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

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 mil...

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Autores principales: Pederiva, Cristina, Gazzotti, Marta, Arca, Marcello, Averna, Maurizio, Banderali, Giuseppe, Biasucci, Giacomo, Brambilla, Marta, Buonuomo, Paola Sabrina, Calabrò, Paolo, Cipollone, Francesco, Citroni, Nadia, D’Addato, Sergio, Del Ben, Maria, Genovesi, Simonetta, Guardamagna, Ornella, Iannuzzo, Gabriella, Iughetti, Lorenzo, Mandraffino, Giuseppe, Maroni, Lorenzo, Mombelli, Giuliana, Muntoni, Sandro, Nascimbeni, Fabio, Passaro, Angelina, Pellegatta, Fabio, Pirro, Matteo, Pisciotta, Livia, Pujia, Roberta, Sarzani, Riccardo, Scicali, Roberto, Suppressa, Patrizia, Zambon, Sabina, Zenti, Maria Grazia, Calandra, Sebastiano, Catapano, Alberico Luigi, Tarugi, Patrizia, Galimberti, Federica, Casula, Manuela, Capra, Maria Elena
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10420921/
https://www.ncbi.nlm.nih.gov/pubmed/37571405
http://dx.doi.org/10.3390/nu15153468
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author Pederiva, Cristina
Gazzotti, Marta
Arca, Marcello
Averna, Maurizio
Banderali, Giuseppe
Biasucci, Giacomo
Brambilla, Marta
Buonuomo, Paola Sabrina
Calabrò, Paolo
Cipollone, Francesco
Citroni, Nadia
D’Addato, Sergio
Del Ben, Maria
Genovesi, Simonetta
Guardamagna, Ornella
Iannuzzo, Gabriella
Iughetti, Lorenzo
Mandraffino, Giuseppe
Maroni, Lorenzo
Mombelli, Giuliana
Muntoni, Sandro
Nascimbeni, Fabio
Passaro, Angelina
Pellegatta, Fabio
Pirro, Matteo
Pisciotta, Livia
Pujia, Roberta
Sarzani, Riccardo
Scicali, Roberto
Suppressa, Patrizia
Zambon, Sabina
Zenti, Maria Grazia
Calandra, Sebastiano
Catapano, Alberico Luigi
Tarugi, Patrizia
Galimberti, Federica
Casula, Manuela
Capra, Maria Elena
author_facet Pederiva, Cristina
Gazzotti, Marta
Arca, Marcello
Averna, Maurizio
Banderali, Giuseppe
Biasucci, Giacomo
Brambilla, Marta
Buonuomo, Paola Sabrina
Calabrò, Paolo
Cipollone, Francesco
Citroni, Nadia
D’Addato, Sergio
Del Ben, Maria
Genovesi, Simonetta
Guardamagna, Ornella
Iannuzzo, Gabriella
Iughetti, Lorenzo
Mandraffino, Giuseppe
Maroni, Lorenzo
Mombelli, Giuliana
Muntoni, Sandro
Nascimbeni, Fabio
Passaro, Angelina
Pellegatta, Fabio
Pirro, Matteo
Pisciotta, Livia
Pujia, Roberta
Sarzani, Riccardo
Scicali, Roberto
Suppressa, Patrizia
Zambon, Sabina
Zenti, Maria Grazia
Calandra, Sebastiano
Catapano, Alberico Luigi
Tarugi, Patrizia
Galimberti, Federica
Casula, Manuela
Capra, Maria Elena
author_sort Pederiva, Cristina
collection PubMed
description 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.
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spelling pubmed-104209212023-08-12 Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group Pederiva, Cristina Gazzotti, Marta Arca, Marcello Averna, Maurizio Banderali, Giuseppe Biasucci, Giacomo Brambilla, Marta Buonuomo, Paola Sabrina Calabrò, Paolo Cipollone, Francesco Citroni, Nadia D’Addato, Sergio Del Ben, Maria Genovesi, Simonetta Guardamagna, Ornella Iannuzzo, Gabriella Iughetti, Lorenzo Mandraffino, Giuseppe Maroni, Lorenzo Mombelli, Giuliana Muntoni, Sandro Nascimbeni, Fabio Passaro, Angelina Pellegatta, Fabio Pirro, Matteo Pisciotta, Livia Pujia, Roberta Sarzani, Riccardo Scicali, Roberto Suppressa, Patrizia Zambon, Sabina Zenti, Maria Grazia Calandra, Sebastiano Catapano, Alberico Luigi Tarugi, Patrizia Galimberti, Federica Casula, Manuela Capra, Maria Elena Nutrients Article 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. MDPI 2023-08-05 /pmc/articles/PMC10420921/ /pubmed/37571405 http://dx.doi.org/10.3390/nu15153468 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Pederiva, Cristina
Gazzotti, Marta
Arca, Marcello
Averna, Maurizio
Banderali, Giuseppe
Biasucci, Giacomo
Brambilla, Marta
Buonuomo, Paola Sabrina
Calabrò, Paolo
Cipollone, Francesco
Citroni, Nadia
D’Addato, Sergio
Del Ben, Maria
Genovesi, Simonetta
Guardamagna, Ornella
Iannuzzo, Gabriella
Iughetti, Lorenzo
Mandraffino, Giuseppe
Maroni, Lorenzo
Mombelli, Giuliana
Muntoni, Sandro
Nascimbeni, Fabio
Passaro, Angelina
Pellegatta, Fabio
Pirro, Matteo
Pisciotta, Livia
Pujia, Roberta
Sarzani, Riccardo
Scicali, Roberto
Suppressa, Patrizia
Zambon, Sabina
Zenti, Maria Grazia
Calandra, Sebastiano
Catapano, Alberico Luigi
Tarugi, Patrizia
Galimberti, Federica
Casula, Manuela
Capra, Maria Elena
Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group
title Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group
title_full Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group
title_fullStr Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group
title_full_unstemmed Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group
title_short Clinical Approach in the Management of Paediatric Patients with Familial Hypercholesterolemia: A National Survey Conducted by the LIPIGEN Paediatric Group
title_sort clinical approach in the management of paediatric patients with familial hypercholesterolemia: a national survey conducted by the lipigen paediatric group
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10420921/
https://www.ncbi.nlm.nih.gov/pubmed/37571405
http://dx.doi.org/10.3390/nu15153468
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