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Olipudase alfa is indicated for the non-central nervous system manifestations of Acid sphingomyelinase deficiency (ASMD). Anaphylaxis is a very rare and life-threatening adverse reaction described for this drug. Here, we report the case of a 2-year-old boy affected by chronic neurovisceral ASMD who experienced signs of hypersensitivity reactions to olipudase alfa since the administered dose of 1 mg/kg during dose escalation and a proper anaphylactic reaction during the second administration of the target therapeutic dose of 3 mg/kg. The treatment was stopped for 15 weeks and then a 7-step desensitization protocol with the infused dose of 0.03 mg/kg was applied. Subsequent gradual dose escalation was resumed, successfully reaching the dose of 0.3 mg/kg. Moreover, biochemical, and radiological disease indexes, which were increased during treatment discontinuation, have gradually improved since the restart of treatment. However, at the second administration of the dose of 0.6 mg/kg, the patient experienced another adverse drug reaction with facial urticarial rash and bronchospasm, requiring the administration of adrenaline, methylprednisolone, and inhaled salbutamol. This case report highlights the need to customize the olipudase alfa desensitization protocol according to individual tolerance and raises the issue of achieving the established therapeutic target in the most sensitive children. Synopsis: We report a case of anaphylaxis to olipudase alfa in a child affected by chronic neurovisceral Acid sphingomyelinase deficiency (ASMD) and describe a 7-step desensitization procedure. This procedure, with the total administered dose of 0.03 mg/kg, followed by gradual dose escalation, allowed to reach the dose of 0.3 mg/kg without adverse reactions; however, at the second administration of the dose of 0.6 mg/kg our patient presented another adverse reaction suggesting the need of a different desensitization strategy.

Abstract Background The coronavirus 2019 (COVID-19) related containment measures led to the disruption of all virus distribution. Bronchiolitis-related hospitalizations shrank during 2020–2021, rebounding to pre-pandemic numbers the following year. This study aims to describe the trend in bronchiolitis-related hospitalization this year, focusing on severity and viral epidemiology. Methods We conducted a retrospective investigation collecting clinical records data from all infants hospitalized for bronchiolitis during winter (1st September-31th March) from September 2018 to March 2023 in six Italian hospitals. No trial registration was necessary according to authorization no.9/2014 of the Italian law. Results Nine hundred fifty-three infants were hospitalized for bronchiolitis this last winter, 563 in 2021–2022, 34 in 2020–2021, 395 in 2019–2020 and 483 in 2018–2019. The mean length of stay was significantly longer this year compared to all previous years (mean 7.2 ± 6 days in 2022–2023), compared to 5.7 ± 4 in 2021–2022, 5.3 ± 4 in 2020–2021, 6.4 ± 5 in 2019–2020 and 5.5 ± 4 in 2018–2019 (p

Abstract Background Limited evidence exists regarding the association between COVID-19 and Long COVID manifestations in children, particularly concerning variants of concern (VOCs). We aimed to characterize a cohort of pediatric patients hospitalized with confirmed acute SARS-CoV-2 and monitor them for Long COVID symptoms. Additionally, it seeks to explore any potential correlations between VOCs and clinical symptoms. Methods We conducted a prospective study involving children hospitalized from November 2021 to March 2023, with confirmed acute SARS-CoV-2 infection. A telephone survey was conducted at 3-6-12 months after discharge. Results We included 167 patients (77 F/90 M). Upon hospital admission, 95.5% of patients presented as symptomatic. Regarding patients for whom it was feasible to determine the SARS-CoV-2 variant (n = 51), the Delta variant was identified in 11 children (21.6%) and Omicron variant in the remaining 40 patients (78.4%: 27.5% BA.1 variant; 15% BA.2 variant; 57.5% BA.5 variant). 19 patients (16.5%) reported experiencing at least one symptom indicative of Long COVID (weight loss 31.6%, inappetence 26.3%, chronic cough 21.1%, fatigue 21.1%, and sleep disturbances, wheezing, abdominal pain and mood disorders 15.8%). In only 4 patients with Long COVID we could identified a specific SARS-CoV-2 variant (3 Omicron: 2 BA.1 and 1 BA.2; 1 Delta). Conclusions this study underscores that long COVID is a significant concern in the pediatric population. Our data reinforce the importance of continuously monitoring the impact of long-COVID in infants, children, and adolescents. A follow-up following SARS-CoV-2 infection is therefore advisable, with symptom investigation tailored to the patient’s age.

