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1. Novel artful applications of vaccines at the horizon

Author

Davide Montin, Giorgio Ottaviano, Maria Sangerardi, Mayla Sgrulletti, Loredana Chini, Rosa Maria Dellepiane, Baldassarre Martire, Caterina Rizzo, and Viviana Moschese

Subjects
diabetes, autoimmunity, Measles Vaccine, Immunology, vaccines, Settore MED/38, Immunity, Innate, mRNA vaccines, Virus Diseases, nonspecific effects, Pediatrics, Perinatology and Child Health, BCG Vaccine, measles, Humans, Immunology and Allergy, BCG
Abstract

Some live vaccines, particularly Bacillus Calmette-Guérin (BCG), oral polio vaccine (OPV), and measles vaccine, can reduce the incidence of all-cause mortality by outreaching the mere control of specific infections and exerting off-target effects. Asides from the prevention of viral infection, some other vaccines, such as those against flu or rotavirus, could reduce the risk of developing autoimmunity. The nonspecific effects of vaccines are mediated by the innate immune system, mainly through the so-called trained innate immunity. These observations paved the way for developing tolerogenic and trained immunity-based vaccines with substantial implications for more effective use of vaccines and combat vaccine hesitancy.

Published
2022

2. Multisystem Inflammatory Syndrome in children: tools for a timely diagnosis in the Emergency Department from an Italian Multicenter Survey

Author

Angela Mauro, Marco Maglione, Fabio Savoia, Matteo Calvi, Angela Amoroso, Maria Sangerardi, Barbara Tubino, Emanuela Piccotti, Marianna Fabi, Serena Salvadeei, Dario Gadda, Federico Marchetti, Fabio Midulla, Danilo Buonsenso, Silvia Magrassi, Filippo Maria Salvini, Rossana Pignataro, Francesca Di Florio, Emanuela Del Giudice, Riccardo Lubrano, Vincenzo Tipo, Mrgherita Rosa, Thaililja Gagliardi, Ludovica Picciano, Michela Stanco, Stefania Gagliardi, Francesca Orlando, Iacopo Ballani, Viola Canzaniga, Francesco Medici, Francesco La Torre, Fabio Cardinale, Jessica Tibaldi, Benedetta Schiappapietra, Matteo D'Alessandro, Maria Bitelli, Giulia Gallotta, Stefano Masi, Alessia Nucci, Massimo Agosti, Annamaria Plebani, Alessandra Iacono, Francesca Cairello, Chiara Ghizzi, Nadia Rossi, Iolanda Schiavone, and Antonia Gentile

Abstract

Objective To assess the most frequent clinical features of Multisystem Inflammatory Syndrome in children (MIS-C) at presentation to the Emergency Department (ED) in a large multicenter cohort of patients, in order to define useful tools for a timely diagnosis.MethodsClinical and laboratory characteristics were retrospectively reviewed for 210 MIS-C patients from 18 Italian pediatric EDs. We assessed correlations between clinical and laboratory parameters and compared features of patients of different age (≤5 years and >5 years). ResultsFever was the main presenting symptom (100%), followed by conjunctivitis (46%), abdominal pain (44%), vomit (41%) and diarrhea (39%). Forty-nine percent of children presented to the ED in critical or nearly critical conditions. A higher prevalence of mucocutaneous involvement was found in younger children (69% versus 47%, p5 years (62% versus 85%, ppConclusionsApart from fever, most common MIS-C manifestations at presentation to the ED are conjunctivitis, abdominal pain, vomit and diarrhea. Younger children more frequently present with mucocutaneous involvement, while gastrointestinal manifestations are more common in older patients. These findings should be considered when MIS-C is suspected in the ED, in order to achieve a timely recognition of the condition.

Published
2022

3. Vaccinations in children and adolescents treated with immune-modifying biologics: update and current developments

Author

Baldassarre Martire, Giorgio Ottaviano, Maria Sangerardi, Mayla Sgrulletti, Loredana Chini, Rosa Maria Dellepiane, Davide Montin, Caterina Rizzo, Claudio Pignata, Gian Luigi Marseglia, and Viviana Moschese

Subjects
Biological Products, Vaccines, Adolescent, Vaccination, Quality of Life, Humans, Immunology and Allergy, Immunization, Biologics, Child, Children, Settore MED/38, Vaccinations
Abstract

Treatment with immune-modifying biologics has positively impacted disease control and quality of life in many patients with immune-mediated disorders. However, the higher susceptibility to common and opportunistic pathogens is of concern. Thus, immunization strategies to control vaccine-preventable diseases represent a critical issue in this population. However, limited data exist on the safety, immunogenicity, and efficacy of available vaccines in patients on biologics, particularly in children. Here, according to published literature and real-life experience and practice, we report the interim indications of the Italian Society of Pediatric Allergology and Immunology (SIAIP) Vaccine Committee and of the Italian Primary Immunodeficiency Network (IPINet) Centers on immunization of children and adolescents receiving biologics. Our aim is to provide a practical guidance for the clinician to ensure optimal protection for patients and the community.

