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Case Report: Liraglutide for Weight Management in Beckwith-Wiedemann Syndromic Obesity

Genetic obesity, including syndromic and non-syndromic forms, represents a minority of cases compared to essential obesity but gene dysregulations lead to complex clinical conditions that make their management particularly difficult. Among them, Beckwith-Wiedemann syndrome (BWS) is a multisystem hum...

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Autores principales: Caputo, Marina, Daffara, Tommaso, Bellone, Simonetta, Mancioppi, Valentina, Marzullo, Paolo, Aimaretti, Gianluca, Prodam, Flavia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8258411/
https://www.ncbi.nlm.nih.gov/pubmed/34239499
http://dx.doi.org/10.3389/fendo.2021.687918
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author Caputo, Marina
Daffara, Tommaso
Bellone, Simonetta
Mancioppi, Valentina
Marzullo, Paolo
Aimaretti, Gianluca
Prodam, Flavia
author_facet Caputo, Marina
Daffara, Tommaso
Bellone, Simonetta
Mancioppi, Valentina
Marzullo, Paolo
Aimaretti, Gianluca
Prodam, Flavia
author_sort Caputo, Marina
collection PubMed
description Genetic obesity, including syndromic and non-syndromic forms, represents a minority of cases compared to essential obesity but gene dysregulations lead to complex clinical conditions that make their management particularly difficult. Among them, Beckwith-Wiedemann syndrome (BWS) is a multisystem human genomic imprinting disorder characterized by overgrowth. We describe the first case of liraglutide treatment in an 18-year-old boy patient affected by BWS complicated by macroglossia, cryptorchidism, nephroblastoma, organomegaly, microscopic lymphocytic colitis, pharmacologically treated arterial hypertension, obesity, and obstructive sleep apnea syndrome. He presented a normal cognitive development. Body mass index at the time of first transition visit in the adult endocrinology department at the age of 18-years-old was 40.6 kg/m(2) without glucose metabolism impairment. Lifestyle interventions failed because of poor compliance. During 20 months of 3.0 mg liraglutide treatment, a weight loss of 19 kg (−13.3%) and BMI reduction of 6.8 points were registered without side effects. To date, liraglutide treatment was effective on obesity in 7 subjects with Prader Willy Syndrome and 14 with melanocortin-4 receptor mutations. The efficacy of liraglutide in BWS could be related to a crosstalk among glucagon-like peptide (GLP)-1 system, mechanisms related to the cyclin-dependent kinase inhibitor 1C (CDKN1C), and dopamine mesolimbic circuit. Clinical trials aiming at a tailored medicine in genetic obesity are needed.
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spelling pubmed-82584112021-07-07 Case Report: Liraglutide for Weight Management in Beckwith-Wiedemann Syndromic Obesity Caputo, Marina Daffara, Tommaso Bellone, Simonetta Mancioppi, Valentina Marzullo, Paolo Aimaretti, Gianluca Prodam, Flavia Front Endocrinol (Lausanne) Endocrinology Genetic obesity, including syndromic and non-syndromic forms, represents a minority of cases compared to essential obesity but gene dysregulations lead to complex clinical conditions that make their management particularly difficult. Among them, Beckwith-Wiedemann syndrome (BWS) is a multisystem human genomic imprinting disorder characterized by overgrowth. We describe the first case of liraglutide treatment in an 18-year-old boy patient affected by BWS complicated by macroglossia, cryptorchidism, nephroblastoma, organomegaly, microscopic lymphocytic colitis, pharmacologically treated arterial hypertension, obesity, and obstructive sleep apnea syndrome. He presented a normal cognitive development. Body mass index at the time of first transition visit in the adult endocrinology department at the age of 18-years-old was 40.6 kg/m(2) without glucose metabolism impairment. Lifestyle interventions failed because of poor compliance. During 20 months of 3.0 mg liraglutide treatment, a weight loss of 19 kg (−13.3%) and BMI reduction of 6.8 points were registered without side effects. To date, liraglutide treatment was effective on obesity in 7 subjects with Prader Willy Syndrome and 14 with melanocortin-4 receptor mutations. The efficacy of liraglutide in BWS could be related to a crosstalk among glucagon-like peptide (GLP)-1 system, mechanisms related to the cyclin-dependent kinase inhibitor 1C (CDKN1C), and dopamine mesolimbic circuit. Clinical trials aiming at a tailored medicine in genetic obesity are needed. Frontiers Media S.A. 2021-06-22 /pmc/articles/PMC8258411/ /pubmed/34239499 http://dx.doi.org/10.3389/fendo.2021.687918 Text en Copyright © 2021 Caputo, Daffara, Bellone, Mancioppi, Marzullo, Aimaretti and Prodam https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Endocrinology
Caputo, Marina
Daffara, Tommaso
Bellone, Simonetta
Mancioppi, Valentina
Marzullo, Paolo
Aimaretti, Gianluca
Prodam, Flavia
Case Report: Liraglutide for Weight Management in Beckwith-Wiedemann Syndromic Obesity
title Case Report: Liraglutide for Weight Management in Beckwith-Wiedemann Syndromic Obesity
title_full Case Report: Liraglutide for Weight Management in Beckwith-Wiedemann Syndromic Obesity
title_fullStr Case Report: Liraglutide for Weight Management in Beckwith-Wiedemann Syndromic Obesity
title_full_unstemmed Case Report: Liraglutide for Weight Management in Beckwith-Wiedemann Syndromic Obesity
title_short Case Report: Liraglutide for Weight Management in Beckwith-Wiedemann Syndromic Obesity
title_sort case report: liraglutide for weight management in beckwith-wiedemann syndromic obesity
topic Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8258411/
https://www.ncbi.nlm.nih.gov/pubmed/34239499
http://dx.doi.org/10.3389/fendo.2021.687918
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