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BACKGROUND Tacrolimus is a narrow therapeutic index drug, requiring consistent levels to maximize transplant success while reducing adverse effects. Elevated tacrolimus level variability (ETLV) is associated with poor outcomes in both pediatric and adult solid organ transplant recipients. We sought to describe the prevalence of ETLV and identify associations with patient-specific factors and poor outcomes. METHODS Tacrolimus levels were evaluated from 118 patients at our single center. As a marker of variability, standard deviations (SD) were calculated for each patient from their entire tacrolimus level data set (global SD), and 1-2 years and 1-5 years post-HT (prediction window SDs). SD ≥3 denoted ETLV. RESULTS There was large variability in tacrolimus levels (median global SD 3.1; IQR 2.3, 4; SD ≥3, n = 64, 54%). Patients with elevated SD (≥3) vs lower SD (

Cardiac arrhythmias occur in 3-40% of patients with acute myocarditis and cause significant morbidity and mortality. Myocardial injury also results in abnormal myocardial deformation. The relationship between left ventricular (LV) deformation, measured by two-dimensional speckle tracking echocardiography (2D-STE), and arrhythmia in pediatric myocarditis is unknown. We evaluated the association between 2D-STE and arrhythmias in children hospitalized with acute myocarditis. We reviewed patients ≤ 18 years hospitalized for acute myocarditis from 2008 to 2018. Arrhythmias were defined as 1) non-sustained or sustained ventricular tachycardia or ventricular fibrillation, 2) sustained supraventricular tachycardia (SVT), 3) high-grade or complete heart block, and 4) any arrhythmia treated with an antiarrhythmic medication. Systolic LV strain values (including LV global longitudinal strain (GLS), global circumferential strain (GCS), and six segments of LV regional long axis strain) were obtained from initial echocardiograms during hospitalization. Of 66 patients hospitalized, 23 (35%) had arrhythmias. SVT was the predominant arrhythmia (74%). Global and regional strain indices were reduced in the arrhythmia group: LV GLS [-8.9 (IQR -13.6, -6.1) vs. -13.7 (IQR -16.9, -9.7), p = 0.038]; basal inferior/septal [-10.7 (IQR -15.5, -7.8) vs. -16.4 (IQR -18, -11.8), p = 0.009]; basal anterior/lateral [-7.1 (IQR -12.8, -4.7) vs. -9.4 (IQR -16.7, -7.4), p = 0.025]; and mid inferior/septal segments [-9 (IQR -13, -7.7) vs. -14.1 (IQR -22.5, -10.7), p = 0.007]. After controlling for age, reductions in GLS and segmental strain in the two basal and two mid-segments were associated with increased arrhythmia occurrence (p0.05). Our findings suggest that echocardiographic LV deformation by 2D-STE may be useful in identifying pediatric patients with acute myocarditis at risk for arrhythmias.

Objective.Sublingual microscopy assesses systemic sclerosis (SSc) vasculopathy. Digital thermal monitoring (DTM) may identify patients at risk for digital ulcer (DU). The purpose of this analysis was to assess sublingual microscopy and DTM in SSc patients with and without previous DU in order to determine the utility of these clinical tools.Methods.SSc registry patients with clinical data who had both DTM and sublingual microscopy on the same day were included in this cross-sectional analysis. DTM quantifies vascular reactivity index (VRI). Sublingual microscopy measures longitudinal red blood cell fraction (RBCfract) and perfused boundary region (PBR). We evaluated the pairwise association between VRI, RBCfract, and PBR in a monotonic relationship using Spearman rank correlation in the DU subset. Correlation coefficients (rs) and their 95% CIs were reported.Results.Ninety patients were included; 29 had digital pits and/or active DU and 61 never had a DU. The only significant clinical feature associated with DU was modified Rodnan skin score (P = 0.003) with DU being higher. The VRI was lower in patients with DU (P = 0.01). The higher the RBCfract, the lower PBR (rs = –0.71, 95% CI –0.86 to –0.47, P < 0.001). VRI was not associated with RBCfract or PBR (P = 0.24 or 0.55, respectively) in the patients with DU.Conclusion.DTM is a useful tool for assessing SSc-DU. While sublingual microscopy measurements did not significantly correlate to VRI in patients with SSc-DU, a longitudinal study may be more helpful in capturing vasculopathy activity prior to possible irreversible damage.

To investigate change in weight-for-age z-scores (WAZ) and risk factors for impaired weight gain between stage 1 palliation (S1P) for single ventricle physiology and discharge.This was a secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative Phase II database. The primary outcome was change in WAZ between S1P and discharge. Risk factors were selected using multivariable mixed effects regression constructed by step-wise model selection, with adjustment for WAZ at S1P and a random effect for center.Of 730 infants who were discharged after S1P, WAZ decreased in 98.6% (-1.5 ± 0.7). WAZ at discharge was-1 but-2 (at risk) in 40% and-2 (failure to thrive) in 35% of participants. Males, higher WAZ at S1P, non-S1P procedures (mostly noncardiac), increased length of stay, necrotizing enterocolitis, and angiotensin-converting enzyme inhibitor use at discharge were associated with a greater decrease in WAZ. Preoperative enteral feeding and respiratory medications were associated with a lesser decrease in the WAZ.Nearly all infants lose weight after S1P with little recovery by hospital discharge. At discharge, three-quarters of the infants in the cohort were at risk for impaired weight gain or had failure to thrive. Most risk factors associated with change in WAZ were unmodifiable or surrogates of disease severity. Novel interventions are needed to minimize the early catabolic effects and promote anabolic recovery after S1P.

