Prenatal vitamin C and fish oil supplement use are associated with human milk microbiota composition in the Canadian CHILD Cohort Study.

Maternal diet may modulate human milk microbiota, but the effects of nutritional supplements are unknown. We examined the associations of prenatal diet and supplement use with milk microbiota composition. Mothers reported prenatal diet intake and supplement use using self-administered food frequency and standardised questionnaires, respectively.

Feasibility study of micronutrient status and body mass index of newly diagnosed pediatric oncology patients: Research commentary.

We conducted a feasibility study to evaluate micronutrients and body mass index (BMI). Fat soluble vitamins A, D, E and trace elements copper (Cu), selenium (Se), and zinc (Zn) levels were evaluated. Weight, height, BMI, and Z-scores were recorded.

High frequency jet ventilation for congenital diaphragmatic hernia.

Background: The optimal role of high frequency jet ventilation (HFJV) in lung protective stabilization of congenital diaphragmatic hernia (CDH) remains uncertain. We aimed to describe our center's experience with HFJV as both a rescue (following failed stabilization with CMV) and primary ventilation mode in the management of CDH.

Methods: Liveborn CDH patients treated from 2013 to 2021 in a single institution were reviewed.

Reporting of Social Determinants of Health in Pediatric Sepsis Studies.

Objective: Standardized, consistent reporting of social determinants of health (SDOH) in studies on children with sepsis would allow for: 1) understanding the association of SDOH with illness severity and outcomes, 2) comparing populations and extrapolating study results, and 3) identification of potentially modifiable socioeconomic factors for policy makers. We, therefore, sought to determine how frequently data on SDOH were reported, which factors were collected and how these factors were defined in studies of sepsis in children.

Data Sources And Selection: We reviewed 106 articles (published between 2005 and 2020) utilized in a recent systematic review on physiologic criteria for pediatric sepsis.

Factors to be Considered in Advancing Pediatric Critical Care Across the World.

This article reviews the many factors that have to be taken into account as we consider the advancement of pediatric critical care (PCC) in multiple settings across the world. The extent of PCC and the range of patients who are cared for in this environment are considered. Along with a review of the ongoing treatment and technology advances in the PCC setting, the structures and systems required to support these services are also considered.

Ductus arteriosus: more than just the patent ductus arteriosus.

The ductus arteriosus is important to fetal circulation. Failure to close at birth is a common event. In this educational pictorial essay, we illustrate the association of the ductus arteriosus with a variety of congenital cardiac, vascular and pulmonary lesions.

Development and validation of the Kids Disability Screen for children with juvenile idiopathic arthritis: results from the CAPRI Registry.

Objective: The aim of this study was to develop and validate a brief disability screen for children with JIA, the Kids Disability Screen (KDS).

Methods: A total of 216 children enrolled in the Canadian Alliance of Pediatric Rheumatology Investigators (CAPRI) Registry in 2017-2018 formed a development cohort, and 220 children enrolled in 2019-2020 formed a validation cohort. At every clinic visit, parents answered two questions derived from the Childhood Health Assessment Questionnaire (CHAQ): 'Is it hard for your child to run and play BECAUSE OF ARTHRITIS?' ('Hard' 0-10), and 'Does your child usually need help from you or another person BECAUSE OF ARTHRITIS?' ('Help', 0-10).

Optimizing skin antisepsis for neonatal surgery: A quality improvement initiative.

Purpose: Surgical site infections (SSIs) are a significant determinant of morbidity in the Neonatal Intensive Care Unit (NICU). Chlorhexidine gluconate/isopropyl alcohol (CHG-IPA) skin prep has demonstrated superiority over Povidone-Iodine (PI) in preventing SSIs in adults, however FDA labeling discourages CHG use in infants <2 months. This project aimed to i) create evidence for safe CHG skin antisepsis in neonates; and ii) evaluate the safety and effectiveness of CHG skin prep for neonatal surgery.

