Seizure Characteristics and EEG Features in Intoxication Type and Energy Deficiency Neurometabolic Disorders in the Pediatric Intensive Care Unit: Single-Center Experience Over 10 Years.

Background: Acute metabolic crises in inborn errors of metabolism (such as urea cycle disorders, organic acidemia, maple syrup urine disease, and mitochondrial disorders) are neurological emergencies requiring management in the pediatric intensive care unit (PICU). There is a paucity of data pertaining to electroencephalograms (EEG) characteristics in this cohort. We hypothesized that the incidence of background abnormalities and seizures in this cohort would be high.

Editorial: The Untapped Potential of Partial Hospitalization Programs.

The prevalence of mental health conditions in adolescents has been increasing worldwide. With the increasing demand, access to effective mental health care has struggled to keep pace. More adolescents are requiring intensive inpatient hospitalizations for high-risk conditions and often lack adequate resources for sub-acute care following discharge.

Carnitine supplementation increases serum concentrations of free carnitine and total acylcarnitine in preterm neonates: A retrospective cohort study.

Objective: Our goal was to determine the efficacy of the American Society for Parenteral and Enteral Nutrition's recommended carnitine dosage of 5 mg/kg/day in maintaining normal serum free carnitine and total acylcarnitine levels in preterm neonates receiving parenteral nutrition (PN).

Study Design: A retrospective cohort study was conducted on neonates born <30 weeks gestation and weighing <1250 g, comparing those who received carnitine supplementation to those without supplementation. Free carnitine and total acylcarnitine data were collected from routine newborn screens in the first days of life and on full enetral feeds.

Associations between sleep, obesity, and asthma in urban minority children.

Study Objectives: Although obesity, asthma, and sleep-disordered breathing are interrelated, there is limited understanding of the independent contributions of body-mass index and pulmonary function on polysomnography in children with asthma.

Methods: We conducted a retrospective chart review on 448 7- to 18-year-old children with asthma who had undergone polysomnography testing between 1/2007-12/2011 to elucidate the association between spirometry variables, body-mass index, and polysomnography parameters, adjusting for asthma and antiallergic medications.

Results: Obese children had poorer sleep architecture and more severe gas exchange abnormalities compared to healthy weight children.

A randomized control trial of a multiplex gastrointestinal PCR panel versus usual testing to assess antibiotics use for patients with infectious diarrhea in the emergency department.

Objective: This study analyzed physician treating behavior through the use of a multiplex gastrointestinal polymerase chain reaction (GI PCR) test compared with usual testing in emergency department (ED) patients with suspected acute infectious diarrhea to assess differences in antibiotic management.

Methods: A prospective, single-center, randomized control trial was designed to investigate antibiotic use in ED patients with moderate to severe suspected infectious diarrhea, comparing those who received GI PCR to those who received usual testing. ED patients with signs of dehydration, inflammation, or persistent symptoms were randomized to either the experimental arm (GI PCR) or the control arm (usual testing or no testing).

Association Between Baseline Use of Angiotensin-Converting Enzyme Inhibitors and Angiotensin Receptor Blockers and Death Among Patients Tested for COVID-19.

Angiotensin-converting enzyme inhibitor (ACEI) and angiotensin receptor blocker (ARB) drugs may modify risk associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Therefore, we assessed whether baseline therapy with ACEIs or ARBs was associated with lower mortality, respiratory failure (noninvasive ventilation or intubation), and renal failure (new renal replacement therapy) in SARS-CoV-2-positive patients. This retrospective registry-based observational cohort study used data from a national database of emergency department patients tested for SARS-CoV-2.

Getting Real: The Maryland Healthcare Ethics Committee Network's COVID-19 Working Group Debriefs Lessons Learned.

Responding to a major pandemic and planning for allocation of scarce resources (ASR) under crisis standards of care requires coordination and cooperation across federal, state and local governments in tandem with the larger societal infrastructure. Maryland remains one of the few states with no state-endorsed ASR plan, despite having a plan published in 2017 that was informed by public forums across the state. In this article, we review strengths and weaknesses of Maryland's response to COVID-19 and the role of the Maryland Healthcare Ethics Committee Network (MHECN) in bridging gaps in the state's response to prepare health care facilities for potential implementation of ASR plans.

BAP1 is a haploinsufficient tumor suppressor linking chronic pancreatitis to pancreatic cancer in mice.