Background:A 10% prevalence of intracranial aneurysms in patients with coarctation of the aorta has been described in a few studies. Our objective is to describe the rate of intracranial aneurysm detection in patients with coarctation of the aorta in the current era. We hypothesise that, with earlier detection and coarctation of the aorta intervention, the rate of intracranial aneurysm is lower than previously reported and screening imaging may only be warranted in older patients or patients with certain risk factors.Methods:This is a retrospective study of 102 patients aged 13 years and older with coarctation who underwent brain computed tomography angiography, magnetic resonance imaging (MRI), or magnetic resonance angiography between January, 2000 and February, 2018.Results:The median age of coarctation repair was 4.4 months (2 days–47 years) and the initial repair was primarily surgical (90.2%). There were 11 former smokers, 4 current smokers, and 13 patients with ongoing hypertension. Imaging modalities included computed tomography angiography (13.7%), MRI (41.2%), and magnetic resonance angiography (46.1%), performed at a median age of 33.3 years, 22.4 years, and 25 years, respectively. There were 42 studies performed for screening, 48 studies performed for neurologic symptoms, and 12 studies performed for both screening and symptoms. There were no intracranial aneurysms detected in this study.Conclusions:These results suggest that the rate of intracranial aneurysms may be lower than previously reported and larger studies should explore the risk of intracranial aneurysms in coarctation of the aorta in the current era.

Background Coronary artery abnormalities in Kawasaki disease (KD) are assessed using echocardiographic z-scores. We hypothesized that changing the coronary artery (CA) z-score model would alter diagnosis and management of children with KD. Methods In this retrospective single-center study of children treated for KD (9/2007–1/2020), we collected echocardiographic measurements for the left anterior descending (LAD), right (RCA), and left main (LMCA) coronary arteries during 3 illness phases and calculated Boston and Pediatric Heart Network (PHN) z-scores. Agreement between Boston and PHN z-scores was assessed using Kappa (κ) and Lin’s Concordance Correlation Coefficients (CCC) and Bland–Altman analysis. Results For 904 echocardiograms from 357 children, the median Boston LAD z-score was lower than the PHN (0.3 [IQR − 0.6, 1.5] vs 1.6 [IQR 0.7, 2.8], CCC 0.94 [95% CI 0.93, 0.95], moderate agreement), aggregated across all illness phases. RCA and LMCA z-scores showed substantial agreement. With conversion from Boston to PHN models, the percentage of individual LAD z-scores ≥ 2.5 increased (14.6% to 32.1%). At least one CA z-score classification changed in 213 children (59.7%) across all phases, and 48 children (13.4%) had a change that altered recommended antithrombotic strategy. Agreement between models differed by age, sex, and race. Conclusions Conversion from Boston to PHN z-scores changed at least 1 CA z-score classification in over half of KD patients and changed recommended antithrombotic management in 13%, largely driven by LAD measurements. Since diagnosis and management of KD and KD-like diseases rely upon CA z-scores, the clinical and research implications of these findings merit further exploration.

Objective:To determine the Final ICU Need in the 24 hours prior to ICU discharge for children with cardiac disease by utilising a single-centre survey.Methods:A cross-sectional survey was utilised to determine Final ICU Need, which was categorised as “Cardiovascular”, “Respiratory”, “Feeding”, “Sedation”, “Systems Issue”, or “Other” for each encounter. Survey responses were obtained from attending physicians who discharged children (≤18 years of age with ICU length of stay >24 hours) from the Cardiac ICU between April 2016 and July 2018.Measurements and results:Survey response rate was 99% (n = 1073), with 667 encounters eligible for analysis. “Cardiovascular” (61%) and “Respiratory” (26%) were the most frequently chosen Final ICU Needs. From a multivariable mixed effects logistic regression model fitted to “Cardiovascular” and “Respiratory”, operations with significantly reduced odds of having “Cardiovascular” Final ICU Need included Glenn palliation (p = 0.003), total anomalous pulmonary venous connection repair (p = 0.024), truncus arteriosus repair (p = 0.044), and vascular ring repair (p < 0.001). Short lengths of stay (Conclusions:Final ICU Need is a novel metric to identify variations in Cardiac ICU utilisation and clinical trajectories. Final ICU Need was significantly influenced by benchmark operation, length of stay, provider, and season. Future applications of Final ICU Need include targeting quality and research initiatives, calibrating provider and family expectations, and identifying provider-level variability in care processes and mental models.

