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2. Pediatric chronic intestinal failure in italy: Report from the 2016 survey on behalf of Italian society for gastroenterology, hepatology and nutrition (SIGENP)

Author

Diamanti, A, Capriati, T, Gandullia, P, Di Leo, G, Lezo, A, Lacitignola, L, Spagnuolo, M, Gatti, S, D'Antiga, L, Verlato, G, Roggero, P, Amarri, S, Baldassarre, M, Cirillo, F, Elia, D, Boldrini, R, Campanozzi, A, Catassi, C, Aloi, M, Romano, C, Candusso, M, Cecchi, N, Bellini, T, Tyndall, E, Fusaro, F, Caldaro, T, Alberti, D, Gamba, P, Lima, M, Bagolan, P, De Ville de Goyet, J, Dall'Oglio, L, Spada, M, Grandi, F, Diamanti A, Capriati T, Gandullia P, Di Leo G, Lezo A, Lacitignola L, Spagnuolo MI, Gatti S, D'Antiga L, Verlato G, Roggero P, Amarri S, Baldassarre ME, Cirillo F, Elia D, Boldrini R, Campanozzi A, Catassi C, Aloi M, Romano C, Candusso M, Cecchi N, Bellini T, Tyndall E, Fusaro F, Caldaro T, Alberti D, Gamba P, Lima M, Bagolan P, De Ville de Goyet J, Dall'Oglio L, Spada M, Grandi F., Diamanti, A, Capriati, T, Gandullia, P, Di Leo, G, Lezo, A, Lacitignola, L, Spagnuolo, M, Gatti, S, D'Antiga, L, Verlato, G, Roggero, P, Amarri, S, Baldassarre, M, Cirillo, F, Elia, D, Boldrini, R, Campanozzi, A, Catassi, C, Aloi, M, Romano, C, Candusso, M, Cecchi, N, Bellini, T, Tyndall, E, Fusaro, F, Caldaro, T, Alberti, D, Gamba, P, Lima, M, Bagolan, P, De Ville de Goyet, J, Dall'Oglio, L, Spada, M, Grandi, F, Diamanti A, Capriati T, Gandullia P, Di Leo G, Lezo A, Lacitignola L, Spagnuolo MI, Gatti S, D'Antiga L, Verlato G, Roggero P, Amarri S, Baldassarre ME, Cirillo F, Elia D, Boldrini R, Campanozzi A, Catassi C, Aloi M, Romano C, Candusso M, Cecchi N, Bellini T, Tyndall E, Fusaro F, Caldaro T, Alberti D, Gamba P, Lima M, Bagolan P, De Ville de Goyet J, Dall'Oglio L, Spada M, and Grandi F.

Abstract

Background: Intestinal failure (IF) is the reduction in functioning gut mass below the minimal level necessary for adequate digestion and absorption of nutrients and fluids for weight maintenance in adults or for growth in children. There is a paucity of epidemiologic data on pediatric IF. The purpose of this study was to determine the prevalence, incidence, regional distribution and underlying diagnosis of pediatric chronic IF (CIF) requiring home parenteral nutrition (HPN) in Italy. Methods: Local investigators were selected in 19 Italian centers either of reference for pediatric HPN or having pediatric gastroenterologists or surgeons on staff and already collaborating with the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition with regard to IF. Data requested in this survey for children at home on Parenteral Nutrition (PN) on 1 December 2016 included patient initials, year of birth, gender, family’s place of residence and underlying diagnosis determining IF. Results: We recorded 145 CIF patients on HPN aged ≤19 years. The overall prevalence was 14.12/million inhabitants (95% CI: 9.20–18.93); the overall incidence was 1.41/million inhabitant years (95% CI: 0.53–2.20). Conclusion: Our survey provides new epidemiological data on pediatric CIF in Italy; these data may be quantitatively useful in developing IF care strategy plans in all developed countries.

Published
2017

4. Metabolic Disorders in HIV-infected Children Metabolic Disorders in HIVinfected Children

Author

Guarino A, Spagnuolo Mi, and Liguoro I

Subjects
medicine.medical_specialty, medicine.diagnostic_test, business.industry, Leptin, Lipohypertrophy, medicine.disease, Endocrinology, Insulin resistance, Lactic acidosis, Diabetes mellitus, Internal medicine, Medicine, business, Lipid profile, Lipoatrophy, Dyslipidemia
Abstract

The introduction of highly active antiretroviral therapy (HAART) for the treatment of acquired immunodeficiency syndrome (AIDS) has resulted in greater survival of patients infected with the human immunodeficiency virus (HIV). However, the use of these drugs has been associated with lipodystrophic syndrome (LS), which is characterized by metabolic alterations (dyslipidemia, insulin resistance, diabetes, and lactic acidosis) and abnormal corporal fat distribution. Clinically, LS may manifest as three different forms: lipohypertrophy (accumulation of fat in the central part of the body), lipoatrophy (loss of fat in the extremities, face and buttocks) and mixed (lipohypertrophy + lipoatrophy). Although its physiopathology has not been elucidated, some mechanisms have been described, including leptin and adiponectin deficiency, mitochondrial dysfunction and use of antiretroviral drugs. The type, dose and duration of the antiretroviral treatment, as well as age and puberty are the main risk factors. LS are also associated with increased incidence of cardiovascular illnesses, atherosclerosis and diabetes mellitus. Follow up must be periodic, consisting of measurement of body fat distribution, evaluation of the lipid profile and insulin resistance.

Published
2014
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28. Diabetes in an infant with cystic fibrosis.