Published
2022

4. Consensus statement of the Italian society of pediatric allergy and immunology for the pragmatic management of children and adolescents with allergic or immunological diseases during the COVID-19 pandemic

Author

Fabio Cardinale, Giorgio Ciprandi, Salvatore Barberi, Roberto Bernardini, Carlo Caffarelli, Mauro Calvani, Giovanni Cavagni, Elena Galli, Domenico Minasi, Michele Miraglia Del Giudice, Viviana Moschese, Elio Novembre, Francesco Paravati, Diego G Peroni, Maria Angela Tosca, Giovanni Traina, Salvatore Tripodi, Gian Luigi Marseglia, Doriana Amato, Caterina Anania, Elisa Anastasio, Rachele Antignani, Stefania Arasi, Martire Baldassarre, Ermanno Baldo, Andrea Barbalace, Simona Barni, Federica Betti, Annamaria Bianchi, Ezio Bolzacchini, Maira Bonini, Paolo Bottau, Sara Bozzetto, Maria Antonia Brighetti, Davide Caimmi, Silvia Caimmi, Luigi Calzone, Caterina Cancrini, Lucia Caminiti, Giulia Capata, Lucetta Capra, Carlo Capristo, Elena Carboni, Francesco Carella, Riccardo Castagnoli, Elena Chiappini, Fernanda Chiera, Iolanda Chinellato, Loredana Chini, Francesca Cipriani, Flavio Civitelli, Pasquale Comberiati, Daniele Contini, Stefania Corrente, Claudio Cravidi, Giuseppe Crisafulli, Barbara Cuomo, Enza D'Auria, Sofia D'Elios, Fabio Decimo, Auro Della Giustina, Rosa Maria Delle Piane, Maria De Filippo, Valentina De Vittori, Lucia Diaferio, Maria Elisa Di Cicco, Dora Di Mauro, Marzia Duse, Silvia Federici, Giuseppe Felice, Maria Grazia Fenu, Giuliana Ferrante, Tiziana Foti, Fabrizio Franceschini, Daniele Ghiglioni, Giuliana Giardino, Mattia Giovannini, Giovanni Cosimo Indirli, Cristiana Indolfi, Massimo Landi, Francesco La Torre, Lucia Maddalena Leone, Amelia Licari, Lucia Liotti, Vassilios Lougaris, Nunzia Maiello, Paride Mantecca, Sara Manti, Marco Maria Mariani, Alberto Martelli, Carla Mastrorilli, Violetta Mastrorilli, Davide Montin, Francesca Mori, Roberta Olcese, Giorgio Ottaviano, Claudia Paglialunga, Giovanni Pajno, Giuseppe Parisi, Stefano Pattini, Luca Pecoraro, Umberto Pelosi, Claudio Pignata, Giampaolo Ricci, Silvia Ricci, Stefano Rizzi, Caterina Rizzo, Sara Rosati, Paolo Rosso, Maria Sangerardi, Angelica Santoro, Francesca Saretta, Lucrezia Sarti, Marco Sartorio, Majla Sgruletti, Annarosa Soresina, Ifigenia Sfika, Mayla Sgrulletti, Nuccia Tesse, Valentina Tranchino, Alessandro Travaglini, Malizia Velia, Elvira Verduci, Mario Vernich, Elisabetta Veronelli, Stefano Volpi, Martina Votto, Anna Maria Zicari, Cardinale, F., Ciprandi, G., Barberi, S., Bernardini, R., Caffarelli, C., Calvani, M., Cavagni, G., Galli, E., Minasi, D., Del Giudice, M. M., Moschese, V., Novembre, E., Paravati, F., Peroni, D. G., Tosca, M. A., Traina, G., Tripodi, S., Marseglia, G. L., Amato, D., Anania, C., Anastasio, E., Antignani, R., Arasi, S., Baldassarre, M., Baldo, E., Barbalace, A., Barni, S., Betti, F., Bianchi, A., Bolzacchini, E., Bonini, M., Bottau, P., Bozzetto, S., Brighetti, M. A., Caimmi, D., Caimmi, S., Calzone, L., Cancrini, C., Caminiti, L., Capata, G., Capra, L., Capristo, C., Carboni, E., Carella, F., Castagnoli, R., Chiappini, E., Chiera, F., Chinellato, I., Chini, L., Cipriani, F., Civitelli, F., Comberiati, P., Contini, D., Corrente, S., Cravidi, C., Crisafulli, G., Cuomo, B., D'Auria, E., D'Elios, S., Decimo, F., Giustina, A. D., Piane, R. M. D., De Filippo, M., De Vittori, V., Diaferio, L., Di Mauro, M. E., Duse, M., Federici, S., Felice, G., Fenu, G., Ferrante, G., Foti, T., Franceschini, F., Ghiglioni, D., Giardino, G., Giovannini, M., Indirli, G. C., Indolfi, C., Landi, M., La Torre, F., Leone, L. M., Licari, A., Liotti, L., Lougaris, V., Maiello, N., Mantecca, P., Manti, S., Mariani, M. M., Martelli, A., Mastrorilli, C., Mastrorilli, V., Montin, D., Mori, F., Olcese, R., Ottaviano, G., Paglialunga, C., Pajno, G., Parisi, G., Pattini, S., Pecoraro, L., Pelosi, U., Pignata, C., Ricci, G., Ricci, S., Rizzi, S., Rizzo, C., Rosati, S., Rosso, P., Sangerardi, M., Santoro, A., Saretta, F., Sarti, L., Sartorio, M., Sgruletti, M., Soresina, A., Sfika, I., Sgrulletti, M., Tesse, N., Tranchino, V., Travaglini, A., Velia, M., Verduci, E., Vernich, M., Veronelli, E., Volpi, S., Votto, M., Zicari, A. M., Cardinale, Fabio, Ciprandi, Giorgio, Barberi, Salvatore, Bernardini, Roberto, Caffarelli, Carlo, Calvani, Mauro, Cavagni, Giovanni, Galli, Elena, Minasi, Domenico, Del