Very early onset perinatal constipation: Can it be cow's milk protein allergy?

Delayed passage of meconium or constipation during the perinatal period is traditionally regarded as a signal to initiate further work up to evaluate for serious diagnoses such as Hirschsprung's disease (HD), meconium ileus due to Cystic Fibrosis, The diagnosis of HD particularly warrants invasive testing to confirm the diagnosis, such as anorectal manometry or rectal suction biopsy. What if there was another etiology of perinatal constipation, that is far lesser known? Cow's milk protein allergy (CMPA) is often diagnosed in infants within the first few weeks of life, however, there are studies that show that the CMPA allergen can be passed from mother to an infant in-utero, therefore allowing symptoms to show as early as day one of life. The presentation is more atypical, with perinatal constipation rather than with bloody stools, diarrhea, and vomiting.

[Neonatal hypoxic ischemic encephalopathy. Progress and new treatments according to the pathophysiological basis of the injury].

Neonatal hypoxic ischemic encephalopathy (HIE) is a type of injury caused by lack of oxygen in the brain during the neonatal period. It is a clinical syndrome clearly recognizable in term and premature newborns secondary to asphyxia at the time of delivery. HIE is estimated to occur at a frequency of 1-3 for each 1000 alive newborns per year in developed countries.

Progression of Enteral Feeding Volumes in Extremely Low Birth Weight Infants in the "Connection Trial".

Objective: Investigate daily feeding volumes and their association with clinical variables in the early postnatal care of premature infants of the "Connection Trial."

Study Design: A total of 641 infants of 510 to 1,000-g birth weight (BW, mean: 847 g) and mean 27 weeks' gestational age at birth (GA) were analyzed for total daily enteral (TDE) feeding volumes of 10, 20, 40, 80, and 120 mL/kg/d and their association with 24 clinical variables. Uni- and multivariable Cox regression models were used to calculate hazard ratios (HR) with 95% confidence intervals as a measure of the chance of reaching each of the TDE volumes.

Adeno-associated virus-mediated gene therapy in a patient with Canavan disease using dual routes of administration and immune modulation.

Gene replacement therapy is a rational therapeutic strategy and clinical intervention for neurodegenerative disorders like Canavan disease, a leukodystrophy caused by biallelic mutations in the aspartoacylase () gene. We aimed to investigate whether simultaneous intravenous (i.v.

External validation and biomarker assessment of a high-risk, data-driven pediatric sepsis phenotype characterized by persistent hypoxemia, encephalopathy, and shock.

Objective: Identification of children with sepsis-associated multiple organ dysfunction syndrome (MODS) at risk for poor outcomes remains a challenge. Data-driven phenotyping approaches that leverage electronic health record (EHR) data hold promise given the widespread availability of EHRs. We sought to externally validate the data-driven 'persistent hypoxemia, encephalopathy, and shock' (PHES) phenotype and determine its association with inflammatory and endothelial biomarkers, as well as biomarker-based pediatric risk-strata.

SERUM SOLUBLE ENDOGLIN IN PEDIATRIC SEPTIC SHOCK-ASSOCIATED MULTIPLE ORGAN DYSFUNCTION SYNDROME.

Background: Endothelial activation is a key driver of multiple organ dysfunction syndrome (MODS). Soluble endoglin (sENG) is expressed by mature and progenitor endothelial cells and thought to have angiogenic properties. We sought to determine the association between sENG and pediatric sepsis-associated MODS.

Engaging Adolescent Heart Transplant Recipients Through In-App Messaging During Mobile Health Intervention.

Adolescents heart transplant recipients experience difficulty with adherence to immunosuppressive medication leading to increased risk of organ rejection, hospitalization, and mortality. Few interventions have been successful to promote medication adherence in adolescent heart transplant patients as most fail to engage the patient in the behavioral change process and support patient-provider communication. The purpose of this study was to explore the nature and degree of in-app communication between adolescent heart transplant recipients and nursing staff during an asynchronous mobile video directly observed therapy intervention.