Abstract Background In pediatric age, Group A Streptococcus (GAS) is responsible for a wide spectrum of clinical manifestations, from mild localized infections to life-threatening invasive diseases. In December 2022, the World Health Organization reported an increased incidence of scarlet fever and invasive GAS infections (iGAS) cases in Europe and the United States. In line with these observations, surveillance has been strengthened in our Region, allowing the identification of certified or highly suspected forms of iGAS. Case presentation We report here 4 emblematic cases of iGAS admitted to our Intensive Care Unit (ICU) in the short time span from mid-February to mid-March 2023. Particularly, we describe a case of pleuropneumonia (4 year old boy) and a case of respiratory failure (2 year old boy), who necessitated Non-Invasive Ventilation support, a case of Streptococcal Toxic Shock Syndrome (6 year old girl), presenting with multi-organ failure, who needed Invasive Ventilation, and a case of meningitis (5 year old girl). All these patients needed intensive care support. Conclusions Accurate differential diagnosis and early treatment both could help to reduce the transmission of GAS and consequently the risk of severe iGAS. These cases confirmed the need for close monitoring and appropriate notification, in order to verify their actual increased incidence.

Abstract Currently, the mechanisms underlying sensory perception and sensory performance in children with food allergies are far from being understood. As well, only recently, single research afforded the oral host-commensal milieu, addressing oral microbial communities in children with peanut allergies. To bridge the current gaps in knowledge both in the sensory and microbial fields, a psychophysiological case–control study was performed in allergic children (n = 29) and a healthy sex-age-matched control group (n = 30). Taste perception, food neophobia, and liking were compared in allergic and non-allergic children. The same subjects were characterized for their oral microbiota composition by addressing saliva to assess whether specific profiles were associated with the loss of oral tolerance in children with food allergies. Our study evidenced an impaired ability to correctly identify taste qualities in the allergic group compared to controls. These results were also consistent with anatomical data related to the fungiform papillae on the tongue, which are lower in number in the allergic group. Furthermore, distinct oral microbial profiles were associated with allergic disease, with significant down-representations of the phylum Firmicutes and of the genera Veillonella spp., Streptococcus spp., Prevotella spp., and Neisseria spp. For the first time, this study emphasizes the link between sensory perception and food allergy, which is a novel and whole-organism view of this pathology. Our data indicated that an impaired taste perception, as regards both functionality and physiologically, was associated with food allergy, which marginally influences the food neophobia attitude. It is also accompanied by compositional shifts in oral microbiota, which is, in turn, another actor of this complex interplay and is deeply interconnected with mucosal immunity. This multidisciplinary research will likely open exciting new approaches to therapeutic interventions.

Pulmonologists may be involved in managing pulmonary diseases in children with complex clinical pictures without a diagnosis. Moreover, they are routinely involved in the multidisciplinary care of children with rare diseases, at baseline and during follow-up, for lung function monitoring. Lysosomal storage diseases (LSDs) are a group of genetic diseases characterised by a specific lysosomal enzyme deficiency. Despite varying pathogen and organ involvement, they are linked by the pathological accumulation of exceeding substrates, leading to cellular toxicity and subsequent organ damage. Less severe forms of LSDs can manifest during childhood or later in life, sometimes being underdiagnosed. Respiratory impairment may stem from different pathogenetic mechanisms, depending on substrate storage in bones, with skeletal deformity and restrictive pattern, in bronchi, with obstructive pattern, in lung interstitium, with altered alveolar gas exchange, and in muscles, with hypotonia. This narrative review aims to outline different pulmonary clinical findings and a diagnostic approach based on key elements for differential diagnosis in some treatable LSDs like Gaucher disease, Acid Sphingomyelinase deficiency, Pompe disease and Mucopolysaccharidosis. Alongside their respiratory clinical aspects, which might overlap, we will describe radiological findings, lung functional patterns and associated symptoms to guide pediatric pulmonologists in differential diagnosis. The second part of the paper will address follow-up and management specifics. Recent evidence suggests that new therapeutic strategies play a substantial role in preventing lung involvement in early-treated patients and enhancing lung function and radiological signs in others. Timely diagnosis, driven by clinical suspicion and diagnostic workup, can help in treating LSDs effectively.

