Physical fitness in children with Marfan and Loeys-Dietz syndrome: associations between cardiovascular parameters, systemic manifestations, fatigue, and pain.

Children with Marfan (MFS) and Loeys-Dietz syndrome (LDS) report limitations in physical activities, sports, school, leisure, and work participation in daily life. This observational, cross-sectional, multicenter study explores associations between physical fitness and cardiovascular parameters, systemic manifestations, fatigue, and pain in children with MFS and LDS. Forty-two participants, aged 6-18 years (mean (SD) 11.5(3.7)), diagnosed with MFS (n = 36) or LDS (n = 6), were enrolled. Physical fitness was evaluated using the Fitkids Treadmill Test's time to exhaustion (TTE) outcome measure. Cardiovascular parameters (e.g., echocardiographic parameters, aortic surgery, cardiovascular medication) and systemic manifestations (systemic score of the revised Ghent criteria) were collected. Pain was obtained by visual analog scale. Fatigue was evaluated by PROMIS® Fatigue-10a-Pediatric-v2.0-short-form and PROMIS® Fatigue-10a-Parent-Proxy-v2.0-short-form. Multivariate linear regression analyses explored associations between physical fitness (dependent variable) and independent variables that emerged from the univariate linear regression analyses (criterion p < .05). The total group (MFS and LDS) and the MFS subgroup scored below norms on physical fitness TTE Z-score (mean (SD) -3.1 (2.9); -3.0 (3.0), respectively). Univariate analyses showed associations between TTE Z-score aortic surgery, fatigue, and pain (criterion p < .05). Multivariate analyses showed an association between physical fitness and pediatric self-reported fatigue that explained 48%; 49%, respectively, of TTE Z-score variance (F (1,18) = 18.6, p ≤ .001, r2 = .48; F (1,15) = 16,3, p = .01, r2 = .49, respectively).    Conclusions: Physical fitness is low in children with MFS or LDS and associated with self-reported fatigue. Our findings emphasize the potential of standardized and tailored exercise programs to improve physical fitness and reduce fatigue, ultimately enhancing the physical activity and sports, school, leisure, and work participation of children with MFS and LDS. What is Known: • Marfan and Loeys-Dietz syndrome are heritable connective tissue disorders and share cardiovascular and systemic manifestations. • Children with Marfan and Loeys-Dietz syndrome report increased levels of disability, fatigue and pain, as well as reduced levels of physical activity, overall health and health-related quality of life. What is New: • Physical fitness is low in children with Marfan and Loeys-Dietz syndrome and associated with self-reported fatigue. • Our findings emphasize the potential of standardized and tailored exercise programs to improve physical fitness and reduce fatigue, ultimately enhancing the physical activity and sports, school, leisure, and work participation of children with Marfan and Loeys-Dietz syndrome.

Metabolic load during morning care and active bed exercises in critically ill patients: An explorative study.

BACKGROUND: To avoid overexertion in critically ill patients, information on the physical demand, i.e., metabolic load, of daily care and active exercises is warranted. OBJECTIVE: The objective of this study was toassess the metabolic load during morning care activities and active bed exercises in mechanically ventilated critically ill patients. METHODS: This study incorporated an explorative observational study executed in a university hospital intensive care unit. Oxygen consumption (VO2) was measured in mechanically ventilated (≥48 h) critically ill patients during rest, routine morning care, and active bed exercises. We aimed to describe and compare VO2 in terms of absolute VO2 (mL) defined as the VO2 attributable to the activity and relative VO2 in mL per kilogram bodyweight, per minute (mL/kg/min). Additional outcomes achieved during the activity were perceived exertion, respiratory variables, and the highest VO2 values. Changes in VO2 and activity duration were tested using paired tests. RESULTS: Twenty-one patients were included with a mean (standard deviation) age of 59 y (12). Median (interquartile range [IQR]) durations of morning care and active bed exercises were 26 min (21-29) and 7 min (5-12), respectively. Absolute VO2 of morning care was significantly higher than that of active bed exercises (p = 0,009). Median (IQR) relative VO2 was 2.9 (2.6-3.8) mL/kg/min during rest; 3.1 (2.8-3.7) mL/kg/min during morning care; and 3.2 (2.7-4) mL/kg/min during active bed exercises. The highest VO2 value was 4.9 (4.2-5.7) mL/kg/min during morning care and 3.7 (3.2-5.3) mL/kg/min during active bed exercises. Median (IQR) perceived exertion on the 6-20 Borg scale was 12 (10.3-14.5) during morning care (n = 8) and 13.5 (11-15) during active bed exercises (n = 6). CONCLUSION: Absolute VO2 in mechanically ventilated patients may be higher during morning care than during active bed exercises due to the longer duration of the activity. Intensive care unit clinicians should be aware that daily-care activities may cause intervals of high metabolic load and high ratings of perceived exertion.

Somatic symptoms, pain, catastrophizing and the association with disability among children with heritable connective tissue disorders.

