Periodontal Manifestations of Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy and Elevated Temperature (CANDLE) Syndrome in an 11 Year Old Patient

Introduction: Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) is an auto inflammatory syndrome caused by an autosomal recessive gene mutation. This very rare syndrome has been reported in only 14 patients worldwide. A number of clinical signs have been reported including joint contractures, muscle atrophy, microcytic anaemia, and panniculitis-induced childhood lipodystrophy. Further symptoms include recurrent fevers, purpuric skin lesions, periorbital erythema and failure to thrive. This is the first reported case of periodontal manifestations associated with CANDLE syndrome. 
Case Presentation: An 11 year old boy was referred to Cork University Dental School and Hospital with evidence of severe periodontal destruction. The patient’s medical condition was managed in Great Ormond Street Children’s Hospital, London. The patient’s dental management included initial treatment to remove teeth of hopeless prognosis followed by prosthodontic rehabilitation using removable partial dentures. This was followed by further non-surgical periodontal treatment and maintenance. In the long term, the potential definitive restorative options, including dental implants, will be evaluated in discussion with the patient’s medical team.
Conclusion: Periodontitis as a manifestation of systemic disease is one of seven categories of periodontitis as defined by the American Academy of Periodontology 1999 classification system. A number of systemic diseases have been associated with advanced periodontal destruction including Diabetes Mellitus, Leukaemia and Papillon-Lefevre Syndrome. In the case described, treatment necessitated a multidisciplinary approach with input from medical and dental specialities for a young patient with severe periodontal destruction associated with CANDLE syndrome.

Physical rehabilitation interventions for adult patients during critical illness: an overview of systematic reviews

Background Physical rehabilitation interventions aim to ameliorate the effects of critical illness-associated muscle dysfunction in survivors. We conducted an overview of systematic reviews (SR) evaluating the effect of these interventions across the continuum of recovery.

Methods Six electronic databases (Cochrane Library, CENTRAL, DARE, Medline, Embase, and Cinahl) were searched. Two review authors independently screened articles for eligibility and conducted data extraction and quality appraisal. Reporting quality was assessed and the Grading of Recommendations Assessment, Development and Evaluation approach applied to summarise overall quality of evidence.

Results Five eligible SR were included in this overview, of which three included meta-analyses. Reporting quality of the reviews was judged as medium to high. Two reviews reported moderate-to-high quality evidence of the beneficial effects of physical therapy commencing during intensive care unit (ICU) admission in improving critical illness polyneuropathy/myopathy, quality of life, mortality and healthcare utilisation. These interventions included early mobilisation, cycle ergometry and electrical muscle stimulation. Two reviews reported very low to low quality evidence of the beneficial effects of electrical muscle stimulation delivered in the ICU for improving muscle strength, muscle structure and critical illness polyneuropathy/myopathy. One review reported that due to a lack of good quality randomised controlled trials and inconsistency in measuring outcomes, there was insufficient evidence to support beneficial effects from physical rehabilitation delivered post-ICU discharge.

Conclusions Patients derive short-term benefits from physical rehabilitation delivered during ICU admission. Further robust trials of electrical muscle stimulation in the ICU and rehabilitation delivered following ICU discharge are needed to determine the long-term impact on patient care. This overview provides recommendations for design of future interventional trials and SR.

Investigating Fractured Flow from Orthopaedics to Rehabilitation

Patients often spend time as inpatients in hospitals outside their home area because of the need to access specialist services. If there is a need for ongoing care after the need for specialist care has passed, patients are transferred (or ‘repatriated’) back to the inpatient care of a hospital in their local Health and Social Care Trust. The need for bed space in specialist units means that there is pressure for this transition to occur in a timely way. We investigated the flow of patients through a trauma and orthopaedics unit using the 6M Design® framework and Vitals Charts® in order to investigate concerns about delayed repatriation. We found that repatriation was part of a complex system that had interdependent components. There was considerable variation in the number of discharges (to any destination) by day of week, with a reduction on Saturdays and Sundays. Understanding that the pressure for quicker repatriation was really due to high work-in-progress led us to model the effects of strategies to address the high work-in-progress. We found that, because only a small proportion of patients require repatriation, expediting the repatriation process by one day for each patient would only reduce WIP by an average of 1.6 patients. Reducing the average length of stay for all trauma and orthopaedics inpatients by one day would reduce the WIP by 10 patients, which would make a much greater impact on the problem of high bed occupancy. Though the smooth and timely repatriation of patients to rehabilitation units is desirable, it is unlikely that efforts to achieve this will have a substantial impact on the problem of high WIP, so other strategies will be required. We will model the effects of strategies to reduce variation in daily discharges by the day of week in a future essay.

