11.2 Disorders of the Adrenal Glands
4 min read•july 24, 2024
Adrenal gland disorders can wreak havoc on the body's hormonal balance. From to , these conditions disrupt normal endocrine function and metabolism, leading to a range of symptoms.
Understanding the differences between primary and secondary is crucial. While both affect production, primary insufficiency impacts too. and round out the list of adrenal gland disorders, each with its unique set of challenges.
Adrenal Gland Disorders: Hyperfunctioning States
Pathophysiology of Cushing's syndrome
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- Pathophysiology
- Excessive cortisol production disrupts normal endocrine function and metabolism
- Most common cause: exogenous glucocorticoid use (prednisone) suppresses natural cortisol production
- Endogenous causes
- Pituitary adenoma (Cushing's disease) secretes excess ACTH stimulating adrenal glands
- Ectopic ACTH-producing tumors (lung cancer) bypass normal feedback mechanisms
- Adrenal tumors autonomously produce cortisol independent of ACTH
- Clinical manifestations
- Central obesity redistributes fat to trunk and face
- Moon face develops from fat deposits in cheeks
- Buffalo hump forms from fat accumulation at base of neck
- Purple striae appear due to skin thinning and easy bruising
- Proximal muscle weakness results from protein catabolism
- occurs from decreased bone formation and increased resorption
- develops from increased gluconeogenesis and insulin resistance
- arises from mineralocorticoid effects and volume expansion
- Mood changes include depression, anxiety, and irritability
- Diagnostic criteria
- 24-hour urinary free cortisol measures cortisol excretion over time
- Late-night salivary cortisol detects loss of normal diurnal rhythm
- assesses feedback inhibition of
- Plasma ACTH levels differentiate ACTH-dependent from ACTH-independent causes
- Imaging studies (CT, MRI) locate tumors in pituitary or adrenal glands
Primary aldosteronism overview
- Causes
- -producing adenoma (Conn's syndrome) autonomously secretes aldosterone
- Bilateral adrenal leads to excessive aldosterone production from both glands
- Pathophysiology
- Excessive aldosterone production increases sodium and water retention
- Increased sodium reabsorption in distal tubules and collecting ducts
- Increased potassium excretion leads to
- Volume expansion raises blood pressure
- Suppression of renin production through negative feedback
- Clinical presentation
- Hypertension (often resistant to treatment) due to volume expansion and vasoconstriction
- Hypokalemia causes muscle weakness and cardiac arrhythmias
- results from increased hydrogen ion excretion
- Muscle weakness occurs from potassium depletion
- Headaches may be related to hypertension
- Polyuria and polydipsia develop from impaired urine concentrating ability
Primary vs secondary adrenal insufficiency
- Primary adrenal insufficiency (Addison's disease)
- Etiology
- Autoimmune adrenalitis destroys adrenal cortex (70-90% of cases)
- Tuberculosis infiltrates adrenal glands (developing countries)
- Fungal infections (histoplasmosis) can invade adrenal tissue
- Metastatic cancer spreads to adrenal glands (lung, breast)
- Characteristics
- Deficiency of both glucocorticoids and mineralocorticoids impairs stress response and electrolyte balance
- Elevated ACTH levels due to lack of negative feedback
- Etiology
- Secondary adrenal insufficiency
- Etiology
- Pituitary tumors compress ACTH-producing cells
- Pituitary or radiation damages ACTH-producing cells
- Prolonged exogenous glucocorticoid use suppresses HPA axis
- Characteristics
- Deficiency of glucocorticoids only affects metabolism and stress response
- Low ACTH levels due to pituitary dysfunction
- Normal mineralocorticoid function preserves electrolyte balance
- Etiology
- Common symptoms of adrenal insufficiency
- and weakness result from cortisol deficiency
- occurs due to decreased appetite and metabolism
- (primary only) caused by increased ACTH and MSH
- develops from fluid and electrolyte imbalances
- (primary only) compensates for mineralocorticoid deficiency
- and may be due to electrolyte disturbances
Pheochromocytoma and neuroblastoma characteristics
- Pheochromocytoma
- Pathophysiology
- Catecholamine-secreting tumor of chromaffin cells in adrenal medulla
- Excessive production of and causes sympathetic overactivity
- Clinical manifestations
- occurs in episodic surges of catecholamines
- Headaches result from vasoconstriction and hypertension
- Sweating is caused by increased metabolic rate and vasodilation
- Palpitations reflect tachycardia and increased cardiac output
- Anxiety develops from central nervous system stimulation
- Pallor results from peripheral vasoconstriction
- Pathophysiology
- Neuroblastoma
- Pathophysiology
- Malignant tumor of sympathetic nervous system affects children
- Originates from neural crest cells that form sympathetic ganglia
- Clinical manifestations
- Abdominal mass is often the presenting sign in young children
- Bone pain indicates metastatic spread to skeletal system
- Proptosis occurs with orbital metastases
- Fever may be due to tumor necrosis or metastases
- Weight loss results from increased metabolism and decreased appetite
- Anemia develops from bone marrow infiltration
- Opsoclonus-myoclonus syndrome (in some cases) presents with rapid eye movements and muscle jerks
- Pathophysiology
Key Terms to Review (35)
24-hour urinary free cortisol test: The 24-hour urinary free cortisol test is a diagnostic procedure used to measure the amount of cortisol excreted in urine over a 24-hour period. This test is crucial in diagnosing disorders related to adrenal gland function, particularly conditions such as Cushing's syndrome, where cortisol levels are abnormally high.
