A Subject with Adrenal Macronodular Hyperplasia Treated by Ketoconazole

以Ketoconazole治療腎上腺結節增生之病例報告

王憶嘉(Yi-Chia Wang) ； 李弘元(Hung-Yuan Li)

《中華民國內分泌暨糖尿病學會會刊》 21卷4期 (2008/07) Pp. 74-81

https://doi.org/10.7101/ED.200807.0074

摘要

非ACTH治療腎上腺結節增生是庫欣氏症的一種罕見表現，我們在此報告一位用ketoconazole治療一年且控制良好之病例，七十四歲男性因體重在一年內增加十公斤，血壓在近五個月來控制不良來門診求醫，由低劑量類固醇抑制試驗的結果，我們診斷他患有庫欣氏症，由於他的ACTH較低，我們安排了腹部電腦斷層檢查，發現腎上腺有巨結節增生，同時，核磁共振檢查並沒有看到腦下腺的腫瘤爲，因此我們高度懷疑他患有非ACTH依賴性的腎上腺結節增生，由於他拒絕手術處理，所以我們使用ketoconazole治療，服藥後他的體重血壓有明顯下降，且血中ACTH及腎上腺素的濃度也回到正常範圍，Ketoconazole是藉由抑制cytochrome P-450相關酵素而減少類固醇製造的藥物，因此可以用來治療庫欣氏症，相對於手術治療，藥物治療相關細節的報告較少，特別是關於有效劑量，藥物開始發揮作用所需時間，以及作用持續霎時間的資料都不多，因此，我們希望藉由此病例，報告並回顧關於ketoconazole的治療經過與反應。

並列摘要

ACTH-independent macronodulat adrenal hyperplasia (AIMAH) is a rare cause of Cushing's syndrome. Here we report a patient with AIMAH treated by ketoconazole for one year with satisfactory results. A 74 year-old man came to our clinic because his body weight increased 10 kg within one year, and blood pressure became out of control in recent five months, Cushing's syndrome was diagnosed by dexamethasone suppression test. Since his baseline ACTH was low, abdominal: computed tomography (CT) was arranged, which revealed macronodular adrenal hyperplasia. Magnetic resonance image (MRI) of his sella turseca did not reveal any pituitary mass. Thus AIMAH was highly suspected. Since he refused surgical intervention, ketoconazole was prescribed. Under medical treatment, he lost his body weight, and his blood pressure, serum ACTH, cortisol were normalized. Ketoconazole is one of the drugs used in Cushing's syndrome through the effect of inhibiting a variety of steroidogenic cytochrome P450-dependent enzymes to lower plasma cortisol levels. In contrast to the fruitful reports of surgical treatment on Cushing's syndrome, the experience on medical treatment is limited, especially the data of the effective dose, how fast it onsets, and how long it can last. Here we share our experience about the details of ketoconazole treatment.

並列關鍵字

ACTH-independent macronodular adrenal hyperplasia ； Cushing's syndrome ； Ketoconazole