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库欣病:通过促肾上腺皮质激素释放激素刺激下的双侧岩下窦同步采样成功实现术前促肾上腺皮质激素分泌型垂体微腺瘤的定位。

Cushing disease: successful preoperative lateralization of an ACTH-producing pituitary microadenoma by simultaneous bilateral inferior petrosal venous sinus sampling with corticotropin-releasing hormone stimulation.

作者信息

Hauffa B P, Stolecke H, Schulte H M

出版信息

Eur J Pediatr. 1986 Dec;145(6):559-62. doi: 10.1007/BF02429066.

Abstract

A 5 10/12-year-old girl with clinical and laboratory signs of endogenous hypercortisolism had evidence of ACTH hypersecretion in a standardized dexamethasone suppression test but had surprisingly low plasma ACTH concentrations before and after ovine corticotropin releasing hormone (oCRH) stimulation. To establish the diagnosis of pituitary disease and to localize the suspected microadenoma, we performed bilateral simultaneous inferior sinus petrosus blood sampling under CRH stimulation (oCRH 1 microgram/kg as an i.v. bolus). No major side effects were noted. Inferior petrosal blood ACTH concentrations did not differ from peripheral blood under basal sampling conditions but were higher in the effluent of the left half of the pituitary 5 min after oCRH stimulation. During transsphenoidal exploration of the sella an ACTH-producing microadenoma was removed from the left portion of the anterior pituitary gland. Signs of hypercortisolism remitted after surgery. Simultaneous bilateral sinus petrosus blood sampling under oCRH stimulation is a useful lateralization procedure even with small ACTH-producing adenomas. This technique may help to avoid unsuccessful surgical exploration in children with Cushing disease.

摘要

一名12岁零10个月的女孩,有内源性皮质醇增多症的临床和实验室体征,在标准地塞米松抑制试验中有促肾上腺皮质激素(ACTH)分泌过多的证据,但在羊促肾上腺皮质激素释放激素(oCRH)刺激前后,其血浆ACTH浓度出奇地低。为了确诊垂体疾病并定位疑似微腺瘤,我们在CRH刺激下(静脉推注1微克/千克oCRH)进行了双侧同时岩下窦采血。未观察到重大副作用。在基础采样条件下,岩下窦血ACTH浓度与外周血无差异,但在oCRH刺激后5分钟,垂体左半侧流出液中的ACTH浓度更高。在经蝶窦探查蝶鞍时,从前叶垂体左侧切除了一个产生ACTH的微腺瘤。术后皮质醇增多症的体征消失。即使是对于小型产生ACTH的腺瘤,oCRH刺激下的双侧同时岩下窦采血也是一种有用的定位方法。这项技术可能有助于避免对库欣病患儿进行不成功的手术探查。

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