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1.
Eur J Pediatr ; 183(11): 4885-4895, 2024 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-39256240

RESUMEN

Multisystem inflammatory syndrome is a severe complication of SARS-CoV-2 infection in children (MIS-C). To date, data on long-term sequelae mainly concern cardiac outcomes. All ≤ 18 year olds consecutively admitted to the Buzzi Children's Hospital with a diagnosis of MIS-C between October 1, 2020, and May 31, 2022, were followed up for up to 12 months by a dedicated multidisciplinary team. They underwent laboratory tests, multi-organ clinical and instrumental assessments, and psychosocial evaluation. 56/62 patients, 40 M, mean age 8.7 years (95% CI 7.7, 9.7), completed the follow-up. Cardiological, gastroenterological, pneumological, and neurological evaluations, including IQ and EEG, were normal. Alterations of HOMA-IR index and/or TyG index, observed in almost all patients during hospitalisation, persisted in about a third of the population at 12 months. At 6 and 12 months respectively, impairment of adaptive functions was observed in 38/56 patients (67.9%) and 25/56 (44.6%), emotional and behavioural problems in 10/56 (17.9%) and 9/56 (16.1%), and decline in QoL in 14/56 (25.0%) and 9/56 (16.1%). Psychosocial well-being impairment was significantly more frequent in the subgroup with persistent glycometabolic dysfunction at 12 months (75% vs. 40.9% p < 0.001). CONLUSION: The mechanisms that might explain the long-term persistence of both metabolic alterations and neuro-behavioural outcomes and their possible relationship are far from being clarified. Our study points out to the potential long-term effects of pandemics and to the importance of a multidisciplinary follow-up to detect potential negative sequelae in different areas of health, both physical and psychosocial. WHAT IS KNOWN: • Multisystem inflammatory syndrome in children (MIS-C) is a severe complication of SARS-CoV-2 infection. • Few data exist on the medium- and long-term outcomes of MIS-C, mostly focused on cardiac involvement. Emerging evidence shows neurological and psychological sequelae at mid- and long-term follow-up. WHAT IS NEW: • This study reveals that MIS-C may lead to long-term glycometabolic dysfunctions joined to impairment in the realm of general well-being and decline in quality of life, in a subgroup of children. • This study highlights the importance of a long-term multidisciplinary follow-up of children hospitalised with MIS-C, in order to detect the potential long-term sequelae in different areas of health, both physical and psychosocial well-being.


Asunto(s)
COVID-19 , Calidad de Vida , Síndrome de Respuesta Inflamatoria Sistémica , Humanos , COVID-19/psicología , COVID-19/complicaciones , COVID-19/epidemiología , Niño , Síndrome de Respuesta Inflamatoria Sistémica/epidemiología , Síndrome de Respuesta Inflamatoria Sistémica/diagnóstico , Femenino , Masculino , Italia/epidemiología , Estudios de Seguimiento , Adolescente , Preescolar , Hospitales Pediátricos , Centros de Atención Terciaria
2.
J Pediatr Gastroenterol Nutr ; 76(4): 505-511, 2023 04 01.
Artículo en Inglés | MEDLINE | ID: mdl-36689921