Author

Lombardi F, Raia V, Spagnuolo MI, Nugnes R, Valerio G, Ciccarelli G, and Franzese A

Abstract

Cystic fibrosis (CF)-related diabetes mellitus is an unusual complication in very young pediatric patients with CF. It is generally associated with more severe clinical manifestations of CF. In this report, we describe a case of diabetes and CF starting in infancy. The patient manifested a form of intermittent diabetes without fasting hyperglycemia, which was exacerbated by steroid treatment during pulmonary disease and occasionally required insulin therapy. Insulin responses to oral and intravenous glucose challenge were low. The clinical and radiological status was stable during the 9-yr follow-up. Our patient demonstrates that diabetes may not only represent a complication of CF, as previously maintained, but can also be a co-morbid condition proceeding along with the exocrine disease. The early occurrence of hyperglycemia in this case may highlight an impairment of beta-cell function, which might be genetically determined. Careful monitoring of the glucose profile and of beta-cell function is indicated in patients with CF to avoid late recognition of diabetes. [ABSTRACT FROM AUTHOR]

Published
2004
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30. Mycobacterium sherrisii visceral disseminated infection in an African HIV-infected adolescent

Author

Andrea Lo Vecchio, Giulia Santoro, Rossella Perna, Francesco Iannelli, Annalisa Del Giudice, Maria Immacolata Spagnuolo, Eugenia Bruzzese, Francesco Santoro, Santoro, F, Santoro, G, Del Giudice, A, Perna, R, Iannelli, F, Spagnuolo, Mi, Bruzzese, E, and Lo Vecchio, A.

Subjects
0301 basic medicine, Microbiology (medical), Adolescent, medicine.drug_class, AIDS-Related Opportunistic Infections, 030106 microbiology, Antibiotics, Biology, lcsh:Infectious and parasitic diseases, 03 medical and health sciences, Moxifloxacin, Clarithromycin, Non-tuberculous mycobacteria, medicine, Humans, lcsh:RC109-216, Abscess, Children, GyrB, Mycobacterium Infections, Clindamycin, HIV, General Medicine, medicine.disease, bacterial infections and mycoses, Anti-Bacterial Agents, Multiple drug resistance, Infectious Diseases, Immunology, Mycobacterium sherrisii, Female, Absce, medicine.drug
Abstract

SummaryA case of visceral disseminated infection by Mycobacterium sherrisii in an African HIV-infected adolescent with multiple abdominal abscesses is reported. Despite multiple drug resistance to first-line antibiotics in vitro, long-term treatment with clarithromycin, moxifloxacin, and clindamycin, together with appropriate antiretroviral treatment, resulted in clinical and radiological cure after 19 months of therapy and follow-up.

Published
2016

31. Factors Affecting Outcome of Tuberculosis in Children in Italy: An Ecological Study

Author

Vania Giacomet, Andrea Lo Vecchio, Eugenia Bruzzese, Emanuele Nicastro, Maria Cristina Fedele, Diana Cerullo, Riccardo Scotto, Maria Immacolata Spagnuolo, Alfredo Guarino, Nicastro E., Scotto R., Cerullo D., Fedele M., Bruzzese E., Giacomet V., Spagnuolo MI., Guarino A., Lo Vecchio A., Nicastro, Emanuele, Scotto, Riccardo, Cerullo, Diana, Fedele, Maria Cristina, Bruzzese, Eugenia, Giacomet, Vania, Spagnuolo, MARIA IMMACOLATA, Guarino, Alfredo, and LO VECCHIO, Andrea

Subjects
Pediatrics, medicine.medical_specialty, 030505 public health, Tuberculosis, business.industry, Incidence (epidemiology), Ecological study, Disease, medicine.disease, Outcome (game theory), Foreign, 03 medical and health sciences, 0302 clinical medicine, Environmental health, Medicine, 030212 general & internal medicine, 0305 other medical science, business, Children, Multiresistant
Abstract

INTRODUCTION: Tuberculosis is a major problem in children depending on their families for management and a re-emerging disease in low incidence countries, where foreign-born cases account for a large proportion of cases. METHODS: We investigated socioeconomic features of families and their impact on management and outcome of children with tuberculosis disease seen at a tertiary care centre for paediatric infectious diseases in Italy. RESULTS: Forty-nine Italian and 30 foreign-origin children were included. Children from foreign families had more complicated diseases (20 % vs 0 %; P = 0.002), harbored more drug resistant strains (20 % vs 2 %; P = 0.011), showed longer hospital stay (12 ± 13.1 vs 5.1 ± 6.5 days; P = 0.012) and higher proportion of missed medical visits (15.7 ± 16 vs 8.6 ± 9.6; P ≤ 0.042) than those from Italian families. Harboring drug resistant strains was an independent risk factor for complicated disease course (OR: 72.98; 95 %CI: 1.54–3468.58; P = 0.029), and this risk is higher in children from Eastern Europe (OR: 10.16; 95 %CI: 1.7–61.9; P = 0.012). CONCLUSIONS: Children from immigrant families showed an increased risk of complicated course of tuberculosis due to a higher rate of resistant strains and raise problems in clinical management. Specific protocols are needed to support these populations ensuring easy access to health services and monitoring.

