Adrenocortical Hyperfunction (Hypercorticism)
Excessive production of adrenocortical hormones is most commonly associated with tissue hyperplasia or hormonally active tumors (adenomas and carcinomas).
- Cushing's Syndrome: Occurs due to cortisol excess. Causes may include ectopic ACTH production, carcinomas, or cortical hyperplasia. If a patient with this syndrome undergoes bilateral adrenalectomy, 8–10% of patients may develop Nelson's syndrome—the formation of an ACTH-secreting pituitary adenoma, which causes profound skin hyperpigmentation.
- Primary Hyperaldosteronism: Aldosterone excess that is entirely independent of the renin-angiotensin system. In 70% of cases, it is caused by a benign tumor—an aldosteroma (Conn's syndrome). It manifests as arterial hypertension, paresthesias, and muscle weakness against the background of hypokalemia and hypernatremia.
Interesting Fact: Liddle's syndrome (pseudohyperaldosteronism) exists. This is a hereditary renal pathology in which receptors are hypersensitive to aldosterone, even though the blood level of the hormone itself remains normal.
Congenital Adrenogenital Syndromes (CAH)
CAH is a group of autosomal recessive disorders rooted in congenital enzymopathy. Due to an enzyme defect, glucocorticoid levels drop, prompting the pituitary gland to produce massive doses of ACTH. This leads to massive bilateral adrenal hyperplasia (their weight may exceed 60 g).
21-hydroxylase deficiency occurs in 90% of cases. There are two main forms:
- Virilizing form: Excess precursors are redirected toward testosterone synthesis. In girls, this leads to female pseudohermaphroditism; in boys, to macrogenitosomia. Children grow rapidly, but bone growth plates close early, leaving them short-statured.
- Salt-wasting form (10% of cases): The enzyme is completely absent, making aldosterone synthesis impossible. It is accompanied by severe urinary loss of sodium and chlorides. Without replacement therapy, it rapidly leads to death.
Adrenocortical Hypofunction (Hypocorticism)
Insufficiency manifests only when at least 80–90% of the cortex on both sides is destroyed.
- Addison's Disease (chronic form): In more than half of cases, it is of autoimmune origin (antibodies against cortical cell cytoplasm are produced). Notably, in autoimmune damage, the medulla remains unaffected. However, if the cause is generalized tuberculosis, both layers are destroyed. Classic symptoms: systemic melanosis (skin darkening), hypotension, weakness, and weight loss.
- Acute Insufficiency (Adrenal Crisis): Can occur upon rapid withdrawal of glucocorticoids or massive hemorrhage into the glands. A striking example is Waterhouse-Friderichsen syndrome, which develops in young children during severe bacterial infections (especially meningococcal). Due to bilateral hemorrhagic infarction of the adrenals, catecholamines disappear, blood vessels dilate paretically, blood pressure drops, and coma ensues.
Pathology of the Medulla
The primary pathology of the medullary substance is pheochromocytoma. This is a rare tumor of mature medullary cells that actively secretes catecholamines (most commonly norepinephrine).
- Features: About 10% of such tumors are malignant. The tumor can also be located outside the adrenals (e.g., in the aortic paraganglion).
- Clinical Presentation: Characterized by a crisis-like course. Triggers for a crisis can include physical exertion or stress. During an attack, there is a sharp spike in blood pressure, tachycardia, headache, profuse sweating, and skin pallor.
- Complications: Catecholamine excess can lead to myocardial infarction, stroke, or sudden death during anesthesia.
Briefly: answers to topical questions
How does Liddle's syndrome differ from primary hyperaldosteronism?
The main difference between Liddle's syndrome and primary hyperaldosteronism is that in Liddle's syndrome, the aldosterone level is within the normal range, whereas in primary hyperaldosteronism, there is increased aldosterone production.
| Characteristic | Primary Hyperaldosteronism | Liddle's Syndrome |
|---|---|---|
| Aldosterone level | Increased production | Within the normal range |
| Pathogenesis | Aldosterone production is independent of the renin-angiotensin system status | Increased sensitivity of renal tubular receptors to aldosterone; increased activity of epithelial sodium channels in the kidneys |
| Etiology | Aldosteronoma; bilateral idiopathic adrenal hyperplasia | Hereditary tubulopathy |
| Manifestation | For aldosteronoma, noted as: usually in women over 40 | In early childhood |
| Clinical and Laboratory Findings | Hyperkaliuria, hypokalemia, hypernatremia, arterial hypertension, muscle weakness, paresthesias, cramps, polyuria | Hypokalemia, metabolic alkalosis, low PRA and PRC, low aldosterone-to-renin ratio |
To remember the pathogenesis differences of Addison's disease: "Autoimmunity eats only the Cortex (AC), while Tuberculosis destroys Totally everything (TT)."
What else to study on this topic
- Rare forms of CAH: 11β-hydroxylase, 17α-hydroxylase, and 3β-hydroxysteroid dehydrogenase deficiency.
- Congenital lipoid adrenal hyperplasia (Prader syndrome).
- Hormonally active tumors: androsteroma and corticoestroma.
- APUD system pathology: carcinoid and carcinoid syndrome.
- Pineal gland tumors (pinealomas) and their systemic effects.
- Multiple endocrine neoplasia syndromes (MEN type 1).
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