Contemporary Outcomes in Tetralogy of Fallot With Absent Pulmonary Valve After Fetal Diagnosis.

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10-year period were collected.

Simulation-Based Discharge Education Program for Caregivers of Children With Tracheostomies.

Objectives: To design, implement, and evaluate a simulation-based education (SBE) program for caregivers of children with tracheostomy.

Methods: Self-reported comfort and confidence in knowledge as well as tracheostomy care skills were assessed before and after a single SBE session for 24 consecutively enrolled caregivers of children with tracheostomies aged <21 years who were hospitalized at an academic medical center from August 2018 to September 2019 by using a survey and checklist, respectively. Mean individual and aggregated scores were compared by using a paired samples t-test, and association between instruments was determined with Spearman correlation.

Cyanosis in a patient after Fontan palliation due to unrecognised hepatic vein to coronary sinus communication.

A 6-year-old male with heterotaxia, abnormal systemic and pulmonary venous drainage, and a history of Fontan completion presented with desaturations and was found by cardiac catheterisation to have a hepatic vein to coronary sinus connection. This was successfully occluded using an Amplatzer Muscular Ventricular Septal Defect Occluder.

The Initial Impact of the Coronavirus Disease 2019 Pandemic on ICU Family Engagement: Lessons Learned From a Collaborative of 27 ICUs.

Objectives: To describe the impact of coronavirus disease 2019 on family engagement among ICUs participating in a multicenter collaborative promoting implementation of family-centered care projects and to report sites' experiences with the collaborative itself prior to its cancelation due to the pandemic in March 2020.

Design: Cross-sectional survey.

Setting: Twenty-seven academic and community ICUs in the United States and South Korea.

Polysomnography use in complex term and preterm infants to facilitate evaluation and management in the neonatal intensive care unit.

Study Objectives: (1) To determine the characteristics of term and preterm infants for whom polysomnography (PSG) was used as a primary diagnostic tool in infants with recurrent desaturation episodes, suspected obstructive apnea, or both, and the prevalence of abnormal studies. (2) To identify the interventions following PSGs. (3) To assess the added value of airway and swallow evaluations.

Reversible Coronary Artery Aneurysm With Delayed Anti-inflammatory Therapy in Multisystem Inflammatory Syndrome in Children.

A 4-year-old boy with multisystem inflammatory syndrome in children before widespread recognition of this disease developed complications, including coronary artery aneurysm, without anti-inflammatory treatment. With delayed treatment, all sequelae resolved. This case demonstrates a natural history supporting the role of anti-inflammatory treatment even with delayed or equivocal diagnosis.

The Deconditioning Effect of the COVID-19 Pandemic on Unaffected Healthy Children.

The COVID-19 pandemic has had devastating direct consequences on the health of affected patients. It has also had a significant impact on the ability of unaffected children to be physically active. We evaluated the effect of deconditioning from social distancing and school shutdowns implemented during the COVID-19 pandemic on the cardiovascular fitness of healthy unaffected children.

Tumor necrosis factor-alpha is associated with mineral bone disorder and growth impairment in children with chronic kidney disease.

Background: Mineral and bone disorder (MBD) and growth impairment are common complications of pediatric chronic kidney disease (CKD). Chronic inflammation detrimentally affects bone health and statural growth in non-CKD settings, but the impact of inflammation on CKD-MBD and growth in pediatric CKD remains poorly understood. This study assessed associations between inflammatory cytokines with biomarkers of CKD-MBD and statural growth in pediatric CKD.

Delayed presentation and sub-optimal outcomes of pediatric patients with acute appendicitis during the COVID-19 pandemic.

Objective: Early presentation and prompt diagnosis of acute appendicitis are necessary to prevent progression of disease leading to complicated appendicitis. We hypothesize that patients had a delayed presentation of acute appendicitis during the COVID-19 pandemic, which affected severity of disease on presentation and outcomes.

Patients And Methods: We conducted a retrospective review of all patients who were treated for acute appendicitis at Morgan Stanley Children's Hospital (MSCH) between March 1, 2020 and May 31, 2020 when the COVID-19 pandemic was at its peak in New York City (NYC).

Perinatal characteristics and delivery room management of infants born through MSAF.

Unlabelled: The immediate delivery room (DR) management of non-vigorous (NV) infants with meconium stained amniotic fluid (MSAF) is controversial. A recent ILCOR suggestion is not to perform routine direct laryngoscopy (DL) with or without tracheal suctioning in NV infants. Our practice is to perform DL and endotracheal (ET) suctioning in targeted NV infants.