RESUMEN

OBJECTIVES: Acute coronavirus disease 2019 infection has been shown to negatively affect body composition among adult and malnourished or obesity children. Our aim is to longitudinally evaluate body composition in children affected by the Multisystem Inflammatory Syndrome (MIS-C). METHODS: In this cohort study, we recruited 40 patients affected by MIS-C, aged 2-18 years old, who were admitted in our clinic between December 2020 and February 2021. Physical examination for each participant included weight, height, body mass index (BMI) z score, circumferences, and skinfolds assessment. The same measurements were repeated during outpatient follow-up at 10 (T2), 30 (T3), 90 (T4), and 180 (T5) days after hospital discharge. Fat mass and fat free mass were calculated according to skinfolds predictive equations for children and adolescents. A control group was randomly selected among patients attending a pediatric nutritional outpatient clinic. RESULTS: BMI z score significantly decrease between preadmission and hospital discharge. Similarly, arm circumference z score, arm muscular area z score, and arm fat area z score significantly decreased, during hospital stay. Fat mass index (FMI) significantly increased over time, peaking at T3. Fat free mass index decreased during hospitalization. CONCLUSIONS: To the best of our knowledge, this is the first study to assess body composition in a numerically large pediatric MIS-C population from acute infection to 6 months after triggering event. FMI and anthropometric parameters linked to fat deposits were significantly higher 6 months after acute event. Thus, limiting physical activity and having sedentary lifestyle may lead to an accumulation of adipose tissue even in healthy children who experienced MIS-C and long hospitalization.


Asunto(s)
COVID-19 , SARS-CoV-2 , Adolescente , Adulto , Niño , Preescolar , Humanos , Antropometría , Composición Corporal , Índice de Masa Corporal , Estudios de Cohortes
3.
Int J Food Sci Nutr ; 73(3): 349-356, 2022 May.
Artículo en Inglés | MEDLINE | ID: mdl-34503383

RESUMEN

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 7.5 and 8.3 years, respectively). Macro- and micronutrient intake was assessed by a three-day food dietary record. Anthropometric measurements were also collected. The median height z-score was significantly lower in the FA group, despite a similar daily energy and protein intake. Calcium, iron and vitamin D intake was suboptimal in both groups, while protein intake was higher than recommended in both groups. Unexpectedly, children with FA consume more protein than controls, while having lower micronutrient intake, especially calcium. Our data suggest the importance of nutritional counseling for children with FA to ensure a balanced nutrient intake while on elimination diet.


Asunto(s)
Calcio , Hipersensibilidad a los Alimentos , Calcio de la Dieta , Estudios de Casos y Controles , Niño , Preescolar , Dieta , Ingestión de Alimentos , Ingestión de Energía , Humanos , Micronutrientes , Estado Nutricional
4.
J Pediatr Gastroenterol Nutr ; 73(4): e98-e104, 2021 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-34091539

RESUMEN

OBJECTIVE: Evaluate accuracy of skinfold thicknesses and body mass index (BMI) for the prediction of fat mass percentage (FM%) in paediatric inflammatory bowel disease (IBD) and to develop population-specific formulae based on anthropometry for estimation of FM%. METHODS: IBD children (n = 30) and healthy controls (HCs, n = 144) underwent anthropometric evaluation and dual-energy X-ray absorptiometry (DEXA) scan, as the clinical reference for measurement of body composition. Body FM% estimated with skinfolds thickness was compared with FM% measured with DEXA. By means of 4 prediction models, population specific formulae for estimation of FM% were developed. RESULTS: No significant difference in terms of FM% measured by DEXA was found between IBD population and HCs (FM% 29.6% vs 32.2%, P = 0.108). Triceps skinfold thickness (TSF, Model 2) was better than BMI (Model 1) at predicting FM% (82% vs 68% of variance). The sum of 2 skinfolds (biceps + triceps; SF2, Model 3) showed an improvement in the prediction of FM% as compared with TSF, Model 2 (86% vs 82% of variance). The sum of 4 skinfolds (biceps + triceps + suprailiac + subscapular; Model 4) showed further improvement in the prediction of FM% as compared with SF2 (88% vs 86% of variance). CONCLUSIONS: The sum of 4 skinfolds is the most accurate in predicting FM% in paediatric IBD. The sum of 2 skinfolds is less accurate but more feasible and less prone to error. The newly developed population-specific formulae could be a valid tool for estimation of body composition in IBD population and an alternative to DEXA measurement.


