Introduction:
The adrenal glands are a pair of triangular-shaped glands located above the kidneys. They are also known as the suprarenal (above the kidney) glands. The adrenal glands are an integral part of the endocrine system and produce hormones directly into the bloodstream. It is structurally divided into the outer cortex and the inner medulla. The medullary complex produces adrenaline and noradrenaline that regulate the flight or fight response. The adrenal cortex produces hormones like cortisol, aldosterone, and androgens. An excess of hormones produced by the adrenal cortex is known as adrenocortical excess.
What Is Adrenocortical Excess?
The adrenal gland can be overactive due to many underlying conditions. In such cases, excessive hormones are produced. Since these hormones control vital activities in the body, an overproduction of these can cause disturbances. This condition is known as adrenocortical hyperfunction.
Depending on the specific hormones, it can be further divided into:
A. Cushing's Syndrome - characterized by excess production of cortisol.
B. Hyperaldosteronism - due to overproduction of aldosterone.
C. Adrenogenital Problems - caused by excess androgen production.
What Causes Adrenocortical Excess?
The underlying condition that causes adrenocortical hyperfunction can be divided based on the hormone affected.
A. Cushing's Syndrome - An elevation in the glucocorticoid (cortisol) levels can be due to the following conditions.
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Long-Term Glucocorticoid Therapy - A vast number of cases presenting with Cushing's syndrome have a history of administration of glucocorticoids or steroids. These medicines suppress the immune system, so they are used to manage autoimmune conditions like rheumatoid arthritis and organ transplants. This is known as iatrogenic illness (illness caused by medical treatment) or exogenous Cushing's syndrome.
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Hypersecretion of ACTH (Adrenocorticotropic Hormone) - The ACTH hormone produced by the pituitary gland instructs the adrenal glands to release cortisol. By default, an overproduction of ACTH will increase cortisol levels. ACTH overproduction is linked to pituitary tumors and other non-endocrine cancer. 70 % to 80 % of Cushing's syndrome cases are due to hypersecretion of ACTH.
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Adrenal Gland Tumors - Cancerous and non-cancerous tumors of the adrenal gland can also cause an overproduction of cortisol levels.
B. Hyperaldosteronism - An excess level of aldosterone hormone due to the following reasons.
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Enlargement of Adrenal Cortex - The condition is known as adrenocortical hyperplasia. It is due to the overgrowth of cells that leads to tissue or organ enlargement. The cause is unknown, but it causes an oversupply of aldosterone.
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Adrenal Tumors - As mentioned above, tumors arising from the adrenal glands also cause an elevated aldosterone level.
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Genetic Reasons - In some families, it is caused by the alteration of the aldosterone-producing genes. This leads to sustained production of steroids.
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Other Causes - Renin is a hormone produced by the kidneys that regulate blood pressure and indirectly stimulates aldosterone production. Alterations in renin production due to renal and other systemic causes can lead to an increase in aldosterone.
C. Adrenogenital Problems - Caused due to elevated androgen levels, thereby leading to disorders of sexual differentiation.
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ACTH Overproduction - ACTH also regulates the formation of androgens. In conditions where ACTH is hyperactive, androgen levels increase along with other adrenocortical hormones.
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Adrenal Tumors and Overgrowth - Like the other two hormones, androgen excess can occur due to adrenal tumors (adenoma and carcinomas) and hyperplasias.
What Are the Symptoms of Adrenocortical Excess?
Symptoms of adrenocortical excess can be discussed under these headings:
A. Symptoms of Cushing’s Syndrome - Cushing's syndrome has a classic clinical presentation.
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Central Obesity - Fat deposition in the upper body, particularly in the back of neck and trunk, causing a buffalo hump appearance. Moon facies or a round face due to fat distribution in the face.
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Thin Arms and Legs - High cortisol levels atrophy of the muscles resulting in thinning of arms and legs.
