End-Organ Function and Exercise Performance in Patients With Fontan Circulation: What Characterizes the High Performers?

Background The physiologic hallmarks of the Fontan circulation-chronically elevated central venous pressures and low cardiac output-have significant effects not only on cardiovascular status but also impact other organ systems. Exercise capacity is limited in many and declines with age, accelerating in adolescence, but with wide variability. We explore the relationship between exercise performance and end-organ function in outpatient subjects with a Fontan circulation.

Frailty Is a Risk Factor for Postoperative Mortality in Patients With Cirrhosis Undergoing Diverse Major Surgeries.

With a rising burden of cirrhosis surgeries, understanding risk factors for postoperative mortality is more salient than ever. The role of baseline frailty has not been assessed in this context. We evaluated the association between patient frailty and postoperative risk among diverse patients with cirrhosis and determined if frailty improves prognostication of cirrhosis surgical risk scores.

Updates on pediatric sepsis.

Sepsis, defined as an infection with dysregulated host response leading to life-threatening organ dysfunction, continues to carry a high potential for morbidity and mortality in children. The recognition of sepsis in children in the emergency department (ED) can be challenging, related to the high prevalence of common febrile infections, poor specificity of discriminating features, and the capacity of children to compensate until advanced stages of shock. Sepsis outcomes are strongly dependent on the timeliness of recognition and treatment, which has led to the successful implementation of quality improvement programs, increasing the reliability of sepsis treatment in many US institutions.

Patients With Acute Ischemic Stroke Who Receive Brain Magnetic Resonance Imaging Demonstrate Favorable In-Hospital Outcomes.

Background Use of inpatient brain magnetic resonance imaging (MRI) in patients with acute ischemic stroke is highly institution dependent and has been associated with increased length and cost of hospital stay. We examined whether inpatient brain MRI in patients with acute ischemic stroke is associated with improved clinical outcomes to justify its resource requirements. Methods and Results The National Inpatient Sample database was queried retrospectively to find 94 003 patients who were admitted for acute ischemic stroke and then received inpatient brain MRI between 2012 and 2014.

The Predictive Role of Model for End-Stage Liver Disease-Lactate and Lactate Clearance for In-Hospital Mortality Among a National Cirrhosis Cohort.

The burden of cirrhosis hospitalizations is increasing. The admission Model for End-Stage Liver Disease-lactate (MELD-lactate) was recently demonstrated to be a superior predictor of in-hospital mortality compared with MELD in limited cohorts. We identified specific classes of hospitalizations where MELD-lactate may be especially useful and evaluated the predictive role of lactate clearance.

Biopsy Validated Study of Biomarkers for Liver Fibrosis and Transplant Prediction in Inherited Cholestasis.

Alagille syndrome (ALGS) and progressive familial intrahepatic cholestasis (PFIC) are inherited cholestatic disorders with risk of developing end-stage liver disease requiring liver transplantation (LT). We investigated aspartate aminotransferase-to-platelet ratio index (APRI), Fibrosis-4 score (FIB-4), and conjugated bilirubin as biomarkers to assess fibrosis severity and risk for LT among children with ALGS and PFIC. This multicenter, cross-sectional study included 64 children with ALGS or PFIC (per genetics or strict clinical criteria) with APRI, FIB-4, and conjugated bilirubin levels collected within ±90 days of their most recent liver biopsy.

Acute Hyperinsulinemia Alters Bone Turnover in Women and Men With Type 1 Diabetes.

Type 1 diabetes (T1D) increases fracture risk across the lifespan. The low bone turnover associated with T1D is thought to be related to glycemic control, but it is unclear whether peripheral hyperinsulinemia due to dependence on exogenous insulin has an independent effect on suppressing bone turnover. The purpose of this study was to test the bone turnover marker (BTM) response to acute hyperinsulinemia.

A Modified Grading System for Early Right Heart Failure Matches Functional Outcomes and Survival After Left Ventricular Assist Devices.

Early right heart failure (ERHF) remains a common complication after continuous-flow left ventricular assist device (cf-LVAD) and has been associated with increased mortality. The specific criteria used to define ERHF remain somewhat arbitrary. Correlating the degree of ERHF with outcomes after LVAD could inform a more clinically relevant definition.

Culturally-Appropriate Orientation Increases the Effectiveness of Mental Health First Aid Training for Bhutanese Refugees: Results from a Multi-state Program Evaluation.