Giudice, Michele Miraglia, Moschese, Viviana, Novembre, Elio, Paravati, Francesco, Peroni, Diego G, Tosca, Maria Angela, Traina, Giovanni, Tripodi, Salvatore, Marseglia, Gian Luigi, SIAIP task force Pignata, Claudio, Cardinale, F, Ciprandi, G, Barberi, S, Bernardini, R, Caffarelli, C, Calvani, M, Cavagni, G, Galli, E, Minasi, D, Del Giudice, M, Moschese, V, Novembre, E, Paravati, F, Peroni, D, Tosca, M, Traina, G, Tripodi, S, Marseglia, G, Amato, D, Anania, C, Anastasio, E, Antignani, R, Arasi, S, Baldassarre, M, Baldo, E, Barbalace, A, Barni, S, Betti, F, Bianchi, A, Bolzacchini, E, Bonini, M, Bottau, P, Bozzetto, S, Brighetti, M, Caimmi, D, Caimmi, S, Calzone, L, Cancrini, C, Caminiti, L, Capata, G, Capra, L, Capristo, C, Carboni, E, Carella, F, Castagnoli, R, Chiappini, E, Chiera, F, Chinellato, I, Chini, L, Cipriani, F, Civitelli, F, Comberiati, P, Contini, D, Corrente, S, Cravidi, C, Crisafulli, G, Cuomo, B, D'Auria, E, D'Elios, S, Decimo, F, Giustina, A, Piane, R, De Filippo, M, De Vittori, V, Diaferio, L, Di Mauro, M, Duse, M, Federici, S, Felice, G, Fenu, G, Ferrante, G, Foti, T, Franceschini, F, Ghiglioni, D, Giardino, G, Giovannini, M, Indirli, G, Indolfi, C, Landi, M, La Torre, F, Leone, L, Licari, A, Liotti, L, Lougaris, V, Maiello, N, Mantecca, P, Manti, S, Mariani, M, Martelli, A, Mastrorilli, C, Mastrorilli, V, Montin, D, Mori, F, Olcese, R, Ottaviano, G, Paglialunga, C, Pajno, G, Parisi, G, Pattini, S, Pecoraro, L, Pelosi, U, Pignata, C, Ricci, G, Ricci, S, Rizzi, S, Rizzo, C, Rosati, S, Rosso, P, Sangerardi, M, Santoro, A, Saretta, F, Sarti, L, Sartorio, M, Sgruletti, M, Soresina, A, Sfika, I, Sgrulletti, M, Tesse, N, Tranchino, V, Travaglini, A, Velia, M, Verduci, E, Vernich, M, Veronelli, E, Volpi, S, Votto, M, Zicari, A, and Fabio Cardinale, Giorgio Ciprandi, Salvatore Barberi, Roberto Bernardini, Carlo Caffarelli, Mauro Calvani, Giovanni Cavagni, Elena Galli, Domenico Minasi, Michele Miraglia Del Giudice, Viviana Moschese, Elio Novembre, Francesco Paravati, Diego G Peroni, Maria Angela Tosca, Giovanni Traina, Salvatore Tripodi, Gian Luigi Marseglia, Doriana Amato, Caterina Anania, Elisa Anastasio, Rachele Antignani, Stefania Arasi, Martire Baldassarre, Ermanno Baldo, Andrea Barbalace, Simona Barni, Federica Betti, Annamaria Bianchi, Ezio Bolzacchini, Maira Bonini, Paolo Bottau, Sara Bozzetto, Maria Antonia Brighetti, Davide Caimmi, Silvia Caimmi, Luigi Calzone, Caterina Cancrini, Lucia Caminiti, Giulia Capata, Lucetta Capra, Carlo Capristo, Elena Carboni, Francesco Carella, Riccardo Castagnoli, Elena Chiappini, Fernanda Chiera, Iolanda Chinellato, Loredana Chini, Francesca Cipriani, Flavio Civitelli, Pasquale Comberiati, Daniele Contini, Stefania Corrente, Claudio Cravidi, Giuseppe Crisafulli, Barbara Cuomo, Enza D'Auria, Sofia D'Elios, Fabio Decimo, Auro Della Giustina, Rosa Maria Delle Piane, Maria De Filippo, Valentina De Vittori, Lucia Diaferio, Maria Elisa Di Cicco, Dora Di Mauro, Marzia Duse, Silvia Federici, Giuseppe Felice, Maria Grazia Fenu, Giuliana Ferrante, Tiziana Foti, Fabrizio Franceschini, Daniele Ghiglioni, Giuliana Giardino, Mattia Giovannini, Giovanni Cosimo Indirli, Cristiana Indolfi, Massimo Landi, Francesco La Torre, Lucia Maddalena Leone, Amelia Licari, Lucia Liotti, Vassilios Lougaris, Nunzia Maiello, Paride Mantecca, Sara Manti, Marco Maria Mariani, Alberto Martelli, Carla Mastrorilli, Violetta Mastrorilli, Davide Montin, Francesca Mori, Roberta Olcese, Giorgio Ottaviano, Claudia Paglialunga, Giovanni Pajno, Giuseppe Parisi, Stefano Pattini, Luca Pecoraro, Umberto Pelosi, Claudio Pignata, Giampaolo Ricci, Silvia Ricci, Stefano Rizzi, Caterina Rizzo, Sara Rosati, Paolo Rosso, Maria Sangerardi, Angelica Santoro, Francesca Saretta, Lucrezia Sarti, Marco Sartorio, Majla Sgruletti, Annarosa Soresina, Ifigenia Sfika, Mayla Sgrulletti, Nuccia Tesse, Valentina Tranchino, Alessandro Travaglini, Malizia Velia, Elvira Verduci, Mario Vernich, Elisabetta Veronelli, Stefano Volpi, Martina Votto, Anna Maria Zicari

