Longitudinal dysphagia assessment in adult patients with nephropathic cystinosis using the Modified Barium Swallow Impairment Profile.

INTRODUCTION/AIMS: Nephropathic cystinosis is a lysosomal storage disorder with known myopathic features, including dysphagia. Evaluation of oropharyngeal swallowing physiology can be standardized using the Modified Barium Swallow Impairment Profile (MBSImP), a validated assessment tool used to analyze and rate swallowing across 17 distinct physiologic domains. Our objective was to better characterize swallowing impairments in nephropathic cystinosis using MBSImP analysis. METHODS: We retrospectively evaluated 40 video fluoroscopic swallowing studies performed at two time points over 1 y in patients with nephropathic cystinosis with various levels of oral and pharyngeal stage dysphagia. Patients completed two self-administered dysphagia outcome measures (the M. D. Anderson Dysphagia Inventory [MDADI] and the 10-item Eating Assessment Tool [EAT-10]). RESULTS: We demonstrated oral stage and pharyngeal stage dysphagia across domains that impacted bolus control, transit, and clearance through both the oral cavity and pharyngeal lumen. Also captured were deficits related to onset and completeness of laryngeal closure that impact airway protection during swallow. There were significant correlations between pharyngeal total score and EAT-10 (r = 0.5, p < 0.001) and between oral total score and EAT-10 (r = 0.7, p < 0.001), MDADI-e (r = -0.6, p < 0.001), MDADI-p (r = -0.5, p < 0.001) and MDADI-c (r = -0.6, p < 0.001). There were no differences in oral or pharyngeal total scores across the 1-y time span. DISCUSSION: This study identifies oral and pharyngeal stage dysphagia as crucial to patients with nephropathic cystinosis and paves the path for future studies of treatment targets.

Clinical myopathy in patients with nephropathic cystinosis.

BACKGROUND: Nephropathic cystinosis is a lysosomal storage disorder. Patient survival years after renal transplantation has revealed systemic complications including distal myopathy and dysphagia. METHODS: We evaluated 20 adult patients with nephropathic cystinosis using patient-reported and clinical outcome measures. Standard motor measures, video fluoroscopy swallow studies, and tests of respiratory function were performed. We also used Rasch analysis of an initial survey to design a 16-item survey focused on upper and lower extremity function, which was completed by 31 additional patients. RESULTS: Distal myopathy and dysphagia were common in patients with nephropathic cystinosis. Muscle weakness ranges from mild involvement of intrinsic hand muscles to prominent distal greater than proximal weakness and contractures. CONCLUSIONS: In addition to further characterization of underlying dysphagia and muscle weakness, we propose a new psychometrically devised, disease specific, functional outcome measures for distal myopathy in patients with nephropathic cystinosis.

Clinical trial readiness study of distal myopathy and dysphagia in nephropathic cystinosis.

BACKGROUND: Nephropathic cystinosis is a lysosomal storage disorder with late-onset systemic complications, such as myopathy and dysphagia. Currently employed outcome measures lack sensitivity and responsiveness for dysphagia and myopathy, a limitation to clinical trial readiness. METHODS: We evaluated 20 patients with nephropathic cystinosis in two visits over the course of a year to identify outcomes sensitive to detect changes over time. Patients also underwent an expiratory muscle strength training program to assess any effects on aspiration and dysphagia. RESULTS: There were significant differences in the Timed Up and Go Test (TUG) and Timed 25-Foot Walk (25-FW) between baseline and 1-y follow-up (P < .05). Maximum expiratory pressure (MEP) and peak cough flow (PCF) significantly improved following respiratory training (P < .05). CONCLUSIONS: Improved respiratory outcomes may enhance patients ability to expel aspirated material from the airway, stave off pulmonary sequelae associated with chronic aspiration, and yield an overall improvement in physical health and well-being.

Loss of appetite in amyotrophic lateral sclerosis is associated with weight loss and decreased calorie consumption independent of dysphagia.

