Diseases associated with excessive production of aldosterone ( aldosteronism )
Endocrinology / / August 12, 2017
In 1956 Van Bushem, Doorenbos, Elings first described case without hyperaldosteronism adrenal tumors at the 17-year-old boy who had malignant hypertension and polydipsia from the age of two.Clinical and biochemical manifestations were the same as in primary aldosteronism.When subtotal adrenalectomy bilateral hyperplasia of the beam and the glomerular zone of the adrenal cortex has been found.A few weeks after the surgery all clinical and biochemical disturbances disappeared.In the future, similar cases have been described where clinical features of primary aldosteronism apparent in early childhood.In these patients, there was no adenoma and bilateral adrenal hyperplasia was found.The clinical course is flowing malignant hypertension with edema of the optic nerve papilla and pronounced thirst and polyuria.Muscle weakness is not the main complaint of these patients.The cause of the disease, Conn opinion, is a genetic defect in the control system of secretion of aldosterone.
This defect is in some kind of
Wolff expressed about this disease different view.He believes that congenital aldosteronism obviously applies to the secondary aldosteronism, and that the primary defect in it to be found in violation of the functions of juxtaglomerular apparatus of kidneys.In confirmation of this, Wolff points to the difference between asymptomatic congenital aldosteronism - the tendency to hyponatremia and malignant hypertension flowing, features that characterize, according to recent data, over-activity of the renin system - angiotensin - aldosterone system.
- Physiology of the adrenal cortex
- Chronic adrenal insufficiency.Addison's disease
- androgen-producing tumors of the adrenal cortex
- Primary aldosteronism (Conn's syndrome)
- Clinical evaluation of laboratory studies of the functional state of the adrenal cortex
- feminizing tumor of the adrenal cortex
It is sometimes difficult to differentiate congenital aldosteronismthe disease, based on the kidney juxtaglomerular apparatus hyperplasia.In the latter disease has a high content of aldosterone in the blood and, therefore, all the symptoms inherent in primary aldosteronism, but due to increased production of renin.diagnostic error in this case may lead to the wrong decision to use as a medical adrenalectomy event.Meanwhile, the removal of the adrenal hyperplasia juxtaglomerular apparatus hyperplasia causes further increase of this apparatus, and therefore, deterioration of the patient.