Asunto(s)
Composición Corporal , Enfermedades Inflamatorias del Intestino , Absorciometría de Fotón , Tejido Adiposo , Antropometría , Índice de Masa Corporal , Niño , Humanos , Enfermedades Inflamatorias del Intestino/diagnóstico , Grosor de los Pliegues Cutáneos
5.
Nutrients ; 16(7)2024 Mar 27.
Artículo en Inglés | MEDLINE | ID: mdl-38612990

RESUMEN

Advancements in food science technology have allowed the development of new products for the therapeutic management of inherited metabolic diseases such as phenylketonuria (PKU). Glycomacropeptide (GMP), a peptide derived from casein, is naturally low in phenylalanine (Phe) and, thus, adequate for protein substitutes (PSs) for the management of PKU in children. This review aims primarily to analyse the differences in the nutritional composition of GMP-based protein substitutes in different formulations (ready to drink, powdered, and bars), and secondarily to assess the quality of these products, comparing their nutritional composition with that of standard amino acid (L-AA) mixtures. Thirty-five GMP-based PSs produced by six different companies were included in this review: twenty-one powdered PSs, eight ready to drink, and six bars. The analysis revealed great heterogeneity not only among the different formulations (powdered, ready to drink, and bars) but also within the same group, in terms of energy content and nutritional composition. GMP-based PSs were shown to have higher contents of sugars and saturated fatty acids compared to L-AA PSs, especially in ready-to-drink formulations and bars. The latter also provided the highest amounts of energy among the GMP-based products. This finding may be related to a higher risk of developing overweight and obesity. The greater palatability of these GMP-based PSs, combined with improved nutritional quality, could not only improve adherence to diet therapy but also reduce the incidence of obesity-related comorbidities in PKU.


Asunto(s)
Caseínas , Fragmentos de Péptidos , Fenilcetonurias , Niño , Humanos , Italia , Obesidad
6.
J Pediatr Endocrinol Metab ; 37(4): 347-352, 2024 Apr 25.
Artículo en Inglés | MEDLINE | ID: mdl-38462926

RESUMEN

OBJECTIVES: A connection between thyroid hormones (THs) and diverse metabolic pathways has been reported. We evaluated thyroid function and tissue sensitivity to THs in children and adolescents with T1D in comparison to euthyroid controls. Additionally, we investigate whether a relationship exists between sensitivity indices and metabolic parameters. METHODS: A retrospective analysis was conducted on 80 pediatric patients diagnosed with T1D. Clinical parameters, TSH, FT3, FT4, and the presence of MS were documented. Additionally, indices of peripheral sensitivity (FT3/FT4 ratio) and central sensitivity (TSH index, TSHI; TSH T4 resistance index, TT4RI; TSH T3 resistance index, TT3RI) were assessed. Thirty healthy subjects were considered as controls. RESULTS: The overall prevalence of MS was 7.27 %, with MS identified in 8 out of 80 (10 %) T1D subjects; none of the controls manifested MS (p<0.01). No significant differences were observed in indexes of tissue sensitivity to THs between subjects with or without MS (all p>0.05). Correlations between THs and indexes of THs tissue sensitivity and metabolic parameters in controls and T1D patients were noted. CONCLUSIONS: This study affirms a heightened prevalence of MS in children with T1D compared to controls and underscores the potential role of THs in maintaining metabolic equilibrium.


Asunto(s)
Diabetes Mellitus Tipo 1 , Síndrome Metabólico , Síndrome de Resistencia a Hormonas Tiroideas , Humanos , Adolescente , Niño , Síndrome Metabólico/epidemiología , Síndrome Metabólico/etiología , Diabetes Mellitus Tipo 1/complicaciones , Triyodotironina , Tiroxina , Estudios Retrospectivos , Tirotropina , Hormonas Tiroideas
8.
Front Pediatr ; 11: 1097152, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37681200