Background Significant variability in morbidity and mortality persists for children with functionally single ventricle congenital heart disease (SV‐CHD) despite standardization in medical and surgical care. We hypothesized that maternal health factors may be associated with an increased risk of poor outcomes in children with SV‐CHD. Methods and Results This retrospective, observational, cohort study included term maternal‐infant pairs with a diagnosis of SV‐CHD who underwent surgical palliation from 2006 to 2015 at Primary Children's Hospital. Pairs lacking maternal variables of interest or infant follow‐up data were excluded. The association of maternal risk factors of abnormal pre‐pregnancy body mass index, abnormal gestational weight gain (20 kg), hypertensive disorders, and gestational diabetes mellitus with death/transplant and hemodynamics were analyzed using regression models. Of 190 infants, 135 (71%) maternal‐infant dyads had complete data for inclusion. Death or transplant occurred in 48 infants (36%) during an average follow‐up of 2.2 years (0.1–11.7 years). Abnormal gestational weight gain was associated with an increased risk of death and/or transplant in logistic regression (odds ratio, 3.22; 95% CI, 1.32–7.86; P =0.01), but not Cox regression (hazard ratio, 1.9; 95% CI, 1.0–3.7; P =0.055). Mean pulmonary artery pressures were higher in the setting of abnormal gestational weight gain (16.5±2.9 versus 14.7±3.0 mm Hg; P P Conclusions Abnormal gestational weight gain (excessive or inadequate) is a novel risk factor for worse outcomes in SV‐CHD. The fetoplacental environment may alter the trajectory of vascular development to impact outcomes in infants with SV‐CHD.

Infants with aortic coarctation may present with left ventricular (LV) dysfunction which may complicate the postoperative course and lead to increased healthcare costs. We aimed to define the prevalence of moderate to severe left ventricular (LV) systolic dysfunction, evaluate time to recovery, and compare health care costs. Single-center retrospective cohort study at a tertiary care hospital was conducted. Infants 6 months old at diagnosis with aortic coarctation were identified using surgical codes for coarctation repair between January 2010 and May 2018. Moderate to severe dysfunction was defined as ejection fraction (EF) 40%. Of 160 infants studied, 18 (11%) had moderate to severe LV dysfunction at presentation. Compared to those with better LV function, infants with moderate to severe LV dysfunction were older at presentation (12 vs. 6 days, p = 0.004), had more postoperative cardiac intensive care unit (ICU) days (5 vs. 3, p 0.001), and more ventilator days (3.5 vs. 1, p 0.001). The median time to normal LV EF (≥ 55%) was 6 days postoperatively (range 1-230 days). Infants presenting with moderate to severe LV dysfunction had higher index hospitalization costs ($90,560 vs. $59,968, p = 0.02), but no difference in cost of medical follow-up for the first year following discharge ($3,078 vs. $2,568, p = 0.46). In the current era, 10% of infants with coarctation present with moderate to severe LV dysfunction that typically recovers. Those with moderate to severe dysfunction had longer duration of mechanical ventilation and postoperative cardiac ICU stays, likely driving higher costs of index hospitalization.

BACKGROUND Understanding the variation in costs of endoscopic sinus surgery (ESS) is critical to defining value. Current published costs of ESS have not identified potential sources of variation. Our objective was to analyze ESS costs to identify sources of variance that could guide value-improving decisions. METHODS ESS cases (n = 1739) performed between 2008 and 2016 were identified from a database of 22 rural to tertiary facilities. Cost and time data were extracted from the database. Medical records were reviewed to confirm procedures. Three bilateral groupings were examined (n = 895 cases from 13 facilities): (1) full ESS (all sinuses); (2) intermediate ESS (total ethmoid, maxillary); and (3) anterior ESS (anterior ethmoid, maxillary). Cost and operative time were analyzed using multivariable gamma regression. RESULTS Median costs for full, intermediate, and anterior ESS were $4281, $3716, and $2549 U.S. dollars (p < 0.001). Median durations were 87, 60, and 58 minutes (p < 0.001). Among patients with no additional procedures, those with full ESS had operative duration, total cost, and supply costs that were 1.37 (95% confidence interval [CI], 1.17 to 1.61), 1.52 (95% CI, 1.32 to 1.75), and 2.40 (95% CI, 1.76 to 3.25) times greater than anterior ESS, respectively (all p < 0.001). Intermediate ESS duration at community urban facilities was 1.87 (95% CI, 1.74 to 2.02) times that of community rural facilities (p < 0.001). CONCLUSION Duration of surgery, extent of surgery, and location of surgery are sources of significant variation in the cost of ESS. These findings will assist healthcare policy makers, hospitals, and surgeons in optimizing the value of ESS.

