Home-Based Resistance Training Programme in Fontan Patients: It Is Feasible, Safe, and Efficient.

Background: Fontan physiology is characterized by a single functional systemic ventricle that reduces cardiac output adaptation and exercise capacity. Peripheral muscle pump is crucial for venous return, and resistance training of the peripheral muscles may be particularly beneficial for cardiac function in these patients. This study explores the feasibility of a training programme including safety and effectiveness, maximal exercise capacity, cardiorespiratory endurance, and quality of life in a Fontan population. Methods: Twelve Fontan patients (18-32 years old) with New York Heart Association functional classes I and II were recruited. The intervention was 12 weeks of home-based exercise programme of 3 sessions per week supervised online by a physiotherapist. Pre- and postintervention evaluations assessed lean body mass, segmental forces with a hand-held dynamometer, maximal oxygen consumption (VO2max), cardiovascular endurance, leg endurance with a 1-minute-sit-to-stand test, and quality of life with a 36-item Short-Form Survey. Results: A total of 11 of 12 participants completed 35 of 36 workouts, exceeding our 80% threshold for completion. No adverse events were reported. Leg muscle strength increased significantly (knee extensors improved by 22.8 ± 22.8 N m [17.7%], P = 0.019). Despite no change in VO2max, endurance time showed a significant improvement of 5.3 minutes (72%). Perceived physical condition evaluated with a self-perception scale improved significantly. There was no significant difference in the lean body mass and in quality of life. Conclusion: In this small group of post-Fontan patients with mild functional impairments, we describe a structured, accessible, and feasible home-based resistance training programme that seems safe and show improvements in strength and perceived physical condition.

Contexte: La physiologie qui découle de l'intervention de Fontan se caractérise par un cœur univentriculaire fonctionnel pour la circulation générale, ce qui réduit l'adaptation du débit cardiaque et la tolérance à l'effort. Le pompage par les muscles périphériques est essentiel au retour veineux, et un entraînement musculaire pourrait donc être bénéfique pour la fonction cardiaque des patients qui ont subi cette intervention. Dans notre étude, nous évaluons la possibilité de mettre en place un programme d'entraînement et examinons l'innocuité et l'efficacité d'un tel programme, la tolérance à l'effort maximal, l'endurance cardiorespiratoire et la qualité de vie chez des patients présentant une circulation de Fontan. Méthodologie: Nous avons recruté 12 patients (âgés de 18 à 32 ans) présentant une circulation de Fontan avec les classes fonctionnelles I et II selon la New York Heart Association. L'intervention prenait la forme d'un programme d'exercice à domicile de trois séances par semaine pendant douze semaines, sous la supervision en ligne d'un physiothérapeute. Les évaluations réalisées avant et après l'intervention incluaient la masse maigre, la force de différents segments corporels évaluée par un dynamomètre portatif, la consommation maximale d'oxygène (VO2max), l'endurance cardiovasculaire, l'endurance des jambes évaluées par un test du lever de la chaise d'une minute et un court questionnaire de 36 questions sur la qualité de vie. Résultats: Au total, 11 des 12 participants ont réalisé 35 entraînements sur 36, ce qui dépassait le seuil de réussite du programme établi à 80 %. Aucun effet indésirable n'a été signalé. La force des muscles des jambes a significativement augmenté (l'amélioration de la force des extenseurs des genoux s'élevait à 22,8 ± 22,8 N m [17,7 %] [p = 0,019]). Malgré une valeur de VO2max restée stable, le temps d'endurance s'est amélioré significativement, soit de 5,3 minutes (72 %). La perception de la condition physique, mesurée sur une échelle d'autoévaluation par les patients, s'est améliorée significativement. Aucune différence significative n'a été relevée dans la masse maigre et la qualité de vie des patients. Conclusion: Dans un petit groupe de patients ayant subi une intervention de Fontan et présentant une atteinte fonctionnelle légère, nous décrivons un programme structuré de musculation à domicile accessible et réalisable qui semble sécuritaire et qui a amélioré la force et de la perception de la condition physique des patients.

Diagnostic Efficacy of a Single-Lead Ambulatory 14-Day ECG Monitor in Symptomatic Children.

