Noninvasive Neurally Adjusted Ventilatory Assist in Infants With Bronchiolitis: Respiratory Outcomes in a Single-Center, Retrospective Cohort, 2016-2018.

OBJECTIVES: To describe our experience of using noninvasive neurally adjusted ventilatory assist (NIV-NAVA) in infants with bronchiolitis, its association with the evolution of respiratory effort, and PICU outcomes. DESIGN: Retrospective analysis of a prospectively curated, high-frequency electronic database. SETTING: A PICU in a university-affiliated maternal-child health center in Canada. PATIENTS: Patients younger than 2 years old who were admitted with a diagnosis of acute bronchiolitis and treated with NIV-NAVA from October 2016 to June 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Patient characteristics, as well as respiratory and physiologic parameters, including electrical diaphragmatic activity (Edi), were extracted from the electronic database. Respiratory effort was estimated using the modified Wood Clinical Asthma Score (mWCAS) and the inspiratory Edi. A comparison in the respiratory effort data was made between the 2 hours before and 2 hours after starting NIV-NAVA. In the two seasons, 64 of 205 bronchiolitis patients were supported with NIV-NAVA. These 64 patients had a median (interquartile range [IQR]) age of 52 days (32-92 d), and there were 36 of 64 males. Treatment with NIV-NAVA was used after failure of first-tier noninvasive respiratory support; 25 of 64 patients (39%) had at least one medical comorbidity. NIV-NAVA initiation was associated with a moderate decrease in mWCAS from 3.0 (IQR, 2.5-3.5) to 2.5 (IQR, 2.0-3.0; p < 0.001). NIV-NAVA initiation was also associated with a statistically significant decrease in Edi ( p < 0.01). However, this decrease was only clinically relevant in infants with a 2-hour baseline Edi greater than 20 µV; here, the before and after Edi was 44 µV (IQR, 33-54 µV) compared with 27 µV (IQR, 21-36 µV), respectively ( p < 0.001). Overall, six of 64 patients (9%) required endotracheal intubation. CONCLUSIONS: In this single-center retrospective cohort, in infants with bronchiolitis who were considered to have failed first-tier noninvasive respiratory support, the use of NIV-NAVA was associated with a rapid decrease in respiratory effort and a 9% intubation rate.

Inadvertent Acute Lipid Injectable Emulsion Overdose Resulting in Fat Overload Syndrome and Pancreatitis in a Patient with TPN Dependence.

We report a case of an acute, inadvertent, 7.5-fold intravenous lipid emulsion overdose with 20% SMOFlipid in an 11-month-old female with tetratricopeptide repeat domain 7A (TTC7A) mutation, intestinal failure, and parenteral nutrition dependence. The overdose resulted in critical deterioration with evidence of fever, metabolic acidosis, respiratory failure, and pancreatitis that resulted in admission to the intensive care unit. This is a unique case of fat overload syndrome with acute pancreatitis following an accidental lipid injectable emulsion overdose in a pediatric patient.

Clostridium septicum myonecrosis in a pediatric patient with a self-reported penicillin allergy.

Infections with Clostridium septicum are especially rare in pediatric patients. C. septicum is the most common cause of spontaneous myonecrosis and is usually associated with comorbid malignancy. Treatment of choice for cases of C. septicum myonecrosis is prompt and thorough surgical debridement and antimicrobial therapy with high dose penicillin. The experience and management of C. septicum infections in patients who are unable to take penicillin are not well described, and the optimal duration of therapy is largely unknown. We describe a case of spontaneous myonecrosis in a 14-year-old receiving cytotoxic chemotherapy for Burkitt's lymphoma who had an anecdotal history of a penicillin allergy. Her infection was initially treated with ceftazidime and metronidazole in concert with debridement but was ultimately cured with 3 weeks of intravenous penicillin therapy following a graded penicillin challenge in hospital. We observed a delayed inflammatory tissue response to a C. septicum skin, soft tissue infection that temporally corresponded to neutrophil reconstitution in our patient with severe neutropenia. Our experience demonstrates that C. septicum myonecrosis can present indolently and progress rapidly and highlights the need for clinical vigilance and repeat "second-look" surgeries. Our case also emphasizes the importance of de-labelling penicillin allergies.