ACTH Stimulation Test: The ACTH stimulation test is a diagnostic procedure used to assess the functioning of the adrenal glands by measuring how they respond to adrenocorticotropic hormone (ACTH). This test is crucial in diagnosing disorders related to adrenal insufficiency, such as Addison's disease or secondary adrenal insufficiency, as it evaluates the glands' ability to produce cortisol in response to ACTH administration.
Adrenal crisis: An adrenal crisis is a life-threatening condition that occurs when the body experiences an acute adrenal insufficiency, leading to a severe deficiency in cortisol. This can happen during times of physical or emotional stress, such as illness, injury, or surgery, especially in individuals with existing adrenal gland disorders. The lack of cortisol can cause symptoms like low blood pressure, confusion, and shock, requiring immediate medical attention to prevent serious complications or death.
Adrenal insufficiency: Adrenal insufficiency is a medical condition characterized by the inadequate production of adrenal hormones, particularly cortisol and aldosterone, from the adrenal glands. This can lead to various metabolic disturbances, causing symptoms such as fatigue, weight loss, low blood pressure, and electrolyte imbalances. Understanding adrenal insufficiency is crucial as it can stem from primary adrenal gland damage, secondary causes due to pituitary dysfunction, or even sudden withdrawal from long-term corticosteroid therapy.
Aldosterone: Aldosterone is a steroid hormone produced by the adrenal cortex that plays a crucial role in regulating sodium and potassium balance in the body. By promoting sodium reabsorption and potassium excretion in the kidneys, it helps maintain fluid balance and blood pressure, making it essential for homeostasis.
Cortisol: Cortisol is a steroid hormone produced by the adrenal glands that plays a vital role in the body's stress response, metabolism, and immune function. It helps regulate blood sugar levels, controls inflammation, and influences memory formation. Imbalances in cortisol levels can lead to various disorders related to adrenal gland function.
Cushing's Syndrome: Cushing's Syndrome is a hormonal disorder caused by prolonged exposure to high levels of cortisol, often resulting from a tumor in the adrenal glands or excessive use of corticosteroid medications. This condition leads to various health issues, including obesity, hypertension, and changes in skin appearance, and is closely associated with the dysfunction of the adrenal glands, which play a key role in regulating stress responses and metabolic processes in the body.
Epinephrine: Epinephrine, also known as adrenaline, is a hormone and neurotransmitter produced by the adrenal glands that plays a critical role in the body's fight-or-flight response. It prepares the body for quick reactions to stress or danger by increasing heart rate, blood pressure, and energy supply. In the context of adrenal gland disorders, understanding epinephrine's function is essential, as imbalances can lead to various health issues such as adrenal insufficiency or pheochromocytoma.
Fatigue: Fatigue is a state of extreme tiredness or exhaustion that can affect physical, mental, and emotional well-being. It often results from various underlying health conditions, lifestyle factors, or chronic diseases and can significantly impact daily functioning and quality of life.
Feedback mechanism: A feedback mechanism is a biological process that helps maintain homeostasis by regulating physiological functions through feedback loops. In this context, feedback mechanisms involve sensors that detect changes in the body, and effectors that respond to those changes, either by amplifying or reducing the original stimulus. This system is crucial for the proper functioning of the adrenal glands, which produce hormones that are vital for various bodily functions.
