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Mitochondrial trifunctional protein deficiency is a long-chain fatty acid disorder that may include manifestations of severe cardiomyopathy and arrhythmias. The pathophysiology for the severe presentation is unclear but is an indicator for worse outcomes. Triheptanoin, a synthetic medium chain triglyceride, has been reported to reverse cardiomyopathy in some individuals, but there is limited literature in severe cases. We describe a neonatal onset of severe disease whose clinical course was not improved despite mechanical support and triheptanoin.
Several metrics exist for matching the size of donor to recipient in paediatric cardiac transplantation. Different centres employ different metrics for size-matching to determine the viability of donor hearts. Inconsistent evidence exists, with limited consensus as to the metric for size-matching that is most predictive of outcomes after cardiac transplantation. Furthermore, any metric must function within the tight timeline available for the assessment of the suitability of the donor. At the time of the writing of this paper, the most commonly used metric for size-matching in paediatric cardiac transplantation is the donor-to-recipient body weight ratio. In this article, we review published literature evaluating commonly used metrics for size-matching in paediatric cardiac transplantation, including weight, height, body surface area, and imaging parameters.
Identify risk factors for central line-associated bloodstream infections (CLABSI) in pediatric intensive care settings in an era with high focus on prevention measures.
Design:
Matched, case–control study.
Setting:
Quaternary children’s hospital.
Patients:
Cases had a CLABSI during an intensive care unit (ICU) stay between January 1, 2015 and December 31, 2020. Controls were matched 4:1 by ICU and admission date and did not develop a CLABSI.
Methods:
Multivariable, mixed-effects logistic regression.
Results:
129 cases were matched to 516 controls. Central venous catheter (CVC) maintenance bundle compliance was >70%. Independent CLABSI risk factors included administration of continuous non-opioid sedative (adjusted odds ratio (aOR) 2.96, 95% CI [1.16, 7.52], P = 0.023), number of days with one or more CVC in place (aOR 1.42 per 10 days [1.16, 1.74], P = 0.001), and the combination of a chronic CVC with administration of parenteral nutrition (aOR 4.82 [1.38, 16.9], P = 0.014). Variables independently associated with lower odds of CLABSI included CVC location in an upper extremity (aOR 0.16 [0.05, 0.55], P = 0.004); non-tunneled CVC (aOR 0.17 [0.04, 0.63], P = 0.008); presence of an endotracheal tube (aOR 0.21 [0.08, 0.6], P = 0.004), Foley catheter (aOR 0.3 [0.13, 0.68], P = 0.004); transport to radiology (aOR 0.31 [0.1, 0.94], P = 0.039); continuous neuromuscular blockade (aOR 0.29 [0.1, 0.86], P = 0.025); and administration of histamine H2 blocking medications (aOR 0.17 [0.06, 0.48], P = 0.001).
Conclusions:
Pediatric intensive care patients with chronic CVCs receiving parenteral nutrition, those on non-opioid sedative infusions, and those with more central line days are at increased risk for CLABSI despite current prevention measures.
We reviewed outcomes in all 36 consecutive children <5 kg supported with the Berlin Heart pulsatile ventricular assist device at the University of Florida, comparing those with acquired heart disease (n = 8) to those with congenital heart disease (CHD) (n = 28).
Methods:
The primary outcome was mortality. The Kaplan-Meier method and log-rank tests were used to assess group differences in long-term survival after ventricular assist device insertion. T-tests using estimated survival proportions were used to compare groups at specific time points.
