Department of Endocrinology, Seth G S Medical College, Mumbai, India.
Endocr Pract. 2011 May-Jun;17(3):369-76. doi: 10.4158/EP10143.OR.
To analyze the clinical presentation, diagnostic evaluation, treatment modalities, and follow-up of pediatric patients with Cushing disease.
In this retrospective analysis, we reviewed records of children (younger than 20 years) with Cushing disease who had undergone transsphenoidal adenomectomy in a tertiary health care center in India during the period of 1988 to 2008. Endogenous hypercortisolism was identified by a serum cortisol value ≥1.8 μg/dL during a low-dose dexamethasone suppression test (LDDST) with or without elevated midnight serum cortisol (≥3.2 μg/dL). Corticotropin dependence was defined by a basal plasma corticotropin concentration ≥5 pg/mL. Patients with normal pituitary imaging underwent bilateral inferior petrosal sinus sampling (BIPSS). Those with persistent or recurrent disease after surgery were treated with second-line interventions on a case-by-case basis.
Twenty-nine boys and 19 girls were included. Mean age was 14.85 (±2.5) years. Weight gain (98%), round facies (98%), and growth arrest (83%) were the most common manifestations. LDDST and midnight cortisol had 100% sensitivity for detecting endogenous hypercortisolism, while midnight corticotropin measurement had 100% sensitivity for defining corticotropin dependence. Magnetic resonance imaging and unstimulated BIPSS had 71% and 89% sensitivity, respectively, for diagnosing Cushing disease. Twenty-seven patients (56%) achieved remission after the first transsphenoidal operation with higher remission rates in those with microadenoma (75%). Basal serum cortisol <5 mg/dL on the fifth postoperative day predicted cure. Eight patients received postoperative radiotherapy, with 4 achieving remission.
Clinical presentation and diagnostic yield with various tests were similar to those previously reported in the literature. Remission rates were poor after first transsphenoidal operation in patients with macroadenoma and outcome was dismal with a second transsphenoidal operation. Serum cortisol concentration <5 mg/dL on the fifth postoperative day predicted cure.
分析儿童库欣病患者的临床表现、诊断评估、治疗方式和随访情况。
本回顾性分析纳入了 1988 年至 2008 年期间在印度一家三级保健中心接受经蝶窦腺瘤切除术的儿童（年龄小于 20 岁）患者的病历资料。采用小剂量地塞米松抑制试验（LDDST），若同时存在午夜血清皮质醇升高（≥3.2μg/dL）或仅存在血清皮质醇升高（≥1.8μg/dL），则可确定内源性皮质醇过多症。根据基础血浆促肾上腺皮质激素浓度≥5pg/mL 确定促肾上腺皮质激素依赖性。垂体影像学正常的患者行双侧岩下窦采血（BIPSS）。术后疾病持续或复发的患者，根据具体情况进行二线干预治疗。
本研究共纳入 29 名男孩和 19 名女孩，平均年龄为 14.85（±2.5）岁。体重增加（98%）、圆脸（98%）和生长停滞（83%）是最常见的表现。LDDST 和午夜皮质醇检测对诊断内源性皮质醇过多症的敏感性均为 100%，而午夜促肾上腺皮质激素测量对确定促肾上腺皮质激素依赖性的敏感性为 100%。磁共振成像和未刺激的 BIPSS 对库欣病的诊断敏感性分别为 71%和 89%。27 名患者（56%）在首次经蝶窦手术后获得缓解，微腺瘤患者的缓解率更高（75%）。术后第 5 天基础血清皮质醇<5mg/dL 预测可治愈。8 名患者接受术后放疗，其中 4 名缓解。
本研究的临床表现和各种检测的诊断结果与文献报道的相似。大腺瘤患者首次经蝶窦手术后缓解率较差，再次经蝶窦手术后预后不佳。术后第 5 天血清皮质醇浓度<5mg/dL 预测可治愈。
