新光吳火獅紀念醫院 外科部 泌尿科1; 輔仁大學 醫學系2
Familiar adrenal aldosterone-producing adenoma
Chih-Yin Yeh1, Yi-Chia Lin1,2, Te-Fu Tsai1,2, Guang-Dar Juang, Chung-Hsin Yeh, Yi-Hong Cheng, Kuang-Yu Chou, Hong-En Chen, Cho-Yen Ho, Tzu-Hsiang Wu , Yi-Bo Chu, Thomas I.S. Hwang1,2Allen W. Chiu1
Division of Urology, Department of Surgery, Shin Kong WHS Memorial Hospital1
School of Medicine, Fu-Jen Catholic University2
Abstract: Primary hyperaldosteronism is the most common cause of secondary hypertension and occurs in about 6% to 20% of adult hypertensive patients, higher in patients with resistant and uncontrolled hypertension. Aldosterone-producing adenoma is present in 50% to 60%, and the remaining is idiopathic or bilateral adrenal hyperplasia. It is about two times more common in women than men. There are three types of familiar hyperaldosteronism have been identified for rare familial forms of the disease. In a majority of these genetic alterations, the end point is Ca influx and membrane depolarization resulting in aldosterone hypersecretion. This report presents four cases of familiar adrenal adenoma instead of only hyperaldosteronism. First case is a 54-year-old female with frequency for one year. Tracing back her personal history showed coronal artery disease status post stenting and hypertension for more than 20 years. Computed Tomography showed left adrenal adenoma. After left adrenalectomy, her frequency cured and blood pressure gradually returns to normal. Then, the other three sisters diagnosed as adrenal adenoma. Distinguishing from familiar hyperaldosteronism which cannot be correctable by surgery, these familiar adrenal aldosterone-producing adenomas were all cured by surgery. Furthermore, essential guidelines necessary to distinguish familiar adenoma from hyperaldosteronism. The literatures were reviewed.