All hands on deck: The multidisciplinary rehabilitation assessment and management of hand function in persons with neuromuscular disorders.

Hand function is important in every aspect of our lives. Across a wide range of neuromuscular disorders-inherited ataxias, motor neuron diseases, polyneuropathies, and myopathies-people can experience losses in hand strength, tone, movement, dexterity, joint range, and sensation. Such changes can adversely affect function and independence in daily activities, reducing participation and quality of life. People with neuromuscular disorders (pwNMD) known to involve the hand should be assessed at regular intervals for changes both clinically and using impairment, performance, function, and patient-reported outcome measures as appropriate. A patient-centered approach to management is recommended, with clinicians partnering with the individual, their caregivers and the interprofessional teams to create personalized solutions that can overcome barriers to participation and best meet the goals of individuals affected by neuromuscular disorders. Management strategies should be multifaceted, and may include exercise, orthoses, assistive devices, technological solutions, environmental or task adaptations, medications, and/or surgery. Exercise recommendations and orthoses should be individualized and evolve based on disease progression, impairments, and functional limitations. While medications and surgery have a small role for specific clinical situations, there is a plethora of assistive and technological solutions to assist with basic and instrumental activities of daily living, work/education, and leisure for pwNMD with reduced hand function. In addition, clinicians should advocate for appropriate accommodations for reduced hand function at work/school, and the development of and adherence to legislation supporting accessibility and inclusion.

Spasticity Management Teams, Evaluations, and Tools: A Canadian Cross-Sectional Survey.

OBJECTIVE: The objective of this study is to determine the physical evaluations and assessment tools used by a group of Canadian healthcare professionals treating adults with spasticity. METHODS: A cross-sectional web-based 19-question survey was developed to determine the types of physical evaluations, tone-related impairment measurements, and assessment tools used in the management of adults with spasticity. The survey was distributed to healthcare professionals from the Canadian Advances in Neuro-Orthopedics for Spasticity Congress database. RESULTS: Eighty study participants (61 physiatrists and 19 other healthcare professionals) completed the survey and were included. Nearly half (46.3%, 37/80) of the participants reported having an inter- or trans-disciplinary team managing individuals with spasticity. Visual observation of movement, available range of motion determination, tone during velocity-dependent passive range of motion looking for a spastic catch, spasticity, and clonus, and evaluation of gait were the most frequently used physical evaluations. The most frequently used spasticity tools were the Modified Ashworth Scale, goniometer, and Goal Attainment Scale. Results were similar in brain- and spinal cord-predominant etiologies. To evaluate goals, qualitative description was used most (37.5%). CONCLUSION: Our findings provide a better understanding of the spasticity management landscape in Canada with respect to staffing, physical evaluations, and outcome measurements used in clinical practice. For all etiologies of spasticity, visual observation of patient movement, Modified Ashworth Scale, and qualitative goal outcomes descriptions were most commonly used to guide treatment and optimize outcomes. Understanding the current practice of spasticity assessment will help provide guidance for clinical evaluation and management of spasticity.

The effect of rurality and distance from care on health outcomes, environmental barriers, and healthcare utilization patterns in persons with traumatic spinal cord injury.

STUDY DESIGN: Cohort study. OBJECTIVES: To evaluate the association between residential living location and health outcomes, environmental barriers, quality of life, and healthcare utilization patterns after traumatic spinal cord injury (tSCI). SETTING: Community setting, Atlantic Canada. METHODS: An ambispective study of data collected on a subset of individuals enrolled in the Rick Hansen Spinal Cord Injury Registry (RHSCIR) from 2012 to 2018. Outcomes were analyzed using two measures of rurality: postal codes at community follow-up (rural versus urban) and residential travel distance to the nearest RHSCIR facility (>100 km versus ≤100 km). Outcomes studied included the Craig Hospital Inventory of Environmental Factors-Short Form (CHIEF-SF), Short Form-36 Version 2 (SF36v2), Life Satisfaction Questionnaire (LISAT-11), Spinal Cord Independence Measure (SCIM), secondary health complications and healthcare utilization patterns. Outcomes were assessed 9 to 24 months post-discharge from initial hospitalization. RESULTS: 104 participants were studied, 21 rural and 83 urban based on postal codes at community follow-up. 59 participants lived more than 100 km away from the nearest RHSCIR facility, while 45 participants lived within 100 km. Individuals from urban area codes reported a greater magnitude of perceived barriers on the policies and work/school subscales of the CHIEF-SF. No differences in function, quality of life, and healthcare utilization patterns according to the measures of rurality were observed. Individuals living >100 km from the nearest RHSCIR facility reported greater rates of sexual dysfunction. CONCLUSIONS: Despite differences in environmental barriers, individuals from urban and rural locations in Eastern Canada reported similar health outcomes and quality of life after tSCI.

