Pralong F P, Gomez F, Guillou L, Mosimann F, Franscella S, Gaillard R C
Department of Medicine, Institute of Pathology, University Hospital and Lausanne Medical School, Switzerland.
J Clin Endocrinol Metab. 1999 Oct;84(10):3817-22. doi: 10.1210/jcem.84.10.6068.
Stimulation ofcortisol secretion by food intake has been implicated in the pathogenesis of some cases of ACTH-independent Cushing's syndrome, via an aberrant response of the adrenal glands to gastric inhibitory polypeptide (GIP). We report here a novel case of food-dependent Cushing's syndrome in a patient with bilateral macronodular adrenal hyperplasia. In this patient we were able to confirm a paradoxical stimulation of cortisol secretion by GIP in vivo as well as in vitro on dispersed tumor adrenal cells obtained at surgery. In addition to GIP, in vitro stimulation of these cultured tumor adrenal cells with leptin, the secreted product of the adipocyte, induced cortisol secretion. By comparison, no such stimulation was observed in vitro in adrenal cells obtained from another patient with bilateral macronodular adrenal hyperplasia and Cushing's syndrome that did not depend on food intake, in tumor cells obtained from a solitary cortisol-secreting adrenal adenoma, and in normal human adrenocortical cells. These results demonstrate that as in previously described cases of food-dependent Cushing's syndrome, GIP stimulated cortisol secretion from the adrenals of the patient reported here. Therefore, they indicate that such a paradoxical response probably represents the hallmark of this rare condition. In addition, they suggest that leptin, which normally inhibits stimulated cortisol secretion in humans, participated in cortisol hypersecretion in this case. Further studies in other cases of food-dependent Cushing's syndrome, however, will be necessary to better ascertain the pathophysiological significance of this finding.
食物摄入对皮质醇分泌的刺激作用已被认为与某些促肾上腺皮质激素非依赖性库欣综合征病例的发病机制有关,这是通过肾上腺对胃抑制性多肽(GIP)的异常反应实现的。我们在此报告一例双侧大结节性肾上腺增生患者的食物依赖性库欣综合征新病例。在该患者中,我们能够在体内以及体外对手术中获取的分散的肿瘤肾上腺细胞证实GIP对皮质醇分泌具有反常刺激作用。除了GIP之外,用脂肪细胞分泌产物瘦素在体外刺激这些培养的肿瘤肾上腺细胞也可诱导皮质醇分泌。相比之下,在从另一名不依赖食物摄入的双侧大结节性肾上腺增生和库欣综合征患者获取的肾上腺细胞中、在从一个分泌皮质醇的孤立肾上腺腺瘤获取的肿瘤细胞中以及在正常人肾上腺皮质细胞中,均未观察到这种体外刺激作用。这些结果表明,正如在先前描述的食物依赖性库欣综合征病例中一样,GIP刺激了此处报告患者肾上腺的皮质醇分泌。因此,这些结果表明这种反常反应可能代表了这种罕见病症的标志。此外,这些结果表明,正常情况下抑制人体刺激后皮质醇分泌的瘦素在该病例中参与了皮质醇分泌过多。然而,对其他食物依赖性库欣综合征病例进行进一步研究对于更好地确定这一发现的病理生理意义将是必要的。