Koppeschaar H P, Croughs R J, van't Verlaat J W, Hendriks M J, Arts C J, Thijssen J H, Schwarz F
Acta Endocrinol (Copenh). 1984 Dec;107(4):471-5. doi: 10.1530/acta.0.1070471.
Transfrontal hypophysectomy was performed in a patient with Cushing's disease and gross enlargement of the pituitary. Despite some reduction of cortisol production active Cushing's syndrome remained due to the presence of a tumour remnant. Medical treatment with the GABA-transaminase inhibitor sodium valproate induced hypocorticism necessitating corticosteroid substitution therapy. Nine months after sodium valproate withdrawal hypercorticism was documented. Re-institution of sodium valproate treatment induced hypocorticism again. As sodium valproate is known to induce a decrease of plasma ACTH in Nelson's syndrome, it is proposed that large tumours present at the time of diagnosis and those appearing after adrenalectomy may represent the spectrum of a single disorder. A prospective trial to study the effects of sodium valproate and other neurotransmitter modulating agents on the size and endocrine function of ACTH secreting macroadenomas is urgently needed.
对一名患有库欣病且垂体明显增大的患者实施了经额垂体切除术。尽管皮质醇分泌有所减少,但由于肿瘤残留,活跃的库欣综合征仍然存在。使用γ-氨基丁酸转氨酶抑制剂丙戊酸钠进行药物治疗导致肾上腺皮质功能减退,需要进行皮质类固醇替代治疗。停用丙戊酸钠九个月后,记录到皮质醇增多。重新使用丙戊酸钠治疗再次导致肾上腺皮质功能减退。由于已知丙戊酸钠会导致尼尔森综合征患者血浆促肾上腺皮质激素(ACTH)降低,因此有人提出,诊断时出现的大肿瘤以及肾上腺切除术后出现的肿瘤可能代表单一疾病的不同表现形式。迫切需要进行一项前瞻性试验,以研究丙戊酸钠和其他神经递质调节剂对分泌ACTH的大腺瘤大小和内分泌功能的影响。