An international survey of the structure and process of care for traumatic spinal cord injury in acute and rehabilitation facilities: lessons learned from a pilot study.

BACKGROUND: To describe the key findings and lessons learned from an international pilot study that surveyed spinal cord injury programs in acute and rehabilitation facilities to understand the status of spinal cord injury care. METHODS: An online survey with two questionnaires, a 74-item for acute care and a 51-item for rehabilitation, was used. A subset of survey items relevant to the themes of specialized care, timeliness, patient-centeredness, and evidence-based care were operationalized as structure or process indicators. Percentages of facilities reporting the structure or process to be present, and percentages of indicators met by each facility were calculated and reported separately for facilities from high-income countries (HIC) and from low and middle-income countries (LMIC) to identify "hard to meet" indicators defined as those met by less than two-thirds of facilities and to describe performance level. RESULTS: A total of 26 acute and 26 rehabilitation facilities from 25 countries participated in the study. The comparison of the facilities based on the country income level revealed three general observations: 1) some indicators were met equally well by both HIC and LMIC, such as 24-hour access to CT scanners in acute care and out-patient services at rehabilitation facilities; 2) some indicators were hard to meet for LMIC but not for HIC, such as having a multidisciplinary team for both acute and rehabilitation settings; and 3) some indicators were hard to meet by both HIC and LMIC, including having peer counselling programs. Variability was also observed for the same indicator between acute and rehabilitation facilities, and a wide range in the total number of indicators met among HIC facilities (acute 59-100%; rehabilitation 36-100%) and among LMIC facilities (acute: 41-82%; rehabilitation: 36-93%) was reported. CONCLUSIONS: Results from this international pilot study found that the participating acute and rehabilitation facilities on average adhered to 74% of the selected indicators, suggesting that the structure and processes to provide ideal traumatic spinal cord injury care were broadly available. Recruiting a representative sample of SCI facilities and incorporating regional attributes in future surveys will be helpful to examine factors affecting adherence to indicators.

Management of sleep-disordered breathing in three spinal cord injury rehabilitation centres around the world: a mixed-methods study.

STUDY DESIGN: Mixed-methods observational study. OBJECTIVE: To describe the sleep-disordered breathing (SDB) management models of three spinal cord injury (SCI) rehabilitation centres that are screening, diagnosing and treating uncomplicated SDB, and to determine their common elements. SETTING: Three specialist SCI rehabilitation centres. METHODS: Data collection at each site included direct observations and interviews with lead clinical staff and an audit of SDB-related clinical practice in 2019. Detailed descriptions of the models of care, including process maps, were developed. A theory-based analysis of the common elements of the three care models was undertaken. RESULTS: At each centre a multidisciplinary team, consisting of medical, allied health and/or nursing staff, provided a comprehensive SDB management service that included screening, diagnosis and treatment. Inpatients with SCI were assessed for SDB with overnight oximetry and/or polygraphy. Further assessment of patient symptoms, respiratory function, and hypercapnia supported the diagnostic process. Treatment with positive airway pressure was initiated on the ward. Having a collaborative, skilled team with strong leadership and adequate resources were the key, common enablers to providing the service. CONCLUSION: It is feasible for multi-disciplinary SCI rehabilitation teams to independently diagnose and treat uncomplicated SDB without referral to specialist sleep services provided they are adequately resourced with equipment and skilled staff. Similar models of care could substantially improve access to SDB treatment for people with SCI. Further research is required to determine the non-inferiority of these alternatives to specialist care.

The CanPain SCI clinical practice guidelines for rehabilitation management of neuropathic pain after spinal cord injury: 2021 update.

