Recurrent pericarditis in a patient with Bardet-Biedl syndrome: A case report.

Bardet-Biedl syndrome is a rare autosomal recessive disorder characterized by rod-cone dystrophy, renal dysfunction, obesity, learning difficulties, hypogonadism, polydactyl, and many other minor features that can affect the cardiovascular, locomotive, neurological, and endocrine systems. We report the case of a 16-year-old boy affected by Bardet-Biedl syndrome who presented with recurrent pericarditis with an optimal response to treatment with Anakinra. To our knowledge, this is the first description of an association between Bardet-Biedl syndrome and recurrent pericarditis.

Bioactive compounds in childhood obesity and associated metabolic complications: Current evidence, controversies and perspectives.

Obesity represents the most frequent chronic disease among children worldwide, with a significant global burden on society. Metabolically unhealthy obesity (MUO) can affect children since their first years of life, and novel therapeutic strategies to tackle metabolic complications are under investigation. This review focuses on bioactive compounds and their possible beneficial effects on obesity, particularly omega-3, docosahexaenoic acid, vitamin D, biotics, polysaccharide macromolecules, polyphenols, inositols, alpha lipoic acid, and bromelaine.

Postoperative apnea after pyloromyotomy for infantile hypertrophic pyloric stenosis.

Objective: Infantile hypertrophic pyloric stenosis (IHPS), which causes gastric outlet obstruction and hypochloremic hypokalemic metabolic alkalosis, could pose a risk of postoperative apnea in patients. The aim of this study is to evaluate the incidence of postoperative apnea in babies admitted to a tertiary-level pediatric surgical center in Milano, Italy with diagnosis of IHPS in 2010-2019. The secondary objective is to evaluate the risk factors for postoperative apnea.

Management of the CDH patient on ECLS.

Congenital diaphragmatic hernia (CDH) is the most common indication for respiratory extracorporeal life support (ECLS) in neonates. The survival rate of CDH neonates treated with ECLS is 50%, and this figure has remained relatively stable over the last few decades. This is likely because the current population of CDH neonates who require ECLS have a higher risk profile [1].

The Effect of Healthy Lifestyle Strategies on the Management of Insulin Resistance in Children and Adolescents with Obesity: A Narrative Review.

Childhood obesity is characterized by an increased risk of several metabolic derangements including insulin resistance (IR). The strongest recommendations to prevent obesity and related complications are a balanced and adequate diet and practicing physical activity from early childhood. In this review, we propose to present the effects of healthy lifestyle strategies, including physical exercise and dietary approaches, on the management of IR and related metabolic derangements.

Screening Questionnaire for Vitamin D Insufficiency in Children with Obesity.

Non-invasive screening tools to identify children at high risk of vitamin D (VitD) deficiency are proactive measures in preventive care. Recently, a validated questionnaire (Evaluation dEficieNCy Questionnaire, EVIDENCe-Q) for identifying newly diagnosed VitD-insufficient adults has been developed. We tested the EVIDENCe-Q modified for children with obesity and evaluated the correlation between VitD and questionnaire scores to adapt this tool to the pediatric population.

Atrioventricular Block in Celiac Disease: An Unusual Clinical Presentation in a Child. A Case-Based Review.

Congenital or acquired atrioventricular block (AVB) is a rare disorder in the pediatric population, while celiac disease (CeD) is a common multisystemic autoimmune disorder that is characterized by intestinal manifestations as they are the typical clinical presentation. Sometimes CeD presents more complex multisystemic involvement which includes the heart. Cardiac involvement, such as dilated cardiomyopathy, myocarditis or conduction disease, have been mainly described in untreated adult patients with or without gastro-intestinal symptoms; rare cases of AVB and CeD have been also reported, particularly in association with extra-cardiac manifestations.

Lung involvement in children with COVID-19 multisystem inflammatory syndrome.

Since the beginning of the COVID-19 pandemic, multisystem inflammatory syndrome in children (MIS-C) has been reported in increasing numbers, mostly focusing on cardiac dysfunction. Very few studies have evaluated lung involvement in terms of imaging findings, while data regarding pulmonary function in children with MIS-C are not available. The purpose of our study was to evaluate lung involvement in MIS-C by imaging and lung function by structured light plethysmography (SLP) at hospital admission and 6 months afterwards.

Precocious puberty and microbiota: The role of the sex hormone-gut microbiome axis.

Puberty is a critical phase of life associated with physiological changes related to sexual maturation, and represents a complex process regulated by multiple endocrine and genetic controls. Puberty is driven by hormones, and it can impact the gut microbiome (GM). GM differences between sex emerge at puberty onset, confirming a relationship between microbiota and sex hormones.

Time of Dietary Energy and Nutrient Intake and Body Mass Index in Children: Compositional Data Analysis from the Childhood Obesity Project (CHOP) Trial.

Meal timing is suggested to influence the obesity risk in children. Our aim was to analyse the effect of energy and nutrient distributions at eating occasions (EO), including breakfast, lunch, supper, and snacks, on the BMI z-score (zBMI) during childhood in 729 healthy children. BMI and three-day dietary protocols were obtained at 3, 4, 5, 6, and 8 years of age, and dietary data were analysed as the percentage of the mean total energy intake (TEI; %E).

