Use of N-Acetylcysteine in Preterm Neonates with Enteral Feeding Intolerance and Intestinal Obstruction: A Case Series and Review of the Literature.

(1) Background: The use of N-acetylcysteine (NAC) to relieve meconium obstruction of prematurity in the first days of life has been reported, with NAC reducing the viscosity of luminal contents by cleaving the disulfide bonds of mucoproteins. However, its use in this population should be further explored since it has been associated with hypernatremia and transient increase in transaminases and bilirubin. (2) Methods: In this retrospective study, we included neonates admitted because of enteral feeding intolerance and intestinal obstruction from 2019 to 2021 who received NAC as a rescue therapy before explorative laparotomy.

A new variant in the GATA6 gene associated with tracheoesophageal fistula, pulmonary vein stenosis and neonatal diabetes.

Introduction: GATA6 is a gene that encodes a transcription factor with a key role in the development of several organ systems, including the development of the pancreas. It is associated with neonatal diabetes but also with other extra-pancreatic anomalies.

Case Presentation: This report describes the association of tracheoesophageal fistula (TEF), pulmonary vein stenosis (PVS), and neonatal diabetes caused by a novel mutation of the GATA6 gene in a small-for-gestational-age male neonate born at 32 weeks of gestation.

Use of levosimendan in hemodynamic management of heart failure in two neonates with intracranial arteriovenous shunts: a case series.

Background: The hemodynamic status of newborns with intracranial arteriovenous shunts (AVSs) may be extremely complex. Mini-invasive hemodynamic monitoring through innovative techniques such as Near-Infrared Spectroscopy (NIRS) and Pressure Recording Analytical Method (PRAM) may help in understanding hemodynamics in newborns with AVSs. Levosimendan is a calcium sensitizer and inodilator, and it is known to improve ventricular function, but its use in newborns is limited.

Early vasopressin infusion improves oxygenation in infants with congenital diaphragmatic hernia.

Objective: Congenital Diaphragmatic Hernia (CDH) is a complex disease including a diaphragmatic defect, lung hypoplasia, and pulmonary hypertension. Despite its increasing use in neonates, the literature on the use of vasopressin in neonates is limited. The aim of this work is to analyze the changes in clinical and hemodynamic variables in a cohort of CDH infants treated with vasopressin.

Hemodynamic Assessment of a Large Pulmonary Arteriovenous Malformation in a Neonate: Case Report and Review of Literature.

Herein we report the case of a neonate with a prenatally diagnosed large pulmonary arteriovenous malformation, managed with minimally invasive hemodynamic monitoring in our Neonatal Intensive Care Unit. The combination of Near-Infrared Spectroscopy and Pressure Recording Analytical Method could guide neonatal management of critical cases of vascular anomalies: immediate data are offered to clinicians, from which therapeutic decisions such as timing of surgical resection are made to achieve a positive outcome. We also systemically collected and summarized information on patients' characteristics of previous cases reported in literature to data, and we compared them to our case.

Changes in Breathing Patterns after Surgery in Severe Laryngomalacia.

Background: Most of the studies regarding the surgical treatment of severe laryngomalacia (LM) have been aimed at describing the efficacy of the treatment in terms of improvement of clinical symptoms or anatomical findings. There are no studies specifically aimed at analyzing the changes in breathing patterns following surgical treatment for severe LM.

Objective: To review the breathing pattern changes before and after corrective surgery in infants with severe LM.

Growth and morbidity in infants with Congenital Diaphragmatic Hernia according to initial lung volume: A pilot study.

Background In congenital diaphragmatic hernia (CDH) survivors, failure to thrive is a well-known complication, ascribed to several factors. The impact of lung volume on growth of CDH survivors is poorly explored. Our aim was to evaluate if, in CDH survivors, lung volume (LV) after extubation correlates with growth at 12 and 24 months of life.

The Role of Lung Function Testing in Newborn Infants With Congenital Thoracic Arterial Anomalies.

Congenital thoracic arterial anomalies (CTAAs), such as complete or incomplete vascular rings, pulmonary artery sling, and innominate artery compression syndrome, may cause severe tracheomalacia and upper airway obstruction. An obstructive ventilatory pattern at lung function testing (LFT) has been suggested in the presence of CTAA. The severity of obstruction may be evaluated by LFT.

Could myocardial function be predictive of successful extubation in newborns and infants?

Objective: To investigate the relationship between cardiac function and extubation readiness in infants using speckle tracking echocardiography.

Working Hypothesis: Cardiac function combined with established clinical parameters may better identify readiness for extubation.

Study Design: Pilot prospective observational study.

Propofol Formulation Affects Myocardial Function in Newborn Infants.

This study aimed to evaluate the effects of propofol in diluted and undiluted formulations on cardiac function in infants. Infants > 30 days received propofol sedation for central line insertion. Cases were divided into two groups: those who received undiluted 1% propofol (P1%); and those who received a diluted formulation (Pd) of equal volumes propofol 1% and 0.

Functional residual capacity and lung clearance index in infants treated for esophageal atresia and tracheoesophageal fistula.