Majority of females with a life-long experience of CAH and parents do not consider females with CAH to be intersex.

Introduction: To assess opinions of females with CAH, and parents of females with CAH, about designating this population "intersex," particularly in legislation about genital surgery during childhood.

Methods: We conducted a mixed-methods (quantitative and qualitative) anonymous cross-sectional online survey of females with CAH (46XX, 16+years old) and independently recruited parents of girls with CAH (2019-2020) diagnosed in first year of life from the United States. A multidisciplinary CAH team drafted the survey in collaboration with women with CAH and parents.

Achieving Country-Wide Scale for Helping Babies Breathe and Helping Babies Survive.

Helping Babies Breathe (HBB) was piloted in 2009 as a program targeted to reduce neonatal mortality (NM). The program has morphed into a suite of programs termed Helping Babies Survive that includes Essential Care for Every Baby. Since 2010, the HBB and Helping Babies Survive training programs have been taught to >850 000 providers in 80 countries.

Neonatal management and outcomes during the COVID-19 pandemic: an observation cohort study.

Background: The risk of vertical and perinatal transmission of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2, which causes COVID-19), the most appropriate management, and the neonate's risk of developing COVID-19 during the perinatal period are unknown. Therefore, we aimed to elucidate best practices regarding infection control in mother-newborn dyads, and identify potential risk factors associated with transmission.

Methods: In this observational cohort study, we identified all neonates born between March 22 and May 17, 2020, at three New York Presbyterian Hospitals in New York City (NY, USA) to mothers positive for SARS-CoV-2 at delivery.

Overview of the ChILD Research Network: A roadmap for progress and success in defining rare diseases.

Children's interstitial and diffuse lung diseases are a diverse group of rare lung disorders that present in childhood with diffuse pulmonary infiltrates and respiratory signs and symptoms. Children with these disorders face high morbidity and mortality and their families must cope with overwhelming uncertainty. Physicians caring for these patients are challenged by a paucity of directed therapies, or even understanding of natural history.

Registries, Risk Calculators, and Risk-Adjusted Outcomes: Current Usage, Limitations, and Future Prospects.

Small study sizes are a limiting factor in assessing outcome measures in pediatric cardiology. It is even more difficult to assess the outcomes of congenital catheterizations where the sample sizes are even smaller, particularly on a individual institutional level. The creation of multicenter registries is a method by which investigators can pool data to better assess quality and outcome measures of these procedures.

Is there a role for therapeutic hypothermia administration in term infants with mild neonatal encephalopathy?

Objective: Identifying term infants presenting with early mild neonatal encephalopathy (NE) as candidates for therapeutic hypothermia (TH) remains unclear. Study objectives were to characterize the neonatal clinical, magnetic resonance imaging (MRI), and longer-term outcome in infants with mild NE treated with TH.

Study Design: Retrospective cohort study of infants admitted with mild or moderate NE treated with TH.

Pediatric renal cell carcinoma.

Purpose Of Review: To review the presentation, natural history and treatment of renal cell carcinoma in children and young adults with renal cell carcinoma (RCC).

Recent Findings: Complete resection of lymph nodes at the time of tumor resection can improve clinical outcomes and limit the need for adjuvant chemotherapy. Genetic alterations that lead to translocation tumors are a therapeutic target of receptor tyrosine kinase inhibitors.

Robotic-Assisted Urologic Surgery in Infants: Positioning, Trocar Placement, and Physiological Considerations.

Pediatric robotic-assisted laparoscopic procedures are becoming increasingly common. They have been shown to be safe in younger patients, including infants. Successful adoption of robotic-assisted surgery in infants requires an understanding of the technical factors unique to this patient population.

Parent-Rated Severity of Illness and Anxiety among Caregivers of Children Born with a Disorder of Sex Development Including Ambiguous Genitalia.

Background/aims: Parents of children born with disorders of sex development (DSD) often experience anxiety, but risk factors, including parental perception of the severity of their child's DSD, have not been examined. We hypothesized that severity of illness (SOI) ratings would relate to parental anxiety, and would be higher for parents of children with a potentially life-threatening DSD (e.g.

Comparative Effectiveness of Levetiracetam vs Phenobarbital for Infantile Epilepsy.

Importance: More than half of infants with new-onset epilepsy have electroencephalographic and clinical features that do not conform to known electroclinical syndromes (ie, nonsyndromic epilepsy). Levetiracetam and phenobarbital are the most commonly prescribed medications for epilepsy in infants, but their comparative effectiveness is unknown.

Objective: To compare the effectiveness of levetiracetam vs phenobarbital for nonsyndromic infantile epilepsy.