BACKGROUND: Loss of appetite has been reported to affect up to half of people with amyotrophic lateral sclerosis (ALS) and to be associated with weight loss. We wished to test whether loss of appetite correlates with reduced dietary intake independent of dysphagia. METHODS: Appetite was measured repeatedly using the Council on Nutrition Appetite Questionnaire (CNAQ) in participants in the Electronic health Application To Measure Outcomes REmotely study. Dietary intake and weight were compared to appetite, ALS Functional Rating Scale-Revised total and bulbar scores (dysphagia). RESULTS: The average baseline CNAQ score was 30.4 (n = 61; SD = 3.9) with 18.0% scoring <28 points (severe loss of appetite). Lower CNAQ scores correlated with greater weight loss since diagnosis (Pearson correlation coefficient, r = -0.34; P = 0.009) and lower baseline energy intake (P = 0.007), independent of dysphagia. CONCLUSIONS: Our results support an association between loss of appetite and decreased calorie intake and weight in ALS which is independent of dysphagia.

Provisional best practices guidelines for the evaluation of bulbar dysfunction in amyotrophic lateral sclerosis.

INTRODUCTION: Universally established comprehensive clinical bulbar scales objectively assessing disease progression in amyotrophic lateral sclerosis (ALS) are currently lacking. The goal of this working group project is to design a best practice set of provisional bulbar ALS guidelines, available for immediate implementation within all ALS clinics. METHODS: ALS specialists across multiple related disciplines participated in a series of clinical bulbar symposia, intending to identify and summarize the currently accepted best practices for the assessment and management of bulbar dysfunction in ALS Results: Summary group recommendations for individual speech, Augmentative and Alternative Communication (AAC), and swallowing sections were achieved, focusing on the optimal proposed level of care within each domain. DISCUSSION: We have identified specific clinical recommendations for each of the 3 domains of bulbar functioning, available for incorporation within all ALS clinics. Future directions will be to establish a formal set of bulbar guidelines through a methodological and evidence-based approach. Muscle Nerve 59:531-531, 2019.

Nutritional counseling with or without mobile health technology: a randomized open-label standard-of-care-controlled trial in ALS.

BACKGROUND: Nutritional status is an important prognostic factor in Amyotrophic Lateral Sclerosis (ALS). We wished to study the safety, tolerability and efficacy of nutritional counseling with or without an mHealth application to maintain or increase body weight in ALS, compared to standard care. METHODS: In this randomized open-label, standard-of-care-controlled, single-center clinical trial, we randomly assigned adults with ALS to one of three nutritional interventions: counseling by their physician or nurse ("standard care"), counseling by a registered dietitian (RD) ("in-person"), or counseling supported by a mHealth app ("mHealth"). Both intervention arms received tailored nutrition recommendations and recorded dietary intake and weight biweekly (mHealth) or monthly (in-person). The primary outcome of weight and secondary and tertiary outcomes of calorie intake, ALSFRS-R, and quality of life (QOL) were recorded at each clinic visit and analyzed in an ITT mixed model analysis. RESULTS: A total of 88 participants were enrolled of whom 78 were included in this analysis. The three arms were well-balanced except for excess males in the mHealth arm and greater weight lost at baseline in the in-person arm. Participants in the mHealth arm increased their calorie intake at month 3 to an average of 94% (95% CI: 85, 103) of recommended calories, compared to 81% (95% CI: 72, 91, p = 0.06 vs. mHealth) in the standard care arm. After 6 months, calorie intake was not different among the three arms. Overall weight was stable across all three groups. QOL scores in the mHealth arm were stable over 3 months (0.3 points, 95% CI: - 1.7, 2.2) compared to worsening in standard care (- 2.1 points, 95% CI: - 4.0, - 0.2, p = 0.09 vs. mHealth), but all scores declined by 6 months. ALSFRS-R total scores declined by an average of - 2.6 points (95% CI: - 5.1, - 0.1) over 6 months in the mHealth arm (p = 0.13 vs. standard care) compared to - 5.8 points (95% CI: - 8.2, - 3.4, p = 0.74 vs. standard care) in the in-person and - 5.2 points (95% CI: - 7.6, - 2.9) in the standard care arm. CONCLUSIONS: Nutritional counseling by a registered dietitian (with or without support by an mHealth app) is safe but did not maintain weight significantly better than standard care in ALS patients. TRIAL REGISTRATION: https://clinicaltrials.gov/ identifier NCT02418546. Registered April 16, 2015.

