Clinical Research Unit, Walter Cantídio University Hospital, Federal University of Ceará, Fortaleza 60416200, CE, Brazil.
Int J Environ Res Public Health. 2021 Sep 15;18(18):9724. doi: 10.3390/ijerph18189724.
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.
先天性全身性脂肪营养不良（CGL）是一种罕见的综合征，其特征为由于脂肪细胞储存脂质的能力缺失，导致几乎完全缺乏皮下脂肪组织。患者表现为全身皮下脂肪缺乏和正常至低体重。他们会出现严重的代谢紊乱、非酒精性脂肪肝、早期心脏异常和感染性并发症。尽管低体重是骨质疏松症的已知危险因素，但据报道，1 型和 2 型 CGL 有较高骨矿物质密度（BMD）的趋势。在这篇综述中，我们讨论了骨髓组织、脂肪因子和胰岛素抵抗在 CGL 患者正常至高 BMD 中的作用。从 Pubmed 和 LILACS 数据库中进行了检索，并获得了截至 2021 年 4 月 10 日发表的 113 篇文章。其中，有 76 篇因未涵盖综述主题而被排除在外。使用找到的研究的参考文献进行了额外文献的手动搜索。阐明在这种独特的胰岛素抵抗模型中导致 BMD 增加的机制，可能有助于从病理生理学和治疗角度理解骨骼、肌肉和脂肪组织之间的相互关系。