Multidisciplinary Management of Children with Occult Spinal Dysraphism: A Comprehensive Journey from Birth to Adulthood.

Occult spinal dysraphism (OSD) comprises different forms of failure in embryogenic development that can lead to genitourinary, spinal, or lower limb alterations, thus determining progressive neurological deterioration. The correct management of children harboring OSD represents a significant issue during their life up to adulthood. However, patients often have to entertain individual consultations with each specialist.

Non-Communicable Diseases and Rare Diseases: A Current and Future Public Health Challenge within Pediatrics.

The global burden of non-communicable diseases (NCDs) and rare diseases constitutes a current and future public health challenge within pediatrics [...

Utility of Functional Lumen Imaging Probe in Long-Term Follow-Up of Children with Esophageal Atresia: A Single-Center Retrospective Study.

Long term follow-up of patients with esophageal atresia (EA) may be hampered by esophageal dysmotility, which affects quality of life and might lead to late complications. The endoluminal functional lumen imaging probe (EndoFlip™ Medtronic, Crospon Inc.) is an innovative diagnostic tool that assesses esophageal distensibility.

Prospective multicentre Italian pregnancy cohort study (SIMPLE) on the associations of maternal first trimester SIMPLE nutritional score with early placental function markers and pregnancy outcomes.

Introduction: Currently, the adherence to nutritional guidelines is low, with alarming rates of obesity worldwide and micronutrient deficiencies documented even in industrialised countries. As a consequence, nutritional screening and counselling represent a critical subject in early pregnancy, aiming to improve pregnancy outcomes and population health.

Methods And Analysis: In this setting, the development of a simple and reproducible nutritional checklist is of utmost importance.

Postoperative Osteopathic Manipulative Treatment in Children with Esophageal Atresia: Potential Benefits on the Anthropometric Parameters.

Esophageal atresia (EA) is a congenital malformation that affects the normal esophageal development. Surgical treatment, although restoring the integrity of the alimentary tract, may lead to long-term sequelae-like developmental abnormalities and musculoskeletal deformities. We evaluated the effects of osteopathic manipulative treatment (OMT) on the recovery of the range of the right upper limb movement and on the rise of the auxological parameters.

SCN2A and arrhythmia: A potential correlation? A case report and literature review.

Variants in SCN2A, encoding the voltage-gated sodium channel Nav1.2, are commonly associated with developmental and epileptic encephalopathy. Although animal studies demonstrated a role for Nav1.

Incidence and risk factors of bacterial sepsis and invasive fungal infection in neonates and infants requiring major surgery: an Italian multicentre prospective study.

Background: Limited data are currently available on the incidence rates and risk factors for bacterial sepsis and invasive fungal infections (IFIs) among neonates and infants undergoing major surgery.

Aim: To assess the incidence of bacterial sepsis and IFI, fungal colonization, risk factors for sepsis, and mortality in neonates and infants aged <3 months undergoing major surgery.

Methods: A multicentre prospective study was conducted involving 13 level-3 neonatal intensive care units in Italy, enrolling all infants aged ≤3 months undergoing major surgery.

The parental psychological distress caused by separation from their critically ill child during the COVID-19 pandemic: A tale of two cities.

Introduction: A child's critical illness is a stressful event for the entire family, causing significant emotional distress among parents and changes to family functioning. The Severe Acute Respiratory Syndrome-Related Coronavirus 2 (SARS-CoV-2) pandemic has abruptly caused modifications in visitation policies of Pediatric Intensive Care Units (PICUs) in many countries. We hypothesized that caregivers with no or severely restricted access to PICUs would demonstrate increased psychological distress as compared to those who had limitless access (LA) to PICUs.

Glut1 deficiency syndrome throughout life: clinical phenotypes, intelligence, life achievements and quality of life in familial cases.

Background: Glut1 deficiency syndrome (Glut1-DS) is a rare metabolic encephalopathy. Familial forms are poorly investigated, and no previous studies have explored aspects of Glut1-DS over the course of life: clinical pictures, intelligence, life achievements, and quality of life in adulthood. Clinical, biochemical and genetic data in a cohort of familial Glut1-DS cases were collected from medical records.

Biotics in atopic diseases: state of the art and future perspectives.

Prevalence of allergic diseases has growing in recent decades, being a significant burden for patients and their families. Different environmental factors, acting in early life, can significantly affect the timing and diversity of bacterial colonization and the immune system development. Growing evidence points to a correlation between early life microbial perturbation and development of allergic diseases.

Self-Perceived Physical Level and Fitness Performance in Children and Adolescents with Inflammatory Bowel Disease.

Background: Inflammatory bowel disease (IBD) patients show a higher risk of developing metabolic and cardiovascular diseases due to the presence of systemic low-grade chronic inflammation. Exercise can improve cardiovascular fitness and modulate the inflammatory processes. We evaluated the physical activity (PA) level and the fitness performance of children and adolescents with IBD.