STUDY DESIGN: Clinical practice guidelines. OBJECTIVES: The objective was to update the 2016 version of the Canadian clinical practice guidelines for the management of neuropathic pain in people with spinal cord injury (SCI). SETTING: The guidelines are relevant for inpatient, outpatient and community SCI rehabilitation settings in Canada. METHODS: The guidelines were updated in accordance with the Appraisal of Guidelines for Research and Evaluation II tool. A Steering Committee and Working Group reviewed the relevant evidence on neuropathic pain management (encompassing screening and diagnosis, treatment and models of care) after SCI. The quality of evidence was scored using Grading of Recommendations Assessment, Development and Evaluation (GRADE). A consensus process was followed to achieve agreement on recommendations and clinical considerations. RESULTS: The working group identified and reviewed 46 additional relevant articles published since the last version of the guidelines. The panel agreed on 3 new screening and diagnosis recommendations and 8 new treatment recommendations. Two key changes to these treatment recommendations included the introduction of general treatment principles and a new treatment recommendation classification system. No new recommendations to model of care were made. CONCLUSIONS: The CanPainSCI recommendations for the management of neuropathic pain after SCI should be used to inform practice.

Consensus-based recommendations for titrating cannabinoids and tapering opioids for chronic pain control.

AIMS: Opioid misuse and overuse have contributed to a widespread overdose crisis and many patients and physicians are considering medical cannabis to support opioid tapering and chronic pain control. Using a five-step modified Delphi process, we aimed to develop consensus-based recommendations on: 1) when and how to safely initiate and titrate cannabinoids in the presence of opioids, 2) when and how to safely taper opioids in the presence of cannabinoids and 3) how to monitor patients and evaluate outcomes when treating with opioids and cannabinoids. RESULTS: In patients with chronic pain taking opioids not reaching treatment goals, there was consensus that cannabinoids may be considered for patients experiencing or displaying opioid-related complications, despite psychological or physical interventions. There was consensus observed to initiate with a cannabidiol (CBD)-predominant oral extract in the daytime and consider adding tetrahydrocannabinol (THC). When adding THC, start with 0.5-3 mg, and increase by 1-2 mg once or twice weekly up to 30-40 mg/day. Initiate opioid tapering when the patient reports a minor/major improvement in function, seeks less as-needed medication to control pain and/or the cannabis dose has been optimised. The opioid tapering schedule may be 5%-10% of the morphine equivalent dose (MED) every 1 to 4 weeks. Clinical success could be defined by an improvement in function/quality of life, a ≥30% reduction in pain intensity, a ≥25% reduction in opioid dose, a reduction in opioid dose to <90 mg MED and/or reduction in opioid-related adverse events. CONCLUSIONS: This five-stage modified Delphi process led to the development of consensus-based recommendations surrounding the safe introduction and titration of cannabinoids in concert with tapering opioids.

A National Spinal Muscular Atrophy Registry for Real-World Evidence.

BACKGROUND: Spinal muscular atrophy (SMA) is a devastating rare disease that affects individuals regardless of ethnicity, gender, and age. The first-approved disease-modifying therapy for SMA, nusinursen, was approved by Health Canada, as well as by American and European regulatory agencies following positive clinical trial outcomes. The trials were conducted in a narrow pediatric population defined by age, severity, and genotype. Broad approval of therapy necessitates close follow-up of potential rare adverse events and effectiveness in the larger real-world population. METHODS: The Canadian Neuromuscular Disease Registry (CNDR) undertook an iterative multi-stakeholder process to expand the existing SMA dataset to capture items relevant to patient outcomes in a post-marketing environment. The CNDR SMA expanded registry is a longitudinal, prospective, observational study of patients with SMA in Canada designed to evaluate the safety and effectiveness of novel therapies and provide practical information unattainable in trials. RESULTS: The consensus expanded dataset includes items that address therapy effectiveness and safety and is collected in a multicenter, prospective, observational study, including SMA patients regardless of therapeutic status. The expanded dataset is aligned with global datasets to facilitate collaboration. Additionally, consensus dataset development aimed to standardize appropriate outcome measures across the network and broader Canadian community. Prospective outcome studies, data use, and analyses are independent of the funding partner. CONCLUSION: Prospective outcome data collected will provide results on safety and effectiveness in a post-therapy approval era. These data are essential to inform improvements in care and access to therapy for all SMA patients.

Optimal Timing of Prenatal Ultrasound in Predicting Birth Weight in Diabetic Pregnancies.