Update on exercise-induced pulmonary hemorrhage.

Exercise-induced pulmonary hemorrhage (EIPH) is an important disease of horses that perform high-intensity athletic activity. EIPH is an ongoing concern for the racing industry because of its high prevalence; potential impact on performance; welfare concerns; and use of prophylactic medications, such as furosemide, on race day. During the last 10 years, significant progress has been made in understanding the pathogenesis and risk factors for EIPH and the impact of the disease on performance and career. This article summarizes the most recent advances in EIPH.

Trimethoprim-sulfonamide: a valid antimicrobial treatment in foals?

Trimethoprim-sulfonamide (TMPS) combinations are widely used to treat a range of infectious diseases in horses, but some equine practitioners are reluctant to use them for treatment of both neonatal and older foals. Considering the emergence of increased antimicrobial resistance, the use of protected antimicrobials commonly prescribed to foals should be avoided and alternative first-line therapy considered, where appropriate. This review examines the characteristics and pharmacokinetics of TMPS and its suitability for treatment of foals. Data regarding dosage and route of administration are reported on the basis of recent publications in foals. The review intends to share significant information about the common infections that are most likely responsive to TMPS treatment in foals and, as such, where TMPS might be considered a suitable first-line therapeutic option.

Listener effort quantifies clinically meaningful progression of dysarthria in people living with amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative motor neuron disease that causes progressive muscle weakness. Progressive bulbar dysfunction causes dysarthria and thus social isolation, reducing quality of life. The Everything ALS Speech Study obtained longitudinal clinical information and speech recordings from 292 participants. In a subset of 120 participants, we measured speaking rate (SR) and listener effort (LE), a measure of dysarthria severity rated by speech pathologists from recordings. LE intra- and inter-rater reliability was very high (ICC 0.88 to 0.92). LE correlated with other measures of dysarthria at baseline. LE changed over time in participants with ALS (slope 0.77 pts/month; p<0.001) but not controls (slope 0.005 pts/month; p=0.807). The slope of LE progression was similar in all participants with ALS who had bulbar dysfunction at baseline, regardless of ALS site of onset. LE could be a remotely collected clinically meaningful clinical outcome assessment for ALS clinical trials.

A Validated Open-Source Shoulder Finite Element Model and Investigation of the Effect of Analysis Precision.

Understanding the loads and stresses on different tissues within the shoulder complex is crucial for preventing joint injury and developing shoulder implants. Finite element (FE) models of the shoulder joint can be helpful in describing these forces and the biomechanics of the joint. Currently, there are no validated FE models of the intact shoulder available in the public domain. This study aimed to develop and validate a shoulder FE model, then make the model available to the orthopaedic research community. Publicly available medical images of the Visible Human Project male subject's right shoulder were used to generate the model geometry. Material properties from the literature were applied to the different tissues. The model simulated abduction in the scapular plane. Simulated glenohumeral (GH) contact force was compared to in vivo data from the literature, then further compared to other in vitro experimental studies. Output variable results were within one standard deviation of the mean in vivo experimental values of the GH contact force in 0°, 10°, 20°, 30°, and 45° of abduction. Furthermore, a comparison among different analysis precision in the Abaqus/Explicit platform was made. The complete shoulder model is available for download at github.com/OSEL-DAM/ShoulderFiniteElementModel.

