The Safety and Efficacy of Transcatheter Pulmonary Valve Replacement Combined with Electrophysiology Procedures.

The objective of this study was to evaluate the safety and efficacy of combining transcatheter pulmonary valve replacement (TPVR) and electrophysiology (EP) procedures. A retrospective review was undertaken to identify TPVR and EP procedures that were concomitantly performed in the cardiac catheterization laboratory at University of Iowa Stead Family Children's Hospital from January 2011 to October 2019. Procedural and follow-up data were compared between patients who underwent TPVR and EP procedures in the same setting to those who received TPVR or EP procedure separately and that were similar in age and cardiac anatomy.

Association of Anemia with Neurodevelopmental Disorders in a Nationally Representative Sample of US Children.

Objective: To examine the associations of anemia with autism spectrum disorder (ASD), attention deficit hyperactivity disorder (ADHD), and learning disability in US children.

Study Design: We included children and adolescents aged 3-17 years from the National Health Interview Survey (NHIS), 1997-2018. Information about physician-diagnosed history of anemia, ASD, ADHD, and learning disability was reported by a parent or guardian.

Comparison of growth and feeding method in infants with and without genetic abnormalities after neonatal cardiac surgery.

Introduction: Congenital heart disease (CHD) is multifactorial in origin, resulting from an interaction between environmental and genetic factors. Multifactorial growth delay is common in infants with CHD. The impact of a genetic abnormality and CHD on the growth of an infant is lacking in the literature.

Coronavirus Disease 2019 (COVID-19) Associated With Febrile Status Epilepticus in a Child.

Infection associated with the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been named coronavirus disease 2019 (COVID-19). The emerging literature suggests that SARS-CoV-2 infection affects children of all age groups. COVID-19 as a cause of febrile seizures and status epilepticus is not yet reported in children.

Long-term Cognitive, Psychological, and Health Outcomes Associated With Child Abuse and Neglect.

Potential long-lasting adverse effects of child maltreatment have been widely reported, although little is known about the distinctive long-term impact of differing types of maltreatment. Our objective for this special article is to integrate findings from the Mater-University of Queensland Study of Pregnancy, a longitudinal prenatal cohort study spanning 2 decades. We compare and contrast the associations of specific types of maltreatment with long-term cognitive, psychological, addiction, sexual health, and physical health outcomes assessed in up to 5200 offspring at 14 and/or 21 years of age.

Cardiomyopathy in limb girdle muscular dystrophy R9, FKRP related.

Introduction: Reported frequencies of cardiomyopathy in limb girdle muscular dystrophy R9 (LGMDR9) vary. We describe the frequency and age at onset of cardiomyopathy in an LDMDR9 cohort.

Methods: Echocardiograms from 56 subjects (157 echocardiograms) with LGMDR9 were retrospectively reviewed.

Narrative review on potential role of gut microbiota in certain substance addiction.

As a neuropsychiatric disorder, substance addiction represents a major public health issue with high prevalence and mortality in many countries. Recently, gut microbiota has been certified to play a part in substance addiction through various mechanisms. Hence, we mainly focused on three substance including alcohol, cocaine and methamphetamine in this review, and summarized their relationships with gut microbiota, respectively.

Flipping syncope: The case of an adolescent athlete with syncopal episodes ultimately diagnosed with catecholaminergic polymorphic ventricular tachycardia.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a channelopathy which can lead to fatal ventricular arrhythmias. The diagnosis can be challenging due to a wide variety of clinical presentations. In this case, we describe the unusual presentation and subsequent workup of a young patient who was ultimately diagnosed with CPVT.

Wilson's Disease Masquerading as Juvenile Idiopathic Arthritis in an Adolescent.

Wilson's disease (WD) is a rare autosomal-recessive inborn error of copper metabolism characterized by the toxic accumulation of copper in liver, brain, cornea, and other tissues. It has a variable clinical presentation. Musculoskeletal presentations are very unusual.

Evaluation of Transcatheter Pulmonary Valve Endocarditis by Dual-Energy Computed Tomography.