The pet food market has grown steadily in part through introduction of novel ingredients. Whole sorghum has been used frequently, but more novelty and nutritional value may be derived if sorghum flour or bran were considered separately. Therefore, the objective of this research was to produce and evaluate sorghum fractions for use in a pet food. A locally grown red sorghum was purchased and milled into different fractions that were later incorporated into extruded dog foods. Yields of flour, mill-feed and germ averaged 69.2%, 28.5% and 0.79%, respectively. Four diets were produced by extrusion: a whole sorghum (WSD), sorghum flour (FLD), sorghum mill-feed (MFD), and control (CON) with equal parts corn, rice and wheat in a completely randomized design experiment. The FLD had the greatest (P

OBJECTIVE: The Pediatric Heart Network sponsored the multicenter Collaborative Learning Study that implemented a clinical practice guideline to facilitate early extubation in infants after repair of isolated coarctation of the aorta and Tetralogy of Fallot. We sought to compare the anesthetic practice in the operating room and sedation-analgesia management in the ICU before and after the implementation of the guideline that resulted in early extubation. DESIGN: Secondary analysis of data from a multicenter study from January 2013 to April 2015. Predefined variables of anesthetic, sedative and analgesia exposure were compared before and after guideline implementation. Propensity score weighted logistic regression analysis was used to determine the independent effect of intraoperative dexmedetomidine administration on early extubation. SETTING: Five children’s hospitals. PATIENTS: 240 study subjects who underwent repair of coarctation of the aorta or Tetralogy of Fallot (119 pre and 121 post-guideline implementation). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Clinical practice guideline implementation was accompanied by a decrease in the median total intraoperative dose of opioids (49.7 vs 24.0 mcg/kg of Fentanyl Equivalents, p

Purpose To calculate the minimal clinically important difference (MCID) of the Patient-Reported Outcomes Measurement Information System (PROMIS) Upper Extremity Computer Adaptive Test (UE CAT) and Quick–Disabilities of the Arm, Shoulder, and Hand (Quick DASH) for ligament reconstruction tendon interposition (LRTI) patients. Methods Adult patients treated with LRTI for trapeziometacarpal OA by fellowship-trained hand surgeons between December 2014 and February 2018 at an academic tertiary institution were included. Outcomes were prospectively collected at each visit by tablet computer, including the Quick DASH, PROMIS UE, Pain Interference, Depression, and Anxiety CATs. Inclusion required a response to the anchor question “How much relief and/or improvement do you feel you have experienced as a result of your treatment?” on a 6-option Likert scale , as well as preoperative (≤120 days before surgery) and follow-up (2–26 weeks) outcomes. We calculated MCID both by an anchor-based approach using the mean score of the minimal change group, and with the 0.5 SD method. Results Of 145 included participants, mean age was 63 ± 8 years and 74% were female. Anchor-based MCID estimates for the total cohort were 4.2 for the PROMIS UE CAT and 8.8 for the QuickDASH. The MCID estimates using the 0.5 SD method were 4.8 and 11.7, respectively. Conclusions We propose MCID values of 4.2 to 4.8 for the PROMIS UE CAT and 8.8 to 11.7 for the QuickDASH when powering clinical studies or when assessing improvement among a cohort of patients who have undergone LRTI surgery. Clinical relevance Minimal clinically important difference estimates are helpful when interpreting clinical outcomes after LRTI and for powering prospective trials.

Purpose Biopsychosocial factors are associated with patient-reported upper extremity disability; however, their impact on postoperative outcomes remains less clear. Our purpose was to test the hypothesis that high levels of preoperative patient-reported upper extremity and psychological disability are associated with lower magnitudes of improvement following trapeziectomy with ligament reconstruction tendon interposition (LRTI). Methods Adult patients undergoing LRTI by fellowship-trained hand surgeons between February 2014 and March 2017 were considered for inclusion. Patient-reported outcomes were collected by tablet computer, including the shortened Disabilities of the Arm, Shoulder, and Hand (QuickDASH), Patient-Reported Outcomes Measurement Information System (PROMIS) Pain Interference (PI) Computer Adaptive Test (CAT), PROMIS Depression CAT, and PROMIS Anxiety CAT. Univariate and multivariable linear regression analyses were performed to identify factors associated with QuickDASH improvement. Results Of 119 included patients, the mean age was 62.7 ± 7.2 years and 74% were women. At a mean of 14 weeks after surgery, the QuickDASH improved by a mean of –10 ± 20 points. The QuickDASH improvement was significantly greater for patients with preoperative QuickDASH scores greater than the median (score change, –19 ± 20) versus those at or below the median (–2 ± 16), and for patients with preoperative PI CAT scores greater than the median (–17 ± 21) versus at or below the median (–4 ± 17). No differences were observed between groups when Anxiety or Depression CAT baseline scores were similarly dichotomized. Multivariable modelling revealed that higher preoperative QuickDASH scores were associated with greater improvement on the QuickDASH; however, no association was observed for preoperative PI CAT, Depression CAT, and Anxiety CAT scores. Conclusions Patients with higher levels of preoperative upper extremity disability experienced more improvement in the early postoperative period following LRTI. Baseline pain interference, depression, and anxiety were not associated with early outcomes. These findings may be useful when identifying patients who may experience the most functional improvement in the immediate postoperative period following LRTI. Type of study/level of evidence Prognostic IV.