SWORD: an intelligent vibratory wearable device to improve rehabilitation in stroke patients

Anualmente ocorrem cerca de 16 milhões AVCs em todo o mundo. Cerca de metade dos sobreviventes irá apresentar défice motor que necessitará de reabilitação na janela dos 3 aos 6 meses depois do AVC. Nos países desenvolvidos, é estimado que os custos com AVCs representem cerca de 0.27% do Produto Interno Bruto de cada País. Esta situação implica um enorme peso social e financeiro. Paradoxalmente a esta situação, é aceite na comunidade médica a necessidade de serviços de reabilitação motora mais intensivos e centrados no doente. Na revisão do estado da arte, demonstra-se o arquétipo que relaciona metodologias terapêuticas mais intensivas com uma mais proficiente reabilitação motora do doente. Revelam-se também as falhas nas soluções tecnológicas existentes que apresentam uma elevada complexidade e custo associado de aquisição e manutenção. Desta forma, a pergunta que suporta o trabalho de doutoramento seguido inquire a possibilidade de criar um novo dispositivo de simples utilização e de baixo custo, capaz de apoiar uma recuperação motora mais eficiente de um doente após AVC, aliando intensidade com determinação da correcção dos movimentos realizados relativamente aos prescritos. Propondo o uso do estímulo vibratório como uma ferramenta proprioceptiva de intervenção terapêutica a usar no novo dispositivo, demonstra-se a tolerabilidade a este tipo de estímulos através do teste duma primeira versão do sistema apenas com a componente de estimulação num primeiro grupo de 5 doentes. Esta fase validará o subsequente desenvolvimento do sistema SWORD. Projectando o sistema SWORD como uma ferramenta complementar que integra as componentes de avaliação motora e intervenção proprioceptiva por estimulação, é descrito o desenvolvimento da componente de quantificação de movimento que o integra. São apresentadas as diversas soluções estudadas e o algoritmo que representa a implementação final baseada na fusão sensorial das medidas provenientes de três sensores: acelerómetro, giroscópio e magnetómetro. O teste ao sistema SWORD, quando comparado com o método de reabilitação tradicional, mostrou um ganho considerável de intensidade e qualidade na execução motora para 4 dos 5 doentes testados num segundo grupo experimental. É mostrada a versatilidade do sistema SWORD através do desenvolvimento do módulo de Tele-Reabilitação que complementa a componente de quantificação de movimento com uma interface gráfica de feedback e uma ferramenta de análise remota da evolução motora do doente. Finalmente, a partir da componente de quantificação de movimento, foi ainda desenvolvida uma versão para avaliação motora automatizada, implementada a partir da escala WMFT, que visa retirar o factor subjectivo da avaliação humana presente nas escalas de avaliação motora usadas em Neurologia. Esta versão do sistema foi testada num terceiro grupo experimental de cinco doentes.

Impact of an intervention to control risk associated with patient transfer.