Background: Newborn babies with esophageal atresia/tracheoesophageal fistula (EA/TEF) are prone to respiratory tract disorders. Functional residual capacity (FRC) and lung clearance index (LCI) are commonly considered useful and sensitive tools to investigate lung function and early detecting airways diseases. The aim of the present study is to report the first series of EA/TEF infants prospectively evaluated for FRC and LCI.

New ATP-binding cassette A3 mutation causing surfactant metabolism dysfunction pulmonary type 3.

Respiratory distress syndrome (RDS) may occur in term and near-term infants because of mutations in surfactant-related genes. ATP-binding cassette A3 (ABCA3), a phospholipid carrier specifically expressed in the alveolar epithelium, is the most frequently involved protein. We report the case of a couple of late-preterm fraternal twin infants of opposite sex carrying the same compound heterozygous ABCA3 mutations, one of which has never been previously reported, with different disease severity, suggesting variable penetrance or sex-related differences.

Effects of ventilation modalities on near-infrared spectroscopy in surgically corrected CDH infants.

Background: Near-infrared spectroscopy (NIRS) is a noninvasive technique for monitoring tissue oxygenation and perfusion. The aim of this study was to evaluate cerebral and splanchnic NIRS changes in CDH operated infants enrolled into the VICI trial and therefore randomized for ventilatory modalities.

Materials And Methods: CDH newborns enrolled into the VICI trial (Netherlands Trial Register, NTR 1310) were randomized at birth for high-frequency oscillatory ventilation (HFOV) or conventional mechanical ventilation (CMV) according to the trial.

Functional residual capacity (FRC) and lung clearance index (LCI) in mechanically ventilated infants: application in the newborn with congenital diaphragmatic hernia (CDH).

Introduction: Functional residual capacity (FRC) and lung clearance index (LCI) are sensitive parameters for early detection of airway disease in infancy. The closed helium dilution method has been applied to assess lung volume and ventilation inhomogeneity (VI) in spontaneously breathing infants.

Aims: The aims of this study were as follows: (1) to assess applicability of the helium gas dilution technique in mechanically ventilated infants with high-risk congenital diaphragmatic hernia (CDH) and to evaluate changes in breathing patterns, lung volume, and VI during the first days of life before and after surgery, and (2) to analyze the possible correlation between changes in lung volume, cerebral hemodynamics, and oxygenation before and after surgical correction of CDH through near-infrared spectroscopy (NIRS) monitoring.

Measuring transcutaneous bilirubin: a comparative analysis of three devices on a multiracial population.

Background: Hyperbilirubinemia can lead to potentially irreversible bilirubin-induced neurotoxicity. Transcutaneous bilirubin (TcB) determination has become a valuable aid in non invasive screening of neonatal jaundice.The aim of this study is to compare the performance of three most widespread transcutaneous bilirubinometers on a multiracial population of term and late pre-term neonates.

Amino acid-based formula as a rescue strategy in feeding very-low-birth-weight infants with intrauterine growth restriction.

Background And Aim: Very-low-birth-weight (VLBW) neonates may develop severe intolerance to standard preterm formula especially if they are associated with intrauterine growth restriction (IUGR). We tested the hypothesis that these infants may tolerate an elemental, amino acid-based formula as a rescue feeding strategy.

Methods: In a prospective, case-control pilot study, we enrolled VLBW IUGR infants enterally fed with standard preterm formula (SPF) at daily increments of 16 mL/kg.

Safety for patients with celiac disease of baked goods made of wheat flour hydrolyzed during food processing.

Background & Aims: Celiac disease (CD) is characterized by an inflammatory response to wheat gluten, rye, and barley proteins. Fermentation of wheat flour with sourdough lactobacilli and fungal proteases decreases the concentration of gluten. We evaluated the safety of daily administration of baked goods made from this hydrolyzed form of wheat flour to patients with CD.

Position and ventilatory response to added dead space in prematurely born infants.

Objectives: Prematurely born infants are at increased risk of sudden infant death syndrome (SIDS) if slept prone.

Working Hypothesis: Prematurely born infants would have an impaired response to an added dead space and lower respiratory muscle strength in the prone compared to the supine position.

Study Design: Prospective study.

Hering-Breuer reflex, lung volume and position in prematurely born infants.

Objectives: To investigate the effect of position on the strength of the Hering-Breuer reflex in prematurely born infants and determine whether any differences seen were related to differences in lung or tidal volume between positions.

Working Hypothesis: Position related differences in the strength of the Hering-Breuer reflex relate to differences in lung or tidal volume.

Study Design: Prospective observational study.

Sourdough bread made from wheat and nontoxic flours and started with selected lactobacilli is tolerated in celiac sprue patients.

This work was aimed at producing a sourdough bread that is tolerated by celiac sprue (CS) patients. Selected sourdough lactobacilli had specialized peptidases capable of hydrolyzing Pro-rich peptides, including the 33-mer peptide, the most potent inducer of gut-derived human T-cell lines in CS patients. This epitope, the most important in CS, was hydrolyzed completely after treatment with cells and their cytoplasmic extracts (CE).