OBJECTIVE: This study sought to determine the optimal timing of ultrasound in the third trimester to predict birth weight accurately in diabetic women with a singleton pregnancy. METHODS: A retrospective cohort study of all diabetic women with a singleton pregnancy treated in Halifax, Nova Scotia, was performed. Estimated fetal weight was derived from ultrasound measures using the Hadlock2 equation. The Mongelli equation was used to predict birth weight. The association between gestational age at ultrasound and accuracy of predicted birth weight was assessed, with accuracy as a continuous variable representing the difference between predicted and actual birth weight and as a categorical variable (with four gestational age categories) representing whether predicted birth weight was within, over, or under 250 g of actual birth weight RESULTS: The cohort of 943 women comprised 121 (12.8%) with type 1 diabetes, 111 (11.7%) with type 2 diabetes, and 711 (75.4%) with gestational diabetes. Ultrasound scans performed at term were the most accurate in predicting birth weight. At this gestational age, the mean difference between predicted and actual birth weight was -30 g (95% confidence interval -109 to -48). After adjusting for maternal body mass index, age, smoking, type of diabetes, and interval between ultrasound examination and delivery, accuracy improved as gestational age at ultrasound increased (P = 0.005). The odds of underpredicting or overpredicting birth weight were not significantly affected by the timing of the ultrasound examination. CONCLUSION: Because the predictive accuracy of ultrasound prediction of birth weight improves with gestational age, fetal growth assessment at term is recommended to aid with delivery planning in women with diabetes.

Accepting new patients who require opioids into family practice: results from the MAAP-NS census survey study.

BACKGROUND: Acceptance to a family practice is key to access and continuity of care. While Canadian patients increasingly report not being able to acquire acceptance to a family practice, little is known about the association between requiring opioids and acceptance. We aim to determine the proportion of family physicians who would accept new patients who require opioids and describe physician and practice characteristics associated with willingness to accept these patients. METHODS: Census telephone survey of family physicians' practices in Nova Scotia, Canada. MEASURES: physician (i.e., age, sex, years in practice) and practice (i.e., number/type of provider in the practice, care hours/week) characteristics and practice-reported willingness to accept new patients who require opioids. RESULTS: The survey was completed for 587 family physicians (83.7% response rate). 354 (60.3%) were taking new patients unconditionally or with conditions; 326 provided a response to whether they would accept new patients who require opioids; 91 (27.9%) reported they would not accept a new patient who requires opioids. Compared to family physicians who would not accept patients who require opioids, in bivariate analysis, those who would, tended to work in larger practices; had fewer years in practice; are female; and provided more patient care. The relationship to number of providers in the practice, having a nurse, and experience persisted in multivariate analysis. CONCLUSIONS: The strongest predictors of willingness to accept patients who require opioids are fewer years in practice (OR = 0.96 [95% CI 0.93, 0.99]) and variables indicating a family physician has support of a larger (OR = 1.19 [95% CI 1.00, 1.42]), interdisciplinary team (e.g., nurses, mental health professionals) (OR = 1.15 [95% CI 1.11, 5.05]). Almost three-quarters (72.1%) of surveyed family physicians would accept patients requiring opioids.

Facilitators and Barriers for Implementing an Internet Clinic for the Treatment of Pressure Injuries.

Background: Pressure injuries (PIs) represent a frequent, often preventable, secondary complication of spinal cord injury (SCI) with serious consequences to health, societal participation, and quality of life. Specialized knowledge and service delivery related to treatment and prevention are typically located within major health centers.Introduction: For persons with SCI living at home, it can be challenging to access specialized PI care. A telehealth approach could help mitigate this challenge. This multisite pilot investigation assessed the feasibility of integrating information technologies within the management of PIs.Materials and Methods: Each study site formed a specialized interdisciplinary care team that identified components of their standard clinical care pathway and examined how they could be integrated with study technologies. A monitoring system was utilized to enable patients and caregivers to exchange clinical information with the care team.Results: Clinician and patient focus groups were completed to identify facilitators and barriers for long-term implementation. Findings demonstrate that this method of service delivery is feasible but requires further development.Discussion: This model of care requires refinement to address technological, regulatory, and clinician acceptance barriers; however, increased access to these services has the potential for improving PI healing or prevention rates in comparison with those not able to access specialized services.Conclusions: This project demonstrates that PI treatment services can be delivered effectively through the internet. Future trials can investigate efficacy and cost-effectiveness of this model of care to inform sustained implementation.

