer, which included missing clinic visits, missing regular blood tests, and undetectable calcineurin inhibitor levels, was significantly lower in the cohort which attended the transfer clinic (18.8 versus 62.5 %, p = 0.03). The median change in estimated glomerular filtration rate (eGFR) in the year following transfer was smaller in the group that attended the transition clinic (-0.9 ± 13.2 ml/min/1.73 m(2)) compared to those who did not (-12.29 ± 14.9 ml/min/1.73 m(2)), p = 0.045. CONCLUSIONS: Attendance at a single kidney transplant transfer clinic was associated with improved adherence and renal function in the year following transfer to adult care. If these changes are sustained, they may improve long-term graft outcomes for adolescent kidney transplant recipients.

CONTEXTE: L'évolution favorable du greffon est plus souvent compromise chez les adolescents et les jeunes adultes transplantés du rein que chez les enfants et les adultes ayant subi la même intervention. Ces jeunes patients qui sont en général atteints de maladies chroniques, rencontrent des difficultés au cours de la période de transition entre le moment de leur transfert des unités de soins pédiatriques vers les unités de soins pour adultes, et celles-ci pourraient contribuer à ce pronostic défavorable. Des améliorations apportées au processus de transition pourraient favoriser l'adhésion de ces jeunes patients à leur protocole de traitement à la suite leur transfert dans les services de soins pour adultes. OBJECTIF: Le but de cette étude est de vérifier si la fréquentation d'une clinique de transfert pouvait améliorer l'adhésion des adolescents et des jeunes adultes greffés du rein à leur traitement, après leur transfert d'un établissement pédiatrique vers des services de soins pour adultes. MÉTHODES: Nous avons développé, au sein de deux centres universitaires de santé, deux cliniques conjointes de transfert pour les transplantés du rein. Ces cliniques étaient formées d'un programme de transplantation rénale pédiatrique, d'un programme de greffe rénale pour adultes et d'une clinique de médecine adolescente. La mise en place d'une clinique de transfert a facilité la communication entre les équipes de transplantation pour adultes et pédiatriques, a permis aux patients adolescents de rencontrer les équipes de transplantation pour adultes et de rencontrer des spécialistes de la médecine adolescente. Nous avons comparé les résultats de 16 jeunes greffés du rein qui avaient été transférés dans les centres de soins pour adultes avant la mise en place de la clinique de transfert avec les résultats de 16 patients qui ont fréquenté la clinique de transfert avant leur transition vers les unités de soins pour adultes. Le principal résultat a été une mesure composite d'adhésion au traitement. La non-adhésion a été définie soit par l'aveu de la part du patient de sa non-observance du traitement médicamenteux, soit par la manifestation de deux des trois comportements suivants dans le suivi du patient : la non-fréquentation de la clinique de transfert, le défaut de se présenter aux rendez-vous pour les analyses sanguines ou un niveau indécelable des inhibiteurs de calcineurine dans l'année suivant le transfert vers les services de soins pour adultes. RÉSULTATS: Les patients des deux groupes présentaient des caractéristiques similaires au début de l'étude, et 43,75 % d'entre eux avaient admis ne pas adhérer entièrement au traitement. Le nombre de comportements identifiés comme signes de non-adhésion au traitement tels que manquer des rendez-vous à la clinique de transfert, ne pas se présenter pour les analyses sanguines ou un niveau d'inhibiteurs de la calcineurine indécelable dans l'année suivant le transfert, étaient nettement inférieurs dans la cohorte de patients qui fréquentait la clinique de transfert que dans celle des patients qui avaient été transférés directement dans les services de soins pour adultes (18,8 % versus 62,5 %, p = 0,03). Qui plus est, les patients ayant fréquenté la clinique de transfert présentaient une variation médiane plus faible du débit de filtration glomérulaire (−0,9 ± 13,2 ml/min/1,73 m2) lorsque comparée à celle du groupe ayant été transféré directement (−12,2 ± 14,9 ml/min/1,73 m2), p = 0,045. CONCLUSIONS: Le fait de fréquenter une clinique de transfert pour les greffés du rein, dans l'année suivant leur transfert dans un centre de soins pour adultes, donne lieu à la fidélisation des jeunes transplantés du rein à l'égard de leur traitement et ceci favorise le rétablissement de leur fonction rénale. Le maintien de ces changements de comportement pourrait améliorer le pronostic à long terme quant à l'évolution du greffon chez les adolescents et les jeunes adultes greffés du rein.