MALT1-Dependent Cleavage of HOIL1 Modulates Canonical NF-κB Signaling and Inflammatory Responsiveness.

Nuclear factor kappa B (NF-κB) is a critical transcription factor involved in regulating cell activation, inflammation, and survival. The linear ubiquitin chain assembly complex (LUBAC) which consists of HOIL1, HOIP, and SHARPIN, catalyzes the linear ubiquitination of target proteins-a post-translational modification that is essential for NF-κB activation. Human germline pathogenic variants that dysregulate linear ubiquitination and NF-κB signaling are associated with immunodeficiency and/or autoinflammation including dermatitis, recurrent fevers, systemic inflammation and enteropathy.

Risk Factors for Developing Posttransplant Diabetes After Pediatric Kidney Transplant in a Canadian Tertiary Care Children's Hospital Between 1995 and 2016.

Background: Posttransplant diabetes mellitus (PTDM) is a serious complication in kidney transplant recipients (KTRs) due to its negative impact on graft and patient survival. Although reported in 3% to 20% of pediatric KTRs, it has not been as well characterized in adults. In this study we describe incidence and risk factors associated with development of PTDM in pediatric KTRs.

NTRK2 Fusion driven pediatric glioblastoma: Identification of oncogenic Drivers via integrative Genome and transcriptome profiling.

This is the first report of a NACC2-NTRK2 fusion in a histological glioblastoma. Oncogenomic analysis revealed this actionable fusion oncogene in a pediatric cerebellar glioblastoma, which would not have been identified through routine diagnostics, demonstrating the value of clinical genome profiling in cancer care.

Potential overdiagnosis of long QT syndrome using exercise stress and QT stand testing in children and adolescents with a low probability of disease.

Background: Long QT syndrome (LQTS) is a dangerous arrhythmia disorder that often presents in childhood and adolescence. The exercise stress test (EST) and QT-stand test may unmask QT interval prolongation at key heart rate transition points in LQTS, but their utility in children is debated.

Objective: To determine if the QT-stand test or EST can differentiate children with a low probability of LQTS from those with confirmed LQTS.

Capsule Endoscopy Complements Magnetic Resonance Enterography and Endoscopy in Evaluating Small Bowel Crohn's Disease.

Aims: Wireless capsule endoscopy (WCE) and magnetic resonance enterography (MRE) are increasingly utilized to evaluate the small bowel (SB) in Crohn's disease (CD). The primary aims were to compare the ability of WCE and MRE to detect SB inflammation in children with newly diagnosed CD, and in the terminal ileum (TI) to compare them to ileo-colonoscopy. Secondary aims were to compare diagnostic accuracy of WCE and MRE and changes in Paris classification after each study.

Developing a Clinical Pathway for Somatic Symptom and Related Disorders in Pediatric Hospital Settings.

Somatic symptom and related disorders (SSRDs) are commonly encountered in pediatric hospital settings. There is, however, a lack of standardization of care across institutions for youth with these disorders. These patients are diagnostically and psychosocially complex, posing significant challenges for medical and behavioral health care providers.

Predicting Which Children with Juvenile Idiopathic Arthritis Will Not Attain Early Remission with Conventional Treatment: Results from the ReACCh-Out Cohort.

Objective: To estimate the probability of early remission with conventional treatment for each child with juvenile idiopathic arthritis (JIA). Children with a low chance of remission may be candidates for initial treatment with biologics or triple disease-modifying antirheumatic drugs (DMARD).

Methods: We used data from 1074 subjects in the Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) cohort.

Perioperative Hypotensive Crisis in an Adolescent with a Pancreatic VIPoma and MEN1-Gene Variant.

Background: Vasoactive intestinal peptide-secreting tumours (VIPomas) lead to high-volume secretory diarrhoea with hypokalaemia, as well as hyperglycaemia and hypercalcaemia. Diagnosis is often delayed.