The aim of the present study was to investigate the nature and prevalence of nonspecific somatic symptoms, pain and catastrophizing in children with Heritable Connective Tissue Disorders (HCTD), and to determine their association with disability. This observational, multicenter study included 127 children, aged 4-18 years, with Marfan syndrome (MFS) (59%), Loeys-Dietz syndrome (LDS) (8%), Ehlers-Danlos syndromes (EDS) (12%) and hypermobile Ehlers-Danlos syndrome (hEDS) (23%). The assessments included the Children's Somatization Inventory or parent proxy (CSI, PCSI), pain visual-analogue scale (VAS), SUPERKIDZ body diagram, Pain Catastrophizing Scale Child or parent proxy (PCS-C, PCS-P) and Childhood Health Assessment Questionnaire (CHAQ-30). Data from children aged ≥8 years were compared to normative data. In children ≥ 8 years (n = 90), pain was present in 59%, with a median of 4 (IQR = 3-9) pain areas. Compared to normative data, the HCTD group reported significantly higher on the CSI (p ≤ 0.001, d = 0.85), VAS pain intensity (p ≤ 0.001, d = 1.22) and CHAQ-30 (p ≤ 0.001, d = 1.16) and lower on the PCS-C (p = 0.017, d = -0.82) and PCS-P (p ≤ 0.001, d = -0.49). The intensity of nonspecific somatic symptoms and pain explained 45% of the variance in disability (r2 = 0.45 F(2,48) = 19.70, p ≤ 0.001). In children ≤ 7 years (n = 37), pain was present in 35% with a median of 5(IQR = 1-13) pain areas. The mean(SD) VAS scores for pain intensity was 1.5(2.9). Functional disability was moderately correlated to the number of pain areas (r = 0.56, p ≤ 0.001), intensity of nonspecific somatic symptoms (r = 0.63, p ≤ 0.001) and pain (r = 0.83, p ≤ 0.001). In conclusion, this study supports the need for comprehensive assessment of nonspecific somatic symptoms, pain, and disability in children with HCTD to allow tailored treatment.

Heritable connective tissue disorders in childhood: Decreased health-related quality of life and mental health.

The psychosocial consequences of growing up with Heritable Connective Tissue Disorders (HCTD) are largely unknown. We aimed to assess Health-Related Quality of Life (HRQoL) and mental health of children and adolescents with HCTD. This observational multicenter study included 126 children, aged 4-18 years, with Marfan syndrome (MFS, n = 74), Loeys-Dietz syndrome (n = 8), molecular confirmed Ehlers-Danlos syndromes (n = 15), and hypermobile Ehlers-Danlos syndrome (hEDS, n = 29). HRQoL and mental health were assessed through the parent and child-reported Child Health Questionnaires (CHQ-PF50 and CHQ-CF45, respectively) and the parent-reported Strengths and Difficulties Questionnaire. Compared with a representative general population sample, parent-reported HRQoL of the HCTD-group showed significantly decreased Physical sum scores (p < 0.001, d = 0.9) and Psychosocial sum scores (p = 0.024, d = 0.2), indicating decreased HRQoL. Similar findings were obtained for child-reported HRQoL. The parent-reported mental health of the HCTD-group showed significantly increased Total difficulties sum scores (p = 0.01, d = 0.3), indicating decreased mental health. While the male and female MFS- and hEDS-subgroups both reported decreased HRQoL, only the hEDS-subgroup reported decreased mental health. In conclusion, children and adolescents with HCTD report decreased HRQoL and mental health, with most adverse outcomes reported in children with hEDS and least in those with MFS. These findings call for systematic monitoring and tailored interventions.

Physical rehabilitation interventions in children with acquired brain injury: a scoping review.

AIM: To synthesize the evidence about the characteristics (frequency, intensity, time, type) and effects of physical rehabilitation interventions on functional recovery and performance in daily functioning in children and young people with acquired brain injury (ABI), including traumatic brain injuries (TBI) and non-TBI, during the subacute rehabilitation phase. METHOD: Using scoping review methodology, a systematic literature search was performed using four databases. Articles were screened by title and abstract and data from eligible studies were extracted for synthesis. RESULTS: Nine of 3009 studies were included. The results demonstrated a variety of intervention characteristics: frequency varied between 1 and 7 days per week; time of intervention varied between 25 minutes and 6 hours a day; intervention types were specified in seven studies; and none of the included studies reported details of intensity of intervention. All studies reported positive results on the International Classification of Functioning, Disability and Health: Children and Youth (ICF-CY) levels of body function and activities after the intervention period, with study designs of included studies being cohort studies without concurrent controls (n=7) or case reports (n=2). INTERPRETATION: Inconsistency in results hampers generalizability to guide clinical practice. Physical interventions during subacute rehabilitation have potential to improve functional recovery with intervention characteristics as an important factor influencing its effectiveness. Future well-designed studies are indicated to gain knowledge and optimize rehabilitation practice in paediatric ABI and high-quality research including outcomes across all ICF-CY domains is needed.

The Better By Moving study: A multifaceted intervention to improve physical activity in adults during hospital stay.

