Division of Endocrinology, Diabetes & Metabolism, Beth Israel Deaconess Medical Center, Boston, MA 02215, USA.
Nat Rev Endocrinol. 2011 Mar;7(3):137-50. doi: 10.1038/nrendo.2010.199. Epub 2010 Nov 16.
Lipodystrophy is a medical condition characterized by complete or partial loss of adipose tissue. Not infrequently, lipodystrophy occurs in combination with pathological accumulation of adipose tissue at distinct anatomical sites. Patients with lipodystrophy exhibit numerous metabolic complications, which indicate the importance of adipose tissue as an active endocrine organ. Not only the total amount but also the appropriate distribution of adipose tissue depots contribute to the metabolic state. Genetic and molecular research has improved our understanding of the mechanisms underlying lipodystrophy. Circulating levels of hormones secreted by the adipose tissue, such as leptin and adiponectin, are greatly reduced in distinct subpopulations of patients with lipodystrophy. This finding rationalizes the use of these adipokines or of agents that increase their circulating levels, such as peroxisome proliferator-activated receptor γ (PPARγ) agonists, for therapeutic purposes. Other novel therapeutic approaches, including the use of growth hormone and growth-hormone-releasing factors, are also being studied as potential additions to the therapeutic armamentarium. New insights gained from research and clinical trials could potentially revolutionize the management of this difficult-to-treat condition.
脂肪营养不良是一种以脂肪组织完全或部分丧失为特征的医学病症。脂肪营养不良常与特定解剖部位病理性脂肪堆积合并发生。脂肪营养不良患者表现出多种代谢并发症，这表明脂肪组织作为一个活跃的内分泌器官的重要性。不仅脂肪组织总量，而且其适当的分布对代谢状态也有贡献。遗传和分子研究提高了我们对脂肪营养不良发病机制的理解。脂肪组织分泌的激素，如瘦素和脂联素，在脂肪营养不良的不同亚群患者中循环水平大大降低。这一发现合理地解释了使用这些脂肪因子或增加其循环水平的药物，如过氧化物酶体增殖物激活受体γ（PPARγ）激动剂，用于治疗目的。其他新的治疗方法，包括生长激素和生长激素释放因子的使用，也正在作为治疗手段的潜在补充进行研究。从研究和临床试验中获得的新见解可能会彻底改变这种难以治疗的疾病的治疗方法。