Provincial Differences in the Diagnosis and Care of Amyotrophic Lateral Sclerosis.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease resulting in muscle weakness, dysarthria and dysphagia, and ultimately respiratory failure leading to death. Half of the ALS patients survive less than 3 years, and 80% of the patients survive less than 5 years. Riluzole is the only approved medication in Canada with randomized controlled clinical trial evidence to slow the progression of ALS, albeit only to a modest degree. The Canadian Neuromuscular Disease Registry (CNDR) collects data on over 140 different neuromuscular diseases including ALS across ten academic institutions and 28 clinics including ten multidisciplinary ALS clinics. METHODS: In this study, CNDR registry data were analyzed to examine potential differences in ALS care among provinces in time to diagnosis, riluzole and feeding tube use. RESULTS: Significant differences were found among provinces, in time to diagnosis from symptom onset, in the use of riluzole and in feeding tube use. CONCLUSIONS: Future investigations should be undertaken to identify factors contributing to such differences, and to propose potential interventions to address the provincial differences reported.

Health Conditions: Effect on Function, Health-Related Quality of Life, and Life Satisfaction After Traumatic Spinal Cord Injury. A Prospective Observational Registry Cohort Study.

OBJECTIVE: To analyze relations among injury, demographic, and environmental factors on function, health-related quality of life (HRQoL), and life satisfaction in individuals with traumatic spinal cord injury (SCI). DESIGN: Prospective observational registry cohort study. SETTING: Specialized acute and rehabilitation SCI centers. PARTICIPANTS: Participants (N=340) from the Rick Hansen Spinal Cord Injury Registry (RHSCIR) who were prospectively recruited from 2004 to 2014 were included. The model cohort participants were 79.1% men, with a mean age of 41.6±17.3 years. Of the participants, 34.7% were motor/sensory complete (ASIA Impairment Scale [AIS] grade A). INTERVENTIONS: None. MAIN OUTCOME MEASURES: Path analysis was used to determine relations among SCI severity (AIS grade and anatomic level [cervical/thoracolumbar]), age at injury, education, number of health conditions, functional independence (FIM motor score), HRQoL (Medical Outcomes Study 36-Item Short-Form Health Survey [Version 2] Physical Component Score [PCS] and Mental Component Score [MCS]), and life satisfaction (Life Satisfaction-11 [LiSat-11]). Model fit was assessed using recommended published indices. RESULTS: Goodness of fit of the model was supported by all indices, indicating the model results closely matched the RHSCIR data. Higher age, higher severity injuries, cervical injuries, and more health conditions negatively affected FIM motor score, whereas employment had a positive effect. Higher age, less education, more severe injuries (AIS grades A-C), and more health conditions negatively correlated with PCS (worse physical health). More health conditions were negatively correlated with a lower MCS (worse mental health), however were positively associated with reduced function. Being married and having higher function positively affected Lisat-11, but more health conditions had a negative effect. CONCLUSIONS: Complex interactions and enduring effects of health conditions after SCI have a negative effect on function, HRQoL, and life satisfaction. Modeling relations among these types of concepts will inform clinicians how to positively effect outcomes after SCI (eg, development of screening tools and protocols for managing individuals with traumatic SCI who have multiple health conditions).

A Delphi-Based Consensus Statement on the Management of Anticoagulated Patients With Botulinum Toxin for Limb Spasticity.

OBJECTIVE: To create a consensus statement on the considerations for treatment of anticoagulated patients with botulinum toxin A (BoNTA) intramuscular injections for limb spasticity. DESIGN: We used the Delphi method. SETTING: A multiquestion electronic survey. PARTICIPANTS: Canadian physicians (N=39) who use BoNTA injections for spasticity management in their practice. INTERVENTIONS: After the survey was sent, there were e-mail discussions to facilitate an understanding of the issues underlying the responses. Consensus for each question was reached when agreement level was ≥75%. MAIN OUTCOME MEASURES: Not applicable. RESULTS: When injecting BoNTA in anticoagulated patients: (1) BoNTA injections should not be withheld regardless of muscles injected; (2) a 25G or smaller size needle should be used when injecting into the deep leg compartment muscles; (3) international normalized ratio (INR) level should be ≤3.5 when injecting the deep leg compartment muscles; (4) if there are clinical concerns such as history of a fluctuating INR, recent bleeding, excessive or new bruising, then an INR value on the day of injection with point-of-care testing or within the preceding 2-3 days should be taken into consideration when injecting deep compartment muscles; (5) the concern regarding bleeding when using direct oral anticoagulants (DOACs) should be the same as with warfarin (when INR is in the therapeutic range); (6) the dose and scheduling of DOACs should not be altered for the purpose of minimizing the risk of bleeding prior to BoNTA injections. CONCLUSIONS: These consensus statements provide a framework for physicians to consider when injecting BoNTA for spasticity in anticoagulated patients. These consensus statements are not strict guidelines or decision-making steps, but rather an effort to generate common understanding in the absence of evidence in the literature.

