论文部分内容阅读
病例患者系白种人,女,于12.8岁时因面部丰满发红、肌肉软弱、昏睡6周首次就医。体检呈柯兴样面容,有皮肤条纹,女子男性化和高血压。实验室检查显示低血钾、硷中毒、高血糖、甲状腺功能低下,血睾酮增高。血浆氢化可的松持续>100μg/dl(2760nmol/L),ACTH250～500pg/ml(55～110pmol/L)。24 h尿17-羟类固醇>100mg(276μmol)、17-酮类固醇70mg(242.6μmol)、游离氢化可的松>11729μg(32.4μmol)。小剂量与大剂量地塞米松抑制试验后血浆氢化可的松无抑制。临床诊断为异位源性ACTH所致柯兴综合征。超声检查显示双侧肾上腺增大,CT扫描提示垂体正常,X线平片显示普遍骨质疏松,腹部超声显示可能胰头增大,超声及CT扫
The case patients were Caucasian and female. At the age of 12.8, the patient was treated for the first time for 6 weeks because of full facial redness, muscle weakness, and lethargy. The examination showed a facial appearance, skin stripes, masculinity, and high blood pressure. Laboratory tests revealed hypokalemia, sputum poisoning, hyperglycemia, hypothyroidism, and elevated testosterone levels. Plasma hydrocortisone persisted >100 μg/dl (2760 nmol/L), ACTH 250–500 pg/ml (55–110 pmol/L). 24 h urine 17-hydroxysteroids >100 mg (276 μmol), 17-ketosteroid 70 mg (242.6 μmol), free hydrocortisone >11 629 μg (32.4 μmol). Plasma hydrocortisone was not inhibited after low-dose and high-dose dexamethasone inhibition tests. The clinical diagnosis is Cushing syndrome caused by ectopic ACTH. Ultrasound examination showed bilateral adrenal enlargement, CT scan showed normal pituitary, X-ray plain radiographs showed common osteoporosis, abdominal ultrasound showed possible pancreatic head enlargement, ultrasound and CT scan