Chronic pancreatitis represents a risk factor for the development of pancreatic cancer. We find that heterozygous loss of histone H2A lysine 119 deubiquitinase BAP1 (BRCA1 Associated Protein-1) associates with a history of chronic pancreatitis and occurs in 25% of pancreatic ductal adenocarcinomas and 40% of acinar cell carcinomas. Deletion or heterozygous loss of Bap1 in murine pancreata causes genomic instability, tissue damage, and pancreatitis with full penetrance.

Sedation, Analgesia, and Neuromuscular Blockade: An Assessment of Practices From 2009 to 2016 in a National Sample of 66,443 Pediatric Patients Cared for in the ICU.

Objectives: To describe the pharmaceutical management of sedation, analgesia, and neuromuscular blockade medications administered to children in ICUs.

Design: A retrospective analysis using data extracted from the national database Health Facts.

Setting: One hundred sixty-one ICUs in the United States with pediatric admissions.

Changes in microbiome diversity following beta-lactam antibiotic treatment are associated with therapeutic versus subtherapeutic antibiotic exposure in cystic fibrosis.

In persons with cystic fibrosis (CF), decreased airway microbial diversity is associated with lower lung function. Conflicting data exist on the impact of short-term antibiotics for treatment of acute pulmonary exacerbations. However, whether differences in antibiotic exposure impacts airway microbiome changes has not been studied.

Predicting CTCF-mediated chromatin interactions by integrating genomic and epigenomic features.

The CCCTC-binding zinc-finger protein (CTCF)-mediated network of long-range chromatin interactions is important for genome organization and function. Although this network has been considered largely invariant, we find that it exhibits extensive cell-type-specific interactions that contribute to cell identity. Here, we present Lollipop, a machine-learning framework, which predicts CTCF-mediated long-range interactions using genomic and epigenomic features.

Loss of KDM6A Activates Super-Enhancers to Induce Gender-Specific Squamous-like Pancreatic Cancer and Confers Sensitivity to BET Inhibitors.

KDM6A, an X chromosome-encoded histone demethylase and member of the COMPASS-like complex, is frequently mutated in a broad spectrum of malignancies and contributes to oncogenesis with poorly characterized mechanisms. We found that KDM6A loss induced squamous-like, metastatic pancreatic cancer selectively in females through deregulation of the COMPASS-like complex and aberrant activation of super-enhancers regulating ΔNp63, MYC, and RUNX3 oncogenes. This subtype of tumor developed in males had concomitant loss of UTY and KDM6A, suggesting overlapping roles, and points to largely demethylase independent tumor suppressor functions.

Microbial diversity within the airway microbiome in chronic pediatric lung diseases.

The study of the airway microbiome in children is an area of emerging research, especially in relation to the role microbial diversity may play in acute and chronic inflammation. Three such pediatric airway diseases include cystic fibrosis, asthma, and chronic lung disease of prematurity. In cystic fibrosis, the presence of Pseudomonas spp.

Different next generation sequencing platforms produce different microbial profiles and diversity in cystic fibrosis sputum.

Background: Cystic fibrosis (CF) is an autosomal recessive disease characterized by recurrent lung infections. Studies of the lung microbiome have shown an association between decreasing diversity and progressive disease. 454 pyrosequencing has frequently been used to study the lung microbiome in CF, but will no longer be supported.

Jan van der Noordaa (1934-2015); A Virologist Pur Sang.

Our loyal friend and colleague, Jan van der Noordaa, passed away unexpectedly at the age of 80 on the evening of 17 June 2015. [..

The ABCA1 domain responsible for interaction with HIV-1 Nef is conformational and not linear.

HIV-1 Nef is an accessory protein responsible for inactivation of a number of host cell proteins essential for anti-viral immune responses. In most cases, Nef binds to the target protein and directs it to a degradation pathway. Our previous studies demonstrated that Nef impairs activity of the cellular cholesterol transporter, ABCA1, and that Nef interacts with ABCA1.

Pharmacokinetics of Aminoglycosides in the Newborn.

Aminoglycosides have played a major role in antimicrobial therapy since their discovery in the 1940s. Their bactericidal efficacy in gram-negative infections, synergism with beta-lactam antibiotics, limited bacterial resistance, and low cost have given these agents a firm place in antimicrobial treatment. After penicillins, aminoglycosides are the most commonly used drugs in the neonatal intensive care unit.