Glucocorticoid therapy: Glucocorticoid therapy involves the use of synthetic glucocorticoids to manage various medical conditions, particularly those related to inflammation and immune response. These medications mimic the action of cortisol, a hormone produced by the adrenal glands, and are commonly used to treat disorders such as adrenal insufficiency, autoimmune diseases, and certain allergies. By modulating the body's inflammatory response, glucocorticoid therapy plays a significant role in the management of conditions resulting from adrenal gland dysfunction.
HPA Axis: The HPA axis, or hypothalamic-pituitary-adrenal axis, is a complex set of interactions among the hypothalamus, pituitary gland, and adrenal glands that regulates the body’s response to stress. This axis plays a crucial role in controlling the release of cortisol, the primary stress hormone, which helps manage various bodily functions during stressful situations. Dysregulation of the HPA axis can lead to disorders of the adrenal glands, affecting overall health and well-being.
Hyperglycemia: Hyperglycemia is a condition characterized by an abnormally high level of glucose in the blood, typically defined as having a blood glucose level above 130 mg/dL when fasting or above 180 mg/dL two hours after eating. This state often arises due to inadequate insulin production, insulin resistance, or increased glucose production by the liver, and it has significant implications for various endocrine disorders, particularly those involving the adrenal and pituitary glands.
Hyperpigmentation: Hyperpigmentation is a skin condition characterized by the darkening of certain areas of the skin due to an excess production of melanin. This increased melanin can be caused by various factors, including hormonal changes, sun exposure, or underlying health conditions. In the context of disorders related to adrenal glands, hyperpigmentation often arises from conditions such as Addison's disease, where there is an imbalance in hormone levels affecting skin pigmentation.
Hyperplasia: Hyperplasia is an increase in the number of cells in a tissue or organ, often in response to a stimulus or stressor. This cellular adaptation can occur as a normal physiological response, such as during growth or healing, or as a pathological condition, indicating an underlying disease process. Understanding hyperplasia is essential for recognizing how the body responds to stress, its implications in adrenal gland disorders, and its association with certain conditions affecting white blood cells and lymphoid tissues.
Hypertension: Hypertension, commonly known as high blood pressure, is a chronic medical condition characterized by consistently elevated blood pressure levels in the arteries. It can lead to serious health complications, including heart disease and stroke, affecting various body systems and organ functions.
Hypokalemia: Hypokalemia is a medical condition characterized by low levels of potassium in the bloodstream, typically defined as a serum potassium concentration of less than 3.5 mEq/L. This condition can lead to various complications, particularly affecting muscle function and cardiac health. Understanding hypokalemia is essential, as it is often linked to disorders involving adrenal gland function and can significantly influence fluid and electrolyte balance in the body.
Hypotension: Hypotension is a medical condition characterized by abnormally low blood pressure, which can lead to inadequate blood flow to organs and tissues. This can result in symptoms such as dizziness, fainting, and fatigue. Understanding hypotension is crucial as it relates to alterations in blood pressure and can also be influenced by disorders affecting the adrenal glands, which are essential for regulating blood pressure through hormone production.
Low-dose dexamethasone suppression test: The low-dose dexamethasone suppression test is a diagnostic tool used to assess the function of the adrenal glands and their production of cortisol. This test helps in determining whether a patient's body appropriately suppresses cortisol secretion in response to dexamethasone, a synthetic glucocorticoid, which is crucial for diagnosing conditions like Cushing's syndrome, where cortisol levels are abnormally high.
Metabolic alkalosis: Metabolic alkalosis is a condition characterized by an increase in blood pH due to an excess of bicarbonate or a loss of hydrogen ions. This imbalance can arise from various factors, including prolonged vomiting, diuretic use, or hormonal disorders affecting the adrenal glands. Understanding metabolic alkalosis helps in recognizing its implications for electrolyte balance and overall acid-base homeostasis in the body.
Mineralocorticoids: Mineralocorticoids are a class of steroid hormones produced by the adrenal cortex that regulate electrolyte and water balance in the body. The most prominent mineralocorticoid is aldosterone, which promotes sodium retention and potassium excretion in the kidneys, playing a crucial role in maintaining blood pressure and fluid balance.
Monitoring electrolyte levels: Monitoring electrolyte levels involves regularly checking the concentration of essential ions in the body, such as sodium, potassium, calcium, and chloride, to maintain proper physiological functions. This process is crucial for assessing and managing conditions related to the adrenal glands, as these glands play a significant role in regulating electrolyte balance through hormone secretion, particularly aldosterone.