OBJECTIVE: 'Better By Moving' is a multifaceted intervention developed and implemented in collaboration with patients and healthcare professionals to improve physical activity in hospitalized adults. This study aimed to understand if, how and why 'Better By Moving' resulted in higher levels of physical activity by evaluating both outcomes and implementation process. DESIGN: Mixed-methods study informed by the Medical Research Council guidance. SETTING: Tertiary hospital. PARTICIPANTS: Adult patients admitted to surgery, haematology, infectious diseases and cardiology wards, and healthcare professionals. MEASURES: Physical activity was evaluated before and after implementation using the Physical Activity Monitor AM400 on one random day during hospital stay between 8 am and 8 pm. Furthermore, the time spent lying on bed, length of stay and discharge destination was investigated. The implementation process was evaluated using an audit trail, surveys and interviews. RESULTS: There was no significant difference observed in physical activity (median [IQR] 23 [12-51] vs 27 [17-55] minutes, P = 0.107) and secondary outcomes before-after implementation. The intervention components' reach was moderate and adoption was low among patients and healthcare professionals. Patients indicated they perceived more encouragement from the environment and performed exercises more frequently, and healthcare professionals signalled increased awareness and confidence among colleagues. Support (priority, resources and involvement) was perceived a key contextual factor influencing the implementation and outcomes. CONCLUSION: Although implementing 'Better By Moving' did not result in significant improvements in outcomes at our centre, the process evaluation yielded important insights that may improve the effectiveness of implementing multifaceted interventions aiming to improve physical activity during hospital stay.

Parenting a child with Marfan syndrome: Distress and everyday problems.

Marfan syndrome (MFS) is a multisystemic, autosomal dominant connective tissue disorder that occurs de novo in 25%. In many families, parent and child(ren) are affected, which may increase distress in parents. To assess distress, 42 mothers (29% MFS) and 25 fathers (60% MFS) of 43 affected children, completed the validated screening-questionnaire Distress thermometer for parents of a chronically ill child, including questions on overall distress (score 0-10; ≥4 denoting "clinical distress") and everyday problems (score 0-36). Data were compared to 1,134 control-group-parents of healthy children. Mothers reported significantly less overall distress (2, 1-4 vs. 3, 1-6; p = .049; r = -.07) and total everyday problems (3, 0-6 vs. 4, 1-8; p = .03; r = -.08) compared to control-group-mothers. Mothers without MFS reported significantly less overall distress compared to mothers with MFS, both of a child with MFS (1, 0-4 vs. 3.5, 2-5; p = .039; r = -.17). No significant differences were found between the father-groups, nor between the group of healthy parents of an affected child living together with an affected partner compared to control-group-parents. No differences in percentages of clinical distress were reported between mothers and control-group-mothers (33 vs. 42%); fathers and control-group-fathers (28 vs. 32%); nor between the other groups. Distress was not associated with the children's MFS characteristics. Concluding, parents of a child with MFS did not show more clinical distress compared to parents of healthy children. However, clinical distress was reported in approximately one-third and may increase in case of acute medical complications. We advise monitoring distress in parents of a child with MFS to provide targeted support.

Feasibility of a home-based interdisciplinary rehabilitation program for patients with Post-Intensive Care Syndrome: the REACH study.

BACKGROUND: Survivors of critical illness experience long-term functional challenges, which are complex, heterogeneous, and multifactorial in nature. Although the importance of rehabilitation interventions after intensive care unit (ICU) discharge is universally recognized, evidence on feasibility and effectiveness of home-based rehabilitation programs is scarce and ambiguous. This study investigates the feasibility of an interdisciplinary rehabilitation program designed for patients with Post-Intensive Care Syndrome (PICS) who are discharged home. METHODS: A mixed method, non-randomized, prospective pilot feasibility study was performed with a 6-month follow-up, comparing the intervention (REACH) with usual care. REACH was provided by trained professionals and included a patient-centered, interdisciplinary approach starting directly after hospital discharge. Primary outcomes were patient safety, satisfaction, adherence, referral need and health care usage. Secondary outcomes, measured at 3 timepoints, were functional exercise capacity, self-perceived health status, health-related quality of life (HRQoL), return to work and psychotrauma. Risk of undernutrition was assessed at baseline. RESULTS: 43 patients with a median mechanical ventilation duration of 8 (IQR:10) days, were included in the study and 79.1% completed 6-month follow-up. 19 patients received the intervention, 23 received usual care. Groups were similar for gender distribution and ICU length of stay. No adverse events occurred. REACH participants showed higher satisfaction with treatment and reported more allied health professional visits, while the usual care group reported more visits to medical specialists. Qualitative analysis identified positive experiences among REACH-professionals related to providing state-of-the-art interventions and sharing knowledge and expertise within an interprofessional network. Similar recovery was seen between groups on all secondary outcomes, but neither group reached reference values for HRQoL at 6 months. Larger return to work rates were seen in the REACH group. Prevalence of undernutrition at hospital discharge was high in both groups (> 80%), warranting the need for careful tuning of physical therapy and nutritional interventions. CONCLUSIONS: This study shows that providing early, home-based rehabilitation interventions for patients with PICS-related symptoms is feasible and perceived positively by patients and professionals. When provided in an interdisciplinary collaborative network state of the art, person-centered interventions can be tailored to individual needs potentially increasing patient satisfaction, adherence, and efficacy. Registered in the Dutch Trial register: NL7792: https://www.trialregister.nl/trial/7792 , registered 7-06-2019.