Published
2016

32. Relationship between severe obesity and gut inflammation in children: what’s next?

Author

Alfredo Guarino, L.R. Assante, Veronica Squeglia, Maria Immacolata Spagnuolo, Adriana Franzese, Rossella Merone, Maria Pia Cicalese, Maria Angela Caiazzo, Giuliana Valerio, Spagnuolo, Mi, Cicalese, Mp, Caiazzo, Ma, Franzese, A, Squeglia, V, Assante, Lr, Valerio, G, Merone, R, Guarino, A., Spagnuolo, MARIA IMMACOLATA, M. P., Cicalese, M. A., Caiazzo, Franzese, Adriana, V., Squeglia, L. R., Assante, G., Valerio, R., Merone, and Guarino, Alfredo

Subjects
Blood Glucose, Male, INSULIN-RESISTANCE, ADIPOSE-TISSUE, BOWEL-DISEASE, CALPROTECTIN, Body Mass Index, metabolism, Leukocyte L1 Antigen Complex, Feces, metabolism, Rectum, Medicine, Child, biology, diagnosis, Fece, digestive, oral, and skin physiology, lcsh:RJ1-570, metabolism, Body Mass Index, C-Reactive Protein, medicine.anatomical_structure, C-Reactive Protein, analysis, Blood Glucose, Female, medicine.symptom, chemistry, Female, Glucose Tolerance Test, Humans, Hyperinsulinism, metabolism, Obesity, Type 2, Gut inflammation, Rectum, Inflammation, diagnosis, Inflammation, Nitric Oxide, digestive system, Diabetes mellitus, Hyperinsulinism, Humans, Obesity, metabolism, Child, Diabetes Mellitu, business.industry, Research, C-reactive protein, lcsh:Pediatrics, Glucose Tolerance Test, medicine.disease, Diabetes Mellitus, Type 2, Biological Marker, Immunology, biology.protein, business, metabolism, Male, Nitric Oxide, metabolism, Body mass index, Leukocyte L1 Antigen Complex, Biomarkers
Abstract

Background Preliminary evidence suggests an association between obesity and gut inflammation. Aims To evaluate the frequency of glucose abnormalities and their correlation with systemic and intestinal inflammation in severely obese children. Patients and Methods Thirty-four children (25 males; median age 10.8 ± 3.4 yrs) with severe obesity (BMI >95%) were screened for diabetes with oral glucose tolerance test (OGTT), systemic inflammation with C-reactive protein (CRP) and gut inflammation with rectal nitric oxide (NO) and faecal calprotectin. Results BMI ranged from 23 to 44 kg/m2, and BMI z-score between 2.08 e 4.93 (median 2.69 ± 0.53). Glucose abnormalities were documented in 71% of patients: type 2 diabetes in 29%, impaired fasting glucose (IFG) in 58%, and impaired glucose tolerance (IGT) in 37.5%. Thirty-one patients (91%) were hyperinsulinemic. CRP was increased in 73.5% with a correlation between BMI z-score and CRP (p 0.03). Faecal calprotectin was increased in 47% patients (mean 77 ± 68), and in 50% of children with abnormal glucose metabolism (mean 76 ± 68 μg/g), with a correlation with increasing BMI z-score. NO was pathological in 88%, and in 87.5% of glucose impairment (mean 6.8 ± 5 μM). Conclusions In this study, the prevalence of glucose abnormalities in obese children is higher than in other series; furthermore, a correlation is present between markers of systemic and intestinal inflammation and glucose abnormalities.

Published
2010

33. Requesting iodine supplementation in children on parenteral nutrition

Author

Maria Immacolata Spagnuolo, M.P. Cicalese, Eugenia Bruzzese, Alfredo Guarino, Cicalese, Mp, Bruzzese, Eugenia, Guarino, Alfredo, Spagnuolo, MARIA IMMACOLATA, Bruzzese, E, Guarino, A, and Spagnuolo, Mi

Subjects
Male, Short Bowel Syndrome, medicine.medical_specialty, Parenteral Nutrition, Adolescent, Thyroid Gland, chemistry.chemical_element, Nutritional Status, Clinical nutrition, Urine, Critical Care and Intensive Care Medicine, Iodine, Gastroenterology, Internal medicine, medicine, Humans, Prospective Studies, Child, Nutrition and Dietetics, Dose-Response Relationship, Drug, business.industry, Thyroid, Nutritional Requirements, Infant, Micronutrient, medicine.disease, Short bowel syndrome, Iodine deficiency, Surgery, Intestinal Diseases, Parenteral nutrition, medicine.anatomical_structure, chemistry, Child, Preschool, Dietary Supplements, Female, Parenteral Nutrition, Total, business, Child Nutritional Physiological Phenomena
Abstract

AIMS: Iodine supplementation of parenterally fed infants recommended by ESPGHAN is 1 microg/kg/day. To assess nutritional and thyroid status of children on parenteral nutrition (PN) through urinary iodine concentration (UIC). PATIENTS AND METHODS: Children (1-17 yrs), undergoing PN and receiving an iodine supply of 1 microg/kg/day, were enrolled from 2000 to 2007. RESULTS: We observed 15 children (10 males, mean age 76.53+/-60.4 months) on PN from 14 to 84 weeks (mean 38.5+/-21.4). Ten were on TPN and five on PPN; nine had short bowel syndrome (SBS) and six had other intestinal diseases requiring PN. Iodine supply in TPN ranged between 1 and 1.6 microg/kg/day (mean 1.1+/-0.3 microg/kg/day), while in PPN it ranged from 2.3 to 2.8 microg/kg/day (mean 2.6+/-0.7 microg/kg/day). We found an inverse correlation between duration of PN in months and UIC (P=0.05). Four weeks after PN onset, UIC

Published
2008

34. Role of physical activity/exercise in the care of children and adolescents with type 1 diabetes

Author

Valerio, G., Maria Immacolata Spagnuolo, Franzese, A., Valerio, G., Spagnuolo, M. I., Franzese, A., 2., Valerio G, Spagnuolo, Mi, and Franzese, Adriana

Subjects
Glycosylated haemoglobin, Type 1 diabetes, Fitne, physical activity, Type 1 diabetes mellitus, Therapy, Exercise, exercise
Abstract

In the last few years the importance of physical activity/exercise in the treatment of type 1 diabetes has been clearly recognized. When associated with normocaloric diet and flexible self management, regular physical activity helps maintain weight, reduces insulin requirement and cardiovascular risk factors, in addition to improving socialization and self-esteem. The effect of physical activity on glucose control is debated, since it depends on a wise adaptation to exercise of carbohydrate intake, insulin dose and glucose self monitoring. Barriers to physical activity in patients with diabetes include fear of severe metabolic derangement. Paediatric diabetologists should develop strategies that allow young subjects with diabetes to participate in a safe and enjoyable manner in activities that are developmentally appropriate.