Nausea: Nausea is an unpleasant sensation in the stomach that often leads to the urge to vomit. It can be triggered by various factors, including gastrointestinal disturbances, hormonal changes, and even stress. Understanding nausea is crucial as it often serves as a symptom indicating underlying issues, such as disorders of the upper gastrointestinal tract, adrenal gland disorders, or even cardiovascular problems like coronary artery disease.
Neuroblastoma: Neuroblastoma is a type of cancer that develops from immature nerve cells called neuroblasts, primarily affecting children. It usually arises in the adrenal glands, which are located on top of the kidneys, but can also occur in nerve tissue along the spine, chest, abdomen, or pelvis. This cancer is known for its aggressive nature and often presents with symptoms related to the affected areas, making early diagnosis challenging.
Norepinephrine: Norepinephrine is a neurotransmitter and hormone that plays a crucial role in the body's response to stress, often referred to as the 'fight or flight' response. It is produced in the adrenal medulla and released into the bloodstream during stressful situations, leading to increased heart rate, blood pressure, and energy availability. This connection is especially relevant in understanding disorders of the adrenal glands, where abnormal levels of norepinephrine can indicate or contribute to various pathophysiological conditions.
Osteoporosis: Osteoporosis is a medical condition characterized by weakened bones, increasing the risk of fractures due to low bone density and deterioration of bone tissue. This condition often develops silently over time, making bones fragile and susceptible to breaks even from minor falls or injuries. Factors like hormonal changes, particularly after menopause, nutritional deficiencies, and sedentary lifestyle contribute to its progression.
Paroxysmal Hypertension: Paroxysmal hypertension is a temporary and sudden increase in blood pressure that occurs in episodes. These episodes can lead to significant fluctuations in blood pressure levels, often resulting from excessive catecholamine release, commonly associated with conditions affecting the adrenal glands such as pheochromocytoma. Understanding this phenomenon is crucial as it can impact cardiovascular health and indicate underlying adrenal disorders.
Patient education on medication adherence: Patient education on medication adherence refers to the process of providing patients with the necessary knowledge and skills to understand their medications and ensure they take them as prescribed. This education is crucial for patients with adrenal gland disorders, as improper medication adherence can lead to serious health complications and ineffective treatment outcomes.
Pheochromocytoma: Pheochromocytoma is a rare tumor that develops in the adrenal glands, leading to excessive production of catecholamines like epinephrine and norepinephrine. This overproduction causes significant alterations in blood pressure and flow, manifesting as episodes of hypertension, palpitations, and sweating. Understanding this condition is essential as it directly relates to disorders of the adrenal glands and their impact on the cardiovascular system.
Primary aldosteronism: Primary aldosteronism is a disorder characterized by the overproduction of the hormone aldosterone from the adrenal glands, which leads to increased sodium retention, potassium excretion, and ultimately hypertension. This condition is significant because it directly impacts blood pressure regulation and can lead to cardiovascular complications if not managed properly.
Salt craving: Salt craving is a strong desire to consume salty foods, often linked to physiological needs or imbalances. This craving can be associated with conditions affecting the adrenal glands, particularly in cases of adrenal insufficiency or disorders like Addison's disease, where the body struggles to maintain appropriate electrolyte balance.
Surgery: Surgery is a medical procedure that involves the manipulation of bodily tissues to diagnose, treat, or prevent diseases and disorders. In the context of adrenal gland disorders, surgery can be crucial for conditions such as tumors or hyperplasia that affect hormone production. Surgical intervention can help restore normal function or alleviate symptoms by removing affected tissues or lesions.
Tumor formation: Tumor formation refers to the process by which abnormal cell growth occurs, leading to the development of a mass of tissue known as a tumor. This can happen due to genetic mutations, environmental factors, or hormonal imbalances that disrupt normal cell cycle regulation. In the context of disorders of the adrenal glands, tumor formation can significantly affect hormone production and lead to various health issues.
Vomiting: Vomiting is the involuntary, forceful expulsion of the contents of the stomach through the mouth. This action is often a reflex response triggered by various factors, including irritation of the gastrointestinal tract, infections, or central nervous system stimuli. It can have significant implications in various medical conditions, especially those related to metabolic or hormonal imbalances, such as disorders affecting the adrenal glands.
Weight Loss: Weight loss refers to the reduction of total body mass, which can occur due to a variety of factors, including decreased caloric intake, increased physical activity, or underlying health conditions. In the context of health, unintentional weight loss can signal serious issues such as malnutrition or disease and is often linked to disruptions in digestion, absorption, or hormonal regulation.