RESUMEN

Introduction: Energy requirements are difficult to estimate in children with cerebral palsy (CP). Resting energy expenditure (REE), necessary to implement personalized nutritional interventions, is most commonly estimated using prediction formulae since indirect calorimetry, the reference method, is not available in all nutrition units. The aims of the present study were: (1) to evaluate the accuracy of the most commonly used REE prediction formulae developed for healthy children, in children with CP; (2) to assess the accuracy of the REE population-specific formula for CP children proposed in our preliminary report; (3) to develop new population-specific methods. Methods: REE was measured by indirect calorimetry in 100 children and adolescents with spastic quadriplegic cerebral palsy (SQCP) and estimated on the basis of predictive formulas selected by the clinicians [World Health Organization (WHO), Harris-Benedict, Schofield weight, Schofield weight & height, Oxford, Mifflin formulae and a population-specific formula for CP children developed in our preliminary report]. Results: 100 children with SQCP (35 girls, 35%) classified as level V according to gross motor function classification system (GMFCS-V); 64% with oral nutrition, 29% total enteral nutrition (nasogastric tube feeding, percutaneous endoscopic gastrostomy, percutaneous endoscopic transgastric jejunostomy) and 7% mixed nutrition. The median (IQR) REE was 41.96 (17.5) kcal/kg/day.Statistical analysis highlighted a proportional bias between the indirect calorimetry and all considered predictive formulae for REE determination. By studying the relationship between the bias and the mean values of REE, specific conversion equations were obtained. With a pre-specified model having as predictors the variable weight and the variable Triceps Skinfold (TSF) and, as response the variable REE measured by indirect calorimetry, a predictive nomogram was developed to estimate the REE in this population of children. Conclusions: We suggest using predictive formulae for healthy children with caution, and where possible carrying out indirect calorimetry to assess REE in children with CP. However, we propose a new tool which could be developed to become an additional help for assessment of REE in the clinical practice.Future objectives will be to obtain a larger sample size, in a multicenter perspective study, to build a specific predictive model for the REE of the studied population.

9.
Front Nutr ; 10: 1096288, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37006933

RESUMEN

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 < 0.001; ρ = 0.645, p < 0.001; ρ = 0.500, p < 0.001, respectively). Iron, phosphorus, vitamin E and vitamin A intakes were also moderately-to-low correlated with ORAC values (ρ = 0.351, p < 0.001; ρ = 0.367, p < 0.001; ρ = 0.346, p < 0.001; and ρ = 0.295, p = 0.004, respectively). We hypothesize that the reduced antioxidant potential of the diet might be related to a reduced variety of the diet in children with food allergies. Our study suggests that the diet of children with food allergies has a lower antioxidant potential (expressed as ORAC value) compared to the diet of healthy children, regardless of the allergenic food excluded from the diet. This issue should be further investigated in prospective, powered studies.

10.
Nutrients ; 16(1)2023 Dec 21.
Artículo en Inglés | MEDLINE | ID: mdl-38201860

RESUMEN

Breastfeeding or standard infant formulas, alongside phenylalanine (Phe)-free protein substitutes, constitute the dietary management for infants with PKU to guarantee protein requirements are met in compliance with metabolic tolerance. This work aims to analyse the nutritional composition of Phe-free infant protein substitutes, in terms of macronutrients, micronutrients and functional components, available for PKU dietary management in Italy. A total of seven infant Phe-free protein substitutes were included in this review, six powder and one liquid. A second analysis was conducted to compare them to the composition of formulas intended for healthy infants, taking into consideration the Commission Delegated Regulation (EU) 2016/127 and Commission Delegated Regulation (EU) 2016/128 for micronutrients. The analysis revealed heterogeneity among protein substitutes suitable for infants with PKU. The energy and protein equivalents (P.Eq.) content are different; all of the substitutes contain docosahexaenoic acid (DHA) and arachidonic acid (ARA), while eicosapentaenoic acid (EPA), fructo-oligosaccharides (FOS), galacto-oligosaccharides (GOS), human milk oligosaccharides (HMOs) and nucleotides are not present in all the substitutes. More attention should be paid to these infant products to ensure metabolic control of PKU, and also promote proper growth, cognitive neurodevelopment, favourable gut microbiota composition, and immune system health, while reducing the risk for non-communicable diseases (NCDs).