Immediate postoperative non-invasive positive pressure ventilation following midface microvascular free flap reconstruction.

BACKGROUND: There is a rare need for postoperative non-invasive positive pressure ventilation (NIPPV) following microvascular reconstruction of the head and neck. In midface reconstruction, the free flap vascular pedicle is especially vulnerable to the compressive forces of positive pressure delivery. CASE: A 60 year old female with Amyotrophic Lateral Sclerosis (ALS) presented with squamous cell carcinoma of the anterior maxilla, for which she underwent infrastructure maxillectomy and fibula free flap reconstruction. To avoid tracheotomy, the patient was extubated postoperatively and transitioned to NIPPV immediately utilizing a full-face positive pressure mask with a soft and flexible sealing layer. The patient was successfully transitioned to NIPPV immediately after extubation. The free flap exhibited no signs of vascular compromise postoperatively, and healed very well. CONCLUSION: Postoperative non-invasive positive pressure ventilation can be successfully applied following complex microvascular midface reconstruction to avoid tracheotomy in select patients without vascular compromise of the free flap.

Clinical evaluation of non-contact infrared thermometers.

Non-contact infrared thermometers (NCITs) are being widely used during the COVID-19 pandemic as a temperature-measurement tool for screening and isolating patients in healthcare settings, travelers at ports of entry, and the general public. To understand the accuracy of NCITs, a clinical study was conducted with 1113 adult subjects using six different commercially available NCIT models. A total of 60 NCITs were tested with 10 units for each model. The NCIT-measured temperature was compared with the oral temperature obtained using a reference oral thermometer. The mean difference between the reference thermometer and NCIT measurement (clinical bias) was different for each NCIT model. The clinical bias ranged from just under - 0.9 °C (under-reporting) to just over 0.2 °C (over-reporting). The individual differences ranged from - 3 to + 2 °C in extreme cases, with the majority of the differences between - 2 and + 1 °C. Depending upon the NCIT model, 48% to 88% of the individual temperature measurements were outside the labeled accuracy stated by the manufacturers. The sensitivity of the NCIT models for detecting subject's temperature above 38 °C ranged from 0 to 0.69. Overall, our results indicate that some NCIT devices may not be consistently accurate enough to determine if subject's temperature exceeds a specific threshold of 38 °C. Model-to-model variability and individual model accuracy in the displayed temperature were found to be outside of acceptable limits. Accuracy and credibility of the NCITs should be thoroughly evaluated before using them as an effective screening tool.

Performance characterization of non-contact infrared thermometers (NCITs) for forehead temperature measurement.

The ability to assess the performance of a non-contact infrared thermometer (NCIT) may be limited due to the algorithms necessary to predict a reference site temperature (e.g., oral) from a measurement of the forehead skin temperature. The algorithm not only adjusts for the difference between the reference site temperature and forehead temperature, but may also account for hardware corrections, bias adjustments and emissivity settings. These algorithms are proprietary to the manufacturer and can be unique for each device. ASTM E1965-98 (2016) is a standard test method for the evaluation of NCITs. It includes forehead thermometers; however, the algorithm must be known or an unadjusted calibration mode must be accessible. This study evaluates 6 NCIT models (10 units of each) against the ASTM standard error criterion using a blackbody source. Units were tested within the manufacturer's operating and temperature measurement range specification. A method to evaluate measurement outliers and characterize each model's performance when the adjustment algorithm is unknown is proposed. Using this method, 5 of the 6 models had a predicted error > 0.3°C.

Perception of and risk factors for type 2 diabetes among students attending an upstate New York college: a pilot study.

