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1. 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

Pulvirenti, F., Sangerardi, M., Plebani, A., Soresina, A., Finocchi, A., Pignata, C., Cirillo, E., Trizzino, A., Aiuti, A., Migliavacca, M., Locatelli, Franco, Bertaina, A., Naviglio, S., Carrabba, M., De Carli, M., Barbaro, M. G. F., Gattorno, M., Quinti, I., Martire, B., Locatelli F. (ORCID:0000-0002-7976-3654), Pulvirenti, F., Sangerardi, M., Plebani, A., Soresina, A., Finocchi, A., Pignata, C., Cirillo, E., Trizzino, A., Aiuti, A., Migliavacca, M., Locatelli, Franco, Bertaina, A., Naviglio, S., Carrabba, M., De Carli, M., Barbaro, M. G. F., Gattorno, M., Quinti, I., Martire, B., and Locatelli F. (ORCID:0000-0002-7976-3654)

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 (< 5 years of age), as scored by proxies. Differently, CGD adults reported higher difficulties both in mental and physical area than norms. Only for children, clinical status had a damaging effect on psychosocial and school dimensions, whereas age had a negative impact on social areas. No significant difference was observed between patients treated or not with HSCT. When compared with patients affected by chronic diseases, CGD children and adults both displayed fewer physical disabilities. Differently, in mental scale adults scored lower than those with rheumatology diseases and had similar impairment in comparison with patients with diabetes mellitus and cancer. This study emphasized the impact of CGD on HRQoL since infancy and its decline in adulthood, with emotional difficulties occurring early. HRQoL impairment should be considered in clinical picture of CGD and pro-actively assessed and managed by clinicians.

Published
2020

2. 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

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, 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, and Zicari, A

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

3. Pediatric health care professionals' vaccine knowledge, awareness and attitude: a survey within the Italian Society of Pediatric Allergy and Immunology.

Author

Del Duca, Elisabetta, Chini, Loredana, Graziani, Simona, Sgrulletti, Mayla, Moschese, Viviana, with the Italian Pediatric Immunology and Allergology Society (SIAIP) Vaccine Committee, Moschese, V., Chini, L., Sgrulletti, M., Dellepiane, R. M., Martire, B., Sangerardi, M., Montin, D., Ottaviano, G., Rizzo, C., Duse, M., and Marseglia, G.

Subjects
VACCINATION, INFLUENZA vaccines, PROFESSIONS, IMMUNIZATION, CONFIDENCE, ATTITUDE (Psychology), MEDICAL personnel, PEDIATRICS, PNEUMOCOCCAL vaccines, QUESTIONNAIRES, PEDIATRIC nursing, DESCRIPTIVE statistics, ROTAVIRUS vaccines, HERPES zoster vaccines, NURSE practitioners
Abstract

Background: Physicians play a key role in driving vaccine acceptance and their recommendations are crucial to address vaccine hesitancy. The aim of the study was to assess knowledge, awareness and attitude of Italian Pediatric Health Care Professionals (pHCPs) on vaccinations. Methods: An anonymous on-line questionnaire was developed within the Vaccine Committee of Italian Society of Pediatric Allergy and Immunology (SIAIP) and spontaneously completed by 231 Pediatricians and Pediatric Nurses (PN). Results: An accurate vaccine education was reported by 70% of pediatricians and 13% of PN but 11% of pediatricians versus 26% of PN consult social media instead of scientific sources for their vaccine update. The investigation on the pHCPs attitudes to vaccination in a personal and family setting highlights poor adherence to vaccinations. Only 63% of pediatricians versus 16% of PN (p < 0.0001) annually received the Flu vaccine. In their family setting 93% of pediatricians versus 51% of PN recommended all vaccinations (p < 0.0001). Anti-flu, anti-rotavirus, anti-zoster and anti-pneumococcal vaccines were not regularly recommended by all pHCPs due to doubts of uselessness (55% of pediatricians versus 40% of PN) and preference for "natural immunity" (44% of pediatricians versus 40% of PN). Conclusions: Our results indicate that pHCPs' attitude and confidence in regards to vaccines remain suboptimal. Current COVID-19 pandemic and the rapid development of vaccines could increase vaccine hesitancy. Due to the documented pHCPs' influence in the parental decision, educational interventions are needed to improve their level of knowledge and counselling skills in order to address parental vaccine hesitancy and to maintain continuity of immunization services. [ABSTRACT FROM AUTHOR]

Published
2021
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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. 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

6. 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

7. Vaccines and allergy: Back to the right places.

Author

Moschese V, Montin D, Ottaviano G, Sgrulletti M, Beni A, Costagliola G, Sangerardi M, Santilli V, Miraglia Del Giudice M, Rizzo C, and Martire B