Purpose Tacrolimus is a narrow therapeutic index drug, requiring consistent levels to maximize solid organ transplant success while reducing adverse effects. High tacrolimus trough level variability (TTLV) is a significant predictor of late rejection in both kidney and liver transplant. We sought to describe the prevalence of high TTLV in our pediatric heart transplant (HT) population, and identify whether this variability is associated with patient specific factors and poor outcomes. Methods Single center, retrospective study of HT patients from 1/2002 - 12/2018. Multi-organ recipients and retransplants were excluded. Records were reviewed for demographics, tacrolimus trough levels (TTLs) and outcomes (rejection, coronary vasculopathy, need for re-transplantation, death). Variability was calculated as the TTL standard deviation (SD) using each patient's TTLs from 1 year post-HT to last follow-up. High TTLV was defined as SD ≥3. Statistical analysis included Kruskal-Wallis, Chi-squared, and Wilcoxon rank sum testing. Results A total of 119 patients were included (male, 57%) with pre-HT diagnoses of congenital heart disease (57%) and cardiomyopathy (42%). The median age at HT was 2.7 yrs (IQR 0.8, 12.2) with a follow-up time post HT of 5.7 yrs (IQR 3.3, 9.9). A total of 6,344 TTLs were included, with a median of 33 TTLs/pt (IQR 14, 76). The median SD for the entire cohort was 3.1 (IQR 2.3, 4.0) with 55% of the population having a SD ≥3. Levels obtained from 1-5 yrs of age were found to have significantly greater variability (median SD 3.1 [IQR 2.4, 4.1]; p-value Conclusion TTLV is high in pediatric HT recipients, especially in those

Interstage weight gain after stage 1 palliation (S1P) has improved. However, the maximal negative impact on weight occurs before discharge, when catabolic demands are high and caloric intake is often limited. Change in weight for age z-score (ΔWAZ) in the immediate post-operative period has not

In adults with congenital heart disease, multiple studies have found high rates of anxiety and depression but there is sparse evidence regarding the effect of pregnancy on mental health. We aim to describe the rate of, and risk factors for, postpartum depression (PPD) in women with heart disease.

Introduction: Significant variability in morbidity and mortality persists for children with single ventricle congenital heart disease (SV-CHD) despite standardization in medical and surgical care. Multiple risk factors for poor outcomes have been described, but they only explain a fraction of the disparity in outcomes. The Developmental Origins of Health and Disease (DOHaD) theory proposes that fetal adaptation to an adverse in utero environment alters the ability to respond to post-natal stressors and may explain additional risk for poor outcomes. This study assessed maternal risk factor association with disparate outcomes following SV palliation. Hypothesis: Maternal factors related to weight, diabetes, hypertension, and smoking may be associated with an increased risk of poor outcomes in children with SV-CHD. Methods: This retrospective observational cohort study included all term maternal-infant pairs with a diagnosis of SV-CHD who underwent surgical palliation from 2006-2015 at Primary Children’s Hospital. Pairs lacking maternal variables of interest or infant follow-up data up to 1 year were excluded. The association of death or transplant with maternal risk factors [abnormal pre-pregnancy BMI, abnormal weight gain during pregnancy (≤ 7 kg or ≥ 20 kg), any type of hypertension or diabetes and smoking] was analyzed using a multivariable logistic regression model. Results: Of 190 infants who qualified, 135 (71%) maternal-infant dyads had complete data for inclusion. Systemic right ventricular morphology was present in 69% and 31% had systemic left ventricular morphology. Death or transplant occurred in 48 infants (36%) during an average follow-up of 2.2 years (0.1-11.7 years). Abnormal weight gain during pregnancy was present in 34 (25%) mothers, of which 24 had excessive (>20 kg) and 10 had inadequate ( Conclusions: Abnormal maternal weight gain (excessive or inadequate) during pregnancy is a novel risk factor that may contribute to worse outcomes in SV-CHD.

INTRODUCTION: Understanding the variation in costs of endoscopic sinus surgery (ESS) is critical to defining value. Current published costs of ESS have not identified potential sources of variation. Our objective was to analyze ESS costs to identify sources of variance that could guide value-improving decisions. METHODS: ESS cases (n=1,739) performed between 2008 and 2016 were identified from a database of 22 rural to tertiary facilities. Cost and time data were extracted from the database. Medical records were reviewed to confirm procedures. Three bilateral groupings were examined (n=895 cases from 13 facilities): 1) Full ESS (all sinuses); 2) Intermediate ESS (total ethmoid, maxillary); 3) Anterior ESS (anterior ethmoid, maxillary). Cost and operative time were analyzed using multivariable gamma regression. RESULTS: Median costs for Full, Intermediate, and Anterior ESS were $4,281, $3,716, and $2,549 (p

