Thrombotic Disorders in the Newborn.

The coagulation and thrombotic systems of an infant are fundamentally different from those of adults and older children. Hemostatic factors have inherently lower circulation levels in infants and are also affected prenatally by conditions of pregnancy. The unique physiology of neonates can contribute to a procoagulant state, which can result in a high level of morbidity and mortality.

Disorders of Coagulation in the Newborn.

The coagulation system in newborns varies from that of children and adults, with many circulating hemostatic factors being lower in the newborn. Infants are also susceptible to diseases and conditions in the pregnant person affecting their coagulation system, which can make it difficult to rapidly identify the cause behind coagulopathy in a neonate. Coagulation disorders can result in high levels of infant morbidity and mortality, which makes early diagnosis and prompt treatment critical.

Variability of Care Practices for Extremely Early Deliveries.

Objectives: Assess temporal changes, intercenter variability, and birthing person (BP) factors relating to interventions for extremely early deliveries.

Methods: Retrospective study of BPs and newborns delivered from 22-24 completed weeks at 13 US centers from 2011-2020. Rates of neonatology consultation, antenatal corticosteroids, cesarean delivery, live birth, attempted resuscitation (AR), and survival were assessed by epoch, center, and gestational age.

The Landscape of Resource Utilization After Resuscitation of 22-, 23-, and 24-Weeks' Gestation Infants.

Objective: To compare estimated healthcare resources needed to care for 22 through 24 weeks' gestation infants.

Study Design: This multicenter, retrospective cohort study included 1505 live in-born and out-born infants 22 through 24 weeks' gestational age at delivery from 6 pediatric tertiary care hospitals from 2011 through 2020. Median neonatal intensive care unit (NICU) length of stay (LOS) for each gestational age was used as a proxy for hospital resource utilization, and the number of comorbidities and medical technology use for each infant were used as estimates of future medical care needs.

Bulk T-cell receptor sequencing confirms clonality in pediatric eosinophilic esophagitis and identifies a food-specific repertoire.

Background: Eosinophilic esophagitis (EoE) involves a chronic immune-mediated response to dietary antigens. Recent work identifies T-cell clonality in children with EoE, however, it is unknown whether this is true in adults or whether there is a restricted food-specific T-cell repertoire. We sought to confirm T-cell receptor (TCR) clonality in EoE and assess for differences with specific food triggers.

When in Doubt, Cut It Out: Biopsy as Key in Diagnosing Cryptococcal Soft Tissue Infection.

Soft tissue infection is an uncommon presentation of in the absence of immunosuppression. Most infected patients present with pneumonia or meningitis, often with signs of disseminated disease, which may be fatal. We present a case of an 81-year-old mildly immunocompromised woman with multiple comorbidities, who presented with an extensive soft tissue infection on her right medial thigh.

Mast Cell Activation in the Systemic Sclerosis Esophagus.

Introduction: Previously, we discovered similar esophageal gene expression patterns in patients with systemic sclerosis (SSc) and eosinophilic esophagitis (EoE) where eosinophil/mast cell-targeted therapies are beneficial. Because SSc and EoE patients experience similar esophageal symptoms, we hypothesized that eosinophil/mast cell-directed therapy may potentially benefit SSc patients. Herein, we determine the association between esophageal mast cell quantities, gene expression and clinical parameters in order to identify SSc patients who may benefit from eosinophil/mast cell-directed therapy.

Transcriptional profiling of pediatric cholestatic livers identifies three distinct macrophage populations.

Background & Aims: Limited understanding of the role for specific macrophage subsets in the pathogenesis of cholestatic liver injury is a barrier to advancing medical therapy. Macrophages have previously been implicated in both the mal-adaptive and protective responses in obstructive cholestasis. Recently two macrophage subsets were identified in non-diseased human liver; however, no studies to date fully define the heterogeneous macrophage subsets during the pathogenesis of cholestasis.

Activated CD8 T-cell Hepatitis in Children With Indeterminate Acute Liver Failure: Results From a Multicenter Cohort.

Objectives: In many pediatric acute liver failure (PALF) cases, a diagnosis is not identified, and the etiology is indeterminate (IND-PALF). Our pilot study found dense CD8 T-cell infiltrates and increased T-cell clonality in liver specimens from IND-PALF patients. We aimed to validate these findings in a multicenter cohort with investigators blinded to diagnosis.

αT-catenin: A developmentally dispensable, disease-linked member of the α-catenin family.

α-Catenins are actin-filament binding proteins and critical subunits of the cadherin-catenin cell-cell adhesive complex. They are found in nominally-defined epithelial (E), neural (N), and testis (T) forms transcribed from three distinct genes. While most of α-catenin research has focused on the developmentally essential founding member, αE-catenin, this review discusses recent studies on αT-catenin (CTNNA3), a developmentally dispensable isoform that is emerging as relevant to cardiac, allergic and neurological diseases.