Subjects
Humans, Child, Anaphylaxis prevention & control, Practice Guidelines as Topic, Vaccines adverse effects, Vaccines administration & dosage, Vaccination adverse effects, Hypersensitivity
Abstract

Hypersensitivity reactions represent one of the most common causes of hesitancy for adherence to national vaccination programs. The majority of hypersensitivity reactions after vaccination are mild, and anaphylaxis is reported to be rare, although it remains challenging to estimate the frequency attributed to each single vaccine, either because of the lower number of administered doses of less common vaccines, or the administration of simultaneous vaccine in most of the vaccination programs. Although literature remains scattered, international consensus guides clinicians in identifying patients who might need the administration of vaccines in protected environments due to demonstrated hypersensitivity to vaccine components or adjuvants. Here we provide the current guidance on hypersensitivity reactions to vaccines and on vaccination of children with allergy disorders., (© 2024 European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)

Published
2024
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8. Vaccinations in Children and Adolescents Treated With Immune-Modifying Biologics: Update and Current Developments.

Author

Martire B, Ottaviano G, Sangerardi M, Sgrulletti M, Chini L, Dellepiane RM, Montin D, Rizzo C, Pignata C, Marseglia GL, and Moschese V

Subjects
Adolescent, Child, Humans, Immunization, Quality of Life, Vaccination, Biological Products therapeutic use, Vaccines therapeutic use
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., (Copyright © 2022 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Published
2022
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9. Novel artful applications of vaccines at the horizon.

Author

Montin D, Ottaviano G, Sangerardi M, Sgrulletti M, Chini L, Dellepiane RM, Martire B, Rizzo C, and Moschese V

Subjects
Humans, Immunity, Innate, Measles Vaccine, BCG Vaccine, Virus Diseases
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., (© 2022 The Authors. Pediatric Allergy and Immunology published by European Academy of Allergy and Clinical Immunology and John Wiley & Sons Ltd.)

Published
2022
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10. One step closer to influenza vaccine inclusiveness.

Author

Sgrulletti M, Ottaviano G, Sangerardi M, Chini L, Dellepiane RM, Martire B, Montin D, Rizzo C, and Moschese V

Subjects
Aged, Child, Female, Humans, Pregnancy, Vaccination, Vaccines, Attenuated, Vaccines, Inactivated, Asthma, Influenza Vaccines, Influenza, Human prevention & control
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., (© 2020 EAACI and John Wiley and Sons A/S. Published by John Wiley and Sons Ltd.)

Published
2020
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11. 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

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 MGF, Gattorno M, Quinti I, and Martire B

Subjects
Adolescent, Adult, Caregivers, Child, Child, Preschool, Cholestyramine Resin, Female, Granulomatous Disease, Chronic psychology, Humans, Immunologic Deficiency Syndromes psychology, Italy epidemiology, Male, Middle Aged, Psychological Distress, Quality of Life, Registries, Severity of Illness Index, Young Adult, Granulomatous Disease, Chronic epidemiology, Immunologic Deficiency Syndromes epidemiology
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 (< 5 years of age), as scored by proxies. Differently, CGD adults reported higher difficulties both in mental and physical area than norms. Only for children, clinical status had a damaging effect on psychosocial and school dimensions, whereas age had a negative impact on social areas. No significant difference was observed between patients treated or not with HSCT. When compared with patients affected by chronic diseases, CGD children and adults both displayed fewer physical disabilities. Differently, in mental scale adults scored lower than those with rheumatology diseases and had similar impairment in comparison with patients with diabetes mellitus and cancer. This study emphasized the impact of CGD on HRQoL since infancy and its decline in adulthood, with emotional difficulties occurring early. HRQoL impairment should be considered in clinical picture of CGD and pro-actively assessed and managed by clinicians.

Published
2020
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12. Hepatic angiodynamic profile in paediatric patients with hereditary haemorrhagic telangiectasia type 1 and type 2.

Author

Giordano P, Francavilla M, Buonamico P, Suppressa P, Lastella P, Sangerardi M, Miniello VL, Scardapane A, Lenato GM, and Sabbà C

Subjects
Activin Receptors, Type II genetics, Adolescent, Age Factors, Arteriovenous Malformations diagnosis, Arteriovenous Malformations epidemiology, Arteriovenous Malformations genetics, Asymptomatic Diseases, Case-Control Studies, Child, Child, Preschool, Computed Tomography Angiography methods, Cross-Sectional Studies, Dilatation, Pathologic, Disease Progression, Endoglin genetics, Female, Genetic Predisposition to Disease, Hepatic Artery diagnostic imaging, Humans, Italy epidemiology, Magnetic Resonance Angiography, Male, Multidetector Computed Tomography, Mutation, Phenotype, Portal Vein abnormalities, Portal Vein diagnostic imaging, Predictive Value of Tests, Prevalence, Telangiectasia, Hereditary Hemorrhagic diagnosis, Telangiectasia, Hereditary Hemorrhagic epidemiology, Telangiectasia, Hereditary Hemorrhagic genetics, Time Factors, Ultrasonography, Doppler, Duplex, Hepatic Artery abnormalities, Liver blood supply
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
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13. Pulmonary Arteriovenous Malformations and Cerebral Abscess Recurrence in a Child With Hereditary Hemorrhagic Telangiectasia.