QUESTION UNDER STUDY: Hospitals transferring patients retain responsibility until admission to the new health care facility. We define safe transfer conditions, based on appropriate risk assessment, and evaluate the impact of this strategy as implemented at our institution. METHODS: An algorithm defining transfer categories according to destination, equipment monitoring, and medication was developed and tested prospectively over 6 months. Conformity with algorithm criteria was assessed for every transfer and transfer category. After introduction of a transfer coordination centre with transfer nurses, the algorithm was implemented and the same survey was carried out over 1 year. RESULTS: Over the whole study period, the number of transfers increased by 40%, chiefly by ambulance from the emergency department to other hospitals and private clinics. Transfers to rehabilitation centres and nursing homes were reassigned to conventional vehicles. The percentage of patients requiring equipment during transfer, such as an intravenous line, decreased from 34% to 15%, while oxygen or i.v. drug requirement remained stable. The percentage of transfers considered below theoretical safety decreased from 6% to 4%, while 20% of transfers were considered safer than necessary. A substantial number of planned transfers could be "downgraded" by mutual agreement to a lower degree of supervision, and the system was stable on a short-term basis. CONCLUSION: A coordinated transfer system based on an algorithm determining transfer categories, developed on the basis of simple but valid medical and nursing criteria, reduced unnecessary ambulance transfers and treatment during transfer, and increased adequate supervision.

Étude de la réponse aiguë à l'exercice intermittent à haute intensité chez le patient coronarien

L'entraînement par intervalles à haute intensité est plus efficace que l'entraînement continu d’intensité modérée pour améliorer la consommation maximale d’oxygène (VO2max) et le profil métabolique des patients coronariens. Cependant, il n’y a pas de publications pour appuyer la prescription d’un type d’exercice intermittent (HIIE) spécifique dans cette population. Nous avons donc comparé les réponses aiguës cardio-pulmonaires de quatre sessions différentes d’exercice intermittent dans le but d’identifier l’exercice optimal chez les patients coronariens. De manière randomisée, les sujets participaient aux sessions d’HIIE, toutes avec des phases d’exercice à 100% de la puissance maximale aérobie (PMA), mais qui variaient selon la durée des phases d’exercice et de récupération (15s ou 1 min) et la nature de la récupération (0% de la PMA ou 50% de la PMA). Chaque session était réalisée sous forme de temps limite et l’exercice était interrompu après 35 minutes. En considérant l’effort perçu, le confort du patient et le temps passé au-dessus de 80% de VO2max, nous avons trouvé que l’exercice optimal consistait à alterner des courtes phases d’exercice de 15s à 100% de la PMA avec des phases de 15s de récupération passive. Ensuite, nous avons comparé les réponses physiologiques de l’HIIE optimisé avec un exercice continu d’intensité modérée (MICE) iso-calorique chez des patients coronariens. En considérant les réponses physiologiques, l’aspect sécuritaire (aucune élévation de Troponin T) et l’effort perçu, le protocole HIIE est apparu mieux toléré et plus efficace chez ces coronariens. Finalement, une simple session d’HIIE n’induit pas d’effets délétères sur la paroi vasculaire, comme démontré avec l’analyse des microparticules endothéliales. En conclusion, l’exercice intermittent à haute intensité est un mode d'entraînement prometteur pour les patients coronariens stables qui devrait faire l’objet d’autres études expérimentales en particulier pour les patients coronariens ischémiques.

Étude des réponses physiologiques aiguës à l’exercice intermittent de haute intensité chez le patient insuffisant cardiaque

L’optimisation de l’exercice par intervalles de haute intensité (EIHI) chez les patients insuffisants cardiaques (IC) n’a jamais été étudiée auparavant. Nous avons comparé les réponses cardio-pulmonaires aiguës lors de 4 différents EIHI dans le but de trouver le protocole optimisé chez les patients IC. Les patients IC étaient aléatoirement alloués à 4 sessions d’EIHI. Chaque phase d’exercice était à une intensité de 100% de la puissance aérobie maximale (PAM), mais de différentes durées (30s ou 90s) et de type de récupération (passive ou active). Chaque protocole d’EIHI durait un maximum de 30 minutes ou jusqu’à épuisement. Considérant le temps total d’exercice, l’adhérence, une perception d’effort moins élevée, le confort du patient ainsi que des temps similaires passés à un haut pourcentage du VO2pic, le mode avec intervalles courts (30s) et récupération passive s’est avéré être le protocole d’EIHI optimisé chez ces patients. Suite à cette étude, nous avons voulu comparer les réponses cardio-pulmonaires aiguës d’un exercice continu d’intensité modéré (ECIM) par rapport à celles de l’EIHI optimisé de dépense énergétique équivalente chez les patients IC. L’objectif de cette étude était de comparer les réponses cardio-pulmonaires, l’adhérence, la perception de l’effort, l’inflammation et les biomarqueurs cardiaques. Comparativement à l’ECIM, l’adhérence, l’efficience et la tolérance étaient plus élevées lors de l’EIHI optimisé chez les patients IC tout en produisant un stimulus physiologique important. L’EIHI n’a causé aucune arythmie significative ou d’effets délétères sur l’inflammation (CRP), le BNP et la nécrose myocardique (C-TnT) chez les patients IC. L’EIHI semble être un mode d’exercice prometteur et devrait être considéré lors de la réadaptation cardiaque chez les patients IC.

