Hübler A, Abendroth K, Keiner T, Stöcker W, Kauf E, Hein G, Stein G
Department of Internal Medicine IV, Friedrich-Schiller-University of Jena, Germany.
Exp Clin Endocrinol Diabetes. 1998;106(1):79-84. doi: 10.1055/s-0029-1211955.
We report on a 33-year-old male patient with generalized acquired lipodystrophy, insulin resistant diabetes mellitus and acanthosis nigricans (Lawrence Syndrome). First probable symptoms of lipodystrophy (weight loss, shrinkage of subcutaneous fatty tissue, and loss of muscular strength) became evident three years ago, with the onset of diabetes mellitus occurring about six months later. The patient suffered from the following clinical symptoms: IDDM with increasing insulin-requirement, extreme reduction of fatty tissue, fatty liver hepatitis with elevated liver enzymes, glomerulopathy, muscular and neuropathic pains, as well as hypertriglyceridaemia. A basal C-peptide concentration is rather high. Definitely, the endogenous insulin secretion is increased. In other words, insulin resistance is documented. In an effort to identify the pathogenetic mechanisms of lipoatrophic diabetes mellitus in this patient and to develop a therapeutic strategy, antibodies against different tissues and endocrinologic regulation were investigated. It was possible to demonstrate the presence of serum autoantibodies against lipocytes of the subcutis and other tissues, against hepatic stellate cells, together with autoantibodies against different endocrine organs. By studying the basis of diabetic abnormalities relating to the growth hormone (GH), the insulin-like growth factor (IGF) dynamics in this patient, i.e. reductions of GH, IGF-I, IGF-II, IGF-Binding protein (IGF-BP) 2 and IGF-BP 3, were detected. An immunosuppressive treatment strategy was not beneficial.
我们报告了一名33岁的男性患者,患有全身性获得性脂肪营养不良、胰岛素抵抗型糖尿病和黑棘皮病(劳伦斯综合征)。脂肪营养不良的最初可能症状(体重减轻、皮下脂肪组织萎缩和肌肉力量丧失)在三年前变得明显,糖尿病发病约在六个月后。该患者出现了以下临床症状:胰岛素依赖型糖尿病且胰岛素需求量增加、脂肪组织极度减少、脂肪肝伴肝酶升高、肾小球病变、肌肉和神经疼痛以及高甘油三酯血症。基础C肽浓度相当高。显然,内源性胰岛素分泌增加。换句话说,存在胰岛素抵抗。为了确定该患者脂肪萎缩性糖尿病的发病机制并制定治疗策略,对针对不同组织和内分泌调节的抗体进行了研究。已证实存在针对皮下脂肪细胞和其他组织、肝星状细胞的血清自身抗体,以及针对不同内分泌器官的自身抗体。通过研究与生长激素(GH)相关的糖尿病异常基础,检测到该患者胰岛素样生长因子(IGF)动态变化,即GH、IGF-I、IGF-II、IGF结合蛋白(IGF-BP)2和IGF-BP 3降低。免疫抑制治疗策略并无益处。