Motivational factors mediate the association of general self-efficacy and performance outcomes in acutely hospitalised older patients.

OBJECTIVES: To study (i) the association of general self-efficacy (GSE) on the course of subjective (i.e. basic and instrumental activities of daily living (ADLs and IADLs) and objective physical performance outcomes (short physical performance battery (SPPB)) among older persons from discharge up to 3 months post-discharge and (ii) the extent to whether motivational factors such as depressive symptoms, apathy and fatigue mediate this association. METHODS: Prospective multi-centre cohort of acutely hospitalised patients aged ≥70 (Hospital-ADL study). Structural equation modelling was used to analyse the structural relationships. RESULTS: The analytic sample included 236 acutely hospitalised patients. GSE had a significant total effect on the course of subjective and objective performance outcomes (ADLs: ß = -0.21, P < 0.001, IADLs: ß = -0.24, P < 0.001 and SPPB: ß = 0.17, P < 0.001). However, when motivational factors as mediator were included into the same model, motivational factors (IADLs: ß = 0.51, P < 0.001; SPPB: ß = 0.49, P < 0.001) but not GSE remained significantly associated with IADLs (ß = -0.06, P = 0.16) and SPPB (ß = 0.002, P = 0.97). Motivational factors partially mediated the relationship between GSE and ADLs (ß = -0.09, P = 0.04). The percentage of mediation was 55, 74 and 99% for ADLs, IADLs and SPPB, respectively. CONCLUSIONS: Motivational factors and GSE are both associated with subjective and objective performance outcomes. However, the relationship between GSE and subjective and objective performance outcomes was highly mediated by motivational factors. Taken together, this suggests that GSE is important to being physically active but not sufficient to becoming more physical active in acutely hospitalised older patients; motivation is important to improving both subjective and objective performance.

Effectiveness of Serious Games to Increase Physical Activity in Children With a Chronic Disease: Systematic Review With Meta-Analysis.

BACKGROUND: Physical activity (PA) is important for children with a chronic disease. Serious games may be useful to promote PA levels among these children. OBJECTIVE: The primary purpose of this systematic review was to evaluate the effectiveness of serious games on PA levels in children with a chronic disease. METHODS: PubMed, EMBASE, PsycINFO, ERIC, Cochrane Library, and CINAHL were systematically searched for articles published from January 1990 to May 2018. Both randomized controlled trials and controlled clinical trials were included to examine the effects of serious games on PA levels in children with a chronic disease. Two investigators independently assessed the intervention, methods, and methodological quality in all articles using the Cochrane risk of bias tool. Both qualitative and quantitative analyses were performed. RESULTS: This systematic review included 9 randomized controlled trials (886 participants). In 2 of the studies, significant between-group differences in PA levels in favor of the intervention group were reported. The meta-analysis on PA levels showed a nonsignificant effect on moderate to vigorous PA (measured in minutes per day) between the intervention and control groups (standardized mean difference 0.30, 95% CI -0.15 to 0.75, P=.19). The analysis of body composition resulted in significantly greater reductions in BMI in the intervention group (standardized mean difference -0.24, 95% CI -0.45 to 0.04, P=.02). CONCLUSIONS: This review does not support the hypothesis that serious games improve PA levels in children with a chronic disease. The meta-analysis on body composition showed positive intervention effects with significantly greater reductions in BMI in favor of the intervention group. A high percentage of nonuse was identified in the study of serious games, and little attention was paid to behavior change theories and specific theoretical approaches to enhance PA in serious games. Small sample sizes, large variability between intervention designs, and limited details about the interventions were the main limitations. Future research should determine which strategies enhance the effectiveness of serious games, possibly by incorporating behavior change techniques.

Physiotherapy With Telerehabilitation in Patients With Complicated Postoperative Recovery After Esophageal Cancer Surgery: Feasibility Study.

BACKGROUND: Improvement of functional status with physiotherapy is an important goal for patients with postoperative complications and an increased length of hospital stay (LoS) after esophagectomy. Supervised physiotherapy with telerehabilitation instead of conventional face-to-face care could be an alternative to treat these patients in their home environment after hospital discharge (T0), but its feasibility has not yet been investigated in detail. OBJECTIVE: The aim of this study was to investigate the feasibility of a 12-week supervised postoperative physiotherapy intervention with telerehabilitation for patients with esophageal cancer who underwent esophagectomy and had postoperative complications or who had an increased LoS. The secondary objective was to investigate the preliminary effectiveness of telerehabilitation on functional recovery compared with usual care. METHODS: A prospective feasibility study with a matched historical comparison group was performed. Feasibility outcomes included willingness and adherence to participate, refusal rate, treatment duration, occurrence of adverse events, and patient satisfaction. Secondary outcome measures were measurements of musculoskeletal and cardiovascular functions and activities according to the domains of the International Classification of Functioning, Disability and Health. RESULTS: A total of 22 patients with esophageal cancer who underwent esophagectomy and had postoperative complications or an increased LoS were included. The mean age at surgery was 64.55 (SD 6.72) years, and 77% (17/22) of patients were male. Moreover, 15 patients completed the intervention. Patient adherence was 99.8% in the first 6 weeks and dropped to 75.6% in the following 6 weeks, with a mean difference of -24.3% (95% CI 1.3 to 47.2; P=.04). At 3 months post operation, no differences in functional status were found between the intervention group and the matched historical comparison group. CONCLUSIONS: This study showed that a postoperative physiotherapeutic intervention with telerehabilitation is feasible for patients with postoperative complications or an increased LoS after esophageal cancer surgery up to 6 weeks after T0.