Results:
Of 82 patients supported with the Berlin Heart at our institution, 49 (49/82 = 59.76%) weighed <10 kg and 36 (36/82 = 43.90%) weighed <5 kg. Of 36 patients <5 kg, 26 (26/36 = 72.22%) were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 36 patients <5 kg was [days]: median = 109, range = 4–305.) Eight out of 36 patients <5 kg had acquired heart disease, and all eight [8/8 = 100%] were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 8 patients <5 kg with acquired heart disease was [days]: median = 50, range = 9–130.) Twenty-eight of 36 patients <5 kg had congenital heart disease. Eighteen of these 28 [64.3%] were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 28 patients <5 kg with congenital heart disease was [days]: median = 136, range = 4–305.) For all 36 patients who weighed <5 kg: 1-year survival estimate after ventricular assist device insertion = 62.7% (95% confidence interval = 48.5–81.2%) and 5-year survival estimate after ventricular assist device insertion = 58.5% (95% confidence interval = 43.8–78.3%). One-year survival after ventricular assist device insertion = 87.5% (95% confidence interval = 67.3–99.9%) in acquired heart disease and 55.6% (95% confidence interval = 39.5–78.2%) in CHD, P = 0.036. Five-year survival after ventricular assist device insertion = 87.5% (95% confidence interval = 67.3–99.9%) in acquired heart disease and 48.6% (95% confidence interval = 31.6–74.8%) in CHD, P = 0.014.
Conclusion:
Pulsatile ventricular assist device facilitates bridge to transplantation in neonates and infants weighing <5 kg; however, survival after ventricular assist device insertion in these small patients is less in those with CHD in comparison to those with acquired heart disease.
We examined 3,046,538 acute respiratory infection (ARI) encounters with 6,103 national telehealth physicians from January 2019 to October 2021. The antibiotic prescribing rates were 44% for all ARIs; 46% were antibiotic appropriate; 65% were potentially appropriate; 19% resulted from inappropriate diagnoses; and 10% were related to coronavirus disease 2019 (COVID-19) diagnosis.
Helium or neopentane can be used as surrogate gas fill for deuterium (D2) or deuterium-tritium (DT) in laser-plasma interaction studies. Surrogates are convenient to avoid flammability hazards or the integration of cryogenics in an experiment. To test the degree of equivalency between deuterium and helium, experiments were conducted in the Pecos target chamber at Sandia National Laboratories. Observables such as laser propagation and signatures of laser-plasma instabilities (LPI) were recorded for multiple laser and target configurations. It was found that some observables can differ significantly despite the apparent similarity of the gases with respect to molecular charge and weight. While a qualitative behaviour of the interaction may very well be studied by finding a suitable compromise of laser absorption, electron density, and LPI cross sections, a quantitative investigation of expected values for deuterium fills at high laser intensities is not likely to succeed with surrogate gases.
We evaluated the effectiveness and tolerability of brivaracetam (BRV), an adjunctive antiseizure medication, as a treatment for focal epilepsy in adults. In this prospective study, we enrolled 51 participants from 3 sites across Canada. At 6 months, 68% (26/38) of participants were still taking BRV, among whom 35% (8/23) attained seizure freedom and 48% (11/23) saw their seizure frequency reduced by over 50%. We did not measure any significant change in irritability, quality of life, depression, and anxiety while treated with BRV. Our findings suggest BRV is effective in reducing seizure frequency among adults with focal epilepsy.
White matter hyperintensity (WMH) burden is greater, has a frontal-temporal distribution, and is associated with proxies of exposure to repetitive head impacts (RHI) in former American football players. These findings suggest that in the context of RHI, WMH might have unique etiologies that extend beyond those of vascular risk factors and normal aging processes. The objective of this study was to evaluate the correlates of WMH in former elite American football players. We examined markers of amyloid, tau, neurodegeneration, inflammation, axonal injury, and vascular health and their relationships to WMH. A group of age-matched asymptomatic men without a history of RHI was included to determine the specificity of the relationships observed in the former football players.