Published
2006

35. Adrenocortical tumor in a boy: final height is not impaired despite a severe advancement of bone age

Author

G. Muzzi, Giuliana Valerio, P Buono, A. Franzese, R. Palmieri, Maria Immacolata Spagnuolo, Francesca Lombardi, Valerio, G, Spagnuolo, Mi, Muzzi, G, Buono, P, Lombardi, F, Palmieri, R, and Franzese, Adriana

Subjects
Adenoma, Male, medicine.medical_specialty, Endocrinology, Diabetes and Metabolism, Adrenocortical Tumor, Physiology, Endocrinology, Medicine, Humans, Testosterone, Adrenal tumors, Bone Development, business.industry, Virilization, 17-alpha-Hydroxyprogesterone, Final height, Bone age, Skeletal maturity, Prognosis, Adrenal Cortex Neoplasms, Body Height, Surgery, Child, Preschool, Pediatrics, Perinatology and Child Health, medicine.symptom, business
Abstract

The long-term sequelae on the growth pattern in successfully resected virilizing adrenal tumors (ACT) have not been clearly defined. We report on 10 years follow-up of a boy with virilizing ACT until the attainment of final height. This is the first clinical description in a boy with a marked advancement of bone age, indicating that despite advanced physical and skeletal maturity the prognosis on growth is good, provided that regression of virilization is obtained.

Published
2003

36. Ursodeoxycholic acid for treatment of cholestasis in children on long term Total Parenteral Nutrition: a pilot study

Author

Angela Vegnente, Maria Immacolata Spagnuolo, Raffaele Iorio, Alfredo Guarino, Spagnuolo, Mi, Iorio, R, Guarino, A, Vegnente, Angela, Spagnuolo, MARIA IMMACOLATA, Iorio, Raffaele, Vegnente, A, and Guarino, Alfredo

Subjects
Parenteral Nutrition - Associated Cholestasis, Male, Resuscitation, medicine.medical_specialty, Time Factors, medicine.medical_treatment, Pilot Projects, Gastroenterology, Cholestasis, Gastrointestinal Agents, Internal medicine, medicine, Humans, Chemotherapy, Hepatology, business.industry, Ursodeoxycholic Acid, Infant, Alanine Transaminase, Bilirubin, gamma-Glutamyltransferase, medicine.disease, Alkaline Phosphatase, Ursodeoxycholic acid, Parenteral nutrition, Treatment Outcome, Alkaline phosphatase, Parenteral Nutrition, Total, Complication, business, medicine.drug
Abstract

BACKGROUND & AIMS: Cholestatic liver disease (CLD) is a frequent and sometimes fatal complication of total parenteral nutrition (TPN) that may require withdrawal of TPN. The aim of this pilot study was to evaluate ursodeoxycholic acid (UDCA) as treatment of TPN-associated CLD. METHODS: Seven children (4 boys and 3 girls) undergoing long-term TPN because of intractable diarrhea syndrome developed cholestasis and were treated with UDCA. Treatment efficacy was evaluated by monitoring clinical and biochemical markers of CLD, including gamma-glutamyl transpeptidase (GGT), alkaline phosphatase (ALP), conjugated bilirubin, and alanine aminotransferase (ALT). RESULTS: In all children, UDCA was associated with the disappearance of signs of CLD and with normalization of biochemical markers of cholestasis within 4-8 weeks. A rebound increase of GGT, ALP, and ALT serum levels was observed in 3 children in whom UDCA was temporarily discontinued while they were still undergoing TPN. However, after reinstitution of UDCA, markers of cholestasis normalized in all cases. UDCA was withdrawn on reinstitution of full oral feeding; there was no relapse of cholestasis. Six children fully recovered. One child died because of the lack of vascular access. CONCLUSIONS: UDCA appears to be an effective treatment for TPN-related cholestasis in children. (Gastroenterology 1996 Sep;111(3):716-9)

Published
1996

37. Successful treatment of acute liver failure due to Wilson's disease: Serendipity or fortuity?

Author

Delle Cave V, Di Dato F, Calvo PL, Spagnuolo MI, and Iorio R

Abstract

Background: Acute liver failure (ALF) may be the first and most dramatic presentation of Wilson's disease (WD). ALF due to WD (WD-ALF) is difficult to distinguish from other causes of liver disease and is a clear indication for liver transplantation. There is no firm recommendation on specific and supportive medical treatment for this condition., Aim: To critically evaluate the diagnostic and therapeutic management of WD-ALF patients in order to improve their survival with native liver., Methods: A retrospective analysis of patients with WD-ALF was conducted in two pediatric liver units from 2018 to 2023., Results: During the study period, 16 children (9 males) received a diagnosis of WD and 2 of them presented with ALF. The first was successfully treated with an unconventional combination of low doses of D-penicillamine and zinc plus steroids, and survived without liver transplant. The second, exclusively treated with supportive therapy, needed a hepatotransplant to overcome ALF., Conclusion: Successful treatment of 1 WD-ALF patient with low-dose D-penicillamine and zinc plus steroids may provide new perspectives for management of this condition, which is currently only treated with liver transplantation., Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article., (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)

Published
2024
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38. Association of Very Rare NOTCH2 Variants with Clinical Features of Alagille Syndrome.