BACKGROUND: Detecting early type 2 diabetes (T2D) risk factors may reduce or prevent the development of the disease. We conducted a pilot study to generate preliminary data on the perception of T2D and further determined the prevalence of T2D risk factors among college students at an upstate New York campus. METHODS: Metabolic profiles were available for 44 college students for cross-sectional analysis. The American Diabetes Association screening guidelines were used to determine risk factors, and perceived susceptibility, perceived seriousness, and self-efficacy were determined with the Health Belief Model's constructs. Sociodemographic and anthropometric data, nutrition knowledge, and metabolic profiles were obtained. RESULTS: The most common T2D risk factors were lack of physical activity (61.4%), decreased high-density lipoprotein cholesterol (HDL-c, 56.8%), high fasting blood glucose (FBG, 45.5%), family history of T2D (43.2%), increased body mass index (BMI, 36.4%), and high blood pressure (15.9%). A high proportion (70%) of participants with detected impaired FBG perceived they were at low risk of developing T2D. Participants with a family history of T2D (mean rank = 24.2) perceived the seriousness of T2D at a similar level as those without family history (mean rank = 21.2), with no significant difference (U = 205, P = 0.430). Nearly 30% of students did not feel confident they could prevent the development of T2D. Pearson's correlations revealed direct relationships between perceived risk of T2D and BMI (r = 0.49, P = 0.001), fat mass percent (r = 0.51, P < 0.001), and waist circumference (r = 0.42, P = 0.005), and an inverse relationship was found with HDL-c (r = - 0.41, P = 0.005). The association of perceived risk of T2D with a family history of T2D revealed a trend toward significance (Chi-squared = 5.746, P = 0.057), and the association of perceived risk of T2D with physical activity was not significant (Chi-squared = 1.520, P = 0.468). The nutrition knowledge score was 74.32 ± 15.97 (recommended is > 75). However, knowledge scores regarding recommended intake of fruits, vegetables, high sodium foods, and whole grains to prevent T2D were only 36.36%, 34.09%, 47.73%, and 63.6%, respectively. CONCLUSIONS: The discordance between college students' perceived risk and prevalence of T2D risk factors warrants strategies to address misperceptions of T2D risk and improve lifestyle behaviors among this study sample.

Magnetic Resonance Imaging Technologist Breast Subspecialty Program; a Quality Improvement Project Gone Right.

We describe a Lean based Quality Improvement Project (QIP) to improve the defect rate of breast magnetic resonance imaging (MRI) studies by developing a MRI Technologist Breast Sub-specialization Program. Key stakeholders (physician and technologist) drove the QIP. Both the overall defect rate and the callback rate (severe defects requiring patients return for repeat imaging) were measured over a 2-month period as 17% and 6%, respectively. Lean visualization tools of Pareto Chart & Fishbone Diagram identified lack of multiple trends, but discerned that most defect causes were within the responsibility of the technologists. Lean Value Stream Map identified technologists' useless work (muda), which was subsequently eliminated. Radiologists collectively defined what made a quality study in a Quality Checklist. Key stakeholders limited the number of technologists based on the study volume (50 studies/technologist/2 years) and reviewed 5 studies recently performed by each technologist. If all 5 studies were defect free per the Quality Checklist, then the technologist was certified to perform breast MRI's by himself/herself. Otherwise, the technologist was on probation. Key stakeholders selected SuperTechs with advanced skill and interest from the certified pool to cover all shifts. Technologists on probation had to complete 5 additional studies defect-free under the supervision of a SuperTech to achieve certification. In addition, SuperTechs were available to backup certified technologists, as needed. Software was implemented at the PACS workstation to flag defective and callback studies. 6 months after the initiation of the QIP, the defect rate decreased from 17% to 2% (p>0.02), and the callback rate decreased from 6% to 0, thus confirming this MRI Breast Program was a QIP gone right.

The Comprehensive Management of Cerebellar Ataxia in Adults.