Subjects
Allergy, Review, 030207 dermatology & venereal diseases, Settore MED/38 - Pediatria Generale E Specialistica, 0302 clinical medicine, COVID-19, Child, Pandemic, Immunologic disease, Asthma, Adolescent, Viral, 030212 general & internal medicine, Disease management (health), Societies, Medical, pandemic, child, adolescent, allergy, asthma, immunologic disease, Incidence (epidemiology), lcsh:RJ1-570, Disease Management, General Medicine, Atopic dermatitis, Settore MED/38, Coronavirus Infections, Decision Making, Humans, Italy, Pneumonia, Viral, Pragmatic Clinical Trials as Topic, SARS-CoV-2, Allergy and Immunology, Betacoronavirus, Consensus, Pandemics, Latex allergy, Human, Telemedicine, Consensu, 03 medical and health sciences, Medical, medicine, Risk factor, Betacoronaviru, business.industry, Coronavirus Infection, lcsh:Pediatrics, Pneumonia, medicine.disease, Immunology, Societies, business, Rare disease
Abstract

The COVID-19 pandemic has surprised the entire population. The world has had to face an unprecedented pandemic. Only, Spanish flu had similar disastrous consequences. As a result, drastic measures (lockdown) have been adopted worldwide. Healthcare service has been overwhelmed by the extraordinary influx of patients, often requiring high intensity of care. Mortality has been associated with severe comorbidities, including chronic diseases. Patients with frailty were, therefore, the victim of the SARS-COV-2 infection. Allergy and asthma are the most prevalent chronic disorders in children and adolescents, so they need careful attention and, if necessary, an adaptation of their regular treatment plans. Fortunately, at present, young people are less suffering from COVID-19, both as incidence and severity. However, any age, including infancy, could be affected by the pandemic.Based on this background, the Italian Society of Pediatric Allergy and Immunology has felt it necessary to provide a Consensus Statement. This expert panel consensus document offers a rationale to help guide decision-making in the management of children and adolescents with allergic or immunologic diseases.

Published
2020

5. One step closer to influenza vaccine inclusiveness

Author

Baldassarre Martire, Rosa Maria Dellepiane, Mayla Sgrulletti, Maria Sangerardi, Viviana Moschese, Caterina Rizzo, Giorgio Ottaviano, Davide Montin, and Loredana Chini

Subjects
Pediatrics, medicine.medical_specialty, Influenza vaccine, Immunology, Vaccines, Attenuated, Virus, 2020 Update from The Italian Society of Pediatric Allergy and Immunology, 03 medical and health sciences, 0302 clinical medicine, children, Influenza, Human, universal vaccine, Humans, Immunology and Allergy, Live attenuated influenza vaccine, Medicine, 030212 general & internal medicine, Child, Immunodeficiency, Aged, Asthma, asthma, children, immunodeficiency, influenza vaccine, pregnancy, universal vaccine, Pregnancy, business.industry, Immunology Section, Incidence (epidemiology), Vaccination, virus diseases, asthma, medicine.disease, Settore MED/38, respiratory tract diseases, Vaccines, Inactivated, 030228 respiratory system, Influenza Vaccines, Pediatrics, Perinatology and Child Health, Supplement Article, Female, pregnancy, influenza vaccine, business, immunodeficiency
Abstract

Flu virus infection is a common cause of acute respiratory illness, with the major incidence in pediatric age, high morbidity, and mortality. The flu vaccine is recommended for all people aged ≥6 months, unless specific contraindications are present. Younger and older age, pregnancy, chronic diseases like asthma, and immunodeficiency are risk factors for severe complications following flu infection. Thus, these categories represent the target for flu vaccine strategies in most countries. Inactivated influenza vaccine (IIV), recombinant influenza vaccine (RIV) or live‐attenuated influenza virus (LAIV) are currently available, with specific precautions and contraindications. We aim to resume the current indications for vaccines in the vulnerable populations to support flu vaccination inclusiveness, in anticipation of a “universal vaccine” strategy.

Published
2020

6. Health-Related Quality of Life and Emotional Difficulties in Chronic Granulomatous Disease: Data on Adult and Pediatric Patients from Italian Network for Primary Immunodeficiency (IPINet)

Author

Maria Carrabba, Baldassarre Martire, Andrea Finocchi, Samuele Naviglio, Marco De Carli, Antonino Trizzino, Isabella Quinti, Emilia Cirillo, Annarosa Soresina, Marco Gattorno, Maria Sangerardi, Alessandro Plebani, Franco Locatelli, Alessandro Aiuti, Federica Pulvirenti, Alice Bertaina, Maria Grazia Foschino Barbaro, Claudio Pignata, Maddalena Migliavacca, Pulvirenti, F., Sangerardi, M., Plebani, A., Soresina, A., Finocchi, A., Pignata, C., Cirillo, E., Trizzino, A., Aiuti, A., Migliavacca, M., Locatelli, F., Bertaina, A., Naviglio, S., Carrabba, M., De Carli, M., Barbaro, M. G. F., Gattorno, M., Quinti, I., and Martire, B.

