Nutrients (Aug 2023)
Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group
- Cristina Pederiva,
- Marta Gazzotti,
- Marcello Arca,
- Maurizio Averna,
- Giuseppe Banderali,
- Giacomo Biasucci,
- Marta Brambilla,
- Paola Sabrina Buonuomo,
- Paolo Calabrò,
- Francesco Cipollone,
- Nadia Citroni,
- Sergio D’Addato,
- Maria Del Ben,
- Simonetta Genovesi,
- Ornella Guardamagna,
- Gabriella Iannuzzo,
- Lorenzo Iughetti,
- Giuseppe Mandraffino,
- Lorenzo Maroni,
- Giuliana Mombelli,
- Sandro Muntoni,
- Fabio Nascimbeni,
- Angelina Passaro,
- Fabio Pellegatta,
- Matteo Pirro,
- Livia Pisciotta,
- Roberta Pujia,
- Riccardo Sarzani,
- Roberto Scicali,
- Patrizia Suppressa,
- Sabina Zambon,
- Maria Grazia Zenti,
- Sebastiano Calandra,
- Alberico Luigi Catapano,
- Patrizia Tarugi,
- Federica Galimberti,
- Manuela Casula,
- Maria Elena Capra
Affiliations
- Cristina Pederiva
- Clinical Service for Dyslipidaemias, Study and Prevention of Atherosclerosis in Childhood, Paediatrics Unit, ASST-Santi Paolo e Carlo, 20142 Milan, Italy
- Marta Gazzotti
- Fondazione SISA (Società Italiana per lo Studio dell’Aterosclerosi), 20133 Milan, Italy
- Marcello Arca
- Dipartimento di Medicina Traslazionale e di Precisione, Università La Sapienza di Roma, 00185 Rome, Italy
- Maurizio Averna
- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University of Palermo, 90127 Palermo, Italy
- Giuseppe Banderali
- Clinical Service for Dyslipidaemias, Study and Prevention of Atherosclerosis in Childhood, Paediatrics Unit, ASST-Santi Paolo e Carlo, 20142 Milan, Italy
- Giacomo Biasucci
- Centre for Paediatric Dyslipidaemias, Paediatrics and Neonatology Unit, Guglielmo da Saliceto Hospital, 29121 Piacenza, Italy
- Marta Brambilla
- Unit of Cell and Molecular Biology in Cardiovascular Diseases, Centro Cardiologico Monzino IRCCS, 20138 Milan, Italy
- Paola Sabrina Buonuomo
- Rare Diseases and Medical Genetic Unit, Ospedale Pediatrico Bambino Gesù, IRCCS, 00163 Rome, Italy
- Paolo Calabrò
- UOC Cardiologia Clinica a Direzione Universitaria e UTIC, AORN “Sant’Anna e San Sebastiano”, 81100 Caserta, Italy
- Francesco Cipollone
- Clinica Medica, Centro di Alta Specializzazione per la Prevenzione dell’Aterosclerosi, Centro di Eccellenza ESH per L’ipertensione Arteriosa, Centro di Riferimento Regionale per le Dislipidemie, Ospedale Policlinico SS Annunziata, 66100 Chieti, Italy
- Nadia Citroni
- Centro Dislipidemie e Aterosclerosi, Ospedale di Trento, APSS-Trento, 38122 Trento, Italy
- Sergio D’Addato
- UO di Medicina Interna Cardiovascolare, Ambulatorio Dislipidemie, Università di Bologna, 40138 Bologna, Italy
- Maria Del Ben
- AO Policlinico Umberto I, 00161 Rome, Italy
- Simonetta Genovesi
- Istituto Auxologico Italiano, IRCCS, 20149 Milan, Italy
- Ornella Guardamagna
- Department of Public Health and Paediatric Sciences, Turin University, 10126 Turin, Italy
- Gabriella Iannuzzo
- Dipartimento di Medicina Clinica e Chirurgia, Università degli Studi di Napoli Federico II, 80131 Naples, Italy
- Lorenzo Iughetti
- U.O.C. Pediatria, Azienda Ospedaliero Universitaria di Modena, 41124 Modena, Italy
- Giuseppe Mandraffino
- Department of Clinical and Experimental Medicine, Lipid Centre, University Hospital G Martino, 98100 Messina, Italy
- Lorenzo Maroni
- Ambulatorio Ipertensione Dislipidemie, UO Medicina Generale, ASST Valle Olona, Ospedale di Gallarate, 21013 Gallarate, Italy
- Giuliana Mombelli
- Centro Dislipidemie ASST Grande Ospedale Metropolitano Niguarda, 20162 Milan, Italy
- Sandro Muntoni
- Dipartimento di Scienze Biomediche, Università Degli Studi di Cagliari, 09124 Cagliari, Italy
- Fabio Nascimbeni
- UO Medicina Interna Metabolica, Lipidology Centre, Baggiovara Hospital, AOU of Modena, 41126 Modena, Italy
- Angelina Passaro
- Centro per lo Studio e il Trattamento Delle Malattie del Metabolismo, Aterosclerosi e Nutrizione Clinica, Azienda Ospedaliera-Universitaria S Anna di Ferrara, 44124 Ferrara, Italy
