Division of Metabolism, Endocrinology and Diabetes (MEND), Department of Internal Medicine, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA.
Division of Endocrinology and Metabolism, Department of Internal Medicine, Ribeirao Preto Medical School, Sao Paulo University, Ribeirao Preto, Brazil.
Expert Rev Endocrinol Metab. 2020 Mar;15(2):95-114. doi: 10.1080/17446651.2020.1735360.
: Lipodystrophy is a heterogeneous group of rare diseases characterized by various degrees of fat loss which leads to serious morbidity due to metabolic abnormalities associated with insulin resistance and subtype-specific clinical features associated with underlying molecular etiology.: This article aims to help physicians address challenges in diagnosing and managing lipodystrophy. We systematically reviewed the literature on PubMed and Google Scholar databases to summarize the current knowledge in lipodystrophy management.: Adipose tissue is a highly active endocrine organ that regulates metabolic homeostasis in the human body through a comprehensive communication network with other organ systems such as the central nervous system, liver, digestive system, and the immune system. The adipose tissue is capable of producing and secreting numerous factors with important endocrine functions such as leptin that regulates energy homeostasis. Recent developments in the field have helped to solve some of the mysteries behind lipodystrophy that allowed us to get a better understanding of adipocyte function and differentiation. From a clinical standpoint, physicians who suspect lipodystrophy should distinguish the disease from several others that may present with similar clinical features. It is also important for physicians to carefully interpret clinical features, laboratory, and imaging results before moving to more sophisticated tests and making decisions about therapy.
脂肪营养不良是一组罕见疾病，其特征为不同程度的脂肪丧失，导致代谢异常相关的胰岛素抵抗和与潜在分子病因学相关的亚型特异性临床特征引起严重发病率。本文旨在帮助医生应对脂肪营养不良的诊断和管理挑战。我们系统地回顾了 PubMed 和 Google Scholar 数据库中的文献，以总结脂肪营养不良管理的现有知识。脂肪组织是一个高度活跃的内分泌器官，通过与中枢神经系统、肝脏、消化系统和免疫系统等其他器官系统的全面通讯网络来调节人体的代谢稳态。脂肪组织能够产生和分泌许多具有重要内分泌功能的因子，如调节能量稳态的瘦素。该领域的最新进展有助于解决脂肪营养不良背后的一些谜团，使我们能够更好地理解脂肪细胞的功能和分化。从临床角度来看，怀疑脂肪营养不良的医生应将该疾病与可能具有相似临床特征的其他几种疾病区分开来。医生在进行更复杂的检查和做出治疗决策之前，仔细解释临床特征、实验室和影像学结果也很重要。
