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Bone Mineral Density in Congenital Generalized Lipodystrophy: The Role of Bone Marrow Tissue, Adipokines, and Insulin Resistance

Congenital Generalized Lipodystrophy (CGL) is a rare syndrome characterized by the almost total absence of subcutaneous adipose tissue due to the inability of storing lipid in adipocytes. Patients present generalized lack of subcutaneous fat and normal to low weight. They evolve with severe metaboli...

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Autores principales: Freire, Erika Bastos Lima, d’Alva, Catarina Brasil, Madeira, Mayara Ponte, Lima, Grayce Ellen da Cruz Paiva, Montenegro, Ana Paula Dias Rangel, Fernandes, Virginia Oliveira, Montenegro Junior, Renan Magalhães
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8465110/
https://www.ncbi.nlm.nih.gov/pubmed/34574647
http://dx.doi.org/10.3390/ijerph18189724
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author Freire, Erika Bastos Lima
d’Alva, Catarina Brasil
Madeira, Mayara Ponte
Lima, Grayce Ellen da Cruz Paiva
Montenegro, Ana Paula Dias Rangel
Fernandes, Virginia Oliveira
Montenegro Junior, Renan Magalhães
author_facet Freire, Erika Bastos Lima
d’Alva, Catarina Brasil
Madeira, Mayara Ponte
Lima, Grayce Ellen da Cruz Paiva
Montenegro, Ana Paula Dias Rangel
Fernandes, Virginia Oliveira
Montenegro Junior, Renan Magalhães
author_sort Freire, Erika Bastos Lima
collection PubMed
description Congenital Generalized Lipodystrophy (CGL) is a rare syndrome characterized by the almost total absence of subcutaneous adipose tissue due to the inability of storing lipid in adipocytes. Patients present generalized lack of subcutaneous fat and normal to low weight. They evolve with severe metabolic disorders, non-alcoholic fatty liver disease, early cardiac abnormalities, and infectious complications. Although low body weight is a known risk factor for osteoporosis, it has been reported that type 1 and 2 CGL have a tendency of high bone mineral density (BMD). In this review, we discuss the role of bone marrow tissue, adipokines, and insulin resistance in the setting of the normal to high BMD of CGL patients. Data bases from Pubmed and LILACS were searched, and 113 articles published until 10 April 2021 were obtained. Of these, 76 were excluded for not covering the review topic. A manual search for additional literature was performed using the bibliographies of the studies located. The elucidation of the mechanisms responsible for the increase in BMD in this unique model of insulin resistance may contribute to the understanding of the interrelationships between bone, muscle, and adipose tissue in a pathophysiological and therapeutic perspective.
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spelling pubmed-84651102021-09-27 Bone Mineral Density in Congenital Generalized Lipodystrophy: The Role of Bone Marrow Tissue, Adipokines, and Insulin Resistance Freire, Erika Bastos Lima d’Alva, Catarina Brasil Madeira, Mayara Ponte Lima, Grayce Ellen da Cruz Paiva Montenegro, Ana Paula Dias Rangel Fernandes, Virginia Oliveira Montenegro Junior, Renan Magalhães Int J Environ Res Public Health Review Congenital Generalized Lipodystrophy (CGL) is a rare syndrome characterized by the almost total absence of subcutaneous adipose tissue due to the inability of storing lipid in adipocytes. Patients present generalized lack of subcutaneous fat and normal to low weight. They evolve with severe metabolic disorders, non-alcoholic fatty liver disease, early cardiac abnormalities, and infectious complications. Although low body weight is a known risk factor for osteoporosis, it has been reported that type 1 and 2 CGL have a tendency of high bone mineral density (BMD). In this review, we discuss the role of bone marrow tissue, adipokines, and insulin resistance in the setting of the normal to high BMD of CGL patients. Data bases from Pubmed and LILACS were searched, and 113 articles published until 10 April 2021 were obtained. Of these, 76 were excluded for not covering the review topic. A manual search for additional literature was performed using the bibliographies of the studies located. The elucidation of the mechanisms responsible for the increase in BMD in this unique model of insulin resistance may contribute to the understanding of the interrelationships between bone, muscle, and adipose tissue in a pathophysiological and therapeutic perspective. MDPI 2021-09-15 /pmc/articles/PMC8465110/ /pubmed/34574647 http://dx.doi.org/10.3390/ijerph18189724 Text en © 2021 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 Review
Freire, Erika Bastos Lima
d’Alva, Catarina Brasil
Madeira, Mayara Ponte
Lima, Grayce Ellen da Cruz Paiva
Montenegro, Ana Paula Dias Rangel
Fernandes, Virginia Oliveira
Montenegro Junior, Renan Magalhães
Bone Mineral Density in Congenital Generalized Lipodystrophy: The Role of Bone Marrow Tissue, Adipokines, and Insulin Resistance
title Bone Mineral Density in Congenital Generalized Lipodystrophy: The Role of Bone Marrow Tissue, Adipokines, and Insulin Resistance
title_full Bone Mineral Density in Congenital Generalized Lipodystrophy: The Role of Bone Marrow Tissue, Adipokines, and Insulin Resistance
title_fullStr Bone Mineral Density in Congenital Generalized Lipodystrophy: The Role of Bone Marrow Tissue, Adipokines, and Insulin Resistance
title_full_unstemmed Bone Mineral Density in Congenital Generalized Lipodystrophy: The Role of Bone Marrow Tissue, Adipokines, and Insulin Resistance
title_short Bone Mineral Density in Congenital Generalized Lipodystrophy: The Role of Bone Marrow Tissue, Adipokines, and Insulin Resistance
title_sort bone mineral density in congenital generalized lipodystrophy: the role of bone marrow tissue, adipokines, and insulin resistance
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8465110/
https://www.ncbi.nlm.nih.gov/pubmed/34574647
http://dx.doi.org/10.3390/ijerph18189724
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