Revisiting post-ICU admission fluid balance across pediatric sepsis mortality risk strata: A secondary analyses from a prospective observational cohort study.

Introduction: Post-ICU admission cumulative positive fluid balance (PFB) is associated with increased mortality among critically ill patients. We sought to test whether this risk varied across biomarker-based risk strata upon adjusting for illness severity, presence of severe acute kidney injury (AKI), and use of renal replacement therapy (CRRT) in pediatric septic shock.

Design: Ongoing multi-center prospective observational cohort.

Impact of Low-Dose Computed Tomography and Physician Education on Referral for Head Shape.

Imaging before specialist evaluation of abnormal head shape is associated with a delay in evaluation and an increase in radiation exposure. A retrospective cohort study was performed to identify referral patterns before and after the implementation of a low-dose computed tomography (LDCT) protocol and physician education to examine the intervention's impact on time to evaluation and radiation exposure. Six hundred sixty-nine patients with an abnormal head shape diagnosis at a single academic medical center between July 1, 2014 and December 1, 2019 were reviewed.

Prognostic and predictive value of endothelial dysfunction biomarkers in sepsis-associated acute kidney injury: risk-stratified analysis from a prospective observational cohort of pediatric septic shock.

Background: Sepsis-associated acute kidney injury (SA-AKI) is associated with high morbidity, with no current therapies available beyond continuous renal replacement therapy (CRRT). Systemic inflammation and endothelial dysfunction are key drivers of SA-AKI. We sought to measure differences between endothelial dysfunction markers among children with and without SA-AKI, test whether this association varied across inflammatory biomarker-based risk strata, and develop prediction models to identify those at highest risk of SA-AKI.

Detrimental effects of PCSK9 loss-of-function in the pediatric host response to sepsis are mediated through independent influence on Angiopoietin-1.

Background: Sepsis is associated with significant mortality. Yet, there are no efficacious therapies beyond antibiotics. PCSK9 loss-of-function (LOF) and inhibition, through enhanced low-density lipoprotein receptor (LDLR) mediated endotoxin clearance, holds promise as a potential therapeutic approach among adults.

Prediction of Outcomes After Heart Transplantation in Pediatric Patients Using National Registry Data: Evaluation of Machine Learning Approaches.

Background: The prediction of posttransplant health outcomes for pediatric heart transplantation is critical for risk stratification and high-quality posttransplant care.

Objective: The purpose of this study was to examine the use of machine learning (ML) models to predict rejection and mortality for pediatric heart transplant recipients.

Methods: Various ML models were used to predict rejection and mortality at 1, 3, and 5 years after transplantation in pediatric heart transplant recipients using United Network for Organ Sharing data from 1987 to 2019.

Antibiotics Use and Its Effects on the Establishment of Feeding Tolerance in Preterm Neonates.

Objective: Antibiotics are one of the most widely used medications in today's neonatal intensive care units. Indiscriminate antibiotic usage persists in preterm newborns who are symptomatic due to factors linked to prematurity rather than being septic. Previous studies in older infants suggest that prior antibiotic administration is associated with possible dysmotility and microbial dysbiosis in the intestinal tract.

Parenteral medication considerations for the ketogenic diet.

Purpose: This initiative conducted a needs assessment regarding the extent of potential risk for accidental carbohydrate exposure in patients on the ketogenic diet in acute care settings at 2 academic medical centers.

Summary: Medications used in the emergency department, intensive care unit, or operating room can contain carbohydrates or be diluted in carbohydrate-containing fluids. Use of these medications can shift patients on the ketogenic diet out of ketosis, causing breakthrough seizures.

Transcatheter Leadless Pacing in Children: A PACES Collaborative Study in the Real-World Setting.