In last decades a simultaneous increase in the prevalence of atopic and autoimmune disorders in pediatric population has been observed. Despite the Th1-Th2 paradigm, supporting the polarization of the immune system with Th1 response involved in autoimmune diseases and Th2 response leading to hypersensitivity reactions, recent evidence suggests a possible coexistence of common pathogenic pathways as result of shared immune dysregulation. Similar genes and other mechanisms such as epithelial barrier damage, gut microbiota dysbiosis and reduced number of T regs and IL-10 contribute to the onset of allergy and autoimmunity. IgA deficiency is also hypothesized to be the crosslink between celiac disease and allergy by lowering gut mucous membrane protection from antigens and allergens. The present narrative review aims to give an overview of the co-occurrence of allergic and autoimmune disorders (celiac disease, inflammatory bowel diseases, type 1 diabetes mellitus, thyroid disease, juvenile idiopathic arthritis) in pediatric population, based on the available evidence. We also highlighted the common pathogenic pathways that may underpin both. Our findings confirm that allergic and autoimmune diseases are commonly associated, and clinicians should therefore be aware of the possible coexistence of these conditions in order to ameliorate disease management and patient care. Particular attention should be paid to the association between atopic dermatitis or asthma and celiac disease or type 1 diabetes and vice versa, for therapeutic interventions. Further studies are needed to better clarify mechanisms involved in the pathogenesis and eventually identify new therapeutic strategies.

Down Syndrome (DS) is the most common chromosomal abnormality compatible with life. The life of patients suffering from DS can be strongly impacted by Recurrent Respiratory tract Infections (RRIs), leading to an increased rate of hospitalisation, a higher need for intensive care and fatality. With a literature review, we summarise here the main etiological factors for RRI in this category of patients, particularly focusing on airway malformations such as tracheomalacia, tracheal bronchus and bronchomalacia, comorbidities associated with the syndrome, like congenital heart diseases, dysphagia, gastroesophageal reflux, musculoskeletal involvement and obesity, and immunologic impairments, involving both innate and adaptive immunity. For these patients, a multidisciplinary approach is imperative as well as some preventive strategies, in particular vaccinations in accordance with their national schedule for immunization.

Abstract Background Cholestasis in extremely premature infants (EPI) constitutes a nutritional challenge and maltodextrins have been reported as a possible strategy for hypoglycaemia. We aim to describe the nutritional management of an EPI with non-syndromic bile duct paucity (NSBDP) and feeding intolerance. Case presentation A patient, born at 27 weeks of gestational age, presented cholestatic jaundice at 20 days of life with a clinical picture of NSBDP. Patient’s growth was insufficient with formula rich in medium-chain triglyceride (MCT) and branched-chain amino acids (BCAA). Due to frequent fasting hypoglicemic episodes, maltodextrins supplements were provided. He subsequently presented severe abdominal distension and painful crises, which required hospital admission and withdrawal of maltodextrins. Hypercaloric extensively hydrolysed formula provided weight gain, glycemic control, and parallel improvement in cholestasis. Conclusions Our case suggests caution with the use of maltodextrins in infants, especially if premature. Commercial preparations for hepatopatic patients contain higher concentrations of MCTs and BCAAs, but personalized strategies must be tailored to each patient.