Wait Times to Rheumatology and Rehabilitation Services for Persons with Arthritis in Quebec

L’arthrite est l’une des causes principales de douleur et d’incapacité auprès de la population canadienne. Les gens atteints d’arthrite rhumatoïde (AR) devraient être évalués par un rhumatologue moins de trois mois suivant l’apparition des premiers symptômes et ce afin de débuter un traitement médical approprié qui leur sera bénéfique. La physiothérapie et l’ergothérapie s’avèrent bénéfiques pour les patients atteints d’ostéoarthrite (OA) et d’AR, et aident à réduire l’incapacité. Notre étude a pour but d’évaluer les délais d’attente afin d’obtenir un rendez-vous pour une consultation en rhumatologie et en réadaptation dans le système de santé public québécois, et d’explorer les facteurs associés. Notre étude est de type observationnel et transversal et s’intéresse à la province de Québec. Un comité d’experts a élaboré trois scénarios pour les consultations en rhumatologie : AR présumée, AR possible, et OA présumée ; ainsi que deux scénarios pour les consultations en réadaptation : AR diagnostiquée, OA diagnostiquée. Les délais d’attente ont été mesurés entre le moment de la requête initiale et la date de rendez-vous fixée. L’analyse statistique consiste en une analyse descriptive de même qu’une analyse déductive, à l’aide de régression logistique et de comparaison bivariée. Parmi les 71 bureaux de rhumatologie contactés, et pour tous les scénarios combinés, 34% ont donné un rendez-vous en moins de trois mois, 32% avaient une attente de plus de trois mois et 34% ont refusé de fixer un rendez-vous. La probabilité d’obtenir une évaluation en rhumatologie en moins de trois mois est 13 fois plus grande pour les cas d’AR présumée par rapport aux cas d’OA présumée (OR=13; 95% Cl [1.70;99.38]). Cependant, 59% des cas d’AR présumés n’ont pas obtenu rendez-vous en moins de trois mois. Cent centres offrant des services publics en réadaptation ont été contactés. Pour tous les scénarios combinés, 13% des centres ont donné un rendez-vous en moins de 6 mois, 13% entre 6 et 12 mois, 24% avaient une attente de plus de 12 mois et 22% ont refusé de fixer un rendez-vous. Les autres 28% restant requéraient les détails d’une évaluation relative à l’état fonctionnel du patient avant de donner un rendez-vous. Par rapport aux services de réadaptation, il n’y avait aucune différence entre les délais d’attente pour les cas d’AR ou d’OA. L’AR est priorisée par rapport à l’OA lorsque vient le temps d’obtenir un rendez-vous chez un rhumatologue. Cependant, la majorité des gens atteints d’AR ne reçoivent pas les services de rhumatologie ou de réadaptation, soit physiothérapie ou ergothérapie, dans les délais prescrits. De meilleures méthodes de triage et davantage de ressources sont nécessaires.

