Single-center experience with pediatric Cushing's disease
Single-center experience with pediatric Cushing's disease
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DOI：
10.3171/ped.2005.103.5.0413
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发表时间：
2005-11-01
影响因子：
4.1
通讯作者：
Laws, ER
中科院分区：
文献类型：
--
作者：
Kanter, AS;Diallo, AO;Laws, ER
Object.尽管外科和放射治疗技术不断进步，小儿库欣病仍然是一个诊断和治疗的挑战。作者报告了33例库欣病患儿的单中心回顾性分析结果，详细介绍了临床表现、诊断评估、治疗过程、并发症和结局。有17名女性和16名男性患者，平均年龄为13岁（范围5-19岁），根据临床和生化标准诊断为库欣病。典型症状包括体重增加（91%）、青春期前生长延迟（83%）、圆脸（61%）、多毛（58%）、头痛（45%）、腹纹（42%）、痤疮（33%）、闭经（24%）和高血压（24%）。在67%的病例中，术前磁共振图像显示垂体病变，在82%的病例中，影像学研究有效地预测了侧化。在7例患者（21%）中进行了岩下窦取样，在所有这些病例中，定侧是100%可靠的。55%的患者接受选择性腺瘤切除术，45%的患者接受垂体次全切除术，并发症包括1例尿崩症，1例持续性低皮质醇血症，需要长期糖皮质激素替代治疗，1例轻微血管损伤，不需要术后管理调整或引起后遗症。无手术相关死亡，无术后脑脊液漏或脑膜炎病例。在平均44个月的随访期内，91%的患者最终达到临床缓解：76%在单独经蝶手术后，另外15%在手术失败后进行辅助放射手术和/或肾上腺切除术后。3例患者（12%）出现疾病复发，并分别在18、81和92个月时接受了第二次外科手术;根据临床和生化标准，所有患者均实现了第二次缓解。3例患者（9%）仍有持续性疾病。小儿库欣氏病是一种罕见的疾病，往往需要多学科的诊断和多模式的治疗方法，成功的长期缓解。
Object. Despite ongoing advances in surgical and radiotherapeutic techniques, pediatric Cushing's disease remains a diagnostic and therapeutic challenge. The authors report on the results of a single-center retrospective review of 33 pediatric patients with Cushing's disease, providing details with respect to clinical presentation, diagnostic evaluation, therapeutic course, complications, and outcomes.Methods. There were 17 female and 16 male patients whose mean age was 13 years (range 5-19 years) in whom a diagnosis of Cushing's disease was based on clinical and biochemical criteria. Typical symptoms included weight gain (91%), prepubertal growth delay (83%), round facies (61%), hirsutism (58%), headache (45%), abdominal striae (42%), acne (33%), amenorrhea (24%), and hypertension (24%). In 67% of the cases, preoperative magnetic resonance images revealed a pituitary lesion and in 82% of the cases the imaging studies effectively predicted lateralization. Inferior petrosal sinus sampling was performed in seven patients (21%), and in all of these cases lateralization was 100% reliable. Fifty-five percent underwent selective adenomectomies and 45% underwent subtotal hypophysectomies.Complications included one case of diabetes insipidus, one of persistent hypocortisolemia necessitating prolonged glucocorticoid replacement therapy, and one minor vascular injury that did not necessitate postoperative management modification or cause sequelae. There were no surgery-related deaths and no cases of postoperative cerebrospinal fluid leakage or meningitis. During a mean follow-up period of 44 months, clinical remission was ultimately achieved in 91% of patients: 76% after transsphenoidal surgery alone and an additional 15% after adjuvant radiosurgery and/or adrenalectomy following surgical failure. Three patients (12%) experienced disease recurrence and underwent a second surgical procedure at 18, 81, and 92 months, respectively; based on clinical and biochemical criteria a second remission was achieved in all. Three patients (9%) remain with persistent disease.Conclusions. Pediatric Cushing's disease is a rare condition, often requiring a multidisciplinary diagnostic and a multimodal therapeutic approach for successful long-term remission.