Evaluation of a Blended Physical Activity Intervention for Older Adults: Mixed Methods Study.

BACKGROUND: Physical activity can prolong the ability of older adults to live independently. Home-based exercises can help achieve the recommended physical activity levels. A blended intervention was developed to support older adults in performing home-based exercises. A tablet and a personal coach were provided to facilitate the self-regulation of exercise behavior. OBJECTIVE: In line with the Medical Research Council framework, this study aimed to carry out process evaluation of a blended intervention. The objectives were (1) to assess the long-term usability of the tablet adopted in the blended intervention and (2) to explore how the tablet, in conjunction with a personal coach, supported older adults in performing home-based exercises. METHODS: The process evaluation was conducted with a mixed-methods approach. At baseline, older adults participating in the blended intervention were asked to fill out a questionnaire about their general experience with information and communication technology (ICT) devices and rate their own skill level. After 6 months, participants filled out the Usefulness, Satisfaction, and Ease of use (USE) questionnaire to assess the usefulness, satisfaction, and ease of use of the tablet. With a random selection of participants, in-depth interviews were held to explore how the tablet and coach supported the self-regulation. The interviews were double coded and analyzed with the directed content analysis method. RESULTS: At baseline, 29% (65/224) of participants who started the intervention (mean age 72 years) filled out the ICT survey and 36% (37/103) of participants who used the tablet for 6 months (mean age 71 years) filled out the USE questionnaire. Furthermore, with 17% (18/103) of participants (mean age 73 years), follow-up interviews were held. The results of the baseline questionnaire showed that the large majority of participants already had experience with a tablet, used it regularly, and reported being skillful in operating ICT devices. After 6 months of use, the participants rated the usefulness, satisfaction, and ease of use of the tablet on average as 3.8, 4.2, and 4.1, respectively, on a 5-point scale. The analysis of the interviews showed that the participants felt that the tablet supported action planning, behavior execution, and self-monitoring. On the other hand, especially during the first few months, the personal coach added value during the goal setting, behavior execution, and evaluation phases of self-regulation. CONCLUSIONS: The results of the process evaluation showed that older adults who participated in the study were positive about the blended intervention that was designed to support them in performing home-based exercises. Participants reported that the tablet helped them to perform the exercises better, more frequently, and safely. It supported them in various phases of self-regulation. The availability of a personal coach was nevertheless crucial. To support physical activity in older adults, a blended approach is promising.

Preparing undergraduate students for clinical work in a complex environment: evaluation of an e-learning module on physiotherapy in the intensive care unit.

BACKGROUND: Intensive Care Units (ICUs) are daunting environments for physiotherapy (PT) students performing clinical rotations. To prepare students for this environment, a newly developed, evidence-based e-learning module was designed and implemented in the undergraduate curriculum. The aim of this study was to investigate whether e-learning is a feasible method in preparing PT students for clinical work in complex ICU environments, as perceived by students and experts. METHODS: A mixed methods proof of concept study was undertaken. Participants were final-year students of an international curriculum, and experts from didactic and clinical fields. An e-learning module consisting of 7 separate chapters based on the latest scientific evidence and clinical expertise was developed, piloted and incorporated into the undergraduate curriculum as a compulsory course to be completed prior to clinical ICU rotations. Data were collected through 3 focus group meetings and 5 semi-structured interviews; these meetings and interviews were audio recorded, transcribed verbatim and analyzed. RESULTS: The study sample comprised of 14 students and 5 experts. Thematic analysis revealed three themes: expected competencies of PT students in ICU, feeling prepared for ICU clinical work and dealing with local variety. The e-learning module enabled students to anticipate clinical situations and PT tasks in the ICU. Higher level clinical reasoning skills, handling of lines and wires and dealing with out-of-textbook situations could not be achieved with the e-learning module alone. CONCLUSIONS: An e-learning module can sufficiently prepare PT students for their clinical tasks in the ICU, as long as it is integrated with, or closely connected to, the students' clinical placement.

Hopelessness and Other Depressive Symptoms in Adults 70 Years and Older as Predictors of All-Cause Mortality Within 3 Months After Acute Hospitalization: The Hospital-ADL Study.