Subjects
Male, Pediatrics, medicine.medical_treatment, burden of disease, chronic granulomatous disease, emotional difficulties, health-related quality of life, hematopoietic stem cell transplantation, PEDsQL, SDQ, SF-12, Hematopoietic stem cell transplantation, Chronic granulomatous disease, Granulomatous Disease, Chronic, Psychological Distress, Severity of Illness Index, Medical microbiology, Quality of life, hemic and lymphatic diseases, Immunology and Allergy, Registries, emotional difficultie, Child, Middle Aged, Settore MED/38, Caregivers, Italy, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Child, Preschool, Female, Psychosocial, Adult, medicine.medical_specialty, Adolescent, Immunology, Cholestyramine Resin, Young Adult, Internal medicine, Diabetes mellitus, medicine, Humans, business.industry, Immunologic Deficiency Syndromes, medicine.disease, Rheumatology, Primary immunodeficiency, Quality of Life, business
Abstract

Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by life-threatening infections, inflammation, and autoimmunity with an impact on health-related quality of life (HRQoL). Few data are available for children, whereas no study has been conducted in adults. Here, we investigated HRQoL and emotional functioning of 19 children and 28 adults enrolled in Italian registry for CGD. PEDsQL and SDQ were used for children and their caregivers, and adults completed the SF-12 questionnaire. Mean scores were compared with norms and with patients affected by chronic diseases. Comparisons were made for CGD patients who underwent or not hematopoietic stem cell transplantation (HSCT). When compared with norms, CGD children exhibited higher difficulties in social/school areas, peer relationship, and conduct/emotional problems (

Published
2020

7. Hepatic angiodynamic profile in paediatric patients with hereditary haemorrhagic telangiectasia type 1 and type 2

Author

Arnaldo Scardapane, Patrizia Suppressa, Paola Giordano, Paolo Buonamico, Patrizia Lastella, Gennaro M. Lenato, Vito Leonardo Miniello, Carlo Sabbà, Maria Sangerardi, and Mariantonietta Francavilla

Subjects
Male, Pathology, Time Factors, Computed Tomography Angiography, Activin Receptors, Type II, Magnetic resonance angiography, 030218 nuclear medicine & medical imaging, Hepatic Artery, 0302 clinical medicine, Prevalence, Child, Paediatric patients, Ultrasonography, Doppler, Duplex, medicine.diagnostic_test, Portal Vein, Ultrasound, Age Factors, Endoglin, Phenotype, Italy, Liver, Child, Preschool, Cohort, Disease Progression, Female, Telangiectasia, Hereditary Hemorrhagic, 030211 gastroenterology & hepatology, Radiology, Cardiology and Cardiovascular Medicine, Nodular regenerative hyperplasia, Dilatation, Pathologic, medicine.medical_specialty, Adolescent, Arteriovenous Malformations, 03 medical and health sciences, Predictive Value of Tests, Multidetector Computed Tomography, medicine, Humans, Genetic Predisposition to Disease, In patient, Hereditary haemorrhagic telangiectasia, Adult patients, business.industry, medicine.disease, Cross-Sectional Studies, Case-Control Studies, Asymptomatic Diseases, Mutation, business, Magnetic Resonance Angiography
Abstract

Abstract. Background: Liver involvement is a common manifestation of hereditary haemorrhagic telangiectasia (HHT). Although a number of studies have been carried out in adult patients, no study has ever been focused on investigating HHT-related hepatic involvement in paediatric patients. The present study aimed for the first time to systematically estimate the prevalence of HHT-associated liver involvement and to characterize HHT-associated hepatic angiodynamic features in paediatric age. Patients and methods: The study was designed as a cross-sectional survey in an HHT paediatric cohort, subclassified as HHT1 and HHT2 according to the mutated gene. The evaluation of the angiodynamic profile was performed by duplex ultrasound examination. Investigation by multi-slice computed tomography (MSCT) or magnetic resonance angiography (MRA) was performed in patients >12 years. Results: MSCT/MRA examination disclosed silent hepatic involvement in 7/20 (35.0 %) children, and nodular regenerative hyperplasia in two cases. Diameter of common hepatic artery was significantly larger in HHT2 (0.45 ± 0.15 cm) compared to HHT1 (0.33 ± 0.09, p < 0.01) and control children (0.32 ± 0.08, p < 0.05). None of the patients had clinical manifestations of liver involvement. Angiodynamic profiles were different between paediatric and adult HHT patients. Conclusions: Liver involvement can be detected in paediatric HHT patients, albeit with a lower frequency compared to adults. Paediatric HHT2 children show a higher frequency of liver involvement and a trend to hepatic artery dilation when compared to HHT1 children.

Published
2017

8. Pulmonary Arteriovenous Malformations and Cerebral Abscess Recurrence in a Child With Hereditary Hemorrhagic Telangiectasia

Author

Paola Giordano, Patrizia Suppressa, Carlo Sabbà, Maria Sangerardi, Maria Luisa Fiorella, Patrizia Lastella, Gennaro M. Lenato, E. Attolini, and Federica Valente

Subjects
Adult, Male, medicine.medical_specialty, Pathology, Adolescent, medicine.medical_treatment, Mucocutaneous zone, Brain Abscess, Telangiectases, Disease, Pulmonary Artery, Recurrence, medicine, Humans, Embolization, Telangiectasia, Abscess, Brain abscess, business.industry, Hematology, Prognosis, medicine.disease, Embolization, Therapeutic, Magnetic Resonance Imaging, Dermatology, Oncology, Pulmonary Veins, Arteriovenous Fistula, Pediatrics, Perinatology and Child Health, Etiology, Telangiectasia, Hereditary Hemorrhagic, medicine.symptom, business
Abstract