Les infections à Clostridium septicum sont particulièrement rares chez les patients d'âge pédiatrique. Le C. septicum, qui est la principale cause de myonécrose spontanée, est généralement associé à des malignités. Le traitement de première intention de la myonécrose à C. septicum consiste à effectuer un débridement chirurgical rapide et minutieux et un traitement antimicrobien au moyen d'une forte dose de pénicilline. L'expérience et la prise en charge des infections à C. septicum ne sont pas bien décrites chez les patients qui ne sont pas en mesure de prendre de la pénicilline, et on ne connaît pas vraiment la durée optimale du traitement. Les auteurs décrivent le cas d'une myonécrose spontanée chez une adolescente de 14 ans qui a reçu une chimiothérapie cytotoxique pour traiter un lymphome de Burkitt et qui a une allergie non démontrée à la pénicilline. Son infection a d'abord été traitée par de la ceftazidime et du métronidazole, conjointement avec le débridement, mais a fini par être guérie au bout de trois semaines de traitement intraveineux à la pénicilline après un test de provocation graduel à la pénicilline à l'hôpital. Les auteurs ont observé une réponse inflammatoire tardive des tissus à une infection de la peau et des tissus mous au C. septicum qui correspondait temporellement à la reconstitution des neutrophiles chez la patiente atteinte d'une grave neutropénie. Selon leur expérience, la myonécrose à C. septicum peut se manifester sous forme indolente et progresser rapidement, ce qui fait ressortir l'importance de la vigilance clinique et des secondes interventions chirurgicales. Ce cas démontre également l'importance de désétiqueter les allergies à la pénicilline.

Contrast-free optical coherence tomography:Systematic evaluation of non-contrast media for intravascular assessment.

BACKGROUND: Coronary revascularization using imaging guidance is rapidly becoming the standard of care. Intravascular optical coherence tomography uses near-infrared light to obtain high resolution intravascular images. Standard optical coherence tomography imaging technique employs iodinated contrast dye to achieve the required blood clearance during acquisition. We sought to systematically evaluate the technical performance of saline as an alternative to iodinated contrast for intravascular optical coherence tomography assessment. METHODS AND RESULTS: We performed bench top optical coherence tomography analysis on nylon tubing with sequential contrast/saline dilutions to empirically derive adjustment coefficients. We then applied these coefficients in vivo in an established rabbit abdominal stenting model with both saline and contrast optical coherence tomography imaging. In this model, we assessed the impact of saline on both quantitative and qualitative vessel assessment. Nylon tubing assessment demonstrated a linear relationship between saline and contrast for both area and diameter. We then derived adjustment coefficients, allowing for accurate calculation of area and diameter when converting saline into both contrast and reference dimensions. In vivo studies confirmed reduced area with saline versus contrast [7.43 (5.67-8.36) mm2 versus 8.2 (6.34-9.39) mm2, p = 0.001] and diameter [3.08 mm versus 3.23 mm, p = 0.001]. Following correction, a strong relationship was achieved in vivo between saline and contrast in both area and diameter without compromising image quality, artefact, or strut assessment. CONCLUSION: Saline generates reduced dimensions compared to contrast during intravascular optical coherence tomography imaging. The relationship across physiologic coronary diameters is linear and can be corrected with high fidelity. Saline does not adversely impact image quality, artefact, or strut assessment.

The impact of implementing a preprinted order form for inpatient management of otherwise healthy children admitted to a tertiary care centre with a diagnosis of bronchiolitis.

OBJECTIVE: To examine the effect on length of stay (LOS) of a preprinted order (PPO) set for children admitted to hospital with a diagnosis of bronchiolitis, as well as on quality improvement measures, medical errors, and resource utilization. PATIENTS AND METHODS: A retrospective chart review was performed of children admitted to the inpatient units at the Children's Hospital of Eastern Ontario (CHEO) with a discharge diagnosis of bronchiolitis. Primary and secondary outcomes were compared between the pre-PPO (December 1, 2014 to June 30, 2015) and post-PPO (December 1, 2015 to June 30, 2016) periods. The primary outcome was LOS in days. Secondary outcomes included the proportion of quality improvement measures reached, the number of medical errors, and resource utilization. RESULTS: A total of 245 patients were included; 122 patients from the pre-PPO period (December 1, 2014 to June 30, 2015) and 123 patients from the post-PPO period (December 1, 2015 to June 30, 2016). Mean LOS was 3.1 days (2.7 to 3.5 days) and 2.8 days (2.4 to 3.2 days) in the pre- and post-PPO periods, respectively (multivariate analysis, P-value = 0.13). There were significant differences between the pre- and post-PPO periods in a number of quality improvement measures, although not in the number of medical errors. Significant reductions in oxygen, corticosteroid, antibiotic, and bronchodilator use were noted post-PPO implementation. CONCLUSION: Although our bronchiolitis PPO did not significantly shorten LOS, it led to important improvements in quality improvement measures and in resource utilization efficiency.

