AIMAHACTH (Adrenocorticotropic Hormone)-Independent Macronodular Adrenocortical Hyperplasia (aka ACTH-Independent Macronodular Adrenal Hyperplasia)
AIMAHACTH (Adrenocorticotropic Hormone)-Independent Macronodular Adrenal Hyperplasia (aka ACTH-Independent Macronodular Adrenocortical Hyperplasia)
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Standard therapy for clinical AIMAH is bilateral adrenalectomy, which can remove the source of serum cortisol radically, with 5-year survival rates being 66% to 70%.
Ameliorating high serum cortisol, improving organ function with medicine, and selecting an appropriate time to perform the operation are keys to cure high-risk AIMAH. Once AIMAH develops into high-risk AIMAH, the disease would develop quickly, accompanied by hypokalemia, hyperglycemia, severe hypertension and exhaustion of multiple organs.
There are various medications, such as ketoconazole, mitotane and metyrapone, to decrease serum cortisol for high-risk AIMAH. In case 3, we chose ketoconazole, which inhibits adrenal steroid production via the inhibition of multiple steroidogenic enzymes at the dose of 400 mg/daily or 800 mg/daily.[sup.8] A major side effect of ketoconazole is hepatotoxicity; therefore, liver function should be monitored routinely during treatment.
The identification of aberrant adrenal hormone receptors provides new opportunities for specific pharmacological therapies in AIMAH.[sup.2] Since multiple receptors could mediate cortisol hypersecretion in AIMAH, these patients should undergo screening for aberrant receptors, as this could affect the therapeutic strategy.
We do not have a lot of experience with AIMAH therapy; however, novel therapeutic agents remain to be tested, with the aim to cure patients in the future.