Predictive value of the pendulum test for assessing knee extensor spasticity.

BACKGROUND: The pendulum test is commonly used to quantify knee extensor spasticity, but it is currently unknown to what extent common pendulum test metrics can detect spasticity in patients with neurological injury or disease, and if the presence of flexor spasticity influences the test outcomes. METHODS: A retrospective analysis was conducted on 131 knees, from 93 patients, across four different patient cohorts. Clinical data included Modified Ashworth Scale (MAS) scores for knee extensors and flexors, and years since diagnosis. BioTone™ measures included extensor strength, passive and active range of motion, and pendulum tests of most affected or both knees. Pendulum test metrics included the relaxation index (RI), 1st flexion amplitude (F1amp) and plateau angle (Plat), where RI=F1amp/Plat. Two-way ANOVA tests were used to determine if pendulum test metrics were influenced by the degree of knee flexor spasticity graded by the MAS, and ANCOVA was used to test for confounding effects of age, years since injury, strength and range of motion (ROM). In order to identify the best pendulum test metrics, Receiver Operator Characteristic analysis and logistic regression (LR) analysis were used to classify knees by spasticity status (none or any) and severity (low/moderate or high/severe). RESULTS: Pendulum test metrics for knee extensors were not influenced by degree of flexor spasticity, age, years since injury, strength or ROM of the limb. RI, F1amp and Plat were > 70% accurate in classifying knees by presence of clinical spasticity (from the MAS), but were less accurate (< 70%) for grading spasticity level. The best classification accuracy was obtained using F1amp and Plat independently in the model rather than using RI alone. CONCLUSIONS: We conclude that the pendulum test has good predictive value for detecting the presence of extensor spasticity, independent of the existence of flexor spasticity. However, the ability to grade spasticity level as measured by MAS using the RI and/or F1amp may be limited. Further study is warranted to explore if the pendulum test is suitable for quantifying more severe spasticity.

Convergent Validity of a Wearable Sensor System for Measuring Sub-Task Performance during the Timed Up-and-Go Test.

BACKGROUND: The timed-up-and-go test (TUG) is one of the most commonly used tests of physical function in clinical practice and for research outcomes. Inertial sensors have been used to parse the TUG test into its composite phases (rising, walking, turning, etc.), but have not validated this approach against an optoelectronic gold-standard, and to our knowledge no studies have published the minimal detectable change of these measurements. METHODS: Eleven adults performed the TUG three times each under normal and slow walking conditions, and 3 m and 5 m walking distances, in a 12-camera motion analysis laboratory. An inertial measurement unit (IMU) with tri-axial accelerometers and gyroscopes was worn on the upper-torso. Motion analysis marker data and IMU signals were analyzed separately to identify the six main TUG phases: sit-to-stand, 1st walk, 1st turn, 2nd walk, 2nd turn, and stand-to-sit, and the absolute agreement between two systems analyzed using intra-class correlation (ICC, model 2) analysis. The minimal detectable change (MDC) within subjects was also calculated for each TUG phase. RESULTS: The overall difference between TUG sub-tasks determined using 3D motion capture data and the IMU sensor data was <0.5 s. For all TUG distances and speeds, the absolute agreement was high for total TUG time and walk times (ICC > 0.90), but less for chair activity (ICC range 0.5-0.9) and typically poor for the turn time (ICC < 0.4). MDC values for total TUG time ranged between 2-4 s or 12-22% of the TUG time measurement. MDC of the sub-task times were higher proportionally, being 20-60% of the sub-task duration. CONCLUSIONS: We conclude that a commercial IMU can be used for quantifying the TUG phases with accuracy sufficient for clinical applications; however, the MDC when using inertial sensors is not necessarily improved over less sophisticated measurement tools.