Participants and Methods:
240 male participants aged 45-74 (60 unexposed asymptomatic men, 60 male former college football players, 120 male former professional football players) underwent semi-structured clinical interviews, magnetic resonance imaging (structural T1, T2 FLAIR, and diffusion tensor imaging), and lumbar puncture to collect cerebrospinal fluid (CSF) biomarkers as part of the DIAGNOSE CTE Research Project. Total WMH lesion volumes (TLV) were estimated using the Lesion Prediction Algorithm from the Lesion Segmentation Toolbox. Structural equation modeling, using Full-Information Maximum Likelihood (FIML) to account for missing values, examined the associations between log-TLV and the following variables: total cortical thickness, whole-brain average fractional anisotropy (FA), CSF amyloid ß42, CSF p-tau181, CSF sTREM2 (a marker of microglial activation), CSF neurofilament light (NfL), and the modified Framingham stroke risk profile (rFSRP). Covariates included age, race, education, APOE z4 carrier status, and evaluation site. Bootstrapped 95% confidence intervals assessed statistical significance. Models were performed separately for football players (college and professional players pooled; n=180) and the unexposed men (n=60). Due to differences in sample size, estimates were compared and were considered different if the percent change in the estimates exceeded 10%.
Results:
In the former football players (mean age=57.2, 34% Black, 29% APOE e4 carrier), reduced cortical thickness (B=-0.25, 95% CI [0.45, -0.08]), lower average FA (B=-0.27, 95% CI [-0.41, -.12]), higher p-tau181 (B=0.17, 95% CI [0.02, 0.43]), and higher rFSRP score (B=0.27, 95% CI [0.08, 0.42]) were associated with greater log-TLV. Compared to the unexposed men, substantial differences in estimates were observed for rFSRP (Bcontrol=0.02, Bfootball=0.27, 994% difference), average FA (Bcontrol=-0.03, Bfootball=-0.27, 802% difference), and p-tau181 (Bcontrol=-0.31, Bfootball=0.17, -155% difference). In the former football players, rFSRP showed a stronger positive association and average FA showed a stronger negative association with WMH compared to unexposed men. The effect of WMH on cortical thickness was similar between the two groups (Bcontrol=-0.27, Bfootball=-0.25, 7% difference).
Conclusions:
These results suggest that the risk factor and biological correlates of WMH differ between former American football players and asymptomatic individuals unexposed to RHI. In addition to vascular risk factors, white matter integrity on DTI showed a stronger relationship with WMH burden in the former football players. FLAIR WMH serves as a promising measure to further investigate the late multifactorial pathologies of RHI.
The Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) is one of the most widely used measures in neuropsychological assessment. Studies of practice effects on the RBANS have largely been limited to studies assessing one or two repeated assessments. The aim of the current study is to examine practice effects across four years after baseline in a longitudinal study of cognitively healthy older adults. Practice effects were estimated using a pseudo-replacement participants approach which has been previously applied in other aging studies.
Participants and Methods:
453 Participants from the Louisiana Aging Brain Study (LABrainS) completed the RBANS Form A on up to four annual assessments after a baseline evaluation. Practice effects were calculated using a modified participants-replacement method where scores of individuals who were administered RBANS Form A multiple times are compared to the baseline scores of matched participants with additional adjustment for attrition effects.
Results:
Practice effects were observed primarily in the immediate memory, delayed memory, and total score indices. For example, an increase of nearly half a standard deviation was observed for delayed memory.
Conclusions:
These findings extend past work on the RBANS and other neuropsychological batteries more broadly in showing the susceptibility of memory measures to practice effects. Given that memory and total score indices of the RBANS have the most robust relationships with diagnostic status and biomarkers for pathological cognitive decline, these findings raise concerns about the ability to recruit those at risk for decline from longitudinal studies using the same form of the RBANS for multiple years.
Publicly available health information is increasingly important for patients and their families. While the average US citizen reads at an 8th-grade level, electronic educational materials for patients and families are often advanced. We assessed the quality and readability of publicly available resources regarding hypoplastic left heart syndrome (HLHS).
Methods:
We queried four search engines for “hypoplastic left heart syndrome”, “HLHS”, and “hypoplastic left ventricle”. The top 30 websites from searches on Google, Yahoo!, Bing, and Dogpile were combined into a single list. Duplicates, commercial websites, physician-oriented resources, disability websites, and broken links were removed. Websites were graded for accountability, content, interactivity, and structure using a two-reviewer system. Nonparametric analysis of variance was performed.