PURPOSE OF REVIEW: In this review, we present the multidisciplinary approach to the management of the many neurological, medical, social, and emotional issues facing patients with cerebellar ataxia. RECENT FINDINGS: Our holistic approach to treatment, developed over the past 25 years in the Massachusetts General Hospital Ataxia Unit, is centered on the compassionate care of the patient and their family, empowering them through engagement, and including the families as partners in the healing process. We present the management of ataxia in adults, beginning with establishing an accurate diagnosis, followed by treatment of the multiple symptoms seen in cerebellar disorders, with a view to maximizing quality of life and effectively living with the consequences of ataxia. We discuss the importance of a multidisciplinary approach to the management of ataxia, including medical and non-medical management and the evidence base that supports these interventions. We address the pharmacological treatment of ataxia, tremor, and other associated movement disorders; ophthalmological symptoms; bowel, bladder, and sexual symptoms; orthostatic hypotension; psychiatric and cognitive symptoms; neuromodulation, including deep brain stimulation; rehabilitation including physical therapy, occupational therapy and speech and language pathology and, as necessary, involving urology, psychiatry, and pain medicine. We discuss the role of palliative care in late-stage disease. The management of adults with ataxia is complex and a team-based approach is essential.

Relationship of serum Vitamin D concentrations with Adipokines and Cardiometabolic risk among non-Hispanic black type 2 diabetic and non-diabetic subjects: a cross-sectional study.

BACKGROUND: To report the association of serum 25-hydroxyvitamin D [25(OH)D] and its association with adipokines and cardiometabolic risk factors in Haitian Americans (HA) and African Americans (AA) by ethnicity and type 2 diabetes (T2D) status. METHODS: A cross-sectional study in 197 HA (92 with T2D and 102 without T2D) and 200 AA (97 with T2D and 103 without T2D) recruited in South Florida. Serum 25(OH)D concentrations and adipokines were analyzed by ELISA and cardiometabolic risk factors were indexed by obesity, glycemic control, insulin sensitivity, lipid profile, and blood pressure. RESULTS: Controlling for age, BMI, energy intake, smoking status and HOMA2-IR in multivariate linear regression analyses, serum 25(OH)D concentrations were significantly associated with WC (R2 = 0.760, B = - 0.092, P = 0.027), HbA1C (R2 = 0.142, B = - 0.012, P = 0.010), and TG (R2 = 0.159, B = - 1.192, P = 0.003) in only HA without T2D. While serum 25(OH)D concentrations were significantly associated with TC (R2 = 0.168, B = - 0.329, P = 0.040), log leptin (R2 = 0.544, B = - 0.007, P = 0.021), and adiponectin (R2 = 0.144, B = 0.111, P = 0.033), but slightly associated with LDL-c (R2 = 0.133, B = - 0.278, P = 0.064) in only AA without T2D. Among individuals with T2D, serum 25(OH)D concentrations were marginally associated with IL-6 (R2 = 0.109, B = 0.076, P = 0.085) in HA with T2D, and there was a trend toward significance with log leptin (R2 = 0.393, B = - 0.006, P = 0.075) in AA with T2D in regression analysis. CONCLUSIONS: The findings that the associations of serum 25(OH)D concentrations with adipokines and cardiometabolic factors differ between HA and AA has clinical and public implications to guide design of T2D preventive strategies that are culturally specific even within the same ethnicity.

Effects of tissue digestion solutions on surface properties of nitinol stents.

Analysis of explanted medical implants can provide a wealth of knowledge about device safety and performance. However, the quality of information may be compromised if the methods used to clean tissue from the device disturb the retrieved condition. Common solutions used to digest tissue may adversely affect the surface of the device and its severity can be material and processing dependent. In this study, two groups of stents made from the same material (Nitinol) were shape set in a salt pot (SP) or further processed by mechanical polishing (MP) and then immersed in one of three tissue digestion solutions (TDS): nitric acid (HNO3 ), sodium hydroxide (NaOH), or papain enzyme (papain). Nickel (Ni) ion concentrations were measured for each stent-TDS combination and post-immersion stent surface constituents, morphology and oxide depths were compared to baseline samples. Exposure to the HNO3 TDS resulted in relatively high Ni ion release and surface damage for both stent types. Papain TDS induced a greater Ni ion release than NaOH TDS, however, both were significantly lower than HNO3 . The NaOH TDS increased the oxide layer thickness on MP stents. In contrast, all other stent immersions resulted in thinner oxide layers. For the Nitinol finishes used in this study, HNO3 is not recommended while papain and NaOH solutions may be appropriate depending on the post-retrieval analysis performed. This study elucidates the importance of preliminary testing for TDS selection and how the surface finish can affect the sensitivity of a material to a TDS. 2017 Wiley Periodicals, Inc. J Biomed Mater Res Part B: Appl Biomater, 106B: 331-339, 2018.

