The New York pilot newborn screening program for lysosomal storage diseases: Report of the First 65,000 Infants.

Purpose: We conducted a consented pilot newborn screening (NBS) for Pompe, Gaucher, Niemann-Pick A/B, Fabry, and MPS 1 to assess the suitability of these lysosomal storage disorders (LSDs) for public health mandated screening.

Methods: At five participating high-birth rate, ethnically diverse New York City hospitals, recruiters discussed the study with postpartum parents and documented verbal consent. Screening on consented samples was performed using multiplexed tandem mass spectrometry.

Is the best interest standard good for pediatrics?

We argue that the "best interest standard" for surrogate decision-making is problematic for a number of reasons. First, reliance on the best interest standard is subjective. Second, it leads to behavior that is intolerant and polarizing.

Importance of birth weight as a risk factor for severe retinopathy of prematurity when gestational age is 30 or more weeks.

Purpose: To determine whether birth weight less than 1500 g is a relevant guideline indicating the need for examination for retinopathy of prematurity (ROP) when gestational age at birth is 30 or more completed weeks.

Design: A retrospective observational cohort study.

Methods: A total of 266 infants in a single institutional neonatal intensive care unit (NICU), whose gestational age at birth was 30 or more weeks but whose birth weight was less than 1500 g, were examined according to published guidelines.

Group B Streptococcus exposure and self-limited respiratory distress in late preterm and term neonates.

Background: Self-limited respiratory distress is a common neonatal respiratory morbidity for which effective treatments are lacking. Supportive care with non-invasive respiratory support is the norm. Animal models suggest that intrapartum exposure to group B Streptococcus (GBS) may cause mild pulmonary hypertension in the neonate, resulting in self-resolving respiratory distress.

Distribution dynamics of recombinant Lactobacillus in the gastrointestinal tract of neonatal rats.

One approach to deliver therapeutic agents, especially proteins, to the gastro-intestinal (GI) tract is to use commensal bacteria as a carrier. Genus Lactobacillus is an attractive candidate for use in this approach. However, a system for expressing exogenous proteins at a high level has been lacking in Lactobacillus.

Antibiotic use in newborns with transient tachypnea of the newborn.

Background: Initiation of empiric antibiotic treatment for possible early-onset sepsis is recommended for late preterm and term neonates with respiratory distress. There is no evidence base to this approach.

Objectives: To determine the incidence of adverse infectious events in neonates with transient tachypnea of the newborn (TTN) managed with a risk-factor-based restrictive antibiotic use policy.

Endotracheal tube: friend or foe? Bacteria, the endotracheal tube, and the impact of colonization and infection.

The microbiology of the endotracheal tube culture plays a role in diagnosing a variety of diseases in the newborn intensive care unit, including subglottic stenosis, bronchopulmonary dysplasia, and ventilator-associated pneumonia. Bacterial production of a biofilm that coats the endotracheal tube acts as a reservoir for infection, prevents eradication, and may play a role in the development of subglottic stenosis. The diagnosis of ventilator-associated pneumonia is limited by the CDC definition as well as currently available diagnostic methods.

Perinatal endocrinology: common endocrine disorders in the sick and premature newborn.

Endocrine disorders are common in infants in the neonatal ICU. They often are associated with prematurity, low birth weight or very low birth weight, and small size for gestational age. They also frequently occur in infants who are critically ill or stressed.

Randomized controlled trial of restrictive fluid management in transient tachypnea of the newborn.

Objective: To determine the effect of mild fluid restriction on the hospital course of neonates with transient tachypnea of the newborn (TTN).

Study Design: In this pilot prospective randomized controlled trial of 64 late preterm and term neonates diagnosed with TTN at a single tertiary care hospital in the United States, patients were randomized to receive standard fluid management or mild fluid restriction. The primary outcome was duration of respiratory support.

Statewide NICU central-line-associated bloodstream infection rates decline after bundles and checklists.

Objective: In 2008, all 18 regional referral NICUs in New York state adopted central-line insertion and maintenance bundles and agreed to use checklists to monitor maintenance-bundle adherence and report checklist use. We sought to confirm whether adopting standardized bundles and using central-line maintenance checklists reduced central-line-associated bloodstream infections (CLABSI).

Methods: This was a prospective cohort study that enrolled all neonates with a central line who were hospitalized in any of 18 NICUs.

Perinatal endocrinology: common endocrine disorders in the sick and premature newborn.