Do We Need an Intubation-Skilled Person at All High-Risk Deliveries?

OBJECTIVE: To evaluate the significance and predictive value of each of the Neonatal Resuscitation Program (NRP)-listed ante- and intrapartum risk factors for the need of neonatal intubation at birth. STUDY DESIGN: In this population-based study, perinatal data of all infants born at ≥ 35 weeks gestation in the province of Nova Scotia between 1994 and 2014, were identified and reviewed from the Nova Scotia Atlee Database. The frequency of occurrence of risk factors, incidence of neonatal intubation at birth, and its relationship with the different NRP-listed risk factors, were examined. Variables that were significant (P < .05) in univariate analyses were entered into the regression model. RESULTS: During the 20-year study period, 176,365 infants ≥ 35 weeks gestation were born. In presence of any of the listed risk factors, 0.3% of infants received intubation at birth compared with 0.08% in absence of any risk factor (P < .001). On logistic regression analysis, only 16 of the NRP-listed risk factors had a significant relationship with intubation at birth (P < .001). Delivery in a tertiary care center did not have an impact. CONCLUSIONS: The presence of an intubation-skilled person at birth may not be indicated in all the NRP-listed ante- and intrapartum risk factors. Stratification of the relative significance of different risk factors may be of importance for the less-resourced health care units providing maternal and newborn care.

Combined Autoimmune Cytopenias Presenting in Childhood.

BACKGROUND: Pediatric patients with chronic and/or refractory autoimmune multi-lineage cytopenias present challenges in both diagnosis and management. Increasing availability of diagnostic testing has revealed an underlying immune dysfunction in patients previously diagnosed with Evans Syndrome. However, the data are sparse and the majority of patients are adults. PROCEDURE: We performed a retrospective chart review to document the natural history of 23 pediatric patients with autoimmune multi-lineage cytopenias followed at three tertiary care pediatric hematology clinics. RESULTS: Investigations revealed seven patients (30.4%) with an autoimmune lymphoproliferative-like syndrome and six patients (26.1%) with other primary immunodeficiencies. Only one (4.3%) patient was suspected to have systemic lupus erythematosus and six patients (26.1%) had other types of autoimmunity. Treatment consisted of immunosuppressive therapy, intravenous gammaglobulin, and splenectomy. Supportive care included granulocyte-colony stimulating factor, and blood product transfusions. Two patients (8.7%) died. Complete remission was achieved in 3 patients (13.0%); of the remaining, 14 patients (60.9%) had chronic immune thrombocytopenic purpura, 10 patients (43.5%) chronic autoimmune neutropenia, and 4 patients (17.4%) chronic autoimmune hemolytic anemia with a median follow up of 5 years (2 months-12 years). CONCLUSIONS: These data suggest that pediatric patients presenting with autoimmune multi-lineage cytopenias should undergo investigation for underlying immune dysregulation, including autoimmune lymphoproliferative syndrome, other primary immunodeficiencies and autoimmune disorders. The development of an international registry for such patients is imperative to improve the understanding of their complex natural history.

Type of Labour in the First Pregnancy and Cumulative Perinatal Morbidity.

OBJECTIVE: To estimate cumulative perinatal morbidity among infants delivered at term, according to the type of labour in the first pregnancy, when the first pregnancy was low risk. METHODS: In a 26-year population-based cohort study (1988-2013) using the Nova Scotia Atlee Perinatal Database, we identified the type of labour in successive pregnancies in low-risk, nulliparous women at term in their first pregnancy (who had at least one subsequent pregnancy), and also identified perinatal outcomes in subsequent deliveries according to the type of labour in the first pregnancy. RESULTS: A total of 37 756 pregnancies satisfied inclusion and exclusion criteria; of these, 1382 (3.7%) had a Caesarean section without labour in the first pregnancy. Rates of most adverse perinatal outcomes were low (≤ 1%). The risks for stillbirth were low in subsequent deliveries, including those that followed CS without labour in the first pregnancy, and the risks for the overall severe perinatal morbidity outcome were less than 10% for all subsequent deliveries. CONCLUSION: The absolute risks for severe perinatal morbidity outcomes in a population of low-risk women (with up to four additional pregnancies) were small, regardless of type of labour in the first pregnancy. This finding provides important information on perinatal outcomes in subsequent pregnancies when considering type of labour in the first pregnancy.