Abstract Background Multisystem inflammatory syndrome in children (MIS-C) is a novel condition temporally associated with SARS-CoV2 infection. Cardiovascular involvement is mainly evident as acute myocardial dysfunction in MIS-C. The aim of this study was to describe the cardiac dysfunction in patients with MIS-C, defining the role of severity in the clinical presentations and outcomes in a single cohort of pediatric patients. Methods A single-center retrospective study on patients diagnosed with MIS-C, according to the Center for Disease Control and Prevention (CDC) definition, and referred to Vittore Buzzi Children’s Hospital in Milan from November 2020 to February 2021. Patients were managed according to a local approved protocol. According to the admission cardiac left ventricular ejection fraction (LVEF), the patients were divided into group A (LVEF

A reduced fruit and vegetable consumption, which implies a decreased intake of antioxidant compounds, seems to play a role in allergic diseases onset. Data on the antioxidant capacity of diet in children with food allergies, who are on an avoidance diet, are still lacking. This pilot study aims to assess the antioxidant potential of diet in Italian children with food allergies, compared to healthy children, using the oxygen radical absorbance capacity (ORAC) method. 95 children (54 with confirmed food allergies and 41 controls), with a median age of 7.8 years, were enrolled and underwent a nutritional assessment. Mean nutrient intakes were compared using the Mann–Whitney test. ORAC resulted significantly lower in allergic children (median 2,908, IQR: 1450;4,716) compared to control children (median 4,392, IQR: 2523;5,836; p = 0.049). Among micronutrients with antioxidant properties, vitamin A intakes were significantly higher in controls than in allergic children. Using Spearman’s correlation, a moderate-to-strong correlation between ORAC and vitamin C, potassium and magnesium was observed (ρ = 0.648, p

BackgroundA severe multisystem inflammatory syndrome in children (MIS-C) related to SARS-CoV-2 has been described after infection. A limited number of reports have analyzed the long-term complications related to pro-inflammatory status in MIS-C. We evaluated multiorgan impairment at the 6-month follow-up in MIS-C.MethodsWe enrolled 33 pediatric patients consecutively hospitalized for MIS-C and monitored for almost 6 months. The inter-relationship of patient's features and disease severity at admission with long term complications was studied by multivariate analysis.ResultsEndo-metabolic derangement, cardiac injury, respiratory, renal and gastrointestinal manifestations and neurological involvement are part of the initial presentation. The most abnormalities appear to resolve within the first few weeks, without significant long term dysfunction at the 6-months follow-up, except for endocrine (non-thyroidal illness syndrome in 12.1%, insulin resistance in 21.2%) and neurological system (27.3% cognitive or psychological, behavioral, adaptive difficulties). Endocrine and heart involvement at admission represent a significant factor on the long term sequelae; however no association between severity score and long-term outcome was noted.ConclusionsThe severity of initial clinical presentation may be associated to organ domain, however it is not related to long term sequelae. The prevalent organ restoration supports a predominant indirect immune-mediated injury triggered by a systemic inflammatory response; however a direct damage due to the viral entry could be not excluded. Eventhought our preliminary results seem to suggest that MIS-C is not a long-term risk condition for children health, a longer follow-up is mandatory to confirm this hypothesis.

Pleuropulmonary blastoma (PPB) is a rare primitive malignant lung cancer that occurs in pediatric age. Its main differential diagnosis is congenital cystic pulmonary malformation (CPAM).A 30-day-old infant with respiratory failure obtained a chest x-ray and a computed tomography scan (CT) which revealed hypertensive pneumothorax with multifocal bilateral cysts. After thoracic drainage, the patient underwent multiple thoracoscopic pulmonary resections. The first histological diagnosis was of type 2 CPAM.During the radiological follow-up, an increase in the number and dimension of the lesions was detected. Thus, a histological revision was performed, leading to the diagnosis of type I PPB, at nine months. The patient subsequently underwent chemotherapy.At the five-year follow-up appointment, chest magnetic resonance (MR) and CT scans showed a dimensional increase in size of the lesions, with the risk of recurrent pneumothorax. An upper right lobectomy and wedge resection of the residual cysts were performed.Control MR scans showed normalization of the lung parenchyma and the patient showed substantial clinical improvement.