Endocrine disorders are common in infants in the neonatal ICU. They often are associated with prematurity, low birth weight or very low birth weight, and small size for gestational age. They also frequently occur in infants who are critically ill or stressed.

Butyrate enhances the intestinal barrier by facilitating tight junction assembly via activation of AMP-activated protein kinase in Caco-2 cell monolayers.

Butyrate, one of the SCFA, promotes the development of the intestinal barrier. However, the molecular mechanisms underlying the butyrate regulation of the intestinal barrier are unknown. To test the hypothesis that the effect of butyrate on the intestinal barrier is mediated by the regulation of the assembly of tight junctions involving the activation of the AMP-activated protein kinase (AMPK), we determined the effect of butyrate on the intestinal barrier by measuring the transepithelial electrical resistance (TER) and inulin permeability in a Caco-2 cell monolayer model.

Multiple factors related to bottle-feeding performance in preterm infants.

Background: A great deal of attention has focused on understanding preterm infant feeding behaviors and on strategies to support the preterm infant during this period; however, comprehensive descriptions of the feeding behavior of preterm infants that incorporate an examination of multiple subsystem levels are lacking.

Objective: To examine various physical indicators related to preterm infants' bottle-feeding performance.

Methods: This was a retrospective, descriptive, exploratory study using a convenience sample.

Bottle-feeding behaviors in preterm infants with and without bronchopulmonary dysplasia.

Objective: This study compared bottle-feeding behaviors in preterm infants with and without bronchopulmonary dysplasia (BPD) during the initial hospitalization.

Method: Individual sucking characteristics and feeding transitional rates were compared in 41 preterm infants (22 boys, 19 girls) with BPD and 99 infants (44 boys, 55 girls) without BPD. Observations of the first bottle feeding and observations of the last feeding before discharge were obtained from medical records of all infants retrospectively.

Late rise of thyroid stimulating hormone in ill newborns.

Objectives: To determine the frequency and characteristics of late rise of thyroid stimulating hormone (LRT) among ill newborns.

Infants And Methods: Data were retrospectively analyzed from infants in intensive care settings with abnormal thyroid tests over 13 months. Thyroid tests were performed by filter paper if neonatal intensive care >4 weeks or serum if clinically indicated.

Necrotising enterocolitis: the state of the science.

Necrotizing enterocolitis is the most common gastrointestinal emergency of the neonate, affecting 5-10% of infants, yet the pathogenesis remains unclear. Widely accepted risk factors include prematurity, enteral feeds, bacterial colonization and mucosal injury. How these or other yet identified factors come together to create the classic clinical and pathologic features is the subject of much research.

Effects of butyrate on intestinal barrier function in a Caco-2 cell monolayer model of intestinal barrier.

Production of short-chain fatty acids (SCFA) in the intestinal lumen may play an important role in the maintenance of the intestinal barrier. However, overproduction/accumulation of SCFA in the bowel may be toxic to the intestinal mucosa and has been hypothesized to play a role in the pathogenesis of neonatal necrotizing enterocolitis (NEC). By using a Caco-2 cell monolayer model of intestinal barrier, we report here that the effect of butyrate on the intestinal barrier is paradoxical.

Routine cervical length in twins and perinatal outcomes.

A retrospective review of twin gestations was undertaken to evaluate whether routine cervical lengths (CLs) in such instances change pregnancy outcome. Data were collected from the ultrasound database and chart review. Exclusion criteria included twins reduced to singletons, twins not delivering at our institution, and incomplete information.

Effect of labor epidural analgesia with and without fentanyl on infant breast-feeding: a prospective, randomized, double-blind study.

Background: The influence of labor epidural fentanyl on the neonate is controversial. The purpose of this study was to determine whether epidural fentanyl has an impact on breast-feeding.

Methods: Women who previously breast-fed a child and who requested labor epidural analgesia were randomly assigned in a double-blinded manner to one of three groups: (1) no fentanyl group, (2) intermediate-dose fentanyl group (intent to administer between 1 and 150 microg epidural fentanyl), or (3) high-dose epidural fentanyl group (intent to administer > 150 microg epidural fentanyl).

Short-chain fatty acid induces intestinal mucosal injury in newborn rats and down-regulates intestinal trefoil factor gene expression in vivo and in vitro.

Background: Luminal administration of short-chain fatty acids (SCFAs) induces dose-dependent intestinal mucosal injury in newborn rats. However, the mechanism underlying the injurious effects of SCFAs on intestinal mucosa in neonates is unclear. Intestinal trefoil factor (ITF) is a factor important for the maintenance and repair of the intestinal mucosal barrier.