Case Description: We present a 13-year-old girl with a distal pancreatic VIPoma diagnosed on her second hospital presentation who became severely hypotensive on anaesthetic induction prior to tumour removal, likely due to the vasodilatory effect of supraphysiological VIP levels.

Prospective Determination of the Incidence and Risk Factors of New-Onset Uveitis in Juvenile Idiopathic Arthritis: The Research in Arthritis in Canadian Children Emphasizing Outcomes Cohort.

Objective: Identification of the incidence of juvenile idiopathic arthritis (JIA)-associated uveitis and its risk factors is essential to optimize early detection. Data from the Research in Arthritis in Canadian Children Emphasizing Outcomes inception cohort were used to estimate the annual incidence of new-onset uveitis following JIA diagnosis and to identify associated risk factors.

Methods: Data were reported every 6 months for 2 years, then yearly to 5 years.

The Muc2 mucin coats murine Paneth cell granules and facilitates their content release and dispersion.

Paneth cells are a key subset of secretory epithelial cells found at the base of small intestinal crypts. Unlike intestinal goblet cells, which secrete the mucin Muc2, Paneth cells are best known for producing an array of antimicrobial factors. We unexpectedly identified Muc2 staining localized around Paneth cell granules.

Optimization of liposomal topotecan for use in treating neuroblastoma.

The purpose of this work was to develop an optimized liposomal formulation of topotecan for use in the treatment of patients with neuroblastoma. Drug exposure time studies were used to determine that topotecan (Hycamtin) exhibited great cytotoxic activity against SK-N-SH, IMR-32 and LAN-1 neuroblastoma human cell lines. Sphingomyelin (SM)/cholesterol (Chol) and 1,2-distearoyl-sn-glycero-3-phosphocholine (DSPC)/Chol liposomes were prepared using extrusion methods and then loaded with topotecan by pH gradient and copper-drug complexation.

Predicting Which Children with Juvenile Idiopathic Arthritis Will Have a Severe Disease Course: Results from the ReACCh-Out Cohort.

Objective: We studied an inception cohort of children with juvenile idiopathic arthritis (JIA) to (1) identify distinct disease courses based on changes over 5 years in 5 variables prioritized by patients, parents, and clinicians; and (2) estimate the probability of a severe disease course for each child at diagnosis.

Methods: Assessments of quality of life, pain, medication requirements, patient-reported side effects, and active joint counts were scheduled at 0, 6, 12, 18, 24, 36, 48, and 60 months. Patients who attended at least 6 assessments were included.

Outcomes of Radiofrequency Perforation for Pulmonary Atresia and Intact Ventricular Septum: A Single-Centre Experience.

Percutaneous radiofrequency perforation (RFP) of the pulmonary valve is used as a primary therapy in neonates with pulmonary atresia and intact ventricular septum (PAIVS). We sought to determine the safety and efficacy of RFP for PAIVS in a single center and assess the pre-intervention anatomical parameters associated with a biventricular outcome. We retrospectively reviewed all cases of PAIVS treated with RFP at a single center from 1999 through 2012.

Respiratory rate and pulse oximetry derived information as predictors of hospital admission in young children in Bangladesh: a prospective observational study.

Objective: Hypoxaemia is a strong predictor of mortality in children. Early detection of deteriorating condition is vital to timely intervention. We hypothesise that measures of pulse oximetry dynamics may identify children requiring hospitalisation.

Echocardiographic Tests of Left Ventricular Function in Pediatric Cardiology: Are We Searching for the Holy Grail?

In this article the utility of echocardiographic tests of left ventricular (LV) function in pediatric cardiology is reviewed. These indices are derived from the basic concepts of cardiac physiology, namely the Frank-Starling curve, pressure volume loops, and the force frequency relation and, to some extent, are all governed by these general principles. Thus, they are prone to be load-dependent and their utility variable.