Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group-Reference-Cited by-同舟云学术

Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group

Published:2023-08-05 Issue:15 Volume:15 Page:3468
ISSN:2072-6643
Container-title:Nutrients
language:en
Short-container-title:Nutrients

Author:

Pederiva Cristina¹^ORCID,Gazzotti Marta²,Arca Marcello³⁴^ORCID,Averna Maurizio⁵⁶,Banderali Giuseppe¹,Biasucci Giacomo⁷^ORCID,Brambilla Marta⁸,Buonuomo Paola⁹,Calabrò Paolo¹⁰¹¹^ORCID,Cipollone Francesco¹²,Citroni Nadia¹³,D’Addato Sergio¹⁴¹⁵^ORCID,Del Ben Maria⁴¹⁶^ORCID,Genovesi Simonetta¹⁷¹⁸^ORCID,Guardamagna Ornella¹⁹,Iannuzzo Gabriella²⁰^ORCID,Iughetti Lorenzo²¹^ORCID,Mandraffino Giuseppe²²^ORCID,Maroni Lorenzo²³,Mombelli Giuliana²⁴,Muntoni Sandro²⁵²⁶,Nascimbeni Fabio²⁷²⁸,Passaro Angelina²⁹³⁰^ORCID,Pellegatta Fabio³¹³²,Pirro Matteo³³,Pisciotta Livia³⁴^ORCID,Pujia Roberta³⁵,Sarzani Riccardo³⁶³⁷,Scicali Roberto³⁸^ORCID,Suppressa Patrizia³⁹,Zambon Sabina⁴⁰,Zenti Maria⁴¹^ORCID,Calandra Sebastiano²⁸,Catapano Alberico³¹,Tarugi Patrizia⁴²,Galimberti Federica³¹^ORCID,Casula Manuela³¹⁴³^ORCID,Capra Maria⁷

Affiliation:

1. Clinical Service for Dyslipidaemias, Study and Prevention of Atherosclerosis in Childhood, Paediatrics Unit, ASST-Santi Paolo e Carlo, 20142 Milan, Italy

2. Fondazione SISA (Società Italiana per lo Studio dell’Aterosclerosi), 20133 Milan, Italy

3. Dipartimento di Medicina Traslazionale e di Precisione, Università La Sapienza di Roma, 00185 Rome, Italy

4. AO Policlinico Umberto I, 00161 Rome, Italy

5. Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, University of Palermo, 90127 Palermo, Italy

6. Istituto di Biofisica, Consiglio Nazionale Delle Ricerche, 90146 Palermo, Italy

7. Centre for Paediatric Dyslipidaemias, Paediatrics and Neonatology Unit, Guglielmo da Saliceto Hospital, 29121 Piacenza, Italy

8. Unit of Cell and Molecular Biology in Cardiovascular Diseases, Centro Cardiologico Monzino IRCCS, 20138 Milan, Italy

9. Rare Diseases and Medical Genetic Unit, Ospedale Pediatrico Bambino Gesù, IRCCS, 00163 Rome, Italy

10. UOC Cardiologia Clinica a Direzione Universitaria e UTIC, AORN “Sant’Anna e San Sebastiano”, 81100 Caserta, Italy

11. Dipartimento di Scienze Mediche Traslazionali, Università Degli Studi Della Campania “Luigi Vanvitelli”, 80131 Naples, Italy

12. 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

13. Centro Dislipidemie e Aterosclerosi, Ospedale di Trento, APSS-Trento, 38122 Trento, Italy

14. UO di Medicina Interna Cardiovascolare, Ambulatorio Dislipidemie, Università di Bologna, 40138 Bologna, Italy

15. IRCCS S Orsola, 40138 Bologna, Italy

16. Dipartimento Scienze Cliniche, Internistiche, Anestesiologiche e Cardiovascolari, Università La Sapenza di Roma, 00161 Rome, Italy

17. Istituto Auxologico Italiano, IRCCS, 20149 Milan, Italy

18. Dipartimento di Medicina e Chirurgia, Università di Milano-Bicocca, 20126 Milan, Italy

19. Department of Public Health and Paediatric Sciences, Turin University, 10126 Turin, Italy

20. Dipartimento di Medicina Clinica e Chirurgia, Università degli Studi di Napoli Federico II, 80131 Naples, Italy

21. U.O.C. Pediatria, Azienda Ospedaliero Universitaria di Modena, 41124 Modena, Italy

22. Department of Clinical and Experimental Medicine, Lipid Centre, University Hospital G Martino, 98100 Messina, Italy

23. Ambulatorio Ipertensione Dislipidemie, UO Medicina Generale, ASST Valle Olona, Ospedale di Gallarate, 21013 Gallarate, Italy

24. Centro Dislipidemie ASST Grande Ospedale Metropolitano Niguarda, 20162 Milan, Italy

25. Dipartimento di Scienze Biomediche, Università Degli Studi di Cagliari, 09124 Cagliari, Italy

26. Centro per le Malattie Dismetaboliche e l’Arteriosclerosi, Associazione ME DI CO Onlus Cagliari, 09123 Cagliari, Italy

27. UO Medicina Interna Metabolica, Lipidology Centre, Baggiovara Hospital, AOU of Modena, 41126 Modena, Italy

28. Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio Emilia, 41125 Modena, Italy

29. 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

30. Dipartimento di Medicina Traslazionale e per la Romagna, Università degli Studi di Ferrara, 44122 Ferrara, Italy

31. IRCCS MultiMedica, 20099 Sesto San Giovanni, Italy

32. Centro per lo Studio dell’Aterosclerosi, Ospedale E Bassini, 20092 Cinisello Balsamo, Italy

33. Sezione Medicina Interna, Angiologia e Malattie da Arteriosclerosi, Dipartimento di Medicina e Chirurgia, Università Degli Studi di Perugia, 06132 Perugia, Italy

34. IRCCS Ospedale Policlinico San Martino UOSD Dietetica e Nutrizione Clinica, Dipartimento di Medicina Interna, Università di Genova, 16132 Genoa, Italy

35. Dipartimento Scienze Mediche Chirurgiche, Università Degli Studi Magna Graecia, 88100 Catanzaro, Italy

36. Clinica Medica e Geriatrica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica Delle Marche, 60126 Ancona, Italy

37. IRCCS-INRCA, 60124 Ancona, Italy

38. Department of Clinical and Experimental Medicine, University of Catania, Ospedale Garibaldi, 95122 Catania, Italy

39. Department of Internal Medicine and Rare Diseases Centre “C. Frugoni”, University Hospital of Bari, 70124 Bari, Italy

40. Dipartimento di Medicina, Università di Padova, 35128 Padua, Italy

41. Servizio di Diabetologia e Malattie Metaboliche “Ospedale P. Pederzoli”, Casa di Cura Privata, 37019 Peschiera del Garda, Italy

42. Department of Life Sciences, University of Modena and Reggio Emilia, 41125 Modena, Italy

43. Epidemiology and Preventive Pharmacology Service (SEFAP), Department of Pharmacological and Biomolecular Sciences, University of Milan, 20133 Milan, Italy

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.

Publisher

MDPI AG

Subject

Food Science,Nutrition and Dietetics

Link

https://www.mdpi.com/2072-6643/15/15/3468/pdf

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