Asunto(s)
Nutrientes , Fenilalanina , Lactante , Humanos , Italia , Micronutrientes , Oligosacáridos
11.
J Clin Med ; 11(7)2022 Mar 27.
Artículo en Inglés | MEDLINE | ID: mdl-35407464

RESUMEN

BACKGROUND: Metabolic syndrome (MS) is closely linked to obesity; however, not all individuals with obesity will develop obesity-related complications and a metabolically healthy obesity (MHO) group is also described. OBJECTIVE: To perform a multivariate analysis (MVA) of the anthropometric and biochemical data in pediatric patients with obesity to reveal a "phenotype" predictive for MS. METHODS: We analyzed 528 children with obesity (OB) and 119 normal-weight pediatric patients (NW). Adiposity indices were recorded, and MS was detected. MVA was performed. RESULTS: Analysis of the structure of correlation of the variables showed that the variables of waist circumference (WC), body mass index (BMI), and estimated fat mass (eFM) were positively correlated with each other as a whole. In addition, the variables of the triglycerides (TG), triglyceride-glucose (TyG) index, and visceral adiposity index were positively correlated with each other as a whole, although none were correlated with the variables of BMI z-score, waist-to-height ratio, WC, eFM, or weight. The variables that related to insulin resistance (IR) and dyslipidemia were crucial for the early stratification of patients at risk of MS. CONCLUSIONS: Independently of body weight, IR, dyslipidemia, hypertriglyceridemia, and fat distribution seem to be the strongest MS risk factors. The early detection of and intervention in these modifiable risk factors are useful to protect children's health.

12.
Nutrients ; 14(15)2022 Aug 04.
Artículo en Inglés | MEDLINE | ID: mdl-35956374

RESUMEN

Gut microbiota is a complex system that starts to take shape early in life. Several factors influence the rise of microbial gut colonization, such as term and mode of delivery, exposure to antibiotics, maternal diet, presence of siblings and family members, pets, genetics, local environment, and geographical location. Breastfeeding, complementary feeding, and later dietary patterns during infancy and toddlerhood are major players in the proper development of microbial communities. Nonetheless, if dysbiosis occurs, gut microbiota may remain impaired throughout life, leading to deleterious consequences, such as greater predisposition to non-communicable diseases, more susceptible immune system and altered gut-brain axis. Children with specific diseases (i.e., food allergies, inborn errors of metabolism, celiac disease) need a special formula and later a special diet, excluding certain foods or nutrients. We searched on PubMed/Medline, Scopus and Embase for relevant pediatric studies published over the last twenty years on gut microbiota dietary patterns and excluded case reports or series and letters. The aim of this review is to highlight the changes in the gut microbiota in infants and children fed with special formula or diets for therapeutic requirements and, its potential health implications, with respect to gut microbiota under standard diets.


Asunto(s)
Microbioma Gastrointestinal , Microbiota , Lactancia Materna , Niño , Dieta , Disbiosis , Femenino , Humanos , Lactante
13.
Nutrients ; 14(21)2022 Nov 06.
Artículo en Inglés | MEDLINE | ID: mdl-36364954

RESUMEN

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. All types of exercise (aerobic, resistance and combined training) effectively reduce IR in pediatric patients with obesity; it seems that aerobic and combined training stimulate greater improvements in IR compared to resistance training. Balanced normocaloric or hypocaloric dietary approaches are also valid strategies to address IR; it is not possible to assess the long-term impact of varying macronutrients on cardiometabolic risk. The glycemic index/load evaluation is a useful dietary approach to glucose metabolism control. Similarly, they should adopt the principle of the Mediterranean diet. Randomized studies with longer monitoring are needed to define the benefits of nutritional supplementation on IR. Considering that healthy style acquisition could track to later ages, programs of healthy lifestyle starting with children offer a better preventive strategy to preserve metabolic control and children's health.