Background: Transcatheter Leadless Pacemakers (TLP) are a safe and effective option for adults with pacing indications. These devices may be an alternative in pediatric patients and patients with congenital heart disease for whom repeated sternotomies, thoracotomies, or transvenous systems are unfavorable. However, exemption of children from clinical trials has created uncertainty over the indications, efficacy, and safety of TLP in the pediatric population.

Iliopsoas haematoma during extracorporeal membrane oxygenation: A registry report from the COVID-19 critical care consortium across 30 countries.

Introduction: Iliopsoas haematoma (IPH) during extracorporeal membrane oxygenation (ECMO) is a rare bleeding complication that can be fatal due to its progression to abdominal compartment syndrome, but its incidence and risk factors are not well known. We have previously reported an IPH incidence rate of 16% in Japan. Among possible reasons for this high incidence, ethnicity has been hypothesised to play a role.

Detrimental effects of PCSK9 loss-of-function in the pediatric host response to sepsis are mediated through independent influence on Angiopoietin-1.

Sepsis is associated with significant mortality, yet there are no efficacious therapies beyond antibiotics and supportive care. In adult sepsis studies, PCSK9 loss-of-function (LOF) and inhibition has shown therapeutic promise, likely through enhanced low-density lipoprotein receptor (LDLR) mediated endotoxin clearance. In contrast, we previously demonstrated higher mortality in septic juvenile hosts with LOF.

Clinical spectrum and currently available treatment of type I interferonopathy Aicardi-Goutières syndrome.

Background: Aicardi-Goutières syndrome (AGS) is a genetically determined disorder with a variable phenotype. Since the original description of AGS, advances in gene sequencing techniques have resulted in a significant broadening of the phenotypic spectrum associated with AGS genes, and new clinical pictures have emerged beyond the classic presentation. The aim of this review is to provide a comprehensive analysis of the clinical spectrum of AGS and report currently available treatments and new immunosuppressive strategies.

Exploring Limited English Proficiency in the Clinical Outcomes of Pediatric Burn Patients.

Introduction: Limited English proficiency (LEP) is linked to lower health care access and worse clinical outcomes. This study aims to explore the potential role of LEP on clinical outcomes of pediatric burn patients.

Methods: We conducted a single-institution retrospective study of burn patients presenting at a tertiary pediatric burn referral program between January 2016 and December 2020.

An ELF4 hypomorphic variant results in NK cell deficiency.

NK cell deficiencies (NKD) are a type of primary immune deficiency in which the major immunologic abnormality affects NK cell number, maturity, or function. Since NK cells contribute to immune defense against virally infected cells, patients with NKD experience higher susceptibility to chronic, recurrent, and fatal viral infections. An individual with recurrent viral infections and mild hypogammaglobulinemia was identified to have an X-linked damaging variant in the transcription factor gene ELF4.

Considerations for Choice of Cranioplasty Material for Pediatric Patients.

Introduction: Optimal material and timing of cranioplasty in the pediatric population continue to be debated. Autologous and alloplastic materials have various indications for use and risk factors for complications.

Methods: A single-center retrospective cohort study was undertaken of all pediatric patients who underwent cranioplasty with any material from 1991-2021.

Extracorporeal membrane oxygenation as a bridge to transplant in neonates with fatal pulmonary conditions: A review.

Neonates with progressive respiratory failure should be referred early for subspecialty evaluation and lung transplantation consideration. ECMO should be considered for patients with severe cardiopulmonary dysfunction and a high likelihood of death while on maximal medical therapy, either in the setting of reversible medical conditions or while awaiting lung transplantation. While ECMO offers hope to neonates that experience clinical deterioration while awaiting transplant, the risks and benefits of this intervention should be considered on an individual basis.

Structural evaluation of child physical abuse in trauma: Social determinants of health at the population level.

Purpose: Child physical abuse (CPA) is closely linked to social factors like insurance status with limited evaluation at a structural population-level. This study evaluates the role of social determinants of health within the built environment on CPA.

Methods: A single-institution retrospective review of pediatric trauma patients was conducted between January 2016 and December 2020.