Author

Giordano P, Sangerardi M, Suppressa P, Lastella P, Attolini E, Valente F, Fiorella ML, Lenato GM, and Sabbà C

Subjects
Adolescent, Adult, Arteriovenous Fistula therapy, Brain Abscess surgery, Embolization, Therapeutic, Humans, Magnetic Resonance Imaging, Male, Prognosis, Recurrence, Telangiectasia, Hereditary Hemorrhagic therapy, Arteriovenous Fistula complications, Brain Abscess etiology, Brain Abscess pathology, Pulmonary Artery abnormalities, Pulmonary Veins abnormalities, Telangiectasia, Hereditary Hemorrhagic complications
Abstract

Background: A 17-year-old boy was referred to our center with a history of brain abscess (BA) recurring after 9 years. The patient reported 2 previous treatments for pulmonary arteriovenous malformations, sporadic nosebleeds, and familial history for epistaxis. Clinical investigations revealed arteriovenous malformations in lung, brain, and liver, as well as mucocutaneous telangiectases. A definite diagnosis of hereditary hemorrhagic telangiectasia was made 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 cases of BA thus far reported highlight the need to raise the suspicion of a pulmonary arteriovenous malformations, both isolated and in the context of a possible hereditary hemorrhagic telangiectasia, for any case of BA of unexplained etiology, in children as well as in adults.

Published
2015
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14. Autoimmune haematological disorders in two Italian children with Kabuki syndrome.

Author

Giordano P, Lassandro G, Sangerardi M, Faienza MF, Valente F, and Martire B

Subjects
Abnormalities, Multiple drug therapy, Abnormalities, Multiple genetics, Anemia, Hemolytic, Autoimmune drug therapy, Anemia, Hemolytic, Autoimmune genetics, Biopsy, Fine-Needle, Child, DNA analysis, DNA-Binding Proteins genetics, DNA-Binding Proteins metabolism, Diagnosis, Differential, Drug Therapy, Combination, Female, Glucocorticoids therapeutic use, Hematologic Diseases drug therapy, Hematologic Diseases genetics, Humans, Immunologic Factors therapeutic use, Male, Neoplasm Proteins genetics, Neoplasm Proteins metabolism, Purpura, Thrombocytopenic, Idiopathic drug therapy, Purpura, Thrombocytopenic, Idiopathic genetics, Vestibular Diseases drug therapy, Vestibular Diseases genetics, Abnormalities, Multiple diagnosis, Anemia, Hemolytic, Autoimmune diagnosis, Face abnormalities, Genetic Testing methods, Hematologic Diseases diagnosis, Immunoglobulins, Intravenous therapeutic use, Prednisolone therapeutic use, Purpura, Thrombocytopenic, Idiopathic diagnosis, Vestibular Diseases diagnosis
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.

Published
2014
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15. Hereditary hemorrhagic telangiectasia: arteriovenous malformations in children.

Author

Giordano P, Lenato GM, Suppressa P, Lastella P, Dicuonzo F, Chiumarulo L, Sangerardi M, Piccarreta P, Valerio R, Scardapane A, Marano G, Resta N, Quaranta N, and Sabbà C

Subjects
Adolescent, Arteriovenous Malformations epidemiology, Arteriovenous Malformations genetics, Child, Child, Preschool, Cross-Sectional Studies, Female, Humans, Infant, Male, Prevalence, Prospective Studies, Telangiectasia, Hereditary Hemorrhagic genetics, Arteriovenous Malformations etiology, Telangiectasia, Hereditary Hemorrhagic complications
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 clinical-radiologic 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., (Copyright © 2013 Mosby, Inc. All rights reserved.)

Published
2013
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16. Association of interleukin-(IL)10 haplotypes and serum IL-10 levels in the progression of childhood immune thrombocytopenic purpura.

Author

Tesse R, Del Vecchio GC, De Mattia D, Sangerardi M, Valente F, and Giordano P

Subjects
Adolescent, Biomarkers blood, Child, Child, Preschool, Chronic Disease, Female, Humans, Infant, Male, Gene Expression Regulation, Haplotypes, Interleukin-10 blood, Interleukin-10 genetics, Polymorphism, Single Nucleotide, Promoter Regions, Genetic, Purpura, Thrombocytopenic, Idiopathic blood, Purpura, Thrombocytopenic, Idiopathic genetics
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), p<0.01)]. 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., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Published
2012
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