Who knows best? Awareness of divided attention difficulty in a neurological rehabilitation setting

Objective: To explore whether patients relearning to walk after acquired brain injury and showing cognitive-motor interference were aware of divided attention difficulty; whether their perceptions concurred with those of treating staff. Design: Patients and neurophysiotherapists (from rehabilitation and disabled wards) completed questionnaires. Factor analyses were applied to responses. Correlations between responses, clinical measures and experimental decrements were examined. Results: Patient/staff responses showed some agreement; staff reported higher levels of perceived difficulty; responses conformed to two factors. One factor (staff/patients alike) reflected expectations about functional/motor status and did not correlate with decrements. The other factor (patients) correlated significantly with dual-task motor decrement, suggesting some genuine awareness of difficulty (cognitive performance prioritized over motor control). The other factor (staff) correlated significantly with cognitive decrement (gait prioritized over sustained attention). Conclusions: Despite some inaccurate estimation of susceptibility; patients and staff do exhibit awareness of divided attention difficulty, but with a limited degree of concurrence. In fact, our results suggest that patients and staff may be sensitive to different aspects of the deficit. Rather than 'Who knows best?', it is a question of 'Who knows what?.

A Gentle/S approach to robot assisted neuro-rehabilitation

Movement disorders (MD) include a group of neurological disorders that involve neuromotor systems. MD can result in several abnormalities ranging from an inability to move, to severe constant and excessive movements. Strokes are a leading cause of disability affecting largely the older people worldwide. Traditional treatments rely on the use of physiotherapy that is partially based on theories and also heavily reliant on the therapists training and past experience. The lack of evidence to prove that one treatment is more effective than any other makes the rehabilitation of stroke patients a difficult task. UL motor re-learning and recovery levels tend to improve with intensive physiotherapy delivery. The need for conclusive evidence supporting one method over the other and the need to stimulate the stroke patient clearly suggest that traditional methods lack high motivational content, as well as objective standardised analytical methods for evaluating a patient's performance and assessment of therapy effectiveness. Despite all the advances in machine mediated therapies, there is still a need to improve therapy tools. This chapter describes a new approach to robot assisted neuro-rehabilitation for upper limb rehabilitation. Gentle/S introduces a new approach on the integration of appropriate haptic technologies to high quality virtual environments, so as to deliver challenging and meaningful therapies to people with upper limb impairment in consequence of a stroke. The described approach can enhance traditional therapy tools, provide therapy "on demand" and can present accurate objective measurements of a patient's progression. Our recent studies suggest the use of tele-presence and VR-based systems can potentially motivate patients to exercise for longer periods of time. Two identical prototypes have undergone extended clinical trials in the UK and Ireland with a cohort of 30 stroke subjects. From the lessons learnt with the Gentle/S approach, it is clear also that high quality therapy devices of this nature have a role in future delivery of stroke rehabilitation, and machine mediated therapies should be available to patient and his/her clinical team from initial hospital admission, through to long term placement in the patient's home following hospital discharge.

Advances in upper limb stroke rehabilitation: a technology push

Strokes affect thousands of people worldwide leaving sufferers with severe disabilities affecting their daily activities. In recent years, new rehabilitation techniques have emerged such as constraint-induced therapy, biofeedback therapy and robot-aided therapy. In particular, robotic techniques allow precise recording of movements and application of forces to the affected limb, making it a valuable tool for motor rehabilitation. In addition, robot-aided therapy can utilise visual cues conveyed on a computer screen to convert repetitive movement practice into an engaging task such as a game. Visual cues can also be used to control the information sent to the patient about exercise performance and to potentially address psychosomatic variables influencing therapy. This paper overviews the current state-of-the-art on upper limb robot-mediated therapy with a focal point on the technical requirements of robotic therapy devices leading to the development of upper limb rehabilitation techniques that facilitate reach-to-touch, fine motor control, whole-arm movements and promote rehabilitation beyond hospital stay. The reviewed literature suggest that while there is evidence supporting the use of this technology to reduce functional impairment, besides the technological push, the challenge ahead lies on provision of effective assessment of outcome and modalities that have a stronger impact transferring functional gains into functional independence.

