From the Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, and the Diabetes, Endocrinology, and Obesity Branch, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, Maryland.
Obstet Gynecol. 2012 Feb;119(2 Pt 2):452-455. doi: 10.1097/AOG.0b013e31822cecf7.
Congenital generalized lipodystrophy is a rare disorder characterized by scant adipose tissue, profound leptin deficiency, and severe insulin resistance, resulting in multiple metabolic derangements, including hyperandrogenism, anovulation, and impaired fecundity.
A young woman with congenital generalized lipodystrophy receiving leptin therapy experienced menarche, conceived spontaneously, and delivered a liveborn male neonate.
Adipose tissue is important to normal female reproductive function. Leptin in particular appears to play a key role in adipose-mediated regulation of fertility.
先天性全身性脂肪营养不良是一种罕见的疾病,其特征是脂肪组织极少、瘦素严重缺乏和严重的胰岛素抵抗,导致多种代谢紊乱,包括高雄激素血症、无排卵和生育能力受损。
一名接受瘦素治疗的先天性全身性脂肪营养不良的年轻女性出现月经初潮,自然受孕,并生下一名活产男婴。
脂肪组织对女性正常生殖功能很重要。瘦素尤其似乎在脂肪介导的生育调节中起着关键作用。