Vein of Galen Aneurysmal Malformation Presenting As Persistent Pulmonary Hypertension in a Newborn: A Case Report.

We present a case of a newborn infant with a vein of Galen aneurysmal malformation (VOGM) who was admitted to our neonatal intensive care unit (NICU) with the diagnosis of persistent pulmonary hypertension of the newborn (PPHN). Further work-up at our institution, which included an echocardiogram and cranial ultrasound revealed VOGM. The patient was transferred to a children's center for further management of the vascular malformation where the patient subsequently died from high cardiac output heart failure.

Self-reported menses physiology is positively modulated by a well-formulated, energy-controlled ketogenic diet vs. low fat diet in women of reproductive age with overweight/obesity.

Weight loss can positively alter female physiology; however, whether dietary carbohydrate- or fat- restriction confer unique effects is less studied. Precisely designed, hypocaloric well-formulated ketogenic diets (KD; ~75% energy for weight maintenance) were compared to isocaloric/isonitrogenous low-fat diet (LFD) on self-reported menses in pre-menopausal overweight and obese women (mean ± SD: 34 ± 10 years, BMI: 32.3 ± 2.

Beneficial Effects of Exercise on Hypertension-Induced Cardiac Hypertrophy in Adolescents and Young Adults.

Purpose Of Review: Hypertension-induced cardiac hypertrophy is widely known as a major risk factor for increased cardiovascular morbidity and mortality. Although exercise is proven to exert overall beneficial effects on hypertension and hypertension-induced cardiac hypertrophy, there are some concerns among providers about potential adverse effects induced by intense exercise, especially in hypertensive athletes. We will overview the underlying mechanisms of physiological and pathological hypertrophy and delineate the beneficial effects of exercise in young people with hypertension and consequent hypertrophy.

The VirTra V-100 Is a Test-Retest Reliable Shooting Simulator for Measuring Accuracy/Precision, Decision-Making, and Reaction Time in Civilians, Police/SWAT, and Military Personnel.

Buga, A, Decker, DD, Robinson, BT, Crabtree, CD, Stoner, JT, Arce, LF, El-Shazly, X, Kackley, ML, Sapper, TN, Anders, JPV, Kraemer, WJ, and Volek, JS. The VirTra V-100 is a test-retest reliable shooting simulator for measuring accuracy/precision, decision-making, and reaction time in civilians, police/SWAT, and military personnel. J Strength Cond Res XX(X): 000-000, 2024-Law-enforcement agencies and the military have increasingly used virtual reality (VR) to augment job readiness.

Implementing Entrustable Professional Activities in Pediatric Fellowships: Facilitating the Process.

Background And Objectives: Entrustable professional activities (EPAs) will be used for initial certification by the American Board of Pediatrics by 2028. Less than half of pediatric fellowships currently use EPAs for assessment, yet all will need to adopt them. Our objectives were to identify facilitators and barriers to the implementation of EPAs to assess pediatric fellows and to determine fellowship program directors' (FPD) perceptions of EPAs and Milestones.

Feasibility and metabolic outcomes of a well-formulated ketogenic diet as an adjuvant therapeutic intervention for women with stage IV metastatic breast cancer: The Keto-CARE trial.

Purpose: Ketogenic diets may positively influence cancer through pleiotropic mechanisms, but only a few small and short-term studies have addressed feasibility and efficacy in cancer patients. The primary goals of this study were to evaluate the feasibility and the sustained metabolic effects of a personalized well-formulated ketogenic diet (WFKD) designed to achieve consistent blood beta-hydroxybutyrate (βHB) >0.5 mM in women diagnosed with stage IV metastatic breast cancer (MBC) undergoing chemotherapy.

Minimum Supervision Levels Required for Pediatric Cardiology Fellowship Graduation by Pediatric Cardiology Fellowship Program Directors.

Entrustable professional activities (EPAs) are "observable essential tasks expected to be performed by a physician for safe patient care in practice." Six Pediatric Cardiology (PC) EPAs and their level of supervision (LOS) scales were developed by medical educators in PC using a modified Delphi process and reviewed by the Subspecialty Pediatrics Investigator Network (SPIN). However, their general use in assessment for PC fellows for graduation requirements has yet to be studied.

Fasting and diurnal blood ketonemia and glycemia responses to a six-week, energy-controlled ketogenic diet, supplemented with racemic R/S-BHB salts.

Background: Single doses of exogenous ketone salts (KS) transiently increase circulating beta-hydroxybutyrate (BHB) (∼1 mM; 1-2 h) regardless of starting levels of ketosis; however, no studies have explored how sustained use of KS influences measures of ketonemia and glycemia.