Prescription software for recovery and rehabilitation using Microsoft Kinect

Brain injuries, including stroke, can be debilitating incidents with potential for severe long term effects; many people stop making significant progress once leaving in-patient medical care and are unable to fully restore their quality of life when returning home. The aim of this collaborative project, between the Royal Berkshire NHS Foundation Trust and the University of Reading, is to provide a low cost portable system that supports a patient's condition and their recovery in hospital or at home. This is done by providing engaging applications with targeted gameplay that is individually tailored to the rehabilitation of the patient's symptoms. The applications are capable of real-time data capture and analysis in order to provide information to therapists on patient progress and to further improve the personalized care that an individual can receive.

Feasibility study on EEG driven robotic system to realize efficient stroke rehabilitation

We aim to develop an efficient robotic system for stroke rehabilitation, in which a robotic arm moves the hemiplegic upper limb when the patient tries to move it. In order to achieve this goal we have considered a method to detect the patient's intended motion using EEG (Electroencephalogram), and have designed a rehabilitation robot based on a Redundant Drive Method. In this paper, we propose an EEG driven rehabilitation robot system and present initial results evaluating the feasibility of the proposed system.

Assessing feasibility and acceptability of study procedures : getting ready for implementation of national stroke guidelines in out-patient health care

BACKGROUND: Even though Swedish national guidelines for stroke care (SNGSC) have been accessible for nearly a decade access to stroke rehabilitation in out-patient health care vary considerably. In order to aid future interventions studies for implementation of SNGSC, this study assessed the feasibility and acceptability of study procedures including analysis of the context in out-patient health care settings. METHODS: The feasibility and acceptability of recruitment, observations and interviews with managers, staff and patients were assessed, as well as the feasibility of surveying health care records. RESULTS: To identify patients from the the hospitals was feasible but not from out-patient care where a need to relieve clinical staff of the recruitment process was identified. Assessing adherence to guidelines and standardized evaluations of patient outcomes through health care records was found to be feasible and suitable assessment tools to evaluate patient outcome were identified. Interviews were found to be a feasible and acceptable tool to survey the context of the health care setting. CONCLUSION: In this feasibility study a variety of qualitative and quantitative data collection procedures and measures were tested. The results indicate what can be used as a set of feasible and acceptable data collection procedures and suitable measures for studying implementation of stroke guidelines in an out-patient health care context.

Unfulfilled rehabilitation needs and dissatisfaction with care 12 months after a stroke : an explorative observational study

BACKGROUND: People who have suffered a stroke commonly report unfulfilled need for rehabilitation. Using a model of patient satisfaction, we examined characteristics in individuals that at 3 months after stroke predicted, or at 12 months were associated with unmet need for rehabilitation or dissatisfaction with health care services at 12 months after stroke. METHODS: The participants (n = 175) received care at the stroke units at the Karolinska University Hospital, Sweden. The dependent variables "unfulfilled needs for rehabilitation" and "dissatisfaction with care" were collected using a questionnaire. Stroke severity, domains of the Stroke Impact Scale (SIS), the Sense of Coherence scale (SOC) and socio demographic factors were used as independent variables in four logistic regression analyses. RESULTS: Unfulfilled needs for rehabilitation at 12 months were predicted by strength (SIS) (odds ratio (OR) 7.05) at three months, and associated with hand function (SIS) (OR 4.38) and poor self-rated recovery (SIS) (OR 2.46) at 12 months. Dissatisfaction with care was predicted by SOC (OR 4.18) and participation (SIS) (OR 3.78), and associated with SOC (OR 3.63) and strength (SIS) (OR 3.08). CONCLUSIONS: Thirty-three percent of the participants reported unmet needs for rehabilitation and fourteen percent were dissatisfied with the care received. In order to attend to rehabilitation needs when they arise, rehabilitation services may need to be more flexible in terms of when rehabilitation is provided. Long term services with scheduled re-assessments and with more emphasis on understanding the experiences of both the patients and their social networks might better be able to provide services that attend to patients' needs and aid peoples' reorientation; this would apply particularly to those with poor coping capacity.