Transcatheter pulmonary valve implantation (TPVI) is now an established alternative to surgery in patients with congenital heart disease and dysfunctional right ventricular outflow tract (RVOT) conduit. However, there is recognition of a higher incidence of infective endocarditis in the patients after TPVI. Transthoracic and transesophageal echocardiography is limited in the evaluation of prosthetic pulmonary valve endocarditis secondary to a metallic artifact and degenerative calcified conduit.

Survey of practices in relation to chronic pulmonary hypertension in neonates in the Canadian Neonatal Network and the National Institute of Child Health and Human Development Neonatal Research Network.

Current knowledge gaps pertaining to diagnosis and management of neonatal chronic pulmonary hypertension (cPH) may result in significant variability in clinical practice. The objective of the study is to understand cPH management practices in neonatal intensive care units affiliated with the Canadian Neonatal Network (CNN) and National Institute of Child Health and Human Development Neonatal Research Network (NRN). A 32-question survey seeking practice details for cPH evaluation, diagnostic criteria, conservative measures, pharmacotherapeutics, and follow-up was e-mailed to a designated physician at each center.

An international survey of pediatric and neonatal clinicians' vascular access practice: PediSIG assessment of vascular access, education, and support (PAVES) catheter selection.

Highlights: There is a wide variance in neonatal and pediatric vascular access workforce models, training, and competency assessments. Pain control during procedures is critical for children, yet it is not consistently used. Procedural support has shown improved patient outcomes, yet is not standardly used for every distressful procedure.

Surgical Management of Bilateral Vocal Fold Paralysis in Children: A Systematic Review and Meta-analysis.

Objectives: To examine and compare the outcomes of various types of glottic widening surgery (GWS) for initial management of bilateral vocal fold paralysis (BVFP) in children, the outcomes of different GWS procedures in children who underwent initial tracheostomy, and the rate of decannulation in children who underwent tracheostomy alone versus tracheostomy followed by GWS.

Data Sources: PubMed, Web of Science, Cochrane Library, and Embase were searched following the PRISMA guidelines (Preferred Reporting Items for Systematic Reviews and Meta-analyses) on September 9, 2019, with no date restriction.

Review Methods: Articles focusing on GWS or tracheostomy for initial management of BVFP were included.

The variability of SMARCA4-related Coffin-Siris syndrome: Do nonsense candidate variants add to milder phenotypes?

SMARCA4 encodes a central ATPase subunit in the BRG1-/BRM-associated factors (BAF) or polybromo-associated BAF (PBAF) complex in humans, which is responsible in part for chromatin remodeling and transcriptional regulation. Variants in this and other genes encoding BAF/PBAF complexes have been implicated in Coffin-Siris Syndrome, a multiple congenital anomaly syndrome classically characterized by learning and developmental differences, coarse facial features, hypertrichosis, and underdevelopment of the fifth digits/nails of the hands and feet. Individuals with SMARCA4 variants have been previously reported and appear to display a variable phenotype.

Molecular characterization of the calcium release channel deficiency syndrome.

We identified a potentially novel homozygous duplication involving the promoter region and exons 1-4 of the gene encoding type 2 cardiac ryanodine receptor (RYR2) that is responsible for highly penetrant, exertion-related sudden deaths/cardiac arrests in the Amish community without an overt phenotype to suggest RYR2-mediated catecholaminergic polymorphic ventricular tachycardia (CPVT). Homozygous RYR2 duplication (RYR2-DUP) induced pluripotent stem cell cardiomyocytes (iPSC-CMs) were generated from 2 unrelated patients. There was no difference in baseline Ca2+ handling measurements between WT-iPSC-CM and RYR2-DUP-iPSC-CM lines.

Multicenter Analysis of Truncal Valve Management and Outcomes in Children with Truncus Arteriosus.

Truncal valve management in patients with truncus arteriosus is a clinical challenge, and indications for truncal valve intervention have not been defined. We sought to evaluate truncal valve dysfunction and primary valve intervention in patients with truncus arteriosus and determine risk factors for later truncal valve intervention. We conducted a retrospective cohort study of children who underwent truncus arteriosus repair at 15 centers between 2009 and 2016.

