The COVID-19 Pandemic and Pregnancy: Impact on Mothers and Newborns.

The severe acute respiratory syndrome coronavirus 2 pandemic has markedly, and likely permanently, changed health care. This includes changing the obstetric and perinatal care of mothers and infants, and by extension, the care of their families. Infection during pregnancy is associated with an increased risk for severe coronavirus disease 2019 illness and related complications that can significantly impact maternal health and the health of the neonate.

Patent Ductus Arteriosus Stent Versus Surgical Aortopulmonary Shunt for Initial Palliation of Cyanotic Congenital Heart Disease with Ductal-Dependent Pulmonary Blood Flow: A Systematic Review and Meta-Analysis.

Background In patients with ductal-dependent pulmonary blood flow, initial palliation includes catheter-based patent ductus arteriosus (PDA) stent or surgical aortopulmonary shunt (APS). This meta-analysis aimed to compare outcomes between PDA stent and APS. Methods and Results A comprehensive literature search yielded six retrospective observational studies.

Impact of esophageal mucosal permeability markers on provocation-induced esophageal reflexes in high-risk infants.

Esophageal distal baseline impedance (DBI) is an indicator of mucosal integrity; lower values suggest increased permeability. Aims were to characterize the (1) effect of DBI category (<900 Ω, 900-2000 Ω, and >2000 Ω) on sensory-motor characteristics of mid-esophageal provocation-induced motility reflexes, and (2) clinical outcomes among high-risk human infants evaluated for gastroesophageal reflux disease. Symptomatic infants (N = 49, 41 ± 3 weeks postmenstrual age) underwent pH-impedance testing to characterize acid reflux index (ARI) and DBI, and pharyngo-esophageal manometry to examine upper esophageal sphincter (UES), peristaltic, and lower esophageal sphincter (LES) functions.

Global resilience and new strategies needed for antimicrobial stewardship during the COVID-19 pandemic and beyond.

Resilience is having the ability to respond to adversity proactively and resourcefully. The coronavirus disease 2019 (COVID-19) pandemic's profound impact on antimicrobial stewardship programs (ASP) requires clinicians to call on their own resilience to manage the demands of the pandemic and the disruption of ASP activities. This article provides examples of ASP resilience from pharmacists and physicians from seven countries with different resources and approaches to ASP-The United States, The United Kingdom, Canada, Nigeria, Lebanon, South Africa, and Colombia.

Heart Failure and Patient-Reported Outcomes in Adults With Congenital Heart Disease from 15 Countries.

Background Heart failure (HF) is the leading cause of mortality and associated with significant morbidity in adults with congenital heart disease. We sought to assess the association between HF and patient-report outcomes in adults with congenital heart disease. Methods and Results As part of the APPROACH-IS (Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease-International Study), we collected data on HF status and patient-reported outcomes in 3959 patients from 15 countries across 5 continents.

Storming the castle: A case report of multi-system dysregulation in a child with Castleman disease.

Castleman disease is a non-clonal, lymphoproliferative disorder rarely seen in children. Presented is a 12-year-old male with progressive abdominal pain, vomiting, and fever. Diagnostic testing revealed multi-organ system involvement and the diagnosis was ultimately made with tissue biopsy.

BNT162b2 Protection against the Omicron Variant in Children and Adolescents.

Background: Spread of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) B.1.1.

Operational and Ethical Considerations for a National Adult Congenital Heart Disease Database.

As more adults survive with congenital heart disease, the need to better understand the long-term complications, and comorbid disease will become increasingly important. Improved care and survival into the early and late adult years for all patients equitably requires accurate, timely, and comprehensive data to support research and quality-based initiatives. National data collection in adult congenital heart disease will require a sound foundation emphasizing core ethical principles that acknowledge patient and clinician perspectives and promote national collaboration.

Safety and effectiveness of recombinant factor XIII-A in congenital factor XIII deficiency: Real-world evidence.

Background: Regular factor XIII (FXIII) prophylaxis is standard treatment for congenital FXIII A-subunit deficiency (FXIII-A CD). Recombinant factor XIII-A (rFXIII-A) was extensively evaluated in the mentor trials.

