522 results match your criteria: "Children' Hospital "Vittore Buzzi"[Affiliation]"

Inborn errors of metabolism (IEMs) represent a complex system model, in need of a shift of approach exploring the main factors mediating the regulation of the system, internal or external and overcoming the traditional concept of biochemical and genetic defects. In this context, among the established factors influencing the metabolic flux, i.e.

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Article Synopsis
  • The study examines right ventricular outflow tract abnormalities (RVOTA) in monochorionic/diamniotic (MC/DA) twin pregnancies that do not have twin-to-twin transfusion syndrome (TTTS).
  • Among 891 MC/DA twin pregnancies analyzed from 2009 to 2018, RVOTA was found in 14 cases (1.6%), with pulmonary stenosis being the most common issue, often accompanied by other complications like amniotic fluid discrepancy and selective fetal growth restriction.
  • The findings suggest that RVOTA can indeed arise in these pregnancies, and it highlights the need for specialized fetal echocardiographic evaluations and delivery in advanced care centers for affected cases.
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(1) Background. Early nutrition and lifestyle before and during pregnancy, breastfeeding, infancy, and early childhood can affect the risk of developing common non-communicable diseases during adulthood such as obesity and metabolic syndrome. To support positive long-term outcomes, it is essential to debunk fake news and provide evidence-based nutritional recommendations.

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A Journey on the Skin Microbiome: Pitfalls and Opportunities.

Int J Mol Sci

September 2021

Department of Biomedical Sciences for Health, University of Milan, 20133 Milan, Italy.

The human skin microbiota is essential for maintaining homeostasis and ensuring barrier functions. Over the years, the characterization of its composition and taxonomic diversity has reached outstanding goals, with more than 10 million bacterial genes collected and cataloged. Nevertheless, the study of the skin microbiota presents specific challenges that need to be addressed in study design.

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Children with obesity tend to have lower level of physical activity compared to non-obese peers. In fact, sedentary behaviors are prevalent in obese children causing difficulties to perform motor tasks and engaging in sport activities. This, in turn, has direct repercussions on adiposity and related comorbidities.

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Liang-Wang syndrome (LIWAS) is a polymalformative syndrome first described in 2019 caused by heterozygous mutation of the KCNMA1 gene encoding the Ca and voltage-activated K channel (BKC). The KCNMA1 variant p.(Gly356Arg) abolishes the function of BKC and blocks the generation of K current.

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Postbiotics - when simplification fails to clarify.

Nat Rev Gastroenterol Hepatol

November 2021

China-Norway Joint Lab on Fish Gastrointestinal Microbiota, Institute of Feed Research, Chinese Academy of Agricultural Sciences, Beijing, China.

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Nutrient intake in school-aged children with food allergies: a case-control study.

Int J Food Sci Nutr

May 2022

International Center for the Assessment of Nutritional Status (ICANS), Department of Food, Environmental and Nutritional Sciences (DeFENS), Università di Milano, Milan, Italy.

Most studies assessed nutrient intake of young children with food allergy (FA) compared to healthy children. We aimed to compare macro- and micronutrient intake of school-aged children with FA to non-allergic children. This case-control study included 93 Italian children (52 with FA and 41 controls, median age 7.

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Obesity is associated with unhealthy lifestyle behaviors and poor Health Related Quality of Life (HRQOL). The cumulative effect of lifestyle behaviors on HRQOL has been demonstrated in chronically ill adolescents, but not in adolescents with obesity. The present study aimed to assess the association between HRQOL and adherence to the Mediterranean Diet (MD) and/or low levels of physical activity (PA) in a large sample of outpatient adolescents with overweight or obesity seeking weight loss treatment.

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Obesity is defined as a condition characterized by an excessive fat accumulation that has negative health consequences. Pediatric obesity is associated with an increased risk for many diseases, including impaired glycemic and lipidic control that may lead to the development of chronic, and potentially disabling, pathologies, such as type 2 diabetes mellitus (T2DM) and cardiovascular events, in adult life. The therapeutic strategy initially starts with interventions that are aimed at changing lifestyle and eating behavior, to prevent, manage, and potentially reverse metabolic disorders.

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An allergy to cow's milk requires the avoidance of cow's milk proteins and, in some infants, the use of a hypoallergenic formula. This review aims to summarize the current evidence concerning different types of hydrolysed formulas (HF), and recommendations for the treatment of IgE- and non-IgE-mediated cow's milk allergy and functional gastrointestinal disorders in infancy, for which some dietary intervention and HF may be of benefit to both immune and motor mechanisms. Current guidelines recommend cow's milk protein (i.

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Acanthosis nigricans (AN) is associated with obesity and type 2 diabetes, where insulin resistance (IR) is considered a predisposing factor. IR can also affect patients with type 1 diabetes (T1D). We evaluated the prevalence of AN in patients with T1D compared to subjects with obesity in order to define the interplay between IR and excess weight.

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We report detailed functional analyses and genotype-phenotype correlations in 392 individuals carrying disease-causing variants in SCN8A, encoding the voltage-gated Na+ channel Nav1.6, with the aim of describing clinical phenotypes related to functional effects. Six different clinical subgroups were identified: Group 1, benign familial infantile epilepsy (n = 15, normal cognition, treatable seizures); Group 2, intermediate epilepsy (n = 33, mild intellectual disability, partially pharmaco-responsive); Group 3, developmental and epileptic encephalopathy (n = 177, severe intellectual disability, majority pharmaco-resistant); Group 4, generalized epilepsy (n = 20, mild to moderate intellectual disability, frequently with absence seizures); Group 5, unclassifiable epilepsy (n = 127); and Group 6, neurodevelopmental disorder without epilepsy (n = 20, mild to moderate intellectual disability).

