One-Year Effect of Elexacaftor/Tezacaftor/Ivacaftor Therapy on HbA1c Levels and Insulin Requirement in Patients with Insulin-Dependent Cystic Fibrosis-Related Diabetes: A Retrospective Observational Study.

Introduction: The impact of ETI therapy on pulmonary function and nutritional status has been widely studied; the literature on the possible outcomes on glycemic control and insulin requirement in patients affected by CFRD is controversial.

Aim: The main objective of our study was to evaluate HbA1c levels in patients with cystic fibrosis-related diabetes (CFRD) after one year of therapy with elexacaftor/tezacaftor/ivacaftor (ETI). The secondary objective was to study the changes in the total daily insulin dose (TDD), pulmonary function and metabolism in this population.

Surgical treatment of cavernous malformation-related epilepsy in children: case series, systematic review, and meta-analysis.

Objective: Cerebral cavernous malformations (CCMs) are cerebral vascular lesions that occasionally occur with seizures. We present a retrospective case series from IRCCS Gaslini Children's Hospital, a systematic review, and meta-analysis of the literature with the goal of elucidating the post-surgery seizure outcome in children with CCMs.

Methods: a retrospective review of children with cavernous malformation related epilepsy who underwent surgery at Gaslini Children's Hospital from 2005 to 2022 was conducted.

Exploring the frontier in robotic pediatric cancer surgery: when to move forward and when to stop.

Purpose: In recent years, the use of robotic-assisted minimally invasive surgery in pediatric oncology has increased. Despite its benefits, its adoption remains limited. This single-center retrospective analysis examines technical nuances, indications, and surgical limitations to prevent complications.

Ventilatory Support, Extubation, and Cerebral Perfusion Changes in Pre-Term Neonates: A Near Infrared Spectroscopy Study.

Early extubation is considered to be beneficial for pre-term neonates. On the other hand, premature extubation can cause lung derecruitment, compromised gas exchange, and need for reintubation, which may be associated with severe brain injury caused by sudden cerebral blood flow changes. We used near infrared spectroscopy (NIRS) to investigate changes in cerebral oxygenation (rScO) and fractional tissue oxygen extraction (+) after extubation in pre-term infants.

The usefulness of point-of-care ultrasound in dehydrated patients in a pediatric emergency department.

Backgrounds: Dehydration is among the most common causes of Pediatric Emergency Department admission; however, no clinical signs, symptoms, or biomarkers have demonstrated sufficient sensitivity, specificity, or reliability to predict dehydration.

Methods: We conducted a prospective, monocentric, observational study at Giannina Gaslini Hospital, a tertiary care pediatric hospital. Our study aimed to compare inferior vena cava ultrasound measurement with volume depletion biomarkers to understand if point-of-care ultrasound could help grade, evaluate, and better manage dehydration in children presenting to the pediatric emergency department.

Individual, environmental and demographic factors may play a role in the prognosis of fatal and non-fatal paediatric drowning.

Aim: Drowning is a leading cause of unintentional death. Ongoing efforts are dedicated to preventing these tragic incidents. Our aim was to evaluate whether demographic, environmental and epidemiological characteristics of drowned children influence their prognosis.

Precocious Puberty Diagnoses Spike, COVID-19 Pandemic, and Body Mass Index: Findings From a 4-year Study.

Context: Since the COVID-19 outbreak, the number of girls with suspected precocious puberty has increased.

Objective: To compare the incidence of idiopathic central precocious puberty (ICPP) during COVID-19 with that of the previous 4 years.

Methods: Anthropometric, biochemical, and radiological parameters were collected between January 2016 and June 2021 from 133 girls who met the Rapidly Progressive ICPP criteria (RP-ICPP).

Influence of maternal oral microbiome on newborn oral microbiome in healthy pregnancies.

Background: Periodontal disease and its bacteria can be responsible for pregnancy complications and transmission of periodontal bacteria from mother to newborn.

Methods: A salivary swab to 60 healthy, full-term newborns and their mothers was taken immediately after birth. The test was performed with Real Time PCR method to evaluate the expression of the gene through DNA amplification.

Difficult Intravascular Access in Pediatric Emergency Department: The Ultrasound-Assisted Strategy (DIAPEDUS Study).

Vein line positioning represents one of the first diagnostic and therapeutic steps in Pediatric Emergency Department (PED); however, the outcome of this maneuver is frequently not as expected, especially for difficult-to-access (DIVA) patients. The standard technique (visual-palpatory) has a low success rate; hence ultrasound (US) assistance has been suggested for DIVA patients, although controversial results have been obtained. Our study compared the success rate of an intravascular (IV) access procedure at the first attempt, with and without ultrasound assistance, in pediatric DIVA patients.

Vein of Galen aneurysmal malformation in newborns: a retrospective study to describe a paradigm of treatment and identify risk factors of adverse outcome in a referral center.

Background: Vein of Galen aneurysmal malformation (VGAM) is a rare cerebral vascular malformation associated with significant morbidity and mortality. Newborns with VGAM without adequate treatment may develop rapidly deteriorating high output heart failure (HOHF) and are at risk for severe neurological outcomes.