Liang-Wang syndrome (LIWAS) is a polymalformative syndrome first described in 2019 caused by heterozygous mutation of the KCNMA1 gene encoding the Ca2+ and voltage-activated K+ channel (BKC). The KCNMA1 variant p.(Gly356Arg) abolishes the function of BKC and blocks the generation of K+ current. The phenotype of this variant includes developmental delay, and visceral and connective tissue malformations. So far, only three cases of LWAS have been described, one of which also had neonatal diabetes (ND). We present the case of a newborn affected by LIWAS carrying the p.(Gly375Arg) variant who manifested diabetes in the first week of life. The description of our case strongly increases the frequency of ND in LIWAS patients and suggests a role of BK inactivation in human insulin secretion. The knowledge on the role of BKC in insulin secretion is very poor. Analyzing the possible mechanisms that could explain the association of LIWAS with ND, we speculate that BK inactivation might impair insulin secretion through the alteration of ion-dependent membrane activities and mitochondrial functions in β-cells, as well as the impaired intra-islet vessel reactivity.

The impact of Coronavirus disease 2019 (COVID-19) on the pediatric population is increasingly recognized. A widespread vaccination in childhood would provide benefits for children and might help ending the pandemic by enhancing community protection. Following recent approval by the European Medicines Agency (EMA) of Comirnaty (Pfizer-BioNTech) for children aged 5–11 years, we aimed to investigate caregivers' intention to vaccinate their children 98%). Three questionnaires were excluded due to compiling errors and 601 were included in the analysis. A total of 311 (51.7%) caregivers stated they would have their child vaccinated, 138 (23%) would refuse to vaccinate their child and 152 (25.3%) were unsure. The intention to vaccinate the child was higher in caregivers vaccinated against COVID-19, in those with a bachelor's degree or higher level of education, and in those with friends/acquaintances who became ill or died due to COVID-19. This study shows that increasing efforts are necessary to provide evidence-based tailored information to caregivers and to promote vaccination in this pediatric age group.

Abstract Background Asthma is a chronic inflammatory disease that is very common among youth worldwide. The burden of this illness is very high not only considering financial costs but also on emotional and social functioning. Guidelines and many researches recommend to develop a good communication between physicians and children/caregiver and their parents. Nevertheless, a previous Italian project showed some criticalities in paediatric severe asthma management. The consensus gathered together experts in paediatric asthma management, experts in narrative medicine and patient associations with the aim of identify simple recommendation to improve communication strategies. Methods Participants to the consensus received the results of the project and a selection of narratives two weeks before the meeting. The meeting was structured in plenary session and in three working groups discussing respectively about communication strategies with children, adolescents and parents. The task of each working group was to identify the most effective (DO) and least effective practices (DON’ T) for 5 phases of the visit: welcome, comprehension of the context, emotions management, duration and end of the visit and endurance of the relationship. Results Participants agreed that good relationships translate into positive outcomes and reached consensus on communication strategies to implement in the different phase of relationships. Conclusions The future challenges identified by the participants are the dissemination of this Consensus document and the implementation of effective communication strategies to improve the management of pediatric asthma.

Introduction: The presentation of eosinophilic myenteric ganglionitis (EMG) can be similar to that of Hirschsprung's disease (HD). In a limited number of cases of pediatric patients, the diagnosis of both EMG and HD are reported. A case of pseudo-obstruction in EMG occurring in a child with HD diagnosis is discussed with literature review.Case Presentation: A boy aged 2 years and 6 months presented with intractable constipation and abdominal distension. Histological HD diagnosis was carried out and transanal Soave pullthrough was performed. At the age of 3 years and 2 months, an infectious enterocolitis occurred. One month later, he presented with constipation, marked abdominal distension and melena. Full thickness colonic biopsies revealed eosinophilic myenteric ganglionitis. Specific IgE tests were positive for several foods. Dietary exclusion was adopted with resolution of clinical symptoms and histologic remission.Conclusion: EMD may occur in patients with HD. At the onset, EMD may be associated with functional intestinal obstruction. The use of an elimination diet proved effective for the relief of symptoms. Long term follow-up is mandatory to define the timing of the reintroduction of foods.

