Miyamura Nobuhiro, Tsutsumi Atsuyuki, Senokuchi Hiroko, Nakamaru Kazuhiko, Kawashima Junji, Sakai Kohji, Taguchi Tetsuya, Tokunaga Hiroshi, Nishida Kenro, Uehara Masaya, Sakakida Michiharu, Araki Eiichi
Department of Metabolic Medicine, Kumamoto University School of Medicine, Kumamoto 860-8556, Japan.
Endocr J. 2003 Jun;50(3):333-40. doi: 10.1507/endocrj.50.333.
ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing's syndrome. Recently, aberrant expression of adrenal receptors for various hormones and/or cytokines has been identified in several cases with AIMAH, which may act as a pathogenetic factor for the disorder. We report here an AIMAH patient with a Rathke's cleft cyst. Endocrinological examinations revealed that the pituitary cyst had no hormonal secretion. Administrations of either AVP or isoproterenol provoked cortisol production in the patient, whereas DDAVP, mosapride or endogenous LH induced by GnRH did not. Reverse transcriptional-PCR analysis of total RNA obtained from the patient's adrenal tissue revealed the expression of mRNA of receptors for V1a, V1b, V2, and LH/hCG. Three of these receptors except for V1a receptor were not expressed in normal adrenal tissue. Hyperosmolar saline infusion promoted the patient's cortisol secretion through the increase in endogenous AVP (peak plasma AVP level reached 90.4 pg/ml during the test). These results suggest that endogenous AVP and catecholamines are involved in the pathophysiology of the patient. Further study will be necessary to clarify the molecular mechanisms that regulate tissue-specific expression of these receptors and their role in the overgrowth of adrenal in AIMAH.
促肾上腺皮质激素非依赖性大结节性肾上腺增生（AIMAH）是库欣综合征的一种罕见病因。最近，在几例AIMAH患者中发现了各种激素和/或细胞因子的肾上腺受体异常表达，这可能是该疾病的致病因素。我们在此报告一例患有拉克氏裂囊肿的AIMAH患者。内分泌检查显示垂体囊肿无激素分泌。给予血管加压素（AVP）或异丙肾上腺素可刺激该患者的皮质醇分泌，而给予去氨加压素、莫沙必利或促性腺激素释放激素（GnRH）诱导的内源性促黄体生成素（LH）则无此作用。对患者肾上腺组织提取的总RNA进行逆转录聚合酶链反应（RT-PCR）分析，结果显示V1a、V1b、V2和LH/hCG受体的mRNA表达。除V1a受体外，其中三种受体在正常肾上腺组织中不表达。高渗盐水输注通过增加内源性AVP促进患者皮质醇分泌（试验期间血浆AVP峰值水平达到90.4 pg/ml）。这些结果表明内源性AVP和儿茶酚胺参与了该患者的病理生理过程。有必要进一步研究以阐明调节这些受体组织特异性表达的分子机制及其在AIMAH肾上腺过度生长中的作用。