Asunto(s)
Resistencia a la Insulina , Obesidad Infantil , Adolescente , Humanos , Niño , Preescolar , Obesidad Infantil/prevención & control , Estilo de Vida , Estilo de Vida Saludable , Ejercicio Físico
14.
Ital J Pediatr ; 48(1): 175, 2022 Sep 15.
Artículo en Inglés | MEDLINE | ID: mdl-36109763

RESUMEN

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.


Asunto(s)
Colestasis , Hipersensibilidad a la Leche , Aminoácidos de Cadena Ramificada , Animales , Conductos Biliares , Bovinos , Femenino , Humanos , Recién Nacido , Masculino , Hipersensibilidad a la Leche/diagnóstico , Hipersensibilidad a la Leche/terapia , Polisacáridos , Triglicéridos
15.
JPGN Rep ; 3(2): e202, 2022 May.
Artículo en Inglés | MEDLINE | ID: mdl-37168918

RESUMEN

Schaaf-Yang syndrome (SYS) is a rare neurodevelopmental disorder whose clinical spectrum includes neurodevelopment delay, dysmorphic features, and gastrointestinal symptoms such as feeding difficulties, gastroesophageal reflux, and chronic constipation. Given the small number of patients diagnosed with this syndrome, our aim is to describe novel clinical features that have not yet been reported. The patient we are describing is a 14-year-old male affected by a severe form of SYS. Initial clinical presentation included respiratory distress at birth, feeding difficulties, and neurodevelopmental delay. Since the age of 8 months, he had been tube fed with a semi-elemental formula, and this was well tolerated. At 9 years of age, the pathological mutation (variant p.Val701fs in MAGEL2 gene) associated with SYS was diagnosed. At 13 years of age, he presented severe gastrointestinal symptoms associated to progressive feeding difficulties. He also suffered from recurrent pancreatitis, late-onset pyloric stenosis and intussusception. Histology showed duodenal villous atrophy with a negative serology for celiac disease. Food protein's hypersensitivity was diagnosed and symptoms resolved after starting an elemental formula.

16.
Nutrients ; 14(9)2022 Apr 20.
Artículo en Inglés | MEDLINE | ID: mdl-35565668

RESUMEN

Since the beginning of the SARS-CoV-2 pandemic, there has been much discussion about the role of diet and antiviral immunity in the context of SARS-CoV-2 infection. Intake levels of vitamins D, C, B12, and iron have been demonstrated to be correlated with lower COVID-19 incidence and mortality. Obesity has been demonstrated to be an independent risk for the severity of COVID-19 infection in adults and also in children. This may be due to different mechanisms, mainly including the gut dysbiosis status observed in obese children. Moreover, the existence of a gut-lung axis added new knowledge to on the potential mechanisms by which diet and dietary substances may affect immune function. The aim of this narrative review is to address the intricate inter-relationship between COVID-19, immune function, and obesity-related inflammation and to describe the role of nutrients and dietary patterns in enhancing the immune system. Two ways to fight against COVID-19 disease exist: one with an antiviral response through immune system boosting and another with antioxidants with an anti-inflammatory effect. In the current pandemic situation, the intake of a varied and balanced diet, rich in micronutrients and bioactive compounds including fibers, should be recommended. However, clinical studies conducted on children affected by SARS-CoV-2 infection and comorbidity are warranted.