Atopic dermatitis is one of the most frequent chronic skin diseases worldwide and often develops within the first few years of life. Recent advancements in our knowledge of its pathophysiology have brought to light the role of genetic predisposition and environmental triggers. With the increasing prevalence of allergic diseases, there is a strong need for a better understanding of the various modifiable eliciting factors of such conditions. The concomitant rise in food allergy and insights into the skin barrier function has highlighted the role of nutrition and diet in the prevention and modification of allergic disorders. Furthermore, the identification of the skin as an important route of sensitization, and the risk of progression to asthma later in life, stress the significance of optimizing our management of skin inflammation in the prevention of allergies. Many nutritional factors, including the type of maternal diet during pregnancy, the duration of breastfeeding, the epicutaneous exposure of allergenic food proteins in the first few years of life, the timing of the introduction of complementary foods, the supplementation of vitamins and probiotics/prebiotics during prenatal and early life, have been assessed as potential targets for the prevention of atopy and eczema. Here, we review the latest data addressing prenatal and perinatal nutritional and dietary interventions in the primary prevention of atopic dermatitis. Also, we define knowledge gaps and targets for future research in the prevention of atopic dermatitis.

Allergic diseases have been linked to genetic and/or environmental factors, such as antibiotic use, westernized high fat and low fiber diet, which lead to early intestinal dysbiosis, and account for the rise in allergy prevalence, especially in western countries. Allergic diseases have shown reduced microbial diversity, including fewer lactobacilli and bifidobacteria, within the neonatal microbiota, before the onset of atopic diseases. Raised interest in microbiota manipulating strategies to restore the microbial balance for atopic disease prevention, through prebiotics, probiotics, or synbiotics supplementation, has been reported. We reviewed and discussed the role of prebiotics and/or probiotics supplementation for allergy prevention in infants. We searched PubMed and the Cochrane Database using keywords relating to “allergy” OR “allergic disorders,” “prevention” AND “prebiotics” OR “probiotics” OR “synbiotics.” We limited our evaluation to papers of English language including children aged 0–2 years old. Different products or strains used, different period of intervention, duration of supplementation, has hampered the draw of definitive conclusions on the clinical impact of probiotics and/or prebiotics for prevention of allergic diseases in infants, except for atopic dermatitis in infants at high-risk. This preventive effect on eczema in high-risk infants is supported by clear evidence for probiotics but only moderate evidence for prebiotic supplementation. However, the optimal prebiotic or strain of probiotic, dose, duration, and timing of intervention remains uncertain. Particularly, a combined pre- and post-natal intervention appeared of stronger benefit, although the definition of the optimal intervention starting time during gestation, the timing, and duration in the post-natal period, as well as the best target population, are still an unmet need.

Soybean (Glycine max) is a species of legume native to East Asia and used in childhood diet for over 2,000 years in the East. Soy protein formulas have been available for almost a century. Nowadays, the increase in cow's milk allergy and vegetarian dietary preferences are driving consumers toward cow's milk alternatives. In this paper, we reviewed the nutritional composition of soy-based infant formula and discussed their possible use in pediatric age, mainly focusing on prevention and treatment of cow's milk allergy. Protein quality is determined by digestibility and amino acid content. Purified or concentrated vegetable proteins (e.g., soy protein and gluten) have high digestibility (>95%), similar to those of animal ones. For some intact vegetable products (e.g., whole cereals and pulses), protein digestibility is lower (80–90%). Food processing and heat treatment also influence protein digestibility. Considering these data, we tried to evaluate the possible use of soybean and derivatives in pediatric age, including the nutritional composition of soy formulas and the clinical indications for their use. Moreover, since plant-based beverages are being perceived as healthy by consumers and their use is growing on the market, we recommend that soy drink should not be used as a substitute for infant formulas or cow's milk in children younger than 24 months.