OBJECTIVE: Depression among older adults predicts mortality after acute hospitalization. Depression is highly heterogeneous in its presentation of symptoms, whereas individual symptoms may differ in predictive value. This study aimed to investigate the prevalence of individual cognitive-affective depressive symptoms during acute hospitalization and investigate the predictive value of both overall and individual cognitive-affective depressive symptoms for mortality between admission up to 3-month postdischarge among older patients. METHODS: A prospective multicenter cohort study enrolled 401 acutely hospitalized patients 70 years and older (Hospitalization-Associated Disability and impact on daily Life Study). The predictive value of depressive symptoms, assessed using the Geriatric Depression Scale 15, during acute hospitalization on mortality was analyzed with multiple logistic regression. RESULTS: The analytic sample included 398 patients (M (SD) = 79.6 (6.6) years; 51% men). Results showed that 9.3% of participants died within 3 months, with symptoms of apathy being most frequently reported. The depression total score during hospitalization was associated with increased mortality risk (admission: odds ratio [OR] = 1.2, 95% confidence interval [CI] = 1.2-1.3; discharge: OR = 1.2, 95% CI = 1.2-1.4). Stepwise multiple logistic regression analyses yielded the finding that feelings of hopelessness during acute hospitalization were a strong unique predictor of mortality (admission: OR = 3.6, 95% CI = 1.8-7.4; discharge: OR = 5.7, 95% CI = 2.5-13.1). These associations were robust to adjustment for demographic factors, somatic symptoms, and medical comorbidities. CONCLUSIONS: Symptoms of apathy were most frequently reported in response to acute hospitalization. However, feelings of hopelessness about their situation were the strongest cognitive-affective predictor of mortality. These results imply that this item is important in identifying patients who are in the last phase of their lives and for whom palliative care may be important.

The diagnostic accuracy of headache measurement instruments: A systematic review and meta-analysis focusing on headaches associated with musculoskeletal symptoms.

AIM: To systematically review the available literature on the diagnostic accuracy of questionnaires and measurement instruments for headaches associated with musculoskeletal symptoms. DESIGN: Articles were eligible for inclusion when the diagnostic accuracy (sensitivity/specificity) was established for measurement instruments for headaches associated with musculoskeletal symptoms in an adult population. The databases searched were PubMed (1966-2018), Cochrane (1898-2018) and Cinahl (1988-2018). Methodological quality was assessed with the Quality Assessment of Diagnostic Accuracy Studies tool (QUADAS-2) and COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) checklist for criterion validity. When possible, a meta-analysis was performed. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) recommendations were applied to establish the level of evidence per measurement instrument. RESULTS: From 3450 articles identified, 31 articles were included in this review. Eleven measurement instruments for migraine were identified, of which the ID-Migraine is recommended with a moderate level of evidence and a pooled sensitivity of 0.87 (95% CI: 0.85-0.89) and specificity of 0.75 (95% CI: 0.72-0.78). Six measurement instruments examined both migraine and tension-type headache and only the Headache Screening Questionnaire - Dutch version has a moderate level of evidence with a sensitivity of 0.69 (95% CI 0.55-0.80) and specificity of 0.90 (95% CI 0.77-0.96) for migraine, and a sensitivity of 0.36 (95% CI 0.21-0.54) and specificity of 0.86 (95% CI 0.74-0.92) for tension-type headache. For cervicogenic headache, only the cervical flexion rotation test was identified and had a very low level of evidence with a pooled sensitivity of 0.83 (95% CI 0.72-0.94) and specificity of 0.82 (95% CI 0.73-0.91). DISCUSSION: The current review is the first to establish an overview of the diagnostic accuracy of measurement instruments for headaches associated with musculoskeletal factors. However, as most measurement instruments were validated in one study, pooling was not always possible. Risk of bias was a serious problem for most studies, decreasing the level of evidence. More research is needed to enhance the level of evidence for existing measurement instruments for multiple headaches.

Marfan syndrome in adolescence: adolescents' perspectives on (physical) functioning, disability, contextual factors and support needs.

Although essential for providing optimal adolescent patient support, knowledge of the impact of Marfan syndrome in adolescence is limited. To explore adolescents' perceived impact of Marfan syndrome on (physical) functioning (activities, participation), disability (limitations, restrictions), contextual factors and support needs, we interviewed 19 adolescents with Marfan syndrome. Audio-recordings were transcribed, coded and analysed using thematic analysis. Identified themes were "difficulties in keeping up with peers" and "being and feeling different from peers". Furthermore, an adolescent Marfan syndrome-specific International Classification of Functioning, Disability and Health for Children and Youth (ICF-CY) model derived from the data describing the adolescent perceived impact of Marfan syndrome on functioning, disability and its contextual factors. Adolescents perceived problems in keeping up with peers in school, sports, leisure and friendships/relationships, and they could not meet work requirements. Moreover, participants perceived to differ from peers due to their appearance and disability. Contextual factors: coping with Marfan syndrome, self-esteem/image, knowledge about Marfan syndrome, support from family/friends/teachers, ability to express needs and peer-group acceptation acted individually as barrier or facilitator for identified themes.Conclusion: Adolescents with Marfan syndrome perceived limitations and restrictions in (physical) functioning. They perceived problems in keeping up with peers and perceived to differ from peers due to their appearance and disability. This warrants awareness and tailored physical, psychosocial, educational and environmental support programmes to improve (physical) functioning and empowerment of adolescents with Marfan syndrome.What is known:• Marfan syndrome is a hereditary connective tissue disorder.• Marfan syndrome affects multiple systems.What is new:• Adolescents with Marfan syndrome perceive (1) problems in keeping up with peers in school, sports, leisure, friendships/relationships and work (2) to differ from peers due to their appearance and disability.• An adolescent Marfan syndrome-specific International Classification of Functioning, Disability and Health for Children and Youth model derived from the data describing the adolescent perceived impact of Marfan syndrome on functioning, disability and contextual factors.