- Fabio Pellegatta
- IRCCS MultiMedica, 20099 Sesto San Giovanni, Italy
- Matteo Pirro
- Sezione Medicina Interna, Angiologia e Malattie da Arteriosclerosi, Dipartimento di Medicina e Chirurgia, Università Degli Studi di Perugia, 06132 Perugia, Italy
- Livia Pisciotta
- IRCCS Ospedale Policlinico San Martino UOSD Dietetica e Nutrizione Clinica, Dipartimento di Medicina Interna, Università di Genova, 16132 Genoa, Italy
- Roberta Pujia
- Dipartimento Scienze Mediche Chirurgiche, Università Degli Studi Magna Graecia, 88100 Catanzaro, Italy
- Riccardo Sarzani
- Clinica Medica e Geriatrica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica Delle Marche, 60126 Ancona, Italy
- Roberto Scicali
- Department of Clinical and Experimental Medicine, University of Catania, Ospedale Garibaldi, 95122 Catania, Italy
- Patrizia Suppressa
- Department of Internal Medicine and Rare Diseases Centre “C. Frugoni”, University Hospital of Bari, 70124 Bari, Italy
- Sabina Zambon
- Dipartimento di Medicina, Università di Padova, 35128 Padua, Italy
- Maria Grazia Zenti
- Servizio di Diabetologia e Malattie Metaboliche “Ospedale P. Pederzoli”, Casa di Cura Privata, 37019 Peschiera del Garda, Italy
- Sebastiano Calandra
- Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, 41125 Modena, Italy
- Alberico Luigi Catapano
- IRCCS MultiMedica, 20099 Sesto San Giovanni, Italy
- Patrizia Tarugi
- Department of Life Sciences, University of Modena and Reggio Emilia, 41125 Modena, Italy
- Federica Galimberti
- IRCCS MultiMedica, 20099 Sesto San Giovanni, Italy
- Manuela Casula
- IRCCS MultiMedica, 20099 Sesto San Giovanni, Italy
- Maria Elena Capra
- Centre for Paediatric Dyslipidaemias, Paediatrics and Neonatology Unit, Guglielmo da Saliceto Hospital, 29121 Piacenza, Italy
- DOI
- https://doi.org/10.3390/nu15153468
- Journal volume & issue
-
Vol. 15,
no. 15
p. 3468
Abstract
Detection and treatment of patients with familial hypercholesterolemia (FH) starting from childhood is fundamental to reduce morbidity and mortality. The activity of National realities such as the LIPIGEN (LIpid transPort disorders Italian GEnetic Network) Paediatric Group, founded in 2018, is a milestone in this context. The aim of this exploratory survey, conducted in October 2021 among Italian lipid clinics included in the LIPIGEN Paediatric Group, was to investigate the current clinical approach in the management and treatment of paediatric patients with suspected FH. A digital questionnaire composed of 20 questions investigating nutritional treatment and nutraceutical and pharmacological therapy for children and adolescents with FH was proposed to the principal investigators of 30 LIPIGEN centres. Twenty-four centres responded to the section referring to children aged < 10 years and 30 to that referring to adolescents. Overall, 66.7% of children and 73.3% of adolescents were given lipid-lowering nutritional treatment as the first intervention level for at least 3–4 months (29.2% and 23.3%) or 6–12 months (58.3% and 53.3%). Nutraceuticals were considered in 41.7% (regarding children) and 50.0% (regarding adolescents) of the centres as a supplementary approach to diet. Lipid-lowering drug therapy initiation was mainly recommended (91.7% and 80.0%). In 83.3% of children and 96.7% of adolescents, statins were the most frequently prescribed drug. We highlighted several differences in the treatment of paediatric patients with suspected FH among Italian centres; however, the overall approach is in line with the European Atherosclerosis Society (EAS) recommendations for FH children and adolescents. We consider this survey as a starting point to reinforce collaboration between LIPIGEN centres and to elaborate in the near future a consensus document on the management of paediatric patients with suspected FH so as to improve and uniform detection, management, and treatment of these patients in our country.
Keywords