Nutrients in foods are not eaten in isolation and food intake interacts in a complex manner, affecting health and disease outcomes. For this reason, focusing on the whole “pattern” of dietary intake instead of the single nutrients or groups of nutrients when studying diseases outcomes is increasingly appealing and growing. Diet diversity refers to the variety of foods being eaten, and the terms, diversity or variety, are often used interchangeably. When the overall diet is characterized by healthy foods, diet diversity will reflect a diversity/variety of healthy foods eaten over a period of time. The introduction of solid foods in the 1st year of life is considered a measure of increased diet diversity. Consuming a diverse range of foods and food allergens in the first year of life may increase intake of important nutrients and positively affect the gut microbiome structure and function. Intake of omega-3 fatty acids and fibers/prebiotics may be particularly important but more information is required about dose and which individuals are most likely to benefit. Increased diet diversity in the first year of life is also associated with reduced food allergy outcomes. In addition to diet diversity, diet indices are considered measures of overall diet quality and can be used as a simple assessment of dietary intake. The focus of this paper is to review and critically address the current knowledge of the association between diet diversity and diet indices and allergy outcomes. Based on the current evidence, we recommend the introduction of solid foods, including common allergenic solids, during the 1st year of life, according to the infant's neuro-developmental abilities and familial or cultural habits. For infants with severe AD and/or FA, medical assessment may be advisable before introducing common food allergens into the diet. Limited evidence exist about the role of diet indices in pregnancy and allergic disease in the offspring, and the most promising results indicate a reduction in childhood wheeze and/or asthma intake.

Asthma is the most frequent chronic disease in children, and its pathogenesis involves genetic, epigenetic, and environmental factors. The rapid rise in the prevalence of asthma registered over the last few decades has stressed the need to identify the environmental and modifiable factors associated with the development of the disease. In particular, there is increasing interest in the role of modifiable nutritional factors specific to both the prenatal and post-natal early life as, during this time, the immune system is particularly vulnerable to exogenous interferences. Several dietary factors, including maternal diet during pregnancy, the duration of breastfeeding, the use of special milk formulas, the timing of the introduction of complementary foods, and prenatal and early life supplementation with vitamins and probiotics/prebiotics, have been addressed as potential targets for the prevention of asthma. In this review, we outline recent findings on the potential role of prenatal and perinatal dietary and nutritional interventions for the primary prevention of pediatric asthma. Moreover, we addressed unmet needs and areas for future research in the prevention of childhood-onset asthma.

Nowadays, food allergies are considered as a wide spectrum of disorders that need different approaches. The “one size fits all” approach is giving way to a “targeted approach,” based on the identification of the patient's phenotype. Thus, the approach of nutritional management of food allergy has moved on from simply being “yes or no” to “how much?”, “in which form?” and “for which patients?” Different factors should be considered in order to make a patient-tailored nutritional plan in clinical practice. Tailored nutritional plans may help to reduce the nutritional, social and economic burden of food allergy.

Abstract The term weaning describes the time period in which a progressive reduction of breastfeeding or the feeding of infant-formula takes place while the infant is gradually introduced to solid foods. It is a crucial time in an infant’s life as not only does it involve with a great deal of rapid change for the child, but it is also associated with the development of food preferences, eating behaviours and body weight in childhood and also in adolescence and adulthood. Therefore, how a child is weaned may have an influence later, on the individual’s entire life. Babies are traditionally first introduced to solid foods using spoon-feeding, in most countries. Beside to traditional approach, an alternative method, promoting infant self-feeding from six months of age, called baby-led weaning or “auto-weaning”, has grown in popularity. This approach causes concern to healthy professionals and parents themselves as data from observational studies pointed out to a potential risk of iron and energy inadequacy as well as choking risk. Aim of this systematic review was to critically examine the current evidence about baby-led weaning approach and to explore the need for future research. A systematic search was conducted in Cochrane library databases and DARE (Database of Abstract of Reviews of Effects), EMBASE and MEDLINE in the period 2000–2018 (up to March 1st) to address some key questions on baby-led weaning. Prisma guidelines for systematic reviews has been followed. After the inclusion/exclusion process, we included for analysis of evidence 12 articles, 10 observational cross-sectional studies and 2 randomized controlled trials. Pooling of results from very different outcomes in the studies included was not possible. Both randomized trials have potential bias; therefore, the quality of the evidence is low. There are still major unresolved issues about baby-led weaning that require answers from research and that should be considered when advices are requested from health professionals by parents willing to approach this method.