Results:
Fifty-two websites were analysed. Inter-rater agreement was high (Kappa = 0.874). Website types included 35 hospital/healthcare organisation (67.3%), 12 open access (23.1%), 4 governmental agency (7.7%), and 1 professional medical society (1.9%). Median total score was 19 of 39 (interquartile range = 15.8–25.3): accountability 5.5 of 17 (interquartile range = 2.0–9.3), content 8 of 12 (interquartile range = 6.4–10.0), interactivity 2 of 6 (interquartile range = 2.0–3.0), and structure 3 of 4 (interquartile range = 2.8–4.0). Accountability was low with 32.7% (n = 17) of sites disclosing authorship and 26.9% (n = 14) citing sources. Forty-two percent (n = 22) of websites were available in Spanish. Total score varied by website type (p = 0.03), with open access sites scoring highest (median = 26.5; interquartile range = 20.5–28.6) and hospital/healthcare organisation websites scoring lowest (median = 17.5; interquartile range = 13.5–21.5). Score differences were driven by differences in accountability (p = 0.001) – content scores were similar between groups (p = 0.25). Overall readability was low, with median Flesch–Kincaid Grade Level of 11th grade (interquartile range = 10th–12th grade).
Conclusions:
Our evaluation of popular websites about HLHS identifies multiple opportunities for improvement, including increasing accountability by disclosing authorship and citing sources, enhancing readability by providing material that is understandable to readers with the full spectrum of educational background, and providing information in languages besides English, all of which would enhance health equity.
Diversification of the medical and cardiothoracic surgical workforce represents an ongoing need. A congenital cardiac surgery shadowing programme for undergraduate students was implemented at the University of Florida Congenital Heart Center.
Methods:
Students shadowing in the Congenital Heart Center from 17 December 2020 through 20 July 2021 were sent a survey through Qualtrics to evaluate the impact of their shadowing experience. The main objectives of the survey were to determine the personal relationship(s) of the students to physicians prior to shadowing, how the presence or absence of physicians in the family of a given student related to the exposure of the student to a medical setting prior to shadowing, and the interest of the students in medicine and cardiothoracic surgery prior to and after the shadowing experience. Survey responses included “Yes/No” questions, scaled responses using a Likert scale, selection lists, and free text responses. When applicable, t-tests were utilised to assess differences between student groups.
Results:
Of the 37 students who shadowed during the study period, 26 (70%) responded. Most students were female (58%, n = 15), and the mean age was 20.9 ± 2.4 years. Students spent a mean duration of 95 ± 138 hours shadowing providers as part of the shadowing programme. Likert scale ratings of interest in the professions of medicine, surgery, and cardiothoracic surgery all increased after the shadowing experience (p < 0.01). Students with a family member in medicine had more clinical exposure prior to the shadowing programme (p < 0.01).
Conclusion:
A surgical shadowing programme at a Congenital Heart Center may have an important formative impact on the views of undergraduate students regarding potential careers in surgery and medicine. Additionally, students without family members in medicine tend to have less prior exposure to medicine and could likely benefit more from this type of shadowing programme.
From its inception, international law has been closely linked to philosophy and political theory, and over time competing theoretical approaches have emerged to explain international law’s nature, form, and efficacy. Even today, we associate the most authoritative names in the discipline – whether founders such as Grotius and Vattel or more modern figures ranging from Kelsen and Lauterpacht to contemporary writers such as Chimni, Chinkin, and Koskenniemi – with specific theoretical perspectives that engage foundational questions about international law’s purpose and content.
Every generation of scholars and practitioners reshapes international legal theory as it rethinks international law and its role in international affairs. Two decades ago, the American Journal of International Law (AJIL) published a “Symposium on Methods in International Law.” The AJIL Symposium took place toward the end of the 1990s, during heady times for international law.