Asunto(s)
COVID-19 , Obesidad Infantil , Adulto , Antivirales , Niño , Humanos , Pandemias , Obesidad Infantil/complicaciones , SARS-CoV-2
17.
Front Neurol ; 13: 1072256, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36698902

RESUMEN

Introduction: X-linked adrenoleukodystrophy (X-ALD) is the most common inherited peroxisomal disorder caused by variants in the ABCD1 gene. The main phenotypes observed in men with X-ALD are primary adrenal insufficiency, adrenomyeloneuropathy, and cerebral ALD (cALD). Cerebral ALD consists of a demyelinating progressive cerebral white matter (WM) disease associated with rapid clinical decline and is fatal if left untreated. Hematopoietic stem cell transplantation is the standard treatment for cALD as it stabilizes WM degeneration when performed early in the disease. For this reason, early diagnosis is crucial, and several countries have already implemented their newborn screening programs (NBS) with the assessment of C26:0-lysophosphatidylcholine (C26:0-LPC) values as screening for X-ALD. Methods: In June 2021, an Italian group in Lombardy launched a pilot study for the implementation of X-ALD in the Italian NBS program. A three-tiered approach was adopted, and it involved quantifying the values of C26:0-LPC and other metabolites in dried blood spots with FIA-MS/MS first, followed by the more specific ultra-performance liquid chromatography-tandem mass spectrometry (UHPLC-MS/MS) technique and, finally, the genetic confirmation via focused NGS. Discussion: Genetically confirmed patients are set to undergo a follow-up protocol and are periodically evaluated to promptly start a specific treatment if and when the first signs of brain damage appear, as suggested by international guidelines. A specific disease monitoring protocol has been created based on literature data and personal direct experience. Conclusion: The primary aim of this study was to develop a model able to improve the early diagnosis and subsequent follow-up and timely treatment of X-ALD. Ethics: The study was approved by the local ethics committee. The research was conducted in the absence of any commercial or financial relationship that could be construed as a potential conflict of interest.

18.
Minerva Pediatr (Torino) ; 73(2): 128-149, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-33880904

RESUMEN

In the last few decades, the importance of a functioning immune system and health status has become more evident. Multiple factors are able to influence the development of chronic diseases and diet is one of the most important environmental factors. Evidence demonstrates that dietary patterns high in fat and low in fiber are associated with the development of non-communicable diseases. Moreover, optimal nutritional status can modulate immune maturation and response to inflammation. During inflammatory conditions, nutritional deficiencies may occur, establishing a vicious circle, consequently a balanced nutritional status is essential to prevent and counteract infections. Dietary diversity can prevent allergic diseases and nutrients such as DHA, arginine, vitamins and trace elements have an impact on physical barriers (such as gut mucosal barrier and skin), on the immune system response and on microbiome modulation. Protein deficiencies can compromise innate and adaptive immune functions; arginine availability can affect the immune response in injured states and other disease processes; EPA and DHA can modulate both innate and adaptive immunity; prebiotics have a beneficial effect on the functioning of the immune system. Zinc, copper, selenium and iron are involved in the correct development and function of the immune system. Vitamins D, E, A, B and C have a role on immune system through different mechanisms of action. Since a complex interplay exists between diet, microbiome and epigenetic factors which determine nutrient-induced changes on the immune function, the effect of each single nutrient may be difficult to study. Well-designed intervention studies, investigating the effects of whole dietary pattern, should be performed to clarify impact of foods on the immune function and disease risk.


Asunto(s)
Fenómenos Fisiológicos Nutricionales Infantiles/inmunología , Dieta , Inmunomodulación , Estado Nutricional/inmunología , Inmunidad Adaptativa , Arginina/inmunología , Arginina/metabolismo , Niño , Fibras de la Dieta/metabolismo , Epigénesis Genética/inmunología , Ácidos Grasos Insaturados/inmunología , Ácidos Grasos Insaturados/metabolismo , Microbioma Gastrointestinal/inmunología , Humanos , Hipersensibilidad/prevención & control , Inmunidad Innata , Infecciones/inmunología , Prebióticos , Desnutrición Proteico-Calórica/complicaciones , Desnutrición Proteico-Calórica/inmunología , Oligoelementos/inmunología , Oligoelementos/metabolismo , Vitaminas/inmunología , Vitaminas/metabolismo
19.
Children (Basel) ; 8(11)2021 Oct 26.
Artículo en Inglés | MEDLINE | ID: mdl-34828680