Allergic diseases represent a global burden. Although the patho-physiological mechanisms are still poorly understood, epithelial barrier dysfunction and Th2 inflammatory response play a pivotal role. Barrier dysfunction, characterized by a loss of differentiation, reduced junctional integrity, and altered innate defence, underpins the pathogenesis of allergic diseases. Epithelial barrier impairment may be a potential therapeutic target for new treatment strategies Up now, monoclonal antibodies and new molecules targeting specific pathways of the immune response have been developed, and others are under investigation, both for adult and paediatric populations, which are affected by atopic dermatitis (AD), asthma, allergic rhinitis (AR), chronic rhinosinusitis with nasal polyps (CRSwNP), or eosinophilic esophagitis (EoE). In children affected by severe asthma biologics targeting IgE, IL-5 and against IL-4 and IL-13 receptors are already available, and they have also been applied in CRSwNP. In severe AD Dupilumab, a biologic which inhibits both IL-4 and IL-13, the most important cytokines involved in inflammation response, has been approved for treatment of patients over 12 years. While a biological approach has already shown great efficacy on the treatment of severe atopic conditions, early intervention to restore epithelial barrier integrity, and function may prevent the inflammatory response and the development of the atopic march.

Background: Few studies have been carried out with the aim of describing the clinical course and follow-up of patients with tracheomalacia. We aim to describe the symptoms at diagnosis and the post-treatment clinical course of patients affected by airway malacia. Methods: We retrospectively analyzed characteristics of pediatric patients with a diagnosis of airway malacia. Patients were classified into three groups: bronchomalacia (BM), tracheomalacia (TM) and tracheo-bronchomalacia (TBM). Demographic and clinical data, diagnostic work-up and surgical treatment were recorded. Results: 13/42 patients were affected by congenital syndromes (30.9%). Esophageal atresia with or without tracheal-esophageal fistula (EA/TEF) was detected in 7/42 patients (16.7%). Cardiovascular anomalies were found in 9/42 (21.4%) and idiopathic forms in 13/42 (30.9%). BM occurred in 7/42 (16.6%), TM in 23/42 (54.7%) and TBM in 12/42 (28.6%). At the diagnosis stage, a chronic cough was reported in 50% of cases with a higher prevalence in EA/TEF (p = 0.005). Surgery was performed in 16/42 (40%) of children. A chronic cough and acute respiratory failure were correlated to the need for surgery. During follow-up, there was no difference in persistence of symptoms between conservative vs surgical treatment (p = 0.47). Conclusion: the management of tracheomalacia remains a challenge for pediatricians. Clinical manifestations, such as a barking cough and acute respiratory failure may suggest the need for surgery. Follow-up is crucial, especially in those patients affected by comorbidities, so as to be able to manage effectively the possible persistence of symptoms, including those that may continue after surgical treatment.

Historically, the lung was not listed and recognized as a major target organ of diabetic injury. The first evidence of diabetic lung involvement was published fifty years ago, with a study conducted in a population of young adults affected by type 1 diabetes (T1D). In recent years, there has been mounting evidence showing that the lung is a target organ of diabetic injury since the beginning of the disease—at the pediatric age. The deeply branched vascularization of the lungs and the abundance of connective tissue, indeed, make them vulnerable to the effects of hyperglycemia, in a way similar to other organs affected by microvascular complications. In this review, we focus on pulmonary function impairment in children and adolescents affected by T1D. We also cover controversial aspects regarding available studies and future perspectives in this field.

We report a case of growth impairment and nutritional deficiencies in a five-month infant fed by unmodified donkey's milk. We discuss the energy and macronutrient daily intake from donkey's milk and the nutritional consequences that can occur if this kind of milk is used unmodified in the first year of life.