[库欣综合症-罕见病例]。

Y Miyachi
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摘要

库欣病与非垂体acth分泌肿瘤的分离很少是直接的。一名16岁的女孩和一名56岁的男子均表现出库欣综合征的典型临床特征。在两例患者中,8mg地塞米松抑制了尿17OHCS,血浆皮质醇和ACTH对静脉给药CRH有反应。颅骨x光片、计算机断层扫描和蝶鞍区磁共振成像(MRI)未见明显异常。第一个病人的脑垂体增强MRI显示一个小的低密度区域,代表微腺瘤。在第二例患者中,增强MRI和下窦取样未能显示垂体瘤。患者死于急性心肌梗死,尸检显示右上肺叶支气管类癌。有必要鉴别库欣综合征患者的异位acth分泌,这些患者表现出库欣病的典型生化特征,但常规放射学评估未能证明垂体瘤的存在。
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[Cushing's syndrome--unusual cases].

The separation of Cushing's disease from non-pituitary ACTH-secreting tumors is rarely straightforward. Both a 16-year-old girl and a 56-year-old man exhibited typical clinical features of Cushing's syndrome. In both patients urinary 17OHCS was suppressed by 8mg dexamethasone and plasma cortisol and ACTH responded to intravenous administration of CRH. X-rays of the skull, a computed tomographic scan and a magnetic resonance imaging (MRI) of the sella turcica area showed no significant abnormality. An enhanced MRI of the pituitary gland in the first patient demonstrated a small low-density area which represented microadenoma. In the second patient, an enhanced MRI and inferior sinus sampling failed to demonstrate a pituitary tumor. The patient died of acute myocardial infarction, and an autopsy revealed a bronchial carcinoid in the right upper pulmonary lobe. It is necessary to differentiate an ectopic ACTH-secretion in patients with Cushing's syndrome who show typical biochemical features of Cushing's disease but fail to demonstrate the presence of a pituitary tumor by routine radiological evaluation.

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[Parathyroid hormone]. [Treatment of hypothalamic-pituitary tumors--experiences at Hiroshima University School of Medicine]. [Future aspects on endocrinology]. [A view of basic endocrinology]. [Comment by a surgeon on Japan Endocrine Society, its past and future].
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