Recognition of the pathogenesis of secondary forms of hypertension is often considered the key to appropriate choice of treatment. We here present the results of a prolonged clinical follow-up (from 1 to 20 years) of a large number of patients with mineralocorticoid excess syndromes (MES), including over 100 patients with primary aldosteronism (PA), 3 cases with dexamethasone-suppressible aldosteronism (DSA), 3 cases of apparent mineralocorticoid excess (AME) Type II, and 4 patients with 17-hydroxylase deficiency (17OHDS). The patients with PA have been divided in two subgroups, one of 69 cases followed between 1973 and 1982, and the second of 37 patients studied between 1983 and 1992; 33 further cases were not evaluated due to poor compliance. In group I, 26 patients underwent surgery (23 unilateral adenoma, 1 primary hyperplasia, 2 bilateral nodular hyperplasia); at 5 years 50% had normal blood pressure, 25% had mild hypertension and 25% had moderate to severe hypertension. Forty-three patients with either adenoma (APA) or idiopathic aldosteronism (IHA) received long-term spironolactone treatment. Among them, 13 required the addition of thiazide and/or beta-blockers, while 13 were switched to an amiloride/thiazide combination (+/- beta blockers) due to side-effects to spironolactone (gynecomastia 6/20 males, menstrual upset or breast pain in 7/23 females). In group II, 12 patients underwent surgery (11 adenoma, 1 primary hyperplasia) with a similar outcome at 3 years as in group I; 25 patients were put on either K canrenoate (11) or Ca++ channel blockers (14) with or without KCl supplementation; in 8 cases these two drugs were combined according to blood pressure levels achieved during the follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)
"It is apparent that laparoscopic adrenalectomy alone is not sufficient to achieve BP control in a significant proportion of patients. In addition, surgery is not without risks (Mantero et al. 1995, Ghose et al. 1999). Therefore, long-term medical therapy might represent an attractive alternative approach for the treatment of PA. "
[Show abstract][Hide abstract] ABSTRACT: Primary aldosteronism (PA) and, in particular, its two commonest subtypes (i.e. idiopathic hyperaldosteronism (IHA) and aldosterone-producing adenoma (APA)) have been recognized as the most common cause of secondary hypertension. While 'conservative' medical treatment with aldosterone receptor antagonists is the therapeutic approach of choice in controlling blood pressure in patients with PA due to IHA, the more invasive (laparoscopic) adrenalectomy seems to be the most suitable therapy for patients with APA. In this review, we focus on the medical approach for the management of APA in cases where surgical excision of the adrenal is not possible.
Endocrine Related Cancer 07/2008; 15(3):693-700. DOI:10.1677/ERC-08-0094 · 4.81 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Background: 17α-hydroxylase deficiency is a rare cause of congenital adrenal hyperplasia and endocrine hypertension. Only around 124 cases were reported in literature. Case: A 35-year-old Taiwanese female presented with severe hypertension (220/130 mmHg), absence of secondary sexual characteristics and primary amenorrhea. Chromosome study revealed 46XX karyotye. The laboratory data revealed hypokalemia, suppressed plasma renin activity, low level of sex steroids with high gonadotropin, morning cortisol level: 1.8µg/dl and ACTH: 235 pg/ml. Her plasma level of aldosterone (sitting position) was at high level of normal (23.6 ng/dl). We made the diagnosis of 17α-hydroxylase deficiency. Plasma aldosterone level was suppressed in most of the reported ca-ses while normal or elevated levels were also described in a considerable number. Her uterus was found to be rudimentary, which was reported in only one genetic female case before. Conclusion: We report a case of 17α-hydroxylase deficiency in genetic female with normal le-vel of plasma aldosterone and rudimentary uterus. ( J Intern Med Taiwan 2002;13: 141-146 )
Journal of Internal Medicine of Taiwan 01/2002; 13(3).
[Show abstract][Hide abstract] ABSTRACT: Disorders of the adrenal cortex and medulla are often associated with hypertension, which can be cured surgically in many cases or may require specific and timely medical treatments. Therefore, knowledge of adrenal physiology, biochemistry, and molecular biology is essential such that an appropriate diagnostic evaluation can be conducted efficiently. The most common hypertensive disorder of the adrenal cortex is primary aldosteronism. Aldosterone-producing adenoma is the most common form of primary aldosteronism and is most likely to be cured by unilateral adrenalectomy when aldosterone production is highly autonomous from renin-angiotensin, lateralizes to one adrenal gland, and is associated with overproduction of 18-hydroxycortiocosterone and C18-methyloxygenated metabolites of cortisol. Variants of adrenal hyperplasia that share these characteristics can also be cured by unilateral adrenalectomy.
World Journal of Urology 03/1999; 17(1):15-21. DOI:10.1007/s003450050099 · 2.67 Impact Factor
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