Background: A 17-year-old boy was referred to our center with ahistory of brain abscess (BA) recurring after 9 years. The patientreported 2 previous treatments for pulmonary arteriovenous mal-formations, sporadic nosebleeds, and familial history for epistaxis.Clinical investigations revealed arteriovenous malformations inlung, brain, and liver, as well as mucocutaneous telangiectases. Adefinite diagnosis of hereditary hemorrhagic telangiectasia wasmade based on clinical criteria and confirmed by genetic analysis.This is the first report of BA recurrence at the end of pediatric age.Conclusions: The present case and the literature review of all casesof BA thus far reported highlight the need to raise the suspicion ofa pulmonary arteriovenous malformations, both isolated and in thecontext of a possible hereditary hemorrhagic telangiectasia, for anycase of BA of unexplained etiology, in children as well as in adults.Key Words: hereditary hemorrhagic telangiectasia, Rendu-Osler-Weber disease, recurrent brain abscess, pulmonary arteriovenousmalformation, diagnostic delay

Published
2015

9. Italian pediatric nutrition survey

Author

Michelangelo Barbaglia, Luigi Marmetucci, Nicoletta Cimadore, Alessandro Monaci, P. Fiore, Sergio Amarri, Elena Brunori, Maddalena Cioni, Carla Russo, Monica Barrani, P. Gandullia, Giovanna Zuin, Giuseppe Parisi, Rita Bellomo Anna, Michele Pinon, Nunzia Miglietti, Francesca Lizzoli, Elisa Mazzoni, Giulia Bardasi, Marisa Zoppo, Giacomo Cagnoli, S. Borodani, L. Forchielli, Monica Tulli, Fina Belli, Michele Salata, Giovanna Verlato, Vittoria Opinto, Roberto Bonaudo, Luisella Angelotti, Giulia Bruni, Elena Uga, Costantino De Giacomo, Antonietta Antonini Monica, Riccardo Guanà, Flavia Urbano, Rosaria Abate, Barbara Santangelo, Chiara Pettinari, Giovanna Fontanella, Patrizia Fusco, L. Lacitignola, Adalberto Brach Del Prever, Gina Ancora, S. Amarri, Laura Lacitignola, Paola Sparano, Marcello Lanari, Stefano Gatti, Francesca Nesi, Valentina De Cosmi, Alessia Frimaire, A Lezo, Francesca Penagini, Carmen Di Scala, Giuseppina Migliore, Roberta Annibali, Grazia Di Leo, Paola Peverelli, Mara Salmaso, Antonella Lezo, Paola Melli, M. Pastore, E. Brunori, Claudia Banzato, M.I. Spagnuolo, Antonella Diamanti, G. Verlato, Angelo Campanozzi, Mariella Pace, Martina Biagioni, Graziano Memmini, Laura Mistura, Sergio Del Vecchio, Annalisa Famiani, Enrico Felici, Germana Casaccia, Graziana Galvagno, Mario Castello, R. Panceri, Paola Accorsi, Martina Fomasi, Francesca Cortinovis, Michela Perrone, Teresa Capriati, Andrea Chiaro, Silvio Ferraris, Nicola Cecchi, Maria Immacolata Spagnuolo, Patrizia Petitti, Cristina Malaventura, Maria Sangerardi, Enrico Gasparrini, Francesco Savino, Luigi Besenzon, Anna Meneghini, Azzurra Guerra, Alessandra Sala, Maria Magistã Anna, Enrico Aidala, Donata Scatã, Gianluigi Palamone, Tiziano Basso, Giuseppe Maggiore, A. Diamanti, Alessandra Mazzocchi, Alessia Morganti, Andreina Stamati Filomena, Paolo Siani, Roberto Panceri, Maria Pastore, Paolo Gandullia, Lezo, A., Diamanti, A., Capriati, T., Gandullia, P., Fiore, P., Lacitignola, L., Gatti, S., Spagnuolo, M. I., Cecchi, N., Verlato, G., Borodani, S., Forchielli, L., Panceri, R., Brunori, E., Pastore, M., Amarri, S., Abate, R., Accorsi, P., Aidala, E., Ancora, G., Angelotti, L., Annibali, R., Antonini Monica, A., Banzato, C., Barbaglia, M., Bardasi, G., Barrani, M., Basso, T., Brach del Prever, A., Belli, F., Bellomo Anna, R., Besenzon, L., Biagioni, M., Bonaudo, R., Bruni, G., Cagnoli, G., Campanozzi, A., Casaccia, G., Castello, M., Chiaro, A., Cimadore, N., Cioni, M., Cortinovis, F., De Cosmi, V., De Giacomo, C., Del Vecchio, S., Di Leo, G., Di Scala, C., Famiani, A., Felici, E., Ferraris, S., Fomasi, M., Fontanella, G., Frimaire, A., Fusco, P., Galvagno, G., Gasparrini, E., Guana, R., Guerra, A., Lanari, M., Lizzoli, F., Maggiore, G., Magista Anna, M., Malaventura, C., Marmetucci, L., Mazzocchi, A., Mazzoni, E., Melli, P., Memmini, G., Meneghini, A., Miglietti, N., Migliore, G., Mistura, L., Monaci, A., Morganti, A., Nesi, F., Opinto, V., Pace, M., Palamone, G., Parisi, G., Penagini, F., Perrone, M., Petitti, P., Pettinari, C., Peverelli, P., Pinon, M., Russo, C., Sala, A., Salata, M., Salmaso, M., Sangerardi, M., Santangelo, B., Savino, F., Scata, D., Siani, P., Sparano, P., Stamati Filomena, A., Tulli, M., Uga, E., Urbano, F., Zoppo, M., Zuin, G., Abate, Rosaria, Accorsi, Paola, Aidala, Enrico, Amarri, Sergio, Ancora, Gina, Angelotti, Luisella, Annibali, Roberta, Antonini Monica, Antonietta, Banzato, Claudia, Barbaglia, Michelangelo, Bardasi, Giulia, Barrani, Monica, Basso, Tiziano, Brach Del Prever, Adalberto, Belli, Fina, Bellomo Anna, Rita, Besenzon, Luigi, Biagioni, Martina, Bonaudo, Roberto, Bruni, Giulia, Brunori, Elena, Cagnoli, Giacomo, Campanozzi, Angelo, Casaccia, Germana, Castello, Mario, Chiaro, Andrea, Cimadore, Nicoletta, Cioni, Maddalena, Cortinovis, Francesca, De Cosmi, Valentina, De Giacomo, Costantino, Del Vecchio, Sergio, Diamanti, Antonella, Di Leo, Grazia, Di Scala, Carmen, Famiani, Annalisa, Felici, Enrico, Ferraris, Silvio, Fomasi, Martina, Fontanella, Giovanna, Frimaire, Alessia, Fusco, Patrizia, Galvagno, Graziana, Gandullia, Paolo, Gasparrini, Enrico, Guanã , Riccardo, Guerra, Azzurra, Lanari, Marcello, Lacitignola, Laura, Lezo, Antonella, Lizzoli, Francesca, Maggiore, Giuseppe, Magistã Anna, Maria, Malaventura, Cristina, Marmetucci, Luigi, Mazzocchi, Alessandra, Mazzoni, Elisa, Melli, Paola, Memmini, Graziano, Meneghini, Anna, Miglietti, Nunzia, Migliore, Giuseppina, Mistura, Laura, Monaci, Alessandro, Morganti, Alessia, Nesi, Francesca, Opinto, Vittoria, Pace, Mariella, Palamone, Gianluigi, Panceri, Roberto, Parisi, Giuseppe, Pastore, Maria, Penagini, Francesca, Perrone, Michela, Petitti, Patrizia, Pettinari, Chiara, Peverelli, Paola, Pinon, Michele, Russo, Carla, Sala, Alessandra, Salata, Michele, Salmaso, Mara, Sangerardi, Maria, Santangelo, Barbara, Savino, Francesco, Scatã , Donata, Siani, Paolo, Spagnuolo, Maria Immacolata, Sparano, Paola, Stamati Filomena, Andreina, Tulli, Monica, Uga, Elena, Urbano, Flavia, Verlato, Giovanna, Zoppo, Marisa, and Zuin, Giovanna