Author

Ferrandino M, Cardiero G, Di Dato F, Cerrato Y, Vitagliano L, Mandato C, Morisco F, Spagnuolo MI, Iorio R, Di Taranto MD, and Fortunato G

Subjects
Humans, Male, Female, Infant, Phenotype, Child, Child, Preschool, High-Throughput Nucleotide Sequencing, Jagged-1 Protein genetics, Mutation, Missense, Mutation, Adolescent, Alagille Syndrome genetics, Receptor, Notch2 genetics
Abstract

Background: Alagille syndrome (ALGS) is a rare autosomal dominant genetic disease caused by pathogenic variants in two genes: Jagged Canonical Notch Ligand 1 ( JAG1 ) and Notch Receptor 2 ( NOTCH2 ). It is characterized by phenotypic variability and incomplete penetrance with multiorgan clinical signs., Methods: Using Next Generation Sequencing (NGS), we analyzed a panel of liver-disease-related genes in a population of 230 patients with cholestasis and hepatopathies. For the rare variants, bioinformatics predictions and pathogenicity classification were performed., Results: We identified eleven rare NOTCH2 variants in 10 patients, two variants being present in the same patient. Ten variants had never been described before in the literature. It was possible to classify only two null variants as pathogenic, whereas the most of variants were missense (8 out of 11) and were classified as uncertain significance variants (USVs). Among patients with ALGS suspicion, two carried null variants, two carried variants predicted to be pathogenic by bioinformatics, one carried a synonymous variant and variants in glycosylation-related genes, and two carried variants predicted as benign in the PEST domain., Conclusions: Our results increased the knowledge about NOTCH2 variants and the related phenotype, allowing us to improve the genetic diagnosis of ALGS.

Published
2024
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39. Bioactives in Oral Nutritional Supplementation: A Pediatric Point of View.

Author

Cecchi N, Romanelli R, Ricevuti F, Carbone MG, Dinardo M, Cesarano E, De Michele A, Messere G, Morra S, Scognamiglio A, and Spagnuolo MI

Subjects
Child, Humans, Administration, Oral, Child Nutritional Physiological Phenomena, Pediatrics, Phytochemicals administration & dosage, Phytochemicals pharmacokinetics, Probiotics administration & dosage, Vitamins administration & dosage, Biological Availability, Dietary Supplements
Abstract

Background: Oral nutritional supplements (ONSs) are crucial for supporting the nutritional needs of pediatric populations, particularly those with medical conditions or dietary deficiencies. Bioactive compounds within ONSs play a pivotal role in enhancing health outcomes by exerting various physiological effects beyond basic nutrition. However, the comprehensive understanding of these bioactives in pediatric ONSs remains elusive., Objective: This systematic narrative review aims to critically evaluate the existing literature concerning bioactive compounds present in oral nutritional supplements from a pediatric standpoint, focusing on their types, sources, bioavailability, physiological effects, and clinical implications., Methods: A systematic search was conducted across the major academic databases, including PubMed, Scopus, and Web of Science, employing predefined search terms related to oral nutritional supplements, bioactives, and pediatrics. Studies published between 2013 and 2024 were considered eligible for inclusion. Data extraction and synthesis were performed according to the PRISMA guidelines., Results: The initial search yielded 558 of articles, of which 72 met the inclusion criteria. The included studies encompassed a diverse range of bioactive compounds present in pediatric ONS formulations, including, but not limited to, vitamins, minerals, amino acids, prebiotics, probiotics, and phytonutrients. These bioactives were sourced from various natural and synthetic origins and were found to exert beneficial effects on growth, development, immune function, gastrointestinal health, cognitive function, and overall well-being in pediatric populations. However, variations in bioavailability, dosing, and clinical efficacy were noted across different compounds and formulations., Conclusions: Bioactive compounds in oral nutritional supplements offer promising avenues for addressing the unique nutritional requirements and health challenges faced by pediatric populations. However, further research is warranted to elucidate the optimal composition, dosage, and clinical applications of these bioactives in pediatric ONS formulations. A deeper understanding of these bioactive compounds and their interplay with pediatric health may pave the way for personalized and effective nutritional interventions in pediatric clinical practice.

Published
2024
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40. Trends and challenges in home enteral feeding methods for children with gastrointestinal disorders: an expert review on bolus feeding delivery methods.

Author

Romano C, Lionetti P, Spagnuolo MI, Amarri S, Diamanti A, Verduci E, Lezo A, and Gatti S

Subjects
Humans, Child, Nutritional Status, Child, Preschool, Infant, Enteral Nutrition methods, Quality of Life, Home Care Services, Gastrointestinal Diseases therapy, Gastrointestinal Diseases diagnosis
Abstract

Introduction: New evidence supports the benefits of bolus feeding for children receiving home enteral feeding (HEN). Current home methods of bolus feeding have certain limitations, particularly in mobile or restless patients. Therefore, innovative delivery methods have been introduced to provide more flexible methods of reducing feeding time and formula handling., Areas Covered: This manuscript presents an expert review of the updates in HEN for children and the results of an online user experience questionnaire about an innovative new cap-based bolus feeding system. A literature bibliographic search was conducted on Medline via PubMed up to September 2023 to collect relevant studies. We presented recent evidence demonstrating a dramatic increase in HEN use among children requiring EN and its benefits on patients' nutritional status and quality of life. In addition, the article examined the clinical and social benefits of bolus feeding and current challenges in delivery methods. We described the benefits of the new system and its user experience., Expert Opinion: The uses and indications for bolus feeding in HEN are increasing among children. However, there are still some unmet needs regarding traditional delivery methods. Innovative techniques can improve flexibility, reduce feeding time, and improve user experience and quality of life.