Interventions for idiopathic toe walking.

BACKGROUND: Idiopathic toe walking (ITW) is an exclusionary diagnosis given to healthy children who persist in walking on their toes after they should typically have achieved a heel-toe gait. The literature discusses conservative and surgical interventions using a variety of treatment modalities. Young children and children without a limitation in ankle dorsiflexion (the upwards movement of the foot towards the shin of the leg) are commonly treated with conservative interventions. Older children who continue toe walking and present with limitations in ankle dorsiflexion are sometimes treated with surgical procedures. This systematic review is needed to evaluate the evidence for any intervention for the treatment of ITW. The conclusions of this review may support decision making by clinicians caring for children with ITW. It may also assist families when deciding on treatment options for their children with ITW. Many of the treatments employed have financial implications for parents or healthcare services. This review also aims to highlight any deficits in the current research base. OBJECTIVES: To assess the effects of conservative and surgical interventions in children with ITW, specifically effects on gait normalisation, ankle range of motion, pain, frequency of recurrence, and any adverse effects. SEARCH METHODS: On 29 April 2019, we searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, CINAHL Plus, and PEDro. We searched the following registers of clinical trials for ongoing and recently completed trials: the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP, apps.who.int/trialsearch), and ClinicalTrials.gov (clinicaltrials.gov). We searched conference proceedings and other grey literature in the BIOSIS databases and System for Information on Grey Literature in Europe (OpenGrey, opengrey.eu). We searched guidelines via the Turning Research Into Practice database (TRIP, tripdatabase.com) and National Guideline Clearinghouse (guideline.gov). We did not apply language restrictions. SELECTION CRITERIA: We considered randomised or quasi-randomised trials for inclusion in the review if they involved participants diagnosed with ITW gait in the absence of a medical condition known to cause toe walking, or associated with toe walking. As there is no universally accepted age group for ITW, this review includes ITW at any age, who have been toe walking for more than six months, who can or cannot walk with a heel-toe gait, and who may or may not have limited dorsiflexion of the ankle joint. DATA COLLECTION AND ANALYSIS: We used standard Cochrane methodological procedures. The primary outcome was improvement in toe walking (defined as greater than 50% of time spent heel-toe walking). Secondary outcomes were active and passive range of motion of the ankle joint, pain, recurrence of ITW after treatment, and adverse events. We assessed the certainty of the evidence using the GRADE framework. MAIN RESULTS: Four studies, comprising 104 participants, met the inclusion criteria. One study did not report data within the appropriate follow-up timeframe and data from two studies were insufficient for analysis. The single study from which we extracted data had 47 participants and was a randomised, controlled, parallel-group trial conducted in Sweden. It tested the hypothesis that combined treatment with serial casting and botulinum toxin type A (BTX) was more effective than serial casting alone in reducing ITW gait.This study found that more participants treated with BTX improved (defined as toe walking less than 50% of the time, as reported by parents) (risk ratio (RR) 1.21, 95% confidence interval (CI) 0.57 to 2.55; 1 trial, 46 participants; very low-certainty evidence). However, there was little or no difference between groups in passive ankle joint dorsiflexion range of movement on the right with the knee extended (mean difference (MD) -1.48º, 95% CI -4.13 to 1.16; 1 trial, 47 participants), on the right with the knee flexed (MD -0.04º, 95% CI -1.80 to 1.73; 1 trial, 46 participants), on the left with the knee flexed (MD 1.07, 95% CI -1.22 to 3.37), or on the left with the knee extended (MD 0.05, 95% CI -0.91 to 1.91). Nor was there a clear difference between the groups in recurrence of toe-walking gait (assessed via severity of toe walking (graded 1 (mild), 2 (moderate), or 3 (severe)) on gait analysis, analysed as continuous data: MD 0.34 points, 95% CI -0.09 to 0.78; 46 participants). In principle, MDs greater than zero (i.e.) positive values) would favour BTX and casting and negative values would favour casting alone. We have not reported effects as better or worse because all results were from evidence of very low certainty. We downgraded the certainty of evidence because of study limitations (outcome assessment was not blinded) and imprecision. Outcomes of pain and active range of motion were not reported in the included study.In terms of adverse events, calf pain was reported twice in the casting-only group and three times in the BTX group. There were three minor skin problems in each group and one reported case of pain directly after BTX injection. The report did not state if calf pain and skin irritation were from the same or different participants. The study authors reported that adverse events did not alter treatment adherence. AUTHORS' CONCLUSIONS: The certainty of evidence from one study, which compared serial casting with serial casting with BTX for ITW in children, was too low for conclusions to be drawn. A further three studies reported outcomes relating to BTX, footwear, exercises, and different types of orthoses as interventions, however the outcome data were too limited to assess their effects.

Long-Term Specialized Physical Therapy in Cervical Dystonia: Outcomes of a Randomized Controlled Trial.