Objective: To assess real-world safety and treatment effectiveness of rFXIII-A prophylaxis from the mentor 6 trial.

Novel truncating variant in associated with cerebellar hypoplasia and velopharyngeal dysfunction.

-related neurodevelopmental disorder is a recently described intellectual disability syndrome often with speech difficulties. Here, we describe an individual with a heterozygous frameshift variant in (NM_182931.2:c.

Avoidance of surgery for head and neck infantile myofibromatosis using imatinib monotherapy.

Describe a novel use for a kinase inhibitor, imatinib, in young children with a known activated somatic mutation in PDGFR-beta. Two patients with infantile myofibromatosis treated with imatinib. Case description of evaluation, diagnosis and treatment decisions for infantile myfibromatosis of the head and neck.

Expanding the clinical phenotype of internal tandem duplication.

Closed spinal dysraphism (SD) is a type of neural tube defect originating during early embryonic development whereby the neural tissue of the spinal defect remains covered by skin, often coinciding with markers of cutaneous stigmata. It is hypothesized that these events are caused by multifactorial processes, including genetic and environmental causes. We present an infant with a unique congenital midline lesion associated with a closed SD.

Effectiveness of BNT162b2 Vaccine against Critical Covid-19 in Adolescents.

Background: The increasing incidence of pediatric hospitalizations associated with coronavirus disease 2019 (Covid-19) caused by the B.1.617.

KRIT1-positive hyperkeratotic cutaneous capillary venous malformation.

Cerebral cavernous malformations (CCM) may present in sporadic or familial forms, with different cutaneous manifestations including deep blue nodules, capillary malformations, and hyperkeratotic cutaneous capillary venous malformations (HCCVM). We report the case of an infant with a KRIT1-positive HCCVM associated with familial CCM. Moreover, histopathology showed positive immunohistochemical stain with GLUT1, further expanding the differential diagnosis of GLUT1-positive vascular anomalies.

Procedural Risk in Congenital Cardiac Catheterization (PREDICT).

Background Advancements in the field, including novel procedures and multiple interventions, require an updated approach to accurately assess patient risk. This study aims to modernize patient hemodynamic and procedural risk classification through the creation of risk assessment tools to be used in congenital cardiac catheterization. Methods and Results Data were collected for all cases performed at sites participating in the C3PO (Congenital Cardiac Catheterization Project on Outcomes) multicenter registry.

Early phase clinical studies of AR-42, a histone deacetylase inhibitor, for neurofibromatosis type 2-associated vestibular schwannomas and meningiomas.

Objectives: Two pilot studies of AR-42, a pan-histone deacetylase inhibitor, in human neurofibromatosis type 2 (NF2), vestibular schwannomas (VS), and meningiomas are presented. Primary endpoints included safety, and intra-tumoral pharmacokinetics (PK) and pharmacodynamics (PD).

Methods: Pilot 1 is a subset analysis of a phase 1 study of AR-42 in solid tumors, which included NF2 or sporadic meningiomas.

Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review.

Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management.

Sustainable generation of patient-led resources in a learning health system.

Background: Patient and Family Advisory Councils (PFACs) are an emerging mechanism to integrate patient and family voices into healthcare. One such PFAC is the Patient Advisory Council (PAC) of the ImproveCareNow (ICN) network, a learning health system dedicated to advancing the care of individuals with pediatric inflammatory bowel disease (IBD). Using quality improvement techniques and co-production, the PAC has made great strides in developing novel patient-led resources.

Invasive mechanical ventilation at 36 weeks post-menstrual age, adverse outcomes with a comparison of recent definitions of bronchopulmonary dysplasia.

Objectives: To determine whether the need for invasive mechanical ventilation (iMV) at 36 weeks PMA in patients with severe bronchopulmonary dysplasia (sBPD) identifies those patients at highest risk for tracheostomy or gastrostomy, and to compare sBPD with recent definitions of BPD.

Study Design: Observational study from Jan 2015 to Sept 2019 using data from the BPD Collaborative Registry.

Results: Five hundred and sixty-four patients with sBPD of whom 24% were on iMV at 36 weeks PMA.