Published
2024
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41. Intractable diarrhea in infancy and molecular analysis: We are beyond the tip of the iceberg.

Author

Diamanti A, Trovato CM, Gandullia P, Lezo A, Spagnuolo MI, Bolasco G, Capriati T, Lacitignola L, Norsa L, Francalanci P, and Novelli A

Subjects
Humans, Infant, Newborn, Intestines, Italy epidemiology, Nutritional Status, Male, Female, Multicenter Studies as Topic, Diarrhea genetics, Inflammatory Bowel Diseases complications
Abstract

Background: Intractable diarrhea (ID) could be defined as a syndrome of severe chronic diarrhea associated with malnutrition not easily resolved by conventional management., Aims: To provide an overview on etiology and management of ID patients in Italy in the last 12 years., Methods: The members of Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) enrolled all ID patients seen between January 1, 2011 and December 31, 2022., Results: 69 children were enrolled (49 M, 20 F; median age at ID onset 9.5 days) from 7 tertiary care pediatric centers. Overall 62 patients had genetic diseases; 3 had infantile Inflammatory Bowel Disease and 1 autoimmune enteropathy in absence of genetic mutations; 2 undefined ID. Defects of intestinal immune-related homeostasis caused ID in 29 patients (42 %)., Conclusion: ID is a rare but challenging problem, although the potential for diagnosis has improved over time. In particular, molecular analysis allowed to identity genetic defects in 90 % of patients and to detect new genetic mutations responsible for ID. Due to both the challenging diagnosis and the treatment for many of these diseases, the close relationship between immune system and digestive tract should require a close collaboration between pediatric immunologists and gastroenterologists, to optimize epidemiologic surveillance and management of ID., Competing Interests: Conflict of interest There is no conflict of interest associated with any of the senior author or other co-authors contributed their efforts in this manuscript., (Copyright © 2023 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

Published
2024
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42. Novel insect-based child nutrition: the position of the nutritional committee of the Italian society of pediatric gastroenterology, hepatology and nutrition (SIGENP).

Author

Norsa L, Agostoni C, Capriati T, Campanozzi A, Diamanti A, Lezo A, Gandullia P, Spagnuolo MI, and Romano C

Subjects
Child, Humans, Italy, Nutritional Status, Child Nutritional Physiological Phenomena, Societies, Medical, Gastroenterology
Abstract

Background: The European Union (EU) approved the placement on European market of insect-based novel foods. Those foods were defined safe for the consumption for all European population, including children., Main Body: The nutrition committee of the Italian society of Paediatric Hepatology and Nutrition (SIGENP) performed literature research to understand benefits and risk of those use of those NF for Italian children. A special attention was reserved to the European Food Safety Agency (EFSA) reports upon which those novel insect-based were approved., Conclusions: Based on the current knowledge, despite a possible ecological advantage, the group of expert suggests additional researches before pronouncing on a possible use for children diet, because of insufficient evidence on nutritional benefits and possible food allergies., (© 2023. The Author(s).)

Published
2023
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45. Effects of COVID-19 pandemic on pediatric tuberculosis: decrease in notification rates and increase in clinical severity.

Author

Lo Vecchio A, Scarano SM, Amato C, Spagnuolo MI, Bruzzese E, and Guarino A

Subjects
Child, Humans, Female, Adolescent, Child, Preschool, Male, Pandemics prevention & control, Cohort Studies, Delayed Diagnosis, Disease Notification, COVID-19 epidemiology, Tuberculosis diagnosis, Tuberculosis epidemiology, Tuberculosis prevention & control, Respiratory Insufficiency epidemiology
Abstract

The outbreak of COVID-19 resulted in a decrease in tuberculosis notification rates globally. We compared tuberculosis incidence rates and disease severity in children seen in our centre prior and during COVID-19 pandemic.We performed a cohort study enrolling children aged under 18 years who received a diagnosis of tuberculosis (January 1st, 2010-December 31st, 2021) at our Pediatric Infectious Diseases Unit. Disease severity was evaluated based on: the classification proposed by Wiseman et al., smear positivity, presence of symptoms at presentation, lung cavitation, extrapulmonary disease, respiratory failure and need for intensive care support. Overall, 168 children (50.6% female, median age 69 months, IQR 95.4) received a diagnosis of tuberculosis, 156 (92.8%) between 2010-2019, before COVID-19 outbreak, and 12 (7.2%) between 2020-2021, during the pandemic. The annual tuberculosis notification rate dropped by 73% in 2021 (0.38/100000, 95%CI 0.1-0.96) compared with 2019 (1.46/100000, 95%CI 0.84-2.37). Compared to the pre-pandemic period, the proportion of children classified as severe was higher in 2020-2021 (5, 41.6% vs 23, 15.7%, p = 0.006) with a higher rate of respiratory failure (2, 16.7%, vs 4, 2.6%, p = 0.01) and an increased need for intensive care support (1, 8.3% vs 1, 0.6%, p = 0.01).   Conclusion: During COVID-19 pandemic we observed a reduction in tuberculosis notification rate in pediatric population and a significant increase in disease severity. This scenario may be the consequence of a delay in diagnosis and an underreporting of cases, rather than the effect of a reduced transmission of tuberculosis. Children reached health-care services only in the need of urgent medical attention. What is Known: • COVID-19 pandemic had a huge impact on national health care systems, resulting in a reduction of access to medical care. What is New: • In Campania Region, Italy, a low tuberculosis incidence country, we witnessed a 75% reduction in tuberculosis notification rate during pandemic. In parallel we demonstrated a significant increase in disease severity, suggesting that the reduction in notification rate may be attributed to an underreporting of cases and consequential diagnostic delay, rather than a reduced transmission of infection., (© 2023. The Author(s).)