RESUMEN

Body mass index (BMI), usually used as a body fatness marker, does not accurately discriminate between amounts of lean and fat mass, crucial factors in determining metabolic syndrome (MS) risk. We assessed the predictive ability of the estimate of FM (eFM) calculated using the following formula: FM = weight - exp(0.3073 × height2 - 10.0155 ×d-growth-standards/standards/body-mass-index-for-age-bmi-for-age weight- 1 + 0.004571 × weight - 0.9180 × ln(age) + 0.6488 × age0.5 + 0.04723×male + 2.8055) (exp = exponential function, score 1 if child was of black (BA), south Asian (SA), other Asian (AO), or other (other) ethnic origin and score 0 if not, ln = natural logarithmic transformation, male = 1, female = 0), to detect MS in 185 prepubertal obese children compared to other adiposity parameters. The eFM, BMI, waist circumference (WC), body shape index (ABSI), tri-ponderal mass index, and conicity index (C-Index) were calculated. Patients were classified as having MS if they met ≥ 3/5 of the following criteria: WC ≥ 95th percentile; triglycerides ≥ 95th percentile; HDL-cholesterol ≤ 5th percentile; blood pressure ≥ 95th percentile; fasting blood glucose ≥ 100 mg/dL; and/or HOMA-IR ≥ 97.5th percentile. MS occurred in 18.9% of obese subjects (p < 0.001), with a higher prevalence in females vs. males (p = 0.005). The eFM was correlated with BMI, WC, ABSI, and Con-I (p < 0.001). Higher eFM values were present in the MS vs. non-MS group (p < 0.001); the eFM was higher in patients with hypertension and insulin resistance (p < 0.01). The eFM shows a good predictive ability for MS. Additional to BMI, the identification of new parameters determinable with simple anthropometric measures and with a good ability for the early detection of MS, such as the eFM, may be useful in clinical practice, particularly when instrumentation to estimate the body composition is not available.

20.
Nutrients ; 13(12)2021 Nov 25.
Artículo en Inglés | MEDLINE | ID: mdl-34959782

RESUMEN

Children with medical complexities, such as multi-system disorders and/or neurological impairments, often experience feeding difficulties and need enteral nutrition. They frequently have impaired motility and digestive-absorbing functions related to their underlying condition. If a cow's milk allergy (CMA) occurs as a comorbidity, it is often misdiagnosed, due to the symptoms' overlap. Many of the commercialized mixtures intended for enteral nutrition are composed of partially hydrolyzed cow's milk proteins, which are not suitable for the treatment of CMA; thus, the exclusion of a concomitant CMA is mandatory in these patients for obtaining symptoms relief. In this review, we focus on the use of elemental and semi-elemental formulas in children with neurological diseases and in preterm infants as clinical "models" of medical complexity. In children with neurodisabilities, when gastrointestinal symptoms persist despite the use of specific enteral formula, or in cases of respiratory and/or dermatological symptoms, CMA should always be considered. If diagnosis is confirmed, only an extensively hydrolyzed or amino-acid based formula, or, as an alternative, extensively hydrolyzed nutritionally adequate formulas derived from rice or soy, should be used. Currently, enteral formulas tailored to the specific needs of preterm infants and children with neurological impairment presenting concomitant CMA have not been marketed yet. For the proper monitoring of the health status of patients with medical complexity, multidisciplinary evaluation and involvement of the nutritional team should be promoted.


Asunto(s)
Nutrición Enteral/métodos , Fórmulas Infantiles/análisis , Hipersensibilidad a la Leche/terapia , Afecciones Crónicas Múltiples/terapia , Hidrolisados de Proteína/administración & dosificación , Animales , Niño , Preescolar , Errores Diagnósticos , Femenino , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro , Masculino , Hipersensibilidad a la Leche/complicaciones , Hipersensibilidad a la Leche/diagnóstico , Enfermedades del Sistema Nervioso/complicaciones , Hidrolisados de Proteína/análisis
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