Subjects
0301 basic medicine, Male, Pediatrics, Hospitalized patients, Endocrinology, Diabetes and Metabolism, Pediatric nutrition, 0302 clinical medicine, Child Development, Endocrinology, Prevalence, 030212 general & internal medicine, Growth Charts, Child, Nutritional support, Wasting, Growth Disorders, Pediatric, Stunting, Nutrition and Dietetics, Nutritional status, Nutrition Surveys, Diabetes and Metabolism, Italy, Malnutrition, Child, Preschool, Female, medicine.symptom, medicine.medical_specialty, Adolescent, Nutritional Status, Socio-culturale, Malnutrition in children, 03 medical and health sciences, Young Adult, medicine, Humans, 030109 nutrition & dietetics, business.industry, Infant, Anthropometry, medicine.disease, Parenteral nutrition, Chronic Disease, business, Child, Hospitalized
Abstract

Introduction the prevalence of malnutrition in children and its impact on clinical outcomes is underrecognized by clinicians in Italy as well as worldwide. A novel definition of pediatric malnutrition has been recently proposed by a working group of the Academy of Nutrition and Dietetics and American Society for Parenteral and Enteral Nutrition (A.S.P.E.N.), based on the correlation between illness and the use of zscores of anthropometric measurements. Aim to investigate the prevalence of malnutrition and related nutritional support among hospitalized children in Italy, in a nationwide survey performed in a single day (16/4/2015). Methods an open access website (http://nday.biomedia.net) was used to collected data from 73 hospitals and 101 wards in 14 Italian regions (1994 patients). Anonymous information was collected on hospitals' characteristics, patient's anthropometry, admission diagnosis, presence of chronic diseases and use of nutritional support: oral nutritional supplements (ONS), enteral nutrition (EN) or parenteral nutrition (PN). Z-scores of anthropometric measurements, calculated with Epi Info 7.1.5, defined nutritional status: wasting was identified by BMI or Weight-for-Length z-score (

Published
2017

10. Association of interleukin-(IL)10 haplotypes and serum IL-10 levels in the progression of childhood immune thrombocytopenic purpura

Author

Federica Valente, Giovanni Carlo Del Vecchio, Riccardina Tesse, Maria Sangerardi, Domenico De Mattia, and Paola Giordano

Subjects
Male, Adolescent, Single-nucleotide polymorphism, Biology, Polymorphism, Single Nucleotide, Immune system, Genetics, medicine, Humans, Child, Promoter Regions, Genetic, Purpura, Thrombocytopenic, Idiopathic, Haplotype, Interleukin, Infant, General Medicine, medicine.disease, Thrombocytopenic purpura, Pathophysiology, Interleukin-10, Interleukin 10, Gene Expression Regulation, Haplotypes, Child, Preschool, Immunology, Chronic Disease, Population study, Female, Biomarkers
Abstract

