Department of Endocrinology, St Bartholomew's Hospital, London EC1A 7BE, UK.
Trends Endocrinol Metab. 1996 Aug;7(6):213-6. doi: 10.1016/1043-2760(96)00089-6.
Cushing's disease is rare in childhood. There is an equal sex incidence, and it accounts for approximately 75% of pediatric causes of Cushing's syndrome. Predominant features are weight gain, growth failure, virilization, and headache. Following confirmation of the presence of inappropriate hypercortisolemia, the accurate differential diagnosis to establish the pituitary as the source of excessive ACTH secretion involves demonstration of > 50% suppression of circulating cortisol during high-dose dexamathasone administration and exaggeration of the cortisol response to corticotropin-releasing hormone (CRH). Imaging of the pituitary reveals a microadenoma in only a minority of cases, but inferior petrosal sinus sampling for ACTH can be of value in confirming the pituitary location of the tumor and possibly its lateralization. Primary therapy is transsphenoidal surgery, which can be supported by direct pituitary irradiation if hypercortisolemia persists. In experienced hands, the therapeutic outcome is good.
库欣病在儿童中较为罕见。其男女发病率相等，约占儿童库欣综合征病因的 75%。主要特征为体重增加、生长障碍、性早熟和头痛。在确认存在不适当的皮质醇过多症后，为确定 ACTH 分泌过多源于垂体，需要进行精确的鉴别诊断，包括在给予大剂量地塞米松时循环皮质醇抑制>50%，以及促肾上腺皮质激素释放激素（CRH）引起的皮质醇反应增强。垂体成像仅能在少数情况下发现微腺瘤，但通过对岩下窦取血样检测 ACTH 可能有助于确定肿瘤的垂体位置，并可能确定其侧别。主要治疗方法是经蝶窦手术，如果皮质醇过多持续存在，可辅以直接垂体照射。在有经验的医生手中，治疗效果良好。
