一个不同寻常的卡尼表示复杂的扩散主要色素在一个肾上腺结节性肾上腺皮质疾病和nonpigmented肾上腺皮质腺瘤和焦主要色素结节性肾上腺皮质疾病。
文章的细节
-
引用
复制到剪贴板 -
东SC,黄DY,杨JW,陈WJ,李CT
一个不同寻常的卡尼表示复杂的扩散主要色素在一个肾上腺结节性肾上腺皮质疾病和nonpigmented肾上腺皮质腺瘤和焦主要色素结节性肾上腺皮质疾病。
Endocr j . 2012; 59 (9): 823 - 30。Epub 2012 6月20。
- PubMed ID
-
22785148 (在PubMed]
- 文摘
-
一个24岁的女性患者cushingoid貌承认2000年5月。内分泌研究显示ACTH-independent库兴氏综合征。一个为期两天的大剂量地塞米松抑制试验(HDDST)揭示矛盾的增加免费24小时尿皮质醇(生)。腹部电脑断层显示左肾上腺结节(直径3 x 2厘米)。一个肾上腺闪烁图(1)(3)(1)I-6beta-iodomethyl-19-norcholesterol显示吸收同位素的左肾上腺和non-visualization右肾上腺整个考试过程。retroperitoneoscopic左肾上腺切除术总在2000年7月进行。左肾上腺的切割面是黄褐色严重。显微镜下,左侧肾上腺结节包含一个nonpigmented肾上腺皮质腺瘤(NP)和另一个焦点主要色素结节性肾上腺皮质疾病(PPNAD FP)混合病变。CYP17展示积极的免疫组织化学研究NP和FP的左肾上腺。非常低的基线早上血浆皮质醇(0.97杯/ dL)和低能的ACTH (8.16 pg / mL)水平测定左肾上腺切除术后1.5个月。 Right adrenal gland recovered its function 6 months after left adrenalectomy. Plasma cortisol could be suppressed to 3.47 mug/dL by overnight low-dose dexamethasone suppression test 65 months after left adrenalectomy. Cushingoid features still did not appear 122 months after left adrenalectomy. In May 2011, this patient was readmitted due to cushingoid characteristics. Paradoxical rise of 24-h UFC to 2-day HDDST was demonstrated. Ultrasonography of thyroid showed bilateral thyroid cysts. Subtotal right adrenalectomy about 80% of right adrenal was performed. Diffuse PPNAD of the right adrenal was proved pathologically. Immunohischemical stain for CYP17 is positive in the right adrenal gland but weaker positive than that in the left adrenal gland. The genetic study of the peripheral blood, left adrenocortical nodule, and right PPNAD all showed p.R16X (c.46C>T) mutation of the PRKAR1A gene.