A total of 69 sows (DNA Line 200 × 400) and their progeny were used to determine if feeding a combination of vitamin D3 and 25(OH)D3 influences neonatal and sow vitamin D3 status. Within 3 d of breeding, sows were allotted to 1 of 3 dietary treatments fortified with 1,500 IU/kg vitamin D3 (CON), 500 IU/kg vitamin D3 + 25 μg/kg 25(OH)D3 (DL), or 1,500 IU/kg vitamin D3 + 50 μg/kg 25(OH)D3 (DH). There were treatment × time interactions for sow and pig serum metabolites (P < 0.001). Therefore, treatment means were compared within time period. At all time periods, sow serum 25(OH)D3 concentrations differed for all treatments with the magnitude of difference largest at weaning (P < 0.011), where serum 25(OH)D3 concentration was always the greatest when sows were fed the DH diet. At birth, piglets from DH fed sows had greater serum 25(OH)D3 concentrations than piglets from sows fed the DL treatment (P = 0.003), with piglets from sows fed CON treatment not differing from either (P > 0.061). At weaning, serum concentrations of 25(OH)D3 in piglets from all sow treatments were different (P < 0.001), with the greatest concentration in piglets from DH sows, followed by CON, and followed by DL. There were no treatment × time interactions for any of the metabolites measured in milk and no main effects for 24,25(OH)2D3 concentration (P > 0.068). Colostrum collected within 12 h of parturition contained less (P = 0.001) 25(OH)D3 than milk collected on d 21 of lactation. Regardless of time, concentrations of 25(OH)D3 in milk were different (P < 0.030), with the largest 25(OH)D3 concentration from DH fed sows, followed by DL and then CON. In conclusion, combining vitamin D3 and 25(OH)D3 in the maternal diet improves the vitamin D3 status of the dam and progeny.

Purpose There is variable practice and debate surrounding prophylactic heart failure (HF) therapy in Duchenne Muscular Dystrophy (DMD) patients with normal systolic function . We sought to determine the impact of HF medications including ACE inhibitors and ARB on the development of moderate systolic dysfunction or death in a cohort of DMD patients. Methods We performed a retrospective cohort study of DMD patients followed at 17 centers across North America from 1/1/2005-12/31/2015. Inclusion criteria were normal systolic function on baseline echocardiogram (ejection fraction (EF) ≥ 55%) and age ≥ 9 years during the study. Moderate systolic dysfunction was defined as an EF≤40% or shortening fraction ≤21% if EF was not available. We compared the composite outcome of moderate systolic dysfunction or death in patients who started HF therapies before evidence of systolic dysfunction (PPX) vs those who did not (non-PPX) using a Cox proportional hazards regression model adjusting for baseline age. We also compared prophylaxis with ACE/ARB (ACE/ARB PPX) to non-PPX. Results 277 males with DMD and normal systolic function were included. Prophylactic HF medications were used in 70 patients (25%) which included ACE/ARB (44, 63%), beta blocker (26, 37%), aldosterone blockade (6, 9%) and digoxin (5, 7%). Medications began at a mean (SD) age of 13.6 (4) years. Mean baseline ages were 10.7 (4.2) and 9.9 (3.9) years in the PPX and non-PPX groups (p=0.16), while mean age to develop moderate systolic dysfunction was 18.6 (5.4) and 16.8 (4.0) in the PPX and non-PPX groups (p=.005). The risk of developing moderate systolic dysfunction or death was 50% lower in the PPX group compared to the non-PPX group (HR=0.50; p=.044) and 79% lower in the ACE/ARB PPX group (N=38) compared to the non-PPX group (HR=0.21; p=0.035) (Figure 1). Conclusion There is variability in the use and choice of prophylactic HF medications in boys with DMD. This cohort showed a benefit to introduction of ACE/ARBs prior to evidence of systolic dysfunction.

Whole-genome prediction (WGP) models that use single-nucleotide polymorphism marker information to predict genetic merit of animals and plants typically assume homogeneous residual variance. However, variability is often heterogeneous across agricultural production systems and may subsequently bias WGP-based inferences. This study extends classical WGP models based on normality, heavy-tailed specifications and variable selection to explicitly account for environmentally-driven residual heteroskedasticity under a hierarchical Bayesian mixed-models framework. WGP models assuming homogeneous or heterogeneous residual variances were fitted to training data generated under simulation scenarios reflecting a gradient of increasing heteroskedasticity. Model fit was based on pseudo-Bayes factors and also on prediction accuracy of genomic breeding values computed on a validation data subset one generation removed from the simulated training dataset. Homogeneous vs. heterogeneous residual variance WGP models were also fitted to two quantitative traits, namely 45-min postmortem carcass temperature and loin muscle pH, recorded in a swine resource population dataset prescreened for high and mild residual heteroskedasticity, respectively. Fit of competing WGP models was compared using pseudo-Bayes factors. Predictive ability, defined as the correlation between predicted and observed phenotypes in validation sets of a five-fold cross-validation was also computed. Heteroskedastic error WGP models showed improved model fit and enhanced prediction accuracy compared to homoskedastic error WGP models although the magnitude of the improvement was small (less than two percentage points net gain in prediction accuracy). Nevertheless, accounting for residual heteroskedasticity did improve accuracy of selection, especially on individuals of extreme genetic merit.

