Definition A clinical syndrome characterized by hypertension, hypokalemia, suppressed plasma renin activity, and neuromuscular dysfunction caused by increased secretion of aldosterone due to adrenocortical tumors or hyperplasia, resulting in water and sodium retention and volume expansion of body fluids. Regulation of aldosterone secretion I. The renin-angiotensin system Low sodium, reduced fluid volume, and low blood pressure can excite the renin-angiotensin II (AT-II) system and stimulate the production of aldosterone in the globus pallidus; Wang Jian, Department of Endocrinology, Nanjing Military General Hospital Increased aldosterone causes sodium retention, fluid volume expansion, and increased blood pressure, and feedback inhibits the renin-AT-II system, resulting in decreased aldosterone production Regulation of aldosterone secretion II. ACTH has a weak stimulatory effect on aldosterone secretion. If applied for a long time, the fasciculus no longer responds to it. Other aldosterone excitatory factors Serotonin, histamine-mediated β-LPH and α-MSH, etc. Antagonists of serotonin and histamine can reduce aldosterone secretion Etiology I. Aldosteronoma (renal tumor) Aldosteronoma (adrenocortical adenoma) is the most common cause, first reported by Conn in 1955, also known as Conn syndrome. It is common in women; unilateral adenoma is common; small size, mostly 1-2 cm, >3 cm is rare; bilateral adenoma is rare II. Idiopathic aldosteronism Pathological changes Diffuse or nodular proliferation of cells in the globular zone of bilateral adrenal glands Etiology Unknown, caused by some extra-adrenal factor that excites aldosterone secretion? Etiological classification III. Aldosterone carcinoma is rare, accounting for about 1% of the disease IV. Glucocorticoid-suppressible aldosteronism (ACTH-dependent aldosteronism) Predisposition Familial, autosomal dominant; may also be sporadic Age and sex Adolescent males are more common Etiology Unknown, aldosterone-secreting cells have abnormal ACTH receptors Features Glucocorticoid (dexamethasone) therapy may be effective Etiology V. Primary adrenocortical hyperplasia Primary adrenocortical hyperplasia Clinical manifestations and biochemical changes are similar to those of aldosteronism VI. Heterogenous aldosteronism is rare, caused by ovarian cancer, and may be associated with precocious puberty, increased estradiol and testosterone in the blood, in addition to primary aldosteronism Pathophysiology and clinical manifestations Hypertension caused by increased aldosterone, decreased sodium excretion from the distal tubules of the kidney, sodium retention, and increased blood volume, is the earliest and most common symptom, and is seen in almost every case at different stages Features Generally does not evolve malignantly; blood pressure is mostly around 170/100 mmHg, but can also be as high as 210/130 mmHg; poor efficacy of antihypertensive drugs Pathophysiology and clinical manifestations II. Neuromuscular dysfunction 1. paroxysmal muscle weakness and paralysis Common, associated with hypokalemia, 1 cm adenoma Inadequate: difficult to define in small adenomas and hyperplasia 10. adrenal CT and MRI non-invasive; little (or no) radioactivity; can detect adenomas up to 1 cm; also valuable in hyperplasia, but sometimes difficult to distinguish in nodular hyperplasia Diagnosis 11. adrenal venous blood sampling for aldosterone and angiography Adenoma: blood aldosterone on the affected side is more than 10 times higher than that on the healthy side Hyperplasia: blood aldosterone is increased on both sides High localization accuracy, but traumatic, many complications of imaging (such as venous embolism, etc.), difficult to master, very valuable for localization and diagnosis Differential diagnosis 1. Secondary aldosteronism Renal artery stenosis, congestive heart failure, cirrhosis, nephrotic syndrome, etc. 3. Adrenal diseases Cortisolism (adenocarcinoma, heterogenous ACTH syndrome) 4. Aldosterone tumors can be cured by surgical treatment. Pre-operative administration of aldosterone and, if necessary, potassium supplementation and a low-sodium diet are required to correct hypokalemia and hypertension. Idiopathic aldosteronism Drug therapy DD spironolactone, 120-240mg/day, divided into 3-4 oral doses For long-term application Male DD breast development, impotence Female DD menstrual disorders If necessary, can switch to aminoglutethimide or aminolol Calcium antagonists (such as nifedipine, etc.), ACEI (enalapril, etc.) have both hypotensive and aldosterone inhibition effects Treatment III. Aldosterone carcinoma Early surgery should be pursued IV. Glucocorticoid suppressible aldosteronism Dexamethasone treatment DD0.75mg, once in 8h, effective after 2-4 weeks Long-term use, maintenance dose of 0.75mg/day Treatment V. Primary adrenocortical hyperplasia Consider surgical treatment DD total excision on one side and major excision on the other side (less effective) Prefer drug therapy