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[食物依赖型库欣综合征:一种新的器质性皮质醇增多症实体]

[Food-dependent Cushing syndrome: a new entity of organic hypercorticism].

作者信息

Matejka G, Vergès B, Vaillant G, Petit J M, Pacaud A, Brun J M

机构信息

Service d'endocrinologie, diabétologie, maladies métaboliques, hôpital du Bocage, Dijon, France.

出版信息

Rev Med Interne. 1996;17(6):461-6. doi: 10.1016/0248-8663(96)86438-1.

Abstract

Diagnosis of Cushing's syndrome is quite difficult in endocrinology. Spontaneous Cushing's syndrome is usually divided into two subgroups, one which is dependent on corticotropin (ACTH) and another one which is not. In the first class are Cushing's disease, the ectopic corticotropin syndrome and the rare ectopic corticotropin-releasing hormone (CRH) syndrome; these ACTH-dependent Cushing's syndrome have usually diffusely enlarged adrenal glands. In the second class are cortisol producing unilateral adrenocortical adenomas or carcinomas, and the recent Cushing's syndrome with food dependent periodic hormonogenesis. This food dependent Cushing's syndrome is an ACTH-independent Cushing's syndrome with multinodular enlargement of both adrenal glands. Pathogenesis is an aberrant adrenal sensitivity to physiologic secretion of gastric inhibitory peptide (GIP). Ectopic expression of GIP receptors on adrenal cells involve pathologic food induced cortisol secretion. Food dependent Cushing's syndrome is a new cause of Cushing's syndrome. Food induced cortisol secretion may have to be explored in the ACTH-independent Cushing's syndrome.

摘要

在内分泌学中,库欣综合征的诊断相当困难。自发性库欣综合征通常分为两个亚组,一组依赖促肾上腺皮质激素(ACTH),另一组则不依赖。第一类包括库欣病、异位促肾上腺皮质激素综合征和罕见的异位促肾上腺皮质激素释放激素(CRH)综合征;这些依赖ACTH的库欣综合征通常肾上腺弥漫性增大。第二类包括分泌皮质醇的单侧肾上腺皮质腺瘤或癌,以及最近发现的食物依赖周期性激素生成的库欣综合征。这种食物依赖的库欣综合征是一种不依赖ACTH的库欣综合征,双侧肾上腺呈多结节性增大。其发病机制是肾上腺对胃抑制肽(GIP)的生理分泌异常敏感。肾上腺细胞上GIP受体的异位表达涉及病理性食物诱导的皮质醇分泌。食物依赖的库欣综合征是库欣综合征的一个新病因。在不依赖ACTH的库欣综合征中,可能需要探索食物诱导的皮质醇分泌情况。

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