Impact of nitinol stent surface processing on in-vivo nickel release and biological response.

Although nitinol is widely used in percutaneous cardiovascular interventions, a causal relationship between nickel released from implanted cardiovascular devices and adverse systemic or local biological responses has not been established. The objective of this study was to investigate the relationship between nitinol surface processing, in-vivo nickel release, and biocompatibility. Nitinol stents manufactured using select surface treatments were implanted into the iliac arteries of minipigs for 6 months. Clinical chemistry profile, complete blood count, serum and urine nickel analyses were performed periodically during the implantation period. After explant, stented arteries were either digested and analyzed for local nickel concentration or fixed and sectioned for histopathological analysis of stenosis and inflammation within the artery. The results indicated that markers for liver and kidney function were not different than baseline values throughout 180 days of implantation regardless of surface finish. In addition, white blood cell, red blood cell, and platelet counts were similar to baseline values for all surface finishes. Systemic nickel concentrations in serum and urine were not significantly different between processing groups and comparable to baseline values during 180 days of implantation. However, stents with non-optimized surface finishing had significantly greater nickel levels in the surrounding artery compared to polished stents. These stents had increased stenosis with potential for local inflammation compared to polished stents. These findings demonstrate that proper polishing of nitinol surfaces can reduce in-vivo nickel release locally, which may aid in minimizing adverse inflammatory reactions and restenosis. STATEMENT OF SIGNIFICANCE: Nitinol is a commonly used material in cardiovascular medical devices. However, relationships between nitinol surface finishing, in-vivo metal ion release, and adverse biological responses have yet to be established. We addressed this knowledge gap by implanting single and overlapped nitinol stents with different surface finishes to assess systemic impact on minipigs (i.e. serum and urine nickel levels, liver and kidney function, immune and blood count) over the 6 month implantation period. In addition, nickel levels and histopathology in stented arteries were analyzed on explant to determine relationships between surface processing and local adverse tissue reactions. The findings presented here highlight the importance of surface processing on in-vivo nickel release and subsequent impact on local biological response for nitinol implants.

Best practices protocol for the evaluation of bulbar dysfunction: summary recommendations from the NEALS bulbar subcommittee symposium.

OBJECTIVE: The aim of this Symposium was to develop a consensus based, bulbar assessment protocol for implementation within NEALS clinics. METHODS: A one-day symposium, held in April 2017, was organized into Speech and Swallowing sections to establish summary recommendations for the assessment of bulbar dysfunction within each group. RESULTS: Summary recommendations included speech referrals and AAC evaluations at initial visit, CNS-BFS, maximum sustained phonation, and speaking rate. Dysarthria evaluation included the speech subsystem involvement of respiration, phonation, resonance, and articulation. Specific recommendations for swallowing were established for each of the following domains: dietary/oral intake, airway defense physiologic capacity, swallow safety screen, patient-reported swallow-related outcomes, oral sensorimotor exam, and pulmonary function. Practice parameters focused upon patient education and unresolved questions included the use of videofluoscopy, monitoring diet progression, and swallow safety screening. CONCLUSIONS: The working goal is to establish a clinical bulbar protocol, designed to be incorporated within ALS clinics and ultimately to formulate a best practice set of bulbar ALS guidelines, available for implementation throughout the international ALS community.