Objective To assess intraocular pressure (IOP) in conditioned equine athletes and document changes with exercise. A secondary objective was to assess associations between IOP and heart rate, as well as with other subjective physical parameters. Sample population Horses were evaluated during 50 mile endurance ride competitions. Data were collected on 69 horses during five different competitions at three different locations with 59 horses ridden once, nine horses ridden in two competitions, and one horse ridden in three competitions for a total of 80 horse-ride combinations. Procedures Intraocular pressure was measured using a TonoVet® tonometer in both eyes of each horse prior to, at two time points during, and immediately after endurance competitions. Heart rates and subjective veterinary scores were recorded on ride cards at each time point. Results For horses with shorter finishing times (≤400 min), IOP decreased by at least 3.1 ± 0.9 mmHg (least square mean estimate ± SEM) from baseline to the end of endurance exercise (P

OBJECTIVE: To evaluate the association between neonatal neurobehavioral state and oral feeding outcomes following congenital heart disease (CHD) surgery. STUDY DESIGN: This single center retrospective cohort study described neonates undergoing cardiac surgery evaluated peri-operatively with the Neonatal Intensive Care Unit Network Neurobehavioral Scale (NNNS). We compared NNNS attention scores, which evaluates neonates’ ability to orient and fixate on stimuli, with the feeding outcomes percentage of feeds taken orally at discharge and time to reach full oral feeds using regression analyses. Models were constructed for both pre-operative and post-operative NNNS evaluations. RESULTS: Between August 2015 and October 2017, 124 neonates underwent 89 pre-operative and 97 post-operative NNNS evaluations. In multivariable Cox regression, higher pre-operative NNNS attention scores were associated with a shorter time to achieve full oral feeds (hazard ratio 1.4; 95% confidence interval: 1.0–2.0; P = .047). This relationship was not seen for post-operative NNNS attention scores or percentage of oral feeds at discharge. Depending on the model, younger age at surgery, increased ventilator days, increased length of stay, and single or two ventricle anatomy with aortic arch obstruction were associated with lower percentage of oral feeds at discharge and/or delay in full oral feeds. CONCLUSIONS: Higher neonatal attention before cardiac surgery is associated with improved feeding outcomes. Prospective assessment of neonatal neurobehavioral state may be a novel approach to predict and target interventions to improve feeding outcomes in CHD. Future studies should examine the impact of intrinsic neurodevelopmental delay versus environmental adaptation on the neurobehavioral state of neonates with CHD.

A total of 36 sows (DNA Line 200 × 400) and their progeny were used to determine if feeding a combination of vitamin D3 and 25(OH)D3 influences progeny muscle fiber morphometrics. Within 3 d of breeding, sows were allotted to 1 of 3 dietary treatments fortified with 1,500 IU/kg vitamin D3 (CON), 500 IU/kg vitamin D3 + 25 μg/kg 25(OH)D3 (DL), or 1,500 IU/kg vitamin D3 + 50 μg/kg 25(OH)D3 (DH). One average body weight male per litter was euthanized within 24 h of birth (n = 36) and at weaning on d 21 of lactation (n = 35). Data were analyzed as a completely randomized design with maternal dietary treatment as the fixed effect. When pigs were sacrificed at birth, there were no treatment effects for fiber morphometric measures (P > 0.170), except for primary fiber number and secondary to primary muscle fiber ratio (P < 0.016). Pigs from CON fed sows had fewer primary fibers than pigs from sows fed the DH treatment (P = 0.014), with the DL treatment not differing from either (P > 0.104). Pigs from CON and DL fed sows had greater secondary to primary fiber ratios compared to pigs from DH sows (P < 0.022), but did not differ from each other (P = 0.994). When pigs were sacrificed at weaning, there were no treatment effects on all fiber morphometric measures (P > 0.129). In conclusion, combining vitamin D3 and 25(OH)D3 in the maternal diet increased primary muscle fibers at birth, but this effect was not seen at weaning.

Purpose Current guidelines for the management of left ventricular systolic dysfunction (LVSD) in Duchenne Muscular Dystrophy (DMD) recommend angiotensin converting enzyme (ACE) inhibitors (class IIa). Little is known about the historic use of HF medications in Duchenne Muscular Dystrophy (DMD) and LVSD. Methods We performed a retrospective cohort study of DMD patients followed at 17 centers across North America from January 1, 2005-December 31, 2015. LVSD was defined as an ejection fraction (EF) Results LVSD was present in 209/436 (47.9%) DMD males including 84 (19.3%) patients with LVSD on baseline echocardiogram with median age 14 (IQR 11-17). 125 (28.7%) patients developed LVSD during the study with a median age at diagnosis of 15 years (IQR 12-17). Of the 209 patients with LVSD, 48 (23.0%) were never on HF medications, 105 (50.2%), 44 (21.0%), 11 (5.3%) and 1 (0.5%) were on single, dual, triple and quad HF therapy respectively. HF medications prescribed during the study period for those with LVSD included angiotensin converting enzyme inhibitor/angiotensin receptor blocker (ACE/ARB) (134, 64.1%), aldosterone antagonist (17, 8.1%), beta blocker (55, 26.3%), and digoxin (12, 5.7%). 154 (73.7%) were not on heart failure medications at study entry (baseline visit) and first line therapies included ACE/ARB (77, 51.0%), beta blockers (32, 21.2%), aldosterone antagonist (2, 1.3%), and digoxin (6, 4.0%). Overall therapies for these 154 patients included ACE/ARB (84, 55.6%), beta blocker (46, 30.5%), aldosterone antagonist (8, 5.3%) and digoxin (8, 5.3%). Conclusion There is significant practice variability that is not consistent with current consensus guidelines in the utilization of HF medications in DMD patients with LVSD. Almost 65% of the cohort were started on an ACE/ARB, however 23% were never prescribed a HF medication. Further studies are needed within the DMD population to understand the efficacy and decision making related to the prescription of common HF medications.