BACKGROUND: The CardioSTAT is a single-lead ambulatory electrocardiography monitor that has been validated for use in adult patients. Recording is made through 2 electrodes positioned in a lead-I configuration, and the device allows monitoring for 2, 7, or 14 days. We sought to investigate the efficacy of this device in children with paroxysmal palpitations. METHODS: In phase I, the quality of tracings from simultaneous CardioSTAT recordings and D1-lead recordings of a standard 12-lead electrocardiography machine in 23 children were compared. Phase II was a prospective observational cohort study comparing arrhythmia detection using the CardioSTAT vs currently used devices (24-hour Holter monitor and the Cardiomemo loop recorder) in 52 children complaining of palpitations. RESULTS: In Phase I, all but 3 rhythm strips were correctly identified. The pacing spikes on 3 strips were not adequately identified by the observers for the CardioSTAT recording. In Phase II, symptomatic episodes were reported in 42%, 73%, and 100% of subjects during monitoring with the Holter, Cardiomemo, and CardioSTATdevices, respectively. An abnormal rhythm was detected in 13%, 23%, and 35% of subjects by the Holter, Cardiomemo, and CardioSTAT monitors, respectively. The underlying rhythm during symptomatic events was determined in 90% of cases with the CardioSTAT monitor, whereas it was determined in only 19% and 29% of cases using the Holter and Cardiomemo monitors, respectively. CONCLUSIONS: The CardioSTAT monitor provided good-quality tracings and was superior to the 24-hour Holter monitor and the Cardiomemo loop recorder in determining the presence or absence of pathologic arrhythmia in the study cohort.

CONTEXTE: Le CardioSTAT est un moniteur d'électrocardiographie ambulatoire à dérivation unique dont l'utilisation a été validée chez les patients adultes. L'enregistrement se fait au moyen de 2 électrodes positionnées dans une configuration de type « dérivation I ¼, et l'appareil permet un suivi pendant 2, 7 ou 14 jours. Nous avons cherché à étudier l'efficacité de cet appareil chez les enfants atteints de palpitations paroxystiques. MÉTHODOLOGIE: Dans la phase I, la qualité des tracés provenant d'enregistrements simultanés de CardioSTAT et d'enregistrements de la dérivation I d'un appareil d'électrocardiographie standard à 12 dérivations a été comparée chez 23 enfants. La phase II était une étude de cohorte observationnelle prospective comparant la détection de l'arythmie à l'aide du moniteur CardioSTAT par rapport aux appareils utilisés actuellement (moniteur Holter à surveillance sur 24 heures et enregistreur d'événements portatif Cardiomémo) chez 52 enfants se plaignant de palpitations. RÉSULTATS: Dans la phase I, toutes les bandes d'enregistrement sauf trois ont été correctement identifiées. Les spicules de stimulation n'ont pas été correctement détectés par les observateurs sur trois bandes d'enregistrement du moniteur CardioSTAT. Dans la phase II, des épisodes symptomatiques ont été signalés chez 42 %, 73 % et 100 % des sujets pendant la surveillance avec les appareils Holter, Cardiomémo et CardioSTAT, respectivement. Un rythme anormal a été détecté chez 13 %, 23 % et 35 % des sujets par les moniteurs Holter, Cardiomémo et CardioSTAT, respectivement. Le rythme sous-jacent pendant les événements symptomatiques a été déterminé dans 90 % des cas avec le moniteur CardioSTAT, alors qu'il n'a été déterminé que dans 19 % et 29 % des cas, respectivement, avec les moniteurs Holter et Cardiomémo. CONCLUSIONS: Le moniteur CardioSTAT a fourni des tracés de bonne qualité et s'est révélé supérieur à l'appareil Holter à surveillance sur 24 heures et à l'enregistreur d'événements portatif Cardiomémo pour déterminer la présence ou l'absence d'arythmie pathologique dans la cohorte étudiée.

A founder mutation in the PLPBP gene in families from Saguenay-Lac-St-Jean region affected by a pyridoxine-dependent epilepsy.