Poor mental health remains a significant source of morbidity and mortality in the Bhutanese refugee community. Mental Health First Aid (MHFA) is a promising intervention that has been used in other immigrant communities to prepare individuals to recognize and respond to mental health warning signs. This was a non-randomized program evaluation.

Applying Educational Theory and Best Practices to Solve Common Challenges of Simulation-based Procedural Training in Emergency Medicine.

Objectives: Procedural competency is an essential prerequisite for the independent practice of emergency medicine. Multiple studies demonstrate that simulation-based procedural training (SBPT) is an effective method for acquiring and maintaining procedural competency and preferred over traditional paradigms ("see one, do one, teach one"). Although newer paradigms informing SBPT have emerged, educators often face circumstances that challenge and undermine their implementation.

Delineating -associated disease: From isolated neuropathy to early onset neurodegeneration.

Objective: To delineate the phenotypic and genotypic spectrum in carriers of mitochondrial mutations in a large international cohort.

Methods: We analyzed in detail the clinical, genetical, and neuroimaging data from 132 mutation carriers from national registries and local databases from Europe, USA, Japan, and China.

Results: We identified 113 clinically affected and 19 asymptomatic individuals with a known pathogenic mutation.

Serum Albumin Is a Marker of Myocardial Fibrosis, Adverse Pulsatile Aortic Hemodynamics, and Prognosis in Heart Failure With Preserved Ejection Fraction.

Background Data regarding the phenotypic correlates and prognostic value of albumin in heart failure with preserved ejection fraction (HFpEF) are scarce. The goal of the current study is to determine phenotypic correlates (myocardial hypertrophy, myocardial fibrosis, detailed pulsatile hemodynamics, and skeletal muscle mass) and prognostic implications of serum albumin in HFpEF. Methods and Results We studied 118 adults with HFpEF.

TGFβ and Hippo Pathways Cooperate to Enhance Sarcomagenesis and Metastasis through the Hyaluronan-Mediated Motility Receptor (HMMR).

High-grade sarcomas are metastatic and pose a serious threat to patient survival. Undifferentiated pleomorphic sarcoma (UPS) is a particularly dangerous and relatively common sarcoma subtype diagnosed in adults. UPS contains large quantities of extracellular matrix (ECM) including hyaluronic acid (HA), which is linked to metastatic potential.

Renal Metastasis From Neuroblastoma Shown on MIBG Imaging.

A 6-year-old girl with high-risk neuroblastoma underwent a I-MIBG scan to monitor the disease status. In addition to the known lesion in the right retroperitoneal region, there were additional foci of increased activity in the right upper quadrate of the abdomen, which were not typical bowel activity. SPECT/CT images located the activity in the cortex of the lower pole of the right kidney, which was partially calcified.

Total and trimester-specific gestational weight gain and infant anthropometric outcomes at birth and 6 months in low-income Hispanic families.

Objective: To describe total and trimester-specific gestational weight gain (GWG) among low-income Hispanic women and determine whether these GWG exposures are associated with infant anthropometric outcomes at birth and 6 months.

Study Design: Data were from 448 mother-infant pairs enrolled in the Starting Early child obesity prevention trial. Prenatal weights were used to calculate total GWG and 2 and 3 trimester GWG rates (kg/week) and categorized as inadequate, adequate, and excessive according to the 2009 Institute of Medicine recommendations.

Race/Ethnicity and Neighborhood Characteristics Are Associated With Bystander Cardiopulmonary Resuscitation in Pediatric Out-of-Hospital Cardiac Arrest in the United States: A Study From CARES.

Background Whether racial and neighborhood characteristics are associated with bystander cardiopulmonary resuscitation ( BCPR ) in pediatric out-of-hospital cardiac arrest ( OHCA ) is unknown. Methods and Results An analysis was conducted of CARES (Cardiac Arrest Registry to Enhance Survival) for pediatric nontraumatic OHCA s from 2013 to 2017. An index (range, 0-4) was created for each arrest based on neighborhood characteristics associated with low BCPR (>80% black; >10% unemployment; <80% high school; median income, <$50 000).

Altered ocular parameters from circadian clock gene disruptions.

The pathophysiology of refractive errors is poorly understood. Myopia (nearsightedness) in particular both blurs vision and predisposes the eye to many blinding diseases during adulthood. Based on past findings of diurnal variations in the dimensions of the eyes of humans and other vertebrates, altered diurnal rhythms of these ocular dimensions with experimentally induced myopia, and evolving evidence that ambient light exposures influence refractive development, we assessed whether disturbances in circadian signals might alter the refractive development of the eye.