Objectives: To determine the response to a hypocaloric, well-formulated ketogenic diet (KD), with and without the inclusion of two daily racemic KS doses (6 g R-BHB + 6 g S-BHB per serving) on 1) daily fasting capillary R-BHB and glucose (R-BHB/GLU), 2) bi-weekly 13 h diurnal BHB and glucose (R-BHB/GLU), 3) three-hours post-KS ingestion kinetics (R-BHB), and 4) bi-weekly fasting plasma enantiomer-specific BHB (R/S-BHB).

Methods: Non-diabetic adults with overweight and obesity were randomized to receive a precisely measured hypocaloric KD (∼75 %en of maintenance) for six weeks, supplemented twice-daily with KS or placebo (PL).

Metabolic and ruck performance effects of a novel, light-weight, energy-dense ketogenic bar.

New Findings: What is the central question of the study? Can a novel, energy-dense and lightweight ketogenic bar (1000 kcal) consumed 3 h before exercise modulate steady-state incline rucksack march ('ruck') performance compared to isocaloric carbohydrate bars in recreationally active, college-aged men? What is the main finding and its importance? Acute ingestion of either nutritional bar sustained ∼1 h of exhaustive rucking with a 30% of body weight rucksack. This proof-of-concept study is the first to demonstrate that carbohydrate bars and lipid bars are equally feasible for preserving ruck performance. Novel ketogenic nutrition bars may have military-relevant applications to lessen carry load without compromising exercise capacity.

Inherited intragenic PBX1 deletion: Expanding the phenotype.

PBX1 encodes the pre-B cell leukemia homeobox transcription factor, a three amino acid loop extension (TALE) homeodomain transcription factor, which forms nuclear complexes with other TALE class homeodomain proteins that ultimately regulate target genes controlling organ patterning during embryogenesis. Heterozygous de novo pathogenic variants in PBX1 resulting in haploinsufficiency are associated with congenital anomalies of the kidneys and urinary tract, most commonly renal hypoplasia, as well as anomalies involving the external ear, branchial arch, heart, and genitalia, and they cause intellectual disability and developmental delay. Affected individuals described thus far have had de novo variants.

Prominent Eustachian Valve and Atrial Septal Defect Presenting With Chronic Hypoxemia in a Teenager.

Prominent Eustachian valves, with obligate right-to-left shunts, have been reported as a cause of neonatal hypoxemia. This anomaly can present as an obstructive structure that inhibits antegrade flow through the tricuspid valve and furthermore contributes to right-to-left atrial shunting in the presence of a patent foramen ovale or atrial septal defect. This case highlights the evaluation and diagnostic workup for chronic hypoxemia in an adolescent female patient and considerations for percutaneous atrial septal defect closure.

Correlation of Echocardiogram and Exercise Test Data in Children with Aortic Stenosis.

Previous pediatric exercise test criteria for aortic stenosis severity were based on cardiac catheterization assessment, whereas current criteria are based on echocardiographic valve gradients. We sought to correlate exercise test criteria with echocardiographic assessment of severity. We report 65 studies, 51 patients (mean age of 13 ± 4 years; 75% males), with aortic stenosis (AS) who had a maximal exercise test between 2005 and 2016.

Identifying Subclinical Coronary Abnormalities and Silent Myocardial Ischemia After Arterial Switch Operation.

The incidence of late coronary complications is reported around 8% after arterial switch operation (ASO) for d-transposition of the great arteries, but the affected patients are usually asymptomatic. Exercise stress test (EST) and myocardial perfusion imaging (MPI) are common non-invasive modalities to screen for silent myocardial ischemia, but their diagnostic reliability in children after ASO is unclear. We retrospectively reviewed asymptomatic patients following ASO with EST, MPI, and coronary imaging studies (CIS) and examined the reliability of each test in identifying abnormal coronary lesions responsible for myocardial ischemia.

Standardization of a Continuous Ramp Ergometer Protocol for Clinical Exercise Testing in Children.

Developing a standardized protocol for pediatric exercise laboratories is challenging. Our objective was to report normal pediatric values for a continuous non-steady state cycle ergometer ramp protocol to achieve 8-10 min of exercise based on sex and weight. One hundred seventeen patients (117) [mean age 13 ± 2.

Coronary artery problems late after arterial switch operation for transposition of the great arteries.

Background: The incidence of late coronary artery abnormalities after arterial switch operation (ASO) for d-loop transposition of the great arteries may be underestimated.

Methods and results: We retrospectively reviewed coronary artery morphology in 40 of 97 patients who survived the first year after ASO. Seven asymptomatic patients developed significant late coronary artery abnormalities.

Trisomy 18 in a 20-year-old woman.

A 20-year-old female with trisomy 18 is described. Survival past infancy is rare in this disorder. Little is known concerning the factors that contribute to prolonged survival with this syndrome.