Pyridoxine-dependent epilepsy (PDE) is a relatively rare subgroup of epileptic disorders. They generally present in infancy as an early onset epileptic encephalopathy or seizures, refractory to standard treatments, with rapid and variable responses to vitamin B6 treatment. Whole exome sequencing of three unrelated families identified homozygous pathogenic mutation c.370_373del, p.Asp124fs in PLPBP gene in five persons. Haplotype analysis showed a single shared profile for the affected persons and their parents, leading to a hypothesis about founder effect of the mutation in Saguenay-Lac-St-Jean region of French Canadians. All affected probands also shared one single mitochondrial haplotype T2b3 and two rare variations in the mitochondrial genome m.801A>G and m.5166A>G suggesting that a single individual female introduced PLPBP mutation c.370_373del, p.Asp124fs in Quebec. The mutation p.Asp124fs causes a severe disease phenotype with delayed myelination and cortical/subcortical brain atrophy. The most noteworthy radiological finding in this Quebec founder mutation is the presence of the temporal cysts that can be used as a marker of the disease. Also, both patients, who are alive, had a history of prenatal supplements taken by their mothers as antiemetic medication with high doses of pyridoxine. In the context of suspected PDE in patients with neonatal refractory seizures, treatment with pyridoxine and/or Pyridoxal-5-phophate has to be started immediately and continued until the results of genetic analysis received. Even with early appropriate treatment, neurological outcome of our patient is still poor.

"I prefer dying fast than dying slowly", how institutional abuse worsens the mental health of stranded Syrian, Afghan and Congolese migrants on Lesbos island following the implementation of EU-Turkey deal.

BACKGROUND: In 2015 and early 2016, close to 1 million migrants transited through Greece, on their way to Western Europe. In early 2016, the closure of the "Balkan-route" and the EU/Turkey-deal led to a drastic reduction in the flow of migrants arriving to the Greek islands. The islands became open detention centers, where people would spend months or years under the constant fear of being returned to Turkey.Syrians were generally granted refugee status in Greece and those arrived before the 20th of March 2016 had the option of being relocated to other European countries. Afghans had some chances of being granted asylum in Greece, whilst most migrants from the Democratic Republic of Congo were refused asylum.In a clinic run by Médecins sans Frontières on Lesbos Island, psychologists observed a deterioration of the migrant's mental health (MH) since March 2016. In order to understand the MH needs for this stranded population it was essential to explore how, and by what factors, their mental health (MH) has been affected on Lesbos Island due to the EU/Turkey-deal. METHODS: This was a qualitative study in which eight service providers' interviews and 12 focus group discussions with male and female Syrian, Afghan and Congolese migrants in two refugee camps on Lesbos Island. Thematic-content analysis was manually applied and triangulation of findings was undertaken to enhance the interpretation of data. RESULTS: Three main themes were generated: 1) Institutional abuse, 2) Continuous traumatic stress (CTS) and 3) MH service provision. Institutional abuse was expressed by inhumane living conditions, lack of information in order to make future decisions, humiliation and depersonalization. This led to CTS that was expressed through being in a state of permanent emergency under lack of protective measures. Delays in appointments, lack of psychiatric care and differences in MH perceptions amongst migrants highlighted the provision of MH services. CONCLUSION: The EU/Turkey-deal reduced migrant flows at a very high price. Decongestion of the camps and the elimination of institutional abuse is urgently needed to reduce CTS and improve migrants' MH.

How to Find Lessons from the Public Health Literature: Example of a Scoping Study Protocol on the Neighborhood Environment.

BACKGROUND: As key determinants of many favorable health and quality of life outcomes, it is important to identify factors associated with mobility and social participation. Although several investigations have been carried out on mobility, social participation and neighborhood environment, there is no clear integration of these results. This paper presents a scoping study protocol that aims to provide a comprehensive understanding of how the physical and social neighborhood environment is associated with or influences mobility and social participation in older adults. METHODS: The rigorous methodological framework for scoping studies is used to synthesize and disseminate current knowledge on the associations or influence of the neighborhood environment on mobility and social participation in aging. Nine databases from public health and other fields are searched with 51 predetermined keywords. Using content analysis, all data are exhaustively analyzed, organized, and synthesized independently by two research assistants. DISCUSSION: A comprehensive synthesis of empirical studies provides decision-makers, clinicians and researchers with current knowledge and best practices regarding neighborhood environments with a view to enhancing mobility and social participation. Such a synthesis represents an original contribution and can ultimately support decisions and development of innovative interventions and clear guidelines for the creation of age-supportive environments. Improvements in public health and clinical interventions might be the new innovation needed to foster health and quality of life for aging population. Finally, the aspects of the associations or influence of the neighborhood environment on mobility and social participation not covered by previous research are identified. CONCLUSIONS: Among factors that impact mobility and social participation, the neighborhood environment is important since interventions targeting it may have a greater impact on an individual's mobility and social participation than those targeting individual factors. Although investigations from various domains have been carried out on this topic, no clear integration of these results is available yet.