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Acute encephalitis in pediatric multisystem inflammatory syndrome associated with COVID-19.

Eur J Paediatr Neurol

September 2021

Vittore Buzzi Children's Hospital, Pediatric Neurology Unit, Via Castelvetro, 32, 20154, Milan, Italy. Electronic address:

Objective: To characterize neurological involvement in multisystem inflammatory syndrome in children (MIS-C) related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection.

Methods: Retrospective analysis of the clinical, electroencephalographic, CSF and neuroradiological parameters recorded in seven children (3 males, aged 3-10 years) affected by MIS-C with acute neurological involvement.

Results: All cases presented acute encephalopathy with drowsiness, irritability, mood deflection and diffuse EEG slowing with periodic posterior complexes.

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Giant congenital exophytic strawberry-like mass in a newborn.

Clin Exp Dermatol

January 2022

Dermatology Unit and Genodermatosis Unit, Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

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For two decades, many scores, questionnaires, and rating systems have been used to evaluate the functional outcome of children with an upper extremity fracture (UEF). However, only a few of these were created specifically for children, and many assess only elbow function. In the absence of any published review on this topic, we set out to identify and categorize different scores used to evaluate the clinical and functional outcomes of surgically treated pediatric UEFs.

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Long-Term Coronavirus Disease 2019 Complications in Inpatients and Outpatients: A One-Year Follow-up Cohort Study.

Open Forum Infect Dis

August 2021

Department of Biomedical and Clinical Sciences "L. Sacco", Pediatric Clinical Research Center "Romeo and Enrica Invernizzi", University of Milan, Milan, Italy.

Background: As the coronavirus pandemic spreads, more and more people are infected with severe acute respiratory syndrome coronavirus 2. The short- and medium-term effects of the infection have been described, but the description of the long-term sequelae is lacking in the literature.

Methods: Patients healed from coronavirus disease 2019 (COVID-19) from February 2020 to May 2020 were considered for inclusion in this study, regardless of the severity of the disease during the acute phase.

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The management of patients with spinal muscular atrophy type 1 (SMA1) is constantly evolving. In just a few decades, the medical approach has switched from an exclusively palliative therapy to a targeted therapy, transforming the natural history of the disease, improving survival time and quality of life and creating new challenges and goals. Many nutritional problems, gastrointestinal disorders and metabolic and endocrine alterations are commonly identified in patients affected by SMA1 during childhood and adolescence.

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Congenital Pouch Colon: Case Series and Review of Evidences for Resection.

J Indian Assoc Pediatr Surg

May 2021

Department of Salute della Donna e del Bambino, Division of Pediatric Surgery, University of Padova, Padova, Italy.

Background: Congenital pouch colon (CPC) is a rare variant of anorectal malformations (ARM) with its highest reported incidence in India. We aimed to describe five patients affected by CPC, in which the tissue from the terminal dilated colon has been successfully used and to discuss our results on the light of an extended revision of the literature.

Materials And Methods: The clinical details of five cases treated for CPC in two Italian Centers were retrospectively reviewed assessing the fate of the terminal dilated colon.

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Purpose: COVID-19 disease may result in a severe multisystem inflammatory syndrome in children (MIS-C), which in turn may alter thyroid function (TF). We assessed TF in MIS-C, evaluating its impact on disease severity.

Methods: We retrospectively considered children admitted with MIS-C to a single pediatric hospital in Milan (November 2019-January 2021).

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"CoVidentary": An online exercise training program to reduce sedentary behaviours in children with type 1 diabetes during the COVID-19 pandemic.

J Clin Transl Endocrinol

September 2021

Laboratory of Adapted Motor Activity (LAMA) - Department of Public Health, Experimental Medicine and Forensic Science, University of Pavia, Pavia, Italy.

Aim: We explored the physical activity (PA) level and the variation in glycaemic control in children with type 1 diabetes (T1D) before and during the lockdown. Then, we proposed an online training program supported by sport-science specialists.

Methods: Parents of children with T1D (<18 years) filled out an online survey.

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Discordant placental echogenicity is observed in MC pregnancies complicated with twin anemia-polycythemia sequence, but could also belong to complicated singleton gestation.

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Background: Previous research shows that a lung ultrasound score (LUS) can anticipate CPAP failure in neonatal respiratory distress syndrome.

Research Question: Can LUS also predict the need for surfactant replacement?

Study Design And Methods: Multicenter, pragmatic study of preterm neonates who underwent lung ultrasound at birth and those given surfactant by masked physicians, who also were scanned within 24 h from administration. Clinical data and respiratory support variables were recorded.

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Background & Aims: it has previously been described that dietary patterns established early in life tracked to late childhood. The aim of the present work was to analyse the association of dietary patterns that tracked from 2 to 8y with cardiometabolic markers at 8y of age.

Methods: The 3 identified patterns at 2y (that previous analyses showed to track to age 8y) were: "Core", loaded for vegetables, fruits, fish, olive oil, etc.

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Mortality in neonates with Gram-negative bloodstream infections has remained unacceptably high. Very few data are available on the impact of resistance profiles, virulence factors, appropriateness of empirical treatment and clinical characteristics on patients' mortality. A survival analysis to investigate 28-day mortality probability and predictors was performed including (I) infants <90 days (II) with an available Enterobacterales blood isolate with (III) clinical, treatment and 28-day outcome data.

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