Published
2023
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47. COVID-19 infection in patients on long-term home parenteral nutrition for chronic intestinal failure.

Author

Pironi L, Jezerski D, Sobocki J, Lal S, Vanuytsel T, Theilla M, Sasdelli AS, Chambrier C, Matysiak K, Aimasso U, Rasmussen HH, Jukes A, Kunecki M, Seguy D, Schneider SM, Daniels J, Poullenot F, Mundi MS, Matras P, Folwarski M, Crivelli A, Wyer N, Ellegard L, Santarpia L, Arvanitakis M, Spaggiari C, Lamprecht G, Guglielmi FW, Lezo A, Layec S, Boluda ER, Guz-Mark A, Gandullia P, Cuerda C, Osland E, Spagnuolo MI, Krznaric Z, Masconale L, Chapman B, Maíz-Jiménez M, Orlandoni P, Martins da Rocha MH, Virgili-Casas MN, Doitchinova-Simeonova M, Czako L, Van Gossum A, D'Antiga L, Ee LC, Warodomwichit D, Taus M, Kolaček S, Thibault R, Verlato G, Serralde-Zúñiga AE, Botella-Carretero JI, Aguayo PS, Olveira G, Chomtho S, Pisprasert V, Moisejevs G, Murillo AZ, Jáuregui MEP, Díez MB, Jahit MS, Densupsoontorn N, Tamer A, Brillanti G, and Joly F

Subjects
Humans, COVID-19 epidemiology, Intestinal Failure, Intestinal Diseases epidemiology, Intestinal Diseases therapy, Parenteral Nutrition, Home adverse effects
Abstract

Background and Aims: To investigate the incidence and the severity of COVID-19 infection in patients enrolled in the database for home parenteral nutrition (HPN) for chronic intestinal failure (CIF) of the European Society for Clinical Nutrition and Metabolism (ESPEN)., Methods: Period of observation: March 1st, 2020 March 1st, 2021., Inclusion Criteria: patients included in the database since 2015 and still receiving HPN on March 1st, 2020 as well as new patients included in the database during the period of observation. Data related to the previous 12 months and recorded on March 1st 2021: 1) occurrence of COVID-19 infection since the beginning of the pandemic (yes, no, unknown); 2) infection severity (asymptomatic; mild, no-hospitalization; moderate, hospitalization no-ICU; severe, hospitalization in ICU); 3) vaccinated against COVID-19 (yes, no, unknown); 4) patient outcome on March 1st 2021: still on HPN, weaned off HPN, deceased, lost to follow up., Results: Sixty-eight centres from 23 countries included 4680 patients. Data on COVID-19 were available for 55.1% of patients. The cumulative incidence of infection was 9.6% in the total group and ranged from 0% to 21.9% in the cohorts of individual countries. Infection severity was reported as: asymptomatic 26.7%, mild 32.0%, moderate 36.0%, severe 5.3%. Vaccination status was unknown in 62.0% of patients, non-vaccinated 25.2%, vaccinated 12.8%. Patient outcome was reported as: still on HPN 78.6%, weaned off HPN 10.6%, deceased 9.7%, lost to follow up 1.1%. A higher incidence of infection (p = 0.04), greater severity of infection (p < 0.001) and a lower vaccination percentage (p = 0.01) were observed in deceased patients. In COVID-19 infected patients, deaths due to infection accounted for 42.8% of total deaths., Conclusions: In patients on HPN for CIF, the incidence of COVID-19 infection differed greatly among countries. Although the majority of cases were reported to be asymptomatic or have mild symptoms only, COVID-19 was reported to be fatal in a significant proportion of infected patients. Lack of vaccination was associated with a higher risk of death., Competing Interests: Conflict of interest statements LP: Participation on a Data Safety Monitoring Board or Advisory Board for Takeda, Consulting fees for Takeda, Northsea, NAPO. SL: Participation on a Data Safety Monitoring Board or Advisory Board for Baxter, Takeda, NorthSea, VectivBio; Grants or contracts from any entity for Baxter, Takeda; Consulting fees for VectivBio, Takeda, Northsea; Support for attending meetings and/or travel for Takeda; Payment or honoraria for lectures for Takeda, Fresenius. PG: none. LS: none. PO: none. NW: none. RT: Royalties or licenses for Royalties for designing the Simple Evaluation of Food Intake® (SEFI®) (Knoë, le Kremlin Bicêtre, France); Consulting fees for Nestlé Health Science; Payment or honoraria for lectures for Baxter, BBraun, Fresenius-Kabi, Nutricia; Support for attending meetings for Nutricia, NHC. PS: none. LE: none. PO: none. L D’A: none. AT: none. ND: Leadership of Pediatric Nutrition Association of Thailand Society of Parenteral Enteral Nutrition of Thailand. ASZ: Payment or honoraria for lectures for Siegfried; Consulting for Takeda; Support for attending meetings for Abbott and Nestlè. MF: Payment or honoraria for lectures for Fresenius Kabi, B Braun, Baxter. GV: none. MIS: none. MT: none. ERB: none. NVC: Payment or honoraria for lectures for Takeda, Nutricia; Payment for expert testimony, Support for attending meetings and Participation on a Data Safety Monitoring Board for Takeda. AL: Consulting fees, Support for attending meetings, Participation on a Data Safety Monitoring Board or Advisory Board for Nestlè; Participation on a Data Safety Monitoring Board or Advisory Board for Takeda; Payment or honoraria for lectures for baxter. LC: none. MA: none. EO: none. AGM: none. AVG: none. VP: honoraria for lectures for Thai Otsuka Pharmaceutical Co., Ltd., Abbott Laboratories Ltd., Nestle (Thai) Ltd., Fresenius Medical Care (Thailand) Ltd., Baxter Healthcare (Thailand) Co., Ltd., Mega Lifesciences PTY Ltd., Novo Nordisk Thailand. MSM: Grants or contracts from any entity for Fresenius Kabi, Nestle, Realfood Blends, VectivBio, Rockfield, Zealand; Consulting fees, Northsea; Participation on a Data Safety Monitoring Board for EndoBarrier. M D-S: none. TV: Grants or contracts from any entity for Vectiv Bio, Takeda; Consulting fees for Vectiv Bio, Zealand Pharma, Takeda, Baxter, Hamni, NorthSea Therapeutics; Payment or honoraria for lectures for Vectiv Bio, Takeda, Baxter; Support for attending meetings for Takeda, Vectiv Bio, Zealand Pharma, Fresenius Kabi; Receipt of equipment, materials, drugs for VectivBio. ZK: Support for attending meetings for Abbott, Fresenius, Nutricia, Nestle, Takeda; Leadership for Croatian Medical Association- The President. FP: none. LM: none. LCE: Consulting fees, Payment or honoraria for lecture and Support for attending meetings for Takeda. UA: Payment or honoraria for lectures for Takeda, Baxter; Support for attending meetings and Participation on a Data Safety Monitoring for Takeda. MK: none. MMJ: none. AC: none. DW: none. GO: none. CC: none. JS: Grants or contracts from any entity and for BBraun, FreseniusKabi, Nestle; Payment or honoraria for lectures for BBraun, OlimpLabs, FreseniusKabi, Baxter, Nestle; Support for attending meetings for FreseniusKabi. FWG: none. CS: none. MBD: none. DS: none. SL: none. SK: Payment or honoraria for lectures, for Abbott, Abela Farm, Danone/Nutricia, Fresenius, GM Pharma, Nestle, Nestle Nutrition Institute, Oktal Pharma, Shire/Takeda; Non-restricted grant delivered to the hospital from BioGaia. BC: none. GM: none. MHMdC: Grants or contracts, Consulting fees, Payment or honoraria, Support for attending meetings, Participation on a Data Safety Monitoring for lectures for Takeda Pharmaceutical Brazil. EPJ: none. FJ: none. DJ: none. GL: none. AZM: none. MT: none. DZ: none. MK: Payment or honoraria for manuscript writing and educational events for Nutricia, FreseniusKabi. ASS: none. GB: none., (Copyright © 2023 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