Overemphasis of the Autism Diagnostic Observation Schedule (ADOS) Evaluation Subverts a Clinician's Ability to Provide Access to Autism Services.

A concerning trend has emerged in the diagnosis and treatment of autism spectrum disorder (ASD) that has a negative impact on care. Quite often, a clinician's diagnosis of ASD using DSM-5 criteria is no longer sufficient for individuals with ASD to access services. Insurance companies, school districts, and developmental disability agencies commonly require an Autism Diagnostic Observation Schedule (ADOS) to be eligible for services.

A novel echocardiographic hemodynamic index for predicting outcome of aortic stenosis patients following transcatheter aortic valve replacement.

OBJECTIVE: Transcatheter aortic valve replacement (TAVR) reduces left ventricular (LV) afterload and improves prognosis in aortic stenosis (AS) patients. However, LV afterload consists of both valvular and arterial loads, and the benefits of TAVR may be attenuated if the arterial load dominates. We proposed a new hemodynamic index, the Relative Valve Load (RVL), a ratio of mean gradient (MG) and valvuloarterial impedance (Zva), to describe the relative contribution of the valvular load to the global LV load, and examined whether RVL predicted patient outcome following TAVR. METHODS: A total of 258 patients with symptomatic severe AS (indexed aortic valve area (AVA)<0.6cm2/m2, AR≤2+) underwent successful TAVR at the University of Ottawa Heart Institute and had clinical follow-up to 1-year post-TAVR. Pre-TAVR MG, AVA, percent stroke work loss (%SWL), Zva and RVL were measured by echocardiography. The primary endpoint was all cause mortality at 1-year post TAVR. RESULTS: There were 53 deaths (20.5%) at 1-year. RVL≤7.95ml/m2 had a sensitivity of 60.4% and specificity of 75.1% for identifying all cause mortality at 1-year post-TAVR and provided better specificity than MG<40 mmHg, AVA>0.75cm2, %SWL≤25% and Zva>5mmHg/ml/m2 despite equivalent or better sensitivity. In multivariable Cox analysis, RVL≤7.95ml/m2 was an independent predictor of all cause mortality (HR 3.2, CI 1.8-5.9; p<0.0001). RVL≤7.95ml/m2 was predictive of all cause mortality in both low flow and normal flow severe AS. CONCLUSIONS: RVL is a strong predictor of all-cause mortality in severe AS patients undergoing TAVR. A pre-procedural RVL≤7.95ml/m2 identifies AS patients at increased risk of death despite TAVR and may assist with decision making on the benefits of TAVR.

Transcatheter Aortic Valve Implantation (TAVI) for Native Aortic Valve Regurgitation　- A Systematic Review.

BACKGROUND: Transcatheter aortic valve implantation (TAVI) has become the standard of care for management of high-risk patients with aortic stenosis. Limited data is available regarding the performance of TAVI in patients with native aortic valve regurgitation (NAVR).Methods and Results:We performed a systematic review from 2002 to 2016. The primary outcome was device success as per VARC-2 criteria. Secondary endpoints included procedural complications, and 30-day and 1-year mortality rates. A total of 175 patients were included from 31 studies. Device success was reported in 86.3% of patients - with device failure driven by moderate aortic regurgitation (AR ≥3+) and/or need for a second device. Procedural complications were rare, with no procedural deaths, myocardial infarctions or annular ruptures reported. Procedural safety was acceptable with a low 30-day incidence of stroke (1.5%). The 30-day and 1-year overall mortality rates were 9.6% and 20.0% (cardiovascular death, 3.8% and 10.1%, respectively). Patients receiving 2nd-generation valves demonstrated similar safety profiles with greater device success compared with 1st-generation valves (96.2% vs. 78.4%). This was driven by the higher incidence of second-valve implantation (23.4% vs. 1.7%) and significant paravalvular leak (8.3% vs. 0.0%). CONCLUSIONS: TAVI demonstrates acceptable safety and efficacy in high-risk patients with severe NAVR. Second-generation valves may afford a similar safety profile with improved device success. Dedicated studies are needed to definitively establish the efficacy of TAVI in this population.

