Tóth Miklós, Vajda Zsolt, Görömbey Zoltán, Molnár Ferenc, Major László, Toke Judit, Szabolcs István, Szücs Nikolette, Kovács László, Kiss Róbert, Czirják Sándor, Füto László, Gláz Edit, Góth Miklós, Rácz Károly
Semmelweis Egyetem, Altalános Orvostudományi Kar II. Belgyógyászati Klinika, Budapest.
Orv Hetil. 2007 May 6;148(18):843-7. doi: 10.1556/OH.2007.28048.
The differentiation of adrenocorticotropic hormone producing pituitary adenoma (Cushing's disease) from the ectopic ACTH syndrome is always a complex and difficult task, and in rare cases it is not possible to differentiate between the two disorders, even with the use of dynamic endocrine tests and the most advanced imaging techniques. Inferior petrosal sinus sampling (IPSS) with subsequent ACTH measurements became the gold-standard method of the differential diagnostic process. 34 patients with ACTH dependent Cushing's syndrome in whom the source of ACTH secretion couldn't be identified unambiguously with imaging techniques and/or dynamic endocrine tests underwent altogether 41 IPSS between 1999 and 2005. The sensitivity of the method was calculated on the basis of 31 samplings of 25 patients who had definite endocrinological diagnosis confirmed by the recovery from Cushing's syndrome after surgical intervention and/or by histological examinations (22 patients with ACTH-producing pituitary adenoma and 3 patients with ectopic ACTH syndrome). As a result of IPSS, pituitary-dependent Cushing's disease was diagnosed with a baseline central to peripheral ACTH ratio of >2.0 or with a ratio of >3.0 after corticotropin releasing hormone (CRH) administration. IPSS correctly identified ACTH-producing pituitary adenoma in 20 of 28 sampling procedures, with a sensitivity of 71.4%. Three patients had true negative and 8 had false negative results. There was no false positive result. Four of the 8 patients with false negative first sampling had a repeat sampling procedure leading to true positive result in each patient. In patients with Cushing's disease having true positive interventions, the basal and 5 minutes post-CRH ACTH concentrations were diagnostic in 14 and 19 cases, respectively. The sensitivity of IPSS within this series, reported for the first time from Hungary, was lower than it was found in much larger series published in international literature. In addition to technical difficulties, the lower sensitivity can be accounted also for the highly selected nature of the patient group.
鉴别促肾上腺皮质激素分泌型垂体腺瘤(库欣病)与异位促肾上腺皮质激素综合征始终是一项复杂且困难的任务,在极少数情况下,即便使用动态内分泌检测和最先进的影像技术,也无法区分这两种疾病。经岩下窦采血(IPSS)并随后测定促肾上腺皮质激素,成为鉴别诊断过程的金标准方法。1999年至2005年期间,34例促肾上腺皮质激素依赖性库欣综合征患者因影像技术和/或动态内分泌检测无法明确促肾上腺皮质激素分泌来源,共接受了41次经岩下窦采血。该方法的敏感性基于25例患者的31次采血计算得出,这些患者经手术干预后库欣综合征缓解和/或经组织学检查确诊为明确的内分泌诊断(22例促肾上腺皮质激素分泌型垂体腺瘤患者和3例异位促肾上腺皮质激素综合征患者)。经岩下窦采血结果显示,垂体依赖性库欣病的诊断标准为基础状态下中央与外周促肾上腺皮质激素比值>2.0,或注射促肾上腺皮质激素释放激素(CRH)后比值>3.0。在28次采血过程中,经岩下窦采血正确识别出20例促肾上腺皮质激素分泌型垂体腺瘤,敏感性为71.4%。3例结果为真阴性,8例为假阴性,无假阳性结果。8例首次采血假阴性的患者中有4例进行了重复采血,结果均为真阳性。在库欣病患者中,干预结果为真阳性的患者中,基础状态下和注射CRH后5分钟的促肾上腺皮质激素浓度分别在14例和19例中具有诊断价值。本研究首次从匈牙利报道该系列中经岩下窦采血的敏感性低于国际文献中报道的更大系列研究。除技术困难外,敏感性较低也可归因于患者群体的高度选择性。