Quality improvement (QI) efforts in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) decreased interstage (hospital discharge post-stage 1 to stage 2 palliation) mortality in infants with hypoplastic left heart syndrome and variants from 9.5% to 5.3% starting in June

AHA guidelines (2004 and 2017) provided similar algorithms for diagnosing incomplete Kawasaki disease (KD). Our aims were to describe differences between subjects treated for incomplete KD where the algorithm was or was not adhered to and evaluate the influence of cardiology consultation on

Although common, the prevalence and impact of treatment on main pulmonary artery (MPA) dilation in Marfan syndrome (MFS) are unclear. We sought to determine the prevalence and progression of MPA dilation (Z score ≥2) and impact of treatment over 3 years using the public dataset from the Pediatric

Vector-borne virus diseases of wheat are recurrent in nature and pose significant threats to crop production worldwide. In the spring of 2011 and 2012, a state-wide sampling survey of multiple commercial field sites and university-managed Kansas Agricultural Experiment Station variety performance trial locations spanning all nine crop-reporting regions of the state was conducted to determine the occurrence of Barley yellow dwarf virus-PAV (BYDV-PAV), Cereal yellow dwarf virus-RPV, Wheat streak mosaic virus (WSMV), High plains virus, Soilborne wheat mosaic virus, and Wheat spindle streak mosaic virus using enzyme-linked immunosorbent assays (ELISA). As a means of directly coupling tiller infection status with tiller grain yield, multiple pairs of symptomatic and nonsymptomatic plants were selected and individual tillers were tagged for virus species and grain yield determination at the variety performance trial locations. BYDV-PAV and WSMV were the two most prevalent species across the state, often co-occurring within location. Of those BYDV-PAV- or WSMV-positive tillers, 22% and 19%, respectively, were nonsymptomatic, a finding that underscores the importance of sampling criteria to more accurately assess virus occurrence in winter wheat fields. Symptomatic tillers that tested positive for BYDV-PAV produced significantly lower grain yields compared with ELISA-negative tillers in both seasons, as did WSMV-positive tillers in 2012. Nonsymptomatic tillers that tested positive for either of the two viruses in 2011 produced significantly lower grain yields than tillers from nonsymptomatic, ELISA-negative plants, an indication that these tillers were physiologically compromised in the absence of virus-associated symptoms. Overall, the virus survey and tagged paired-tiller sampling strategy revealed effects of virus infection on grain yield of individual tillers of plants grown under field conditions and may provide a complementary approach toward future estimates of the impact of virus incidence on crop health in Kansas.

Objective—To compare efficacy and duration of effect on corneal sensitivity of 0.5% proparacaine hydrochloride, 0.5% bupivacaine hydrochloride, 2% lidocaine hydrochloride, and 2% mepivacaine hydrochloride solutions following ocular administration in clinically normal horses. Animals—68 clinically normal horses. Procedures—60 horses were assigned to receive 1 anesthetic agent in 1 eye. For each of another 8 horses, 1 eye was treated with each of the anesthetic agents in random order with a 1-week washout period between treatments. Corneal sensitivity was assessed via corneal touch threshold (CTT) measurements obtained with a Cochet-Bonnet aesthesiometer before and at 1 minute, at 5-minute intervals from 5 to 60 minutes, and at 10-minute intervals from 60 to 90 minutes after application of 0.2 mL of anesthetic agent. General linear mixed models were fitted to the CTT data from each of the 2 experimental groups to assess the effects of the anesthetic agents over time, accounting for repeated observations within individual horses. Results—Corneal sensitivity decreased immediately following topical application of each anesthetic agent; effects persisted for 35 minutes for proparacaine and mepivacaine treatments, 45 minutes for lidocaine treatment, and 60 minutes for bupivacaine treatment. Maximal CTT reduction was achieved following application of bupivacaine or proparacaine solution, whereas mepivacaine solution was least effective. Conclusions and Clinical Relevance—Ocular application of each evaluated anesthetic agent reduced corneal sensitivity in horses; although 0.5% proparacaine or 2% lidocaine solution appeared to induce adequate short-duration corneal anesthesia, use of 0.5% bupivacaine solution may be more appropriate for procedures requiring longer periods of corneal anesthesia.