ALDOSTEROMA (synonym: primary aldosteronism, Conn's syndrome) — the hormone-producing tumor of bark of an adrenal gland which is marking out the increased quantity of Aldosteronum.
Primary aldosteronism was for the first time described by J. W. Conn in 1955. The term «aldosterom» is entered by O. V. Nikolaev (1963) and was addition to the classification of gormonalnoaktivny tumors of bark of an adrenal gland published in 1947. It is localized And. in one of adrenal glands; bilateral tumors are observed approximately in 6% of cases.
People of young age get sick usually, women are more often. Cases of development are described And. at children. Malignant And. meet seldom.
Some researchers, in particular Dzherni (I. The village of Gerny, 1970), consider that 5 — 8% of all patients with a hypertension suffer from primary aldosteronism and can be cured in the surgical way.
Morphologically And. reminds adenoma of bark of an adrenal gland of ocherous-yellow color, in the thin connective tissue capsule, as a rule, of the small sizes (1 — 3 cm in dia.). The microscopic structure is presented by spongiocytes (light cells) of bark of an adrenal gland which arrangement reminds a structure of a glomerular or puchkovy zone.
A clinical picture
In clinical symptomatology it is possible to allocate three basic groups of symptoms: cardiovascular, renal, neuromuscular.
Supersecretion of Aldosteronum (see) breaks water and electrolytic balance in an organism towards a gipokaliyemichesky alkalosis and a delay of sodium. Sodium collects intracellularly in this connection patients have no peripheral hypostases. Accumulation of sodium in a wall of vessels leads to an overhydratation and narrowing of their gleam that increases a vascular tone and peripheric resistance and leads to development of hypertensia. High arterial pressure meets almost constantly. Against the background of constant hypertensia the crises reminding attacks at a pheochromocytoma (see), but, unlike the last can be observed, they are followed more often by bradycardia.
As a result of hypertensia changes of an eyeground from a hypertensive vasomotor spasm to the expressed retinopathy with hemorrhages and a papilledema develop. At children of change of an eyeground come especially quickly and often lead to a vision disorder, up to a blindness.
For patients And. the polyuria and a nocturia with an isosthenuria and alkali reaction of urine is characteristic. At certain patients the daily urine reaches 10 l. Restriction of liquid does not promote improvement of concentration function of kidneys.
Almost all patients complain of muscular weakness. Paresthesias, spasms, sometimes sluggish paralyzes are quite often observed that is caused by deficit of potassium in fabrics.
Changes of an ECG are characteristic of a hypopotassemia: reduction of an interval S — T, quite often negative tooth of T in 1,2, V5, V6 assignments. Content of potassium and chlorides in blood serum is usually reduced, but some patients can have a normal level of potassium. The hypernatremia is observed seldom. More often the level of sodium in blood corresponds to normal indicators. Excretion with urine of Aldosteronum is more often raised, sometimes by 3 — 5 times in comparison with normal indicators. Activity of a renin in a blood plasma low, however at a combination of Ampere-second activity of a renin can be increased by primary damage of kidneys (similar observations are described in literature).
the Diagnosis is made on the basis of clinical symptoms and laboratory researches. For diagnosis And. (especially normokaliyemichesky form) also the test with hypothiazid (100 mg) which at is used And. gives the expressed decrease in potassium in blood. For the purpose of topical diagnosis And. most often apply a suprarenorentgenografiya, sometimes arteriography and a flebografiya (see) adrenal glands. The technique of a flebografiya by catheterization of average adrenal veins presents the known difficulties therefore recently some researchers give preference to an occlusal flebografiya of the lower vena cava. A renovascular hypertension make an angiography for differential diagnosis of Ampere-second (see) also define a renin of blood. At patients And. activity of a renin of a blood plasma is sharply reduced, at renovascular hypertensia — usually high.
the Forecast in the absence of a malignancy and renal disturbances favorable.
After removal And. the water and electrolytic balance is recovered, blood pressure is normalized or considerably the arterial hypertension decreases if irreversible changes in kidneys did not manage to develop.
Treatment surgical. At the known localization of a tumor apply lumbar or thoracolumbar accesses on the relevant party. When localization And. it is not possible to define, use transabdominal access more often that allows to examine both adrenal glands at the same time. In the course of a preparation for surgery patients receive drugs of potassium and antagonists of Aldosteronum, in particular Aldactonum (see. Spironolactonums ). In the postoperative period the close check behind a condition of electrolytic balance and administration of salts of potassium according to results of a research of balance of potassium is necessary. After removal of a unilateral tumor purpose of steroid and angiotonic hormones is not shown.
Bibliography: Nikolaev O. V. and E. I Cockroaches. Hormonal and active tumors of bark of an adrenal gland, M., 1963, bibliogr.; Ratner N. A., Gerasimova E. N. and Gerasimenko P. P. Giperaldosteronizm, M., 1968, bibliogr.; Soffer L., Dorfman R. and Gebrilav of L. Epinephral glands of the person, the lane with English, M., 1966, bibliogr.; Conn J. W., Knopf R. F. and. Nesbit R. M. Clinical characteristics of primary aldosteronism from an analysis of 145 cases, Amer. J. Surg., v. 107, p. 159, 1964, bibliogr.
V. I. Kertsman, O. V. Nikolaev.