Development of the Supported Employment, Comprehensive Cognitive Enhancement, and Social Skills program for adults on the autism spectrum: Results of initial study.

The population of adults on the autism spectrum continues to increase, and vocational outcomes are particularly poor. Longitudinal studies of adults with autism spectrum and without intellectual disability have shown consistent and persistent deficits across cognitive, social, and vocational domains, indicating a need for effective treatments of functional disabilities as each impact employment. This initial pilot study is an open trial investigation of the feasibility, acceptability, and initial estimates of outcomes for the newly developed Supported Employment, Comprehensive Cognitive Enhancement, and Social Skills intervention, a manualized "soft skills" curriculum, to enhance both cognitive and social development in adults with autism spectrum. A total of eight adults with autism spectrum, without intellectual disability (78% males), participated in the study. Results support the original hypothesis that adults with autism spectrum can improve both cognitive (i.e. executive functioning) and social cognitive (i.e. social thinking and social communication) abilities. Further Supported Employment, Comprehensive Cognitive Enhancement, and Social Skills was found to be feasible, acceptable, and highly satisfactory for participants and parents. Employment rates more than doubled post-intervention, with an increase from 22% to 56% of participants employed. Conclusion is that Supported Employment, Comprehensive Cognitive Enhancement, and Social Skills has promise as an intervention that can be easily embedded into exiting supported employment vocational training programs to improve cognitive, social, and vocational outcomes.

Predicting Acute Kidney Injury following Transcatheter Aortic Valve Replacement.

PURPOSE: Acute kidney injury occurs in up to a quarter of patients following transcatheter aortic valve replacement (TAVR) and has been associated with increased short and long-term mortality rates. A variety of patient characteristics predictive of post-TAVR acute kidney injury (AKI) have been identified, however discrepancies among studies exist almost uniformly. We investigated the hypothesis that the change in glomerular filtration rate (ΔGFR) in response to contrast administered during pre-TAVR coronary angiography is predictive of ΔGFR post-TAVR. METHODS: The study comprised 195 patients who underwent TAVR at a single center between August 2008 and June 2015 and were prospectively included in the CAPITAL TAVR registry. Multiple linear regression analysis was conducted to estimate the effect of independent variables on the change in renal function post-TAVR. RESULTS: There was no relationship identified between the ΔGFR post-angiogram and the ΔGFR post-TAVR (r=0.043, P=0.582). Multiple linear regression analysis revealed that a significant amount of the change in renal function post-TAVR can be explained by the patient's baseline creatinine (beta coefficient, -0.310, P.

Parent perceptions of an adapted evidence-based practice for toddlers with autism in a community setting.

Although data from parent-implemented Naturalistic Developmental Behavioral Interventions have shown positive effects on decreasing core symptoms of autism, there has been limited examination of the effectiveness of Naturalistic Developmental Behavioral Interventions in community settings. In addition, parent perspectives of their involvement in parent-implemented early intervention programs have not been well studied. Using both qualitative and quantitative data to examine parent perspectives and the perceived feasibility of parent training by community providers, 13 families were followed as they received training in the Naturalistic Developmental Behavioral Intervention, Project ImPACT. Data indicate that parent training by community providers is feasible and well received, and parents find value in participating in intervention and perceive benefit for their children. Recommendations for adaptation of program elements and future research are discussed.

BUILDING A RESEARCH-COMMUNITY COLLABORATIVE TO IMPROVE COMMUNITY CARE FOR INFANTS AND TODDLERS AT-RISK FOR AUTISM SPECTRUM DISORDERS.

This article describes the formation and initial outcomes of a research-community collaborative group that was developed based on community-based participatory research principles. The group includes a transdisciplinary team of practitioners, funding agency representatives, researchers, and families of children with autism spectrum disorders, who partnered to improve community-based care for infants and toddlers at risk for autism through the implementation of evidence-based practices. Data from this group provide support for the feasibility of developing and sustaining a highly synergistic and productive research-community collaborative group who shares common goals to improve community care.