Goñi Iriarte María José
Servicio de Endocrinología, Hospital de Navarra, Pamplona, Navarra, Spain.
Endocrinol Nutr. 2009 May;56(5):251-61. doi: 10.1016/S1575-0922(09)71408-1. Epub 2009 Jul 1.
The present article reviews: Corticotrophin (ACTH) independent bilateral macronodular adrenal hyperplasia, which is characterized by aberrant adrenal receptors due to either ectopic expression or to overexpression (eutopic expression). Micronodular adrenal hyperplasia, which provokes small pigmented nodules in the adrenal gland with atrophy of the internodal tissue. These nodules may not be visible on imaging tests. The term subclinical Cushing's syndrome, coined in 1981, should be used in patients with clinically non-functioning adrenal adenomas but who show autonomous cortisol production that is insufficient to generate overt Cushing's syndrome. This entity must be distinguished from preclinical Cushing's syndrome, given that the subclinical form does not necessarily herald the development of symptoms of hypercortisolism. Cushing's syndrome is uncommon in children and adolescents. Regarding the general incidence of the disease, only 10% of cases are diagnosed in this age group. The most common cause of endogenous Cushing's disease in children older than 7 years is ACTH-dependent Cushing's disease (85%). The association of Cushing's syndrome and pregnancy is highly uncommon, since hypercortisolism usually causes amenorrhea, oligomenorrhea and infertility due to inhibition of gonadotropin secretion. One hundred thirty-six pregnancies have been described in 122 women, with a gestational age at diagnosis of 18.4+/-1 weeks.
促肾上腺皮质激素（ACTH）非依赖性双侧大结节性肾上腺增生，其特征是由于异位表达或过表达（原位表达）导致肾上腺受体异常。微结节性肾上腺增生，可在肾上腺中引起小的色素沉着结节，并伴有节间组织萎缩。这些结节在影像学检查中可能不可见。1981年提出的亚临床库欣综合征一词，应用于临床无功能肾上腺腺瘤但显示自主性皮质醇分泌但不足以产生明显库欣综合征的患者。鉴于亚临床形式不一定预示着高皮质醇血症症状的发展，必须将该实体与临床前库欣综合征区分开来。库欣综合征在儿童和青少年中并不常见。关于该疾病的总体发病率，该年龄组仅10%的病例得到诊断。7岁以上儿童内源性库欣病最常见的原因是ACTH依赖性库欣病（85%）。库欣综合征与妊娠的关联非常罕见，因为高皮质醇血症通常会因抑制促性腺激素分泌而导致闭经、月经过少和不孕。已描述122名女性中有136次妊娠，诊断时的孕周为18.4±1周。