OBJECTIVE: To evaluate the effectiveness of a specialized physical therapy (SPT) program on disability in cervical dystonia (CD) compared to regular physical therapy (RPT). DESIGN: A single-blinded randomized controlled trial. SETTING: This study was performed by a physical therapist in a primary health care setting. Measurements were performed at baseline, 6 and 12 months in the botulinum toxin (BoNT) outpatient clinic of the neurology department. PARTICIPANTS: Patients with primary CD and stable on BoNT treatment for 1 year (N=96). MAIN OUTCOME MEASURES: The primary outcome was disability assessed with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS). Secondary outcomes were pain, anxiety, depression, quality of life (QOL), and health related costs over 12 months. RESULTS: A total of 72 participants (30 men, 42 women) finished the study: 40 received SPT, 32 RPT. No significant between group differences were found after 12 months of treatment (P=.326). Over these 12 months both groups improved significantly (P<.001) on the TWSTRS disability scale compared to baseline (SPT 1.7 points, RPT 1.0 points). Short Form 36 (SF-36) General Health Perceptions (P=.046) and self-perceived improvement (P=.007) showed significantly larger improvements after 12 months in favor of SPT. Total health related costs after 12 months were $1373±556 for SPT compared to $1614±917 for RPT. CONCLUSION: SPT revealed no significant differences compared to RPT after 12 months of treatment on the TWSTRS disability scale. Both groups showed similar improvements compared to baseline. Positive results in the SPT group were higher patient perceived effects and general health perception. Treatment costs were lower in the SPT group. With lower costs and similar effects, the SPT program seems to be the preferred program to treat CD.

Effects of Postacute Multidisciplinary Rehabilitation Including Exercise in Out-of-Hospital Settings in the Aged: Systematic Review and Meta-analysis.

OBJECTIVE: Many older individuals receive rehabilitation in an out-of-hospital setting (OOHS) after acute hospitalization; however, its effect on mobility and unplanned hospital readmission is unclear. Therefore, a systematic review and meta-analysis were conducted on this topic. DATA SOURCES: Medline OVID, Embase OVID, and CINAHL were searched from their inception until February 22, 2018. STUDY SELECTION: OOHS (ie, skilled nursing facilities, outpatient clinics, or community-based at home) randomized trials studying the effect of multidisciplinary rehabilitation were selected, including those assessing exercise in older patients (mean age ≥65y) after discharge from hospital after an acute illness. DATA EXTRACTION: Two reviewers independently selected the studies, performed independent data extraction, and assessed the risk of bias. Outcomes were pooled using fixed- or random-effect models as appropriate. The main outcomes were mobility at and unplanned hospital readmission within 3 months of discharge. DATA SYNTHESIS: A total of 15 studies (1255 patients) were included in the systematic review and 12 were included in the meta-analysis (7 assessing mobility using the 6-minute walk distance [6MWD] test and 7 assessing unplanned hospital readmission). Based on the 6MWD, patients receiving rehabilitation walked an average of 23 m more than controls (95% confidence interval [CI]=: -1.34 to 48.32; I2: 51%). Rehabilitation did not lower the 3-month risk of unplanned hospital readmission (risk ratio: 0.93; 95% CI: 0.73-1.19; I2: 34%). The risk of bias was present, mainly due to the nonblinded outcome assessment in 3 studies, and 7 studies scored this unclearly. CONCLUSION: OOHS-based multidisciplinary rehabilitation leads to improved mobility in older patients 3 months after they are discharged from hospital following an acute illness and is not associated with a lower risk of unplanned hospital readmission within 3 months of discharge. However, the wide 95% CIs indicate that the evidence is not robust.

Feasibility of Exercise Testing in Patients Who Are Critically Ill: A Prospective, Observational Multicenter Study.

OBJECTIVE: To evaluate the feasibility and safety of exercise testing and to describe the physiological response to exercise of patients in the Intensive Care Unit (ICU). DESIGN: A prospective observational multicenter study. SETTING: Two mixed medical-surgical ICUs. PARTICIPANTS: Patients (N=37; with no primary neurological disorders, 59% men; median age 50y; ICU length of stay 14.5d; Acute Physiology and Chronic Health Evaluation IV 73.0) who had been mechanically ventilated for more than 48 hours and were hemodynamically stable enough to perform physical exercise. INTERVENTIONS: A passive or active incremental exercise test, depending on muscle strength, on a bed-based cycle ergometer. MAIN OUTCOME MEASURES: Feasibility and safety were evaluated based on protocol adherence and adverse events. Physiological responses to exercise quantified as changes in respiratory frequency (RF), oxygen uptake (Vo2), carbon dioxide output (Vco2), respiratory exchange ratio (RER), and blood lactate. RESULTS: Thirty-seven patients of whom 18 were mechanically ventilated underwent the exercise test. The active incremental test was performed by 28, and the passive test by 9 participants. Thirty-three (89%) accomplished the test according to the protocol and 1 moderate severe adverse event (bradycardia; heart rate 44) occurred shortly after the test. RF, Vo2, Vco2, and lactate increased significantly, whereas RER did not change during the active incremental exercise test. No changes were observed during the passive exercise test. CONCLUSIONS: It is safe and feasible to perform exercise testing on a bed-based cycle ergometer in patients who are critically ill and a physiological response could be measured. Future research should investigate the clinical value of exercise testing in daily ICU practice and whether exercise capacity and its limiting factors could be determined by incremental exercise testing.