Published
2023
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48. Pediatric Nutrition in Different Countries.

Author

Salatto A and Spagnuolo MI

Subjects
Child, Humans, Child Nutritional Physiological Phenomena, Habits
Abstract

In this Special Issue, titled "Pediatric Nutrition in Different Countries", we give concise and straightforward information on the nutritional habits of children in different countries worldwide [...].

Published
2023
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49. Pitfalls and Risks of "New Eating Disorders": Let the Expert Speak!

Author

Salatto A, Riccio MP, Garotti R, Bravaccio C, and Spagnuolo MI

Subjects
Humans, Child, Retrospective Studies, Eating, Anorexia Nervosa diagnosis, Feeding and Eating Disorders diagnosis, Feeding and Eating Disorders epidemiology, Avoidant Restrictive Food Intake Disorder
Abstract

Since the post-pandemic period, there has been an increase in the incidence of eating disorders (EADs) and a lowering of the age of onset. In addition to the 'classic' forms, there has also been an increase in new forms of EADs. This article proposes a brief review of the literature concerning mainly two of these new disorders: atypical anorexia and avoidant/restrictive food intake disorder. In addition, a brief overview is proposed of the most frequently raised questions that clinicians may face when dealing with EADs. The answers are provided by doctors from the Federico II University of Naples, who additionally offer the most common red flags on the topic derived from long clinical experience. This article is proposed to be a brief operational guide for all clinicians working in the pediatric area in order to provide diagnostic clues and useful elements to refer patients to specialists for a correct and multidisciplinary treatment.

Published
2023
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50. Teduglutide in pediatric intestinal failure: A position statement of the Italian society of pediatric gastroenterology, hepatology and nutrition (SIGENP).

Author

Diamanti A, Lezo A, D'Antiga L, Gandullia P, Spagnuolo MI, Roggero P, Capriati T, and Lionetti P

Subjects
Child, Gastrointestinal Agents therapeutic use, Glucagon-Like Peptide 2 therapeutic use, Humans, Infant, Peptides, State Medicine, Gastroenterology, Intestinal Failure, Short Bowel Syndrome drug therapy
Abstract

In recent years, the spectrum of possible treatments for Intestinal Failure (IF)-Short Bowel Syndrome (SBS) has been enriched by the implementation of GLP-2 analogues. In Italy, teduglutide (Ted), an analogue of GLP-2, was approved in January 2021 by the Italian Regulatory Agency for Drugs (AIFA) for IF-SBS patients ≥1 year old. According to the Agency indications, Ted can now be prescribed by regional reference centers, with costs fully charged to the National Health Service. Following pediatric-use approval in our country and in light of scarce evidence in childhood, the pediatric network for IF of the Italian Society for Pediatric Gastroenterology, Hepatology and Nutrition (SIGENP) planned to share management methods of Ted in pediatric IF. The main purpose was to identify the best candidates from a cost-effective perspective. Thus, focusing on available literature and on expert opinions, the present position statement provides consensus-based recommendations on the use of Ted for pediatric gastroenterologists and nutritionists treating children with SBS., Competing Interests: Conflict of Interest Some authors have participated as consultants on advisory boards and as speakers at Takeda-sponsored conferences, (Copyright © 2022. Published by Elsevier Ltd.)

Published
2022
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