Derangement of genetic and immunological factors seems to have a pivotal role in the pathophysiology of immune thrombocytopenic purpura (ITP). We investigated interleukin(IL)-10 genetically determined expression in children with an acute progression of ITP (n=41) compared to young patients with chronic ITP (n=44) and healthy controls (n=60), and attempted to correlate IL-10 production with the course of the disease. We genotyped our study population for three single nucleotide polymorphisms at positions -1082 (A/G), -819 (C/T) and -592 (C/A) in the promoter region of the IL-10 gene. IL-10 levels were measured by enzyme-linked immunoassay. The IL-10 production in our study population was significantly higher in patients carrying the GCC haplotype than those bearing ACC and ATA haplotypes (6.9 ± 1.5 vs 3.6 ± 0.8 vs 3.3 ± 0.3, p=0.03). The serum concentration of IL-10 was significantly higher in patients with an acute course of their disease, who mainly carried the GCC haplotype (92%), compared to chronic subjects, bearing the non-GCC haplotypes, and controls [17 pg/mL (1.7-18) vs 3.5 pg/mL (0.6-11) vs 3 pg/mL (1-7), p0.01)]. Our findings show that patients carrying the GCC-high producer IL-10 haplotype have an acute development of ITP and that IL-10 levels might represent a useful predictive biomarker of the disease course.

Published
2012

11. Hereditary Hemorrhagic Telangiectasia: Arteriovenous Malformations in Children

Author

Nicola Quaranta, Paola Piccarreta, Raffaella Valerio, Maria Sangerardi, Arnaldo Scardapane, Carlo Sabbà, Patrizia Suppressa, Nicoletta Resta, Gennaro M. Lenato, Franca Dicuonzo, Patrizia Lastella, Luigi Chiumarulo, Giuseppe Marano, and Paola Giordano

Subjects
Male, medicine.medical_specialty, Adolescent, Cross-sectional study, Magnetic resonance angiography, Arteriovenous Malformations, Prevalence, Humans, Medicine, Clinical significance, Prospective Studies, Child, Prospective cohort study, Telangiectasia, medicine.diagnostic_test, business.industry, Infant, Magnetic resonance imaging, Arteriovenous malformation, Endoglin, medicine.disease, Cross-Sectional Studies, Child, Preschool, Pediatrics, Perinatology and Child Health, Female, Telangiectasia, Hereditary Hemorrhagic, Radiology, medicine.symptom, business
Abstract

Objective To evaluate the clinical features in a large cohort of pediatric patients with genetically confirmed hereditary hemorrhagic telangiectasia (HHT) and to identify possible predictors of arteriovenous malformation (AVM) onset or clinical significance. Study design Prospective cross-sectional survey of all children subjected to screening for AVMs in the multidisciplinary HHT center. All patients proved to be carriers of endoglin mutations or activin A receptor type-II-like kinase 1 mutations, defined as HHT1 and HHT2, respectively. A full clinical-radiological protocol for AVM detection was adopted, independent from presence or absence of AVM-related symptoms. Results Forty-four children (mean age, 10.3 years; range, 1-18) were subjected to a comprehensive clinicalradiologic evaluation. This investigation disclosed cerebrovascular malformations in 7 of 44 cases, pulmonary AVMs in 20 of 44 cases, and liver AVMs in 23 of 44 cases. Large visceral AVMs were found in 12 of 44 children and were significantly more frequent in patients with HHT1. Only large AVMs were associated with symptoms and complications. Conclusions Children with HHT have a high prevalence of AVMs; therefore, an appropriate clinical and radiological screening protocol is advisable. Large AVMs can be associated with complications in childhood, whereas small AVMs probably have no clinical risk. (J Pediatr 2013;163:179-86).

Published
2013

12. Autoimmune haematological disorders in two Italian children with Kabuki Syndrome

Author

Maria Sangerardi, Baldassarre Martire, Maria Felicia Faienza, Paola Giordano, Federica Valente, and Giuseppe Lassandro

Subjects
Male, Pediatrics, medicine.medical_specialty, Anemia, Prednisolone, Biopsy, Fine-Needle, Psychological intervention, Case Report, Disease, Diagnosis, Differential, Therapeutic approach, medicine, Humans, Immunologic Factors, KDM6A, Abnormalities, Multiple, Genetic Testing, Child, Glucocorticoids, Genetic testing, Purpura, Thrombocytopenic, Idiopathic, Kabuki syndrome, medicine.diagnostic_test, Autoimmune haematological disorders, business.industry, KMT2D, Immunoglobulins, Intravenous, DNA, medicine.disease, Hematologic Diseases, Immune thrombocytopenia, Neoplasm Proteins, DNA-Binding Proteins, Vestibular Diseases, Face, Immunology, MLL2, Drug Therapy, Combination, Female, Anemia, Hemolytic, Autoimmune, Differential diagnosis, business, Immune haemolytic anaemia, medicine.drug
Abstract

Kabuki syndrome (also called Niikawa-Kuroki syndrome) is a rare genetic disease described for the first time in Japan, characterised by anomalies in multiple organ systems and often associated with autoimmune disorders and impaired immune response. We herein report the clinical history, the therapeutic approach and the outcome of two children with Kabuki syndrome who developed autoimmune haematological disorders (haemolytic anaemia and immune thrombocytopenia). Factors regarding differential diagnosis and interventions in better management of this syndrome and its complications are discussed. This is the first report of Italian children with autoimmune haematological disorders complicating Kabuki syndrome.

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