Clinical evaluation of the Prismaflex™ HF 20 set and Prismaflex™ system 7.10 for acute continuous kidney replacement therapy (CKRT) in children.

Background: Continuous kidney replacement therapy (CKRT) is a common modality for treatment of severe acute kidney injury (AKI) in children. Adult technologies routinely utilized to provide this therapy have a large extracorporeal volume. The Prismaflex™ HF20 filter set has a relatively low extracorporeal blood volume of 60 mL, which provides technological benefit for smaller children compared with current filter sets available in the USA.

Effect on Patient Safety of a Resident Physician Schedule without 24-Hour Shifts.

Background: The effects on patient safety of eliminating extended-duration work shifts for resident physicians remain controversial.

Methods: We conducted a multicenter, cluster-randomized, crossover trial comparing two schedules for pediatric resident physicians during their intensive care unit (ICU) rotations: extended-duration work schedules that included shifts of 24 hours or more (control schedules) and schedules that eliminated extended shifts and cycled resident physicians through day and night shifts of 16 hours or less (intervention schedules). The primary outcome was serious medical errors made by resident physicians, assessed by intensive surveillance, including direct observation and chart review.

Parental attitudes and family helmet use for all-terrain vehicles and bicycles.

Background: Helmets prevent head trauma in both all-terrain vehicle (ATV) and bicycle crashes. This pilot study's objective was to compare family helmet use and participant attitudes regarding helmets for ATVs versus bicycles.

Methods: A convenience sampling of adults attending a 2017 university-sponsored health fair who had at least one child < 18 years living at home were surveyed.

Loss of ventricular preexcitation during noninvasive testing does not exclude high-risk accessory pathways: A multicenter study of WPW in children.

Background: Abrupt loss of ventricular preexcitation on noninvasive evaluation, or nonpersistent preexcitation, in Wolff-Parkinson-White syndrome (WPW) is thought to indicate a low risk of life-threatening events.

Objective: The purpose of this study was to compare accessory pathway (AP) characteristics and occurrences of sudden cardiac arrest (SCA) and rapidly conducted preexcited atrial fibrillation (RC-AF) in patients with nonpersistent and persistent preexcitation.

Methods: Patients 21 years or younger with WPW and invasive electrophysiology study (EPS) data, SCA, or RC-AF were identified from multicenter databases.

Pulmonary Hemosiderosis in a Child With Systemic Lupus Erythematosus: A Rare Presentation.

Pulmonary hemorrhage is an uncommon manifestation in children and is often associated with systemic lupus erythematosus. We report a case of an adolescent girl who presented to our hospital with recurrent episodes of fever, cough, and breathlessness. Later on, she was diagnosed with pulmonary hemosiderosis as a manifestation of systemic lupus erythematosus.

Creatinine filtration kinetics in critically Ill neonates.

Background: Creatinine values are unreliable within the first weeks of life; however, creatinine is used most commonly to assess kidney function. Controversy remains surrounding the time required for neonates to clear maternal creatinine.

Methods: Eligible infants had multiple creatinine lab values and were admitted to the neonatal intensive care unit (NICU).

Noggin regulates foregut progenitor cell programming, and misexpression leads to esophageal atresia.

Esophageal atresia (EA/TEF) is a common congenital abnormality present in 1 of 4000 births. Here we show that atretic esophagi lack Noggin (NOG) expression, resulting in immature esophagus that contains respiratory glands. Moreover, when using mouse esophageal organoid units (EOUs) or tracheal organoid units (TOUs) as a model of foregut development and differentiation in vitro, NOG determines whether foregut progenitors differentiate toward esophageal or tracheal epithelium.

Laboratory misdiagnosis of von Willebrand disease in post-menarchal females: A multi-center study.

Increased awareness of von Willebrand Disease (VWD) has led to more frequent diagnostic laboratory testing, which insurers often dictate be performed at a facility with off-site laboratory processing, instead of a coagulation facility with onsite processing. Off-site processing is more prone to preanalytical variables causing falsely low levels of von Willebrand Factor (VWF) due to the additional transport required. Our aim was to determine the percentage of discordance between off-site and onsite specimen processing for VWD in this multicenter, retrospective study.