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Weakness and Fatigue - It is an indirect effect caused due to the loss of muscle mass by the overaction of cortisol.
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Facial and Body Hair Growth - This condition is known as hirsutism, where male-pattern hair growth is seen on the face, back, and chest.
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Menstrual Abnormalities - Circulating cortisol levels cause an irregular menstrual cycle in 80 % of women with Cushing's syndrome.
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Hypertension - Cortisol increases the heart rate and blood pressure as a stress response. Hypertension is a common feature of Cushing's syndrome.
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High Blood Sugar - Glucocorticoids inhibit glucose uptake by the cells resulting in hyperglycemia. It is also an important cause of secondary diabetes or diabetes mellitus.
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The Fragile Skin and Stretch Marks - Cortisol causes thinning of the skin by affecting the skin proteins. Consequently, the skin is delicate and can easily bruise. Stretch marks can be seen in the abdominal area.
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Bone Weakness - Bone resorption results in the development of a condition called osteoporosis, which increases the susceptibility to fractures.
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Psychiatric Abnormalities - Includes mental disturbances, anxiety, mood swings, depression, psychosis, etc.
B. Symptoms of Hyperaldosteronism:
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Hypertension - In hyperaldosteronism due to adrenal tumors and hyperplasias, the serum renin concentrations are low. As a result, sodium retention increases, resulting in increased blood pressure.
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Decreased Potassium Levels - A condition known as hypokalemia results from increased potassium excretion by the kidneys. It causes neuromuscular manifestations like weakness, numbness, and visual disturbances.
C. Symptoms of the Adrenogenital Problem:
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In Females - Virilization causes females to develop male characteristics. This includes abnormal hair growth on the face and chest, acne, changes in the menstruation cycle, smaller breasts, infertility, enlarged clitoris resembling a penis, shallow vagina, etc.
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In Males - Feminization causes males to gain feminine characteristics. Males can also develop virilization that may cause infertility, enlarged penis size, early or delayed puberty, etc.
How Is Adrenocortical Hyperfunction Diagnosed?
The diagnosis of adrenocortical excess can be made by:
A. Clinical Examination - A physical examination for clinical signs (particularly in Cushing's syndrome), a thorough history of symptoms, and past medical history helps determine the diagnosis.
B. Blood Tests - Beneficial to measure ACTH levels in the blood. Depending on the underlying cause, the ACTH levels can be high (in pituitary tumors) or low (in adrenal tumors).
C. Urine Tests - To measure the corticosteroid levels.
D. Dexamethasone Suppression Test - Involves administering Dexamethasone (a steroid medication). Helps in determining the underlying condition.
E. Advanced Imaging - Includes non-invasive techniques like MRI (magnetic resonance imaging) and CT (computed tomography) scan to visualize the size and location of tumors causing adrenocortical hyperfunction.
What Is the Treatment for Adrenocortical Excess?
The choice of treatment mainly depends on the underlying condition. Surgery is the first line of approach for pituitary and adrenal glands tumors. Removing the gland is the most common treatment for adrenal tumors and hyperplasias. If the tumors are cancerous, then multimodality therapy, including surgery, chemotherapy, and radiotherapy, is the choice. Other treatment options include hormone-suppressing and inhibiting drugs. For patients with long-term steroid therapy, reducing the dose or changing the medication will improve the symptoms. Other treatment options include hormone-suppressing and inhibiting drugs.
Conclusion:
Adrenocortical hyperfunction is a condition where the cortex of the adrenal gland produces excess hormones. The adrenal cortex is responsible for producing cortisol, aldosterone, and androgens. These hormones have specific functions, an excess of which can lead to disturbances in metabolism and sexual development. The overproduction is due to underlying conditions like pituitary and adrenal tumors, adrenal hyperplasias, long-term steroid therapy, etc. Most commonly, adrenocortical excess manifests as Cushing's syndrome. The treatment mainly depends on identifying the underlying condition.