Objective: To describe the clinical course and management of newborns with VGAM, and identify which echocardiographic and neuroradiologic factors may be associated with severe heart failure at birth and adverse short term outcomes.

Pharmacological pain and sedation interventions for the prevention of intraventricular hemorrhage in preterm infants on assisted ventilation - an overview of systematic reviews.

Background: Germinal matrix hemorrhage and intraventricular hemorrhage (GMH-IVH) may contribute to neonatal morbidity and mortality and result in long-term neurodevelopmental sequelae. Appropriate pain and sedation management in ventilated preterm infants may decrease the risk of GMH-IVH; however, it might be associated with harms.

Objectives: To summarize the evidence from systematic reviews regarding the effects and safety of pharmacological interventions related to pain and sedation management in order to prevent GMH-IVH in ventilated preterm infants.

Acute pediatric encephalitis: etiology, course, and outcome of a 12-year single-center immunocompetent cohort.

Background: Encephalitis is an uncommon but severe disorder due to an inflammation of the brain parenchyma, usually diagnosed on clinical, laboratory, electroencephalographic, and neuroradiological features. New causes of encephalitis have been reported in recent years, so diagnostic criteria have changed over time. We report on a single-center experience of a pediatric Hospital, the hub of its region, over 12 years (2008-2021), with the evaluation of all children managed for acute encephalitis.

The Transport of Respiratory Distress Syndrome Twin Newborns: The 27-Year-Long Experience of Gaslini Neonatal Emergency Transport Service Using Both Single and Double Ventilators.

Objective: Twin pregnancy rates have increased in the past 30 years. We describe the experience of the Neonatal Emergency Transport Service of the Gaslini Hospital, Genoa, Italy, in the transport of twin newborns.

Methods: This was a retrospective study (1996-2021); 7,852 medical charts from the Neonatal Emergency Transport Service were reviewed.

Clinical Evaluation of the Eurosets Trilly Oxygenator During Cardiopulmonary Bypass in a Pediatric Population.

The equipment selected for cardiopulmonary bypass (CPB) in pediatric cardiac surgery critically influences the safety, efficiency, efficacy and pathophysiological impact in perioperative use and the post-operative outcome. In this report, we present a single-center retrospective analysis of the clinical efficacy, efficiency and safety of the Trilly oxygenator (Eurosets Srl, Medolla, MO, Italy), which has an integrated arterial filter. It has a blood flow capacity of 500 to 3500ml/min, an AAMI index of 4.

Corrigendum: Pubertal timing in children with Silver Russell syndrome compared to those born small for gestational age.

[This corrects the article DOI: 10.3389/fendo.2022.

Influence of isolated low-grade intracranial haemorrhages on the neurodevelopmental outcome of infants born very low birthweight.

Aim: To determine whether isolated low-grade germinal matrix-intraventricular haemorrhages (LG-GMH-IVH) and low-grade punctate cerebellar haemorrhages (LG-CBH) contribute to the neurodevelopment of infants born preterm with very low birthweight (VLBW).

Method: A prospective observational cohort study was conducted on infants born with VLBW hospitalized from January 2012 to July 2017 who had undergone serial cranial ultrasounds since birth and magnetic resonance susceptibility-weighted imaging of the brain at term-corrected age. Only those with VLBW carrying isolated LG-GMH-IVH (grades 1 or 2) or isolated LG-CBH (punctate cerebellar haemorrhages ≤4 mm in diameter) or absence of lesions (no-lesion) were enrolled and followed up to 3 years.

Increased Frequency of Diabetic Ketoacidosis: The Link With COVID-19 Pandemic.

Aims: Diabetic ketoacidosis is the most severe metabolic derangement due to prolonged insulin deficiency as in type 1 diabetes. Diabetic ketoacidosis, a life-threatening condition, is often diagnosed late. A timely diagnosis is mandatory to prevent its consequences, mainly neurological.

Simultaneous Quantification of Ivacaftor, Tezacaftor, and Elexacaftor in Cystic Fibrosis Patients' Plasma by a Novel LC-MS/MS Method.

The new breakthrough cystic fibrosis (CF) drug combination of ivacaftor (IVA), tezacaftor (TEZ), and elexacaftor (ELX), namely "caftor" drugs, directly modulates the activity and trafficking of the defective CF transmembrane conductance regulator protein (CFTR) underlying the CF disease. The role of therapeutic drug monitoring (TDM) of caftor drugs in clinical settings has recently been established. The availability of reliable and robust analytical methods for the quantification of IVA, TEZ, and ELX is essential to support dose-concentration-effect studies.

Neurodevelopmental Outcome at 3 Years of Age in Very Low Birth Weight Infants According to Brain Development and Lesions.

Background: During the last decades, severe brain lesions affecting very low birth weight (<1500 gr, VLBW) infants were gradually substituted by milder lesions with debatable prognoses.

Objective: The objective of this study is to define type, frequency and 3 years of neurodevelopmental outcome of prematurity-related brain lesions in a modern cohort of VLBW infants.

Methods: VLBW infants admitted to our NICU in 5 years period with brain MRI at term-equivalent age were included.