Adrenal Disorder
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Questions and Answers

What is a condition that may result from abrupt withdrawal of chronic exogenous steroids?

  • Cushing Syndrome
  • Tertiary Adrenal Insufficiency
  • Addison's Disease (correct)
  • Secondary Adrenal Insufficiency

    • Which hormone is primarily decreased in primary adrenal insufficiency?

  • Adrenocorticotropic hormone (ACTH)
  • Dehydroepiandrosterone (DHEA)
  • Cortisol (correct)
  • Aldosterone

    • What percentage of the adrenal cortex needs to be destroyed to cause symptoms of adrenal insufficiency?

  • 100%
  • 70%
  • 90% (correct)
  • 50%

    • Which of the following is a key feature of Addison's disease?

    <p>Life-threatening if untreated</p> Signup and view all the answers

    Which physiological condition primarily leads to secondary adrenal insufficiency?

    <p>Decreased ACTH due to pituitary dysfunction</p> Signup and view all the answers

    What is the most common cause of primary hyperaldosteronism?

    <p>Bilateral adrenal cortical hyperplasia</p> Signup and view all the answers

    Which condition is characterized by severe abdominal distention and is often associated with a firm, irregular mass that can cross the midline?

    <p>Neuroblastoma</p> Signup and view all the answers

    What is the hallmark clinical feature of hyperaldosteronism?

    <p>Hypokalemic hypertension</p> Signup and view all the answers

    In the context of adrenal tumors, which statement is true regarding incidentalomas?

    <p>Tumors under 3 cm are generally benign unless symptomatic.</p> Signup and view all the answers

    What age group is most commonly affected by unilateral aldosterone-producing adrenocortical adenoma?

    <p>Ages 30 and 50</p> Signup and view all the answers

    Which of the following is not a treatment option for neuroblastoma?

    <p>Medication for hypertension</p> Signup and view all the answers

    What is the most common cause of Addison's disease in developed countries?

    <p>Autoimmunity</p> Signup and view all the answers

    Which of the following laboratory findings is indicative of Addison's disease?

    <p>Hyponatremia</p> Signup and view all the answers

    What happens to renin levels in primary hyperaldosteronism?

    <p>They decrease due to volume expansion</p> Signup and view all the answers

    What type of adrenal insufficiency is indicated by an elevated serum ACTH level?

    <p>Addison's disease</p> Signup and view all the answers

    What is opsoclonus-myoclonus syndrome most commonly associated with?

    <p>Neuroblastoma</p> Signup and view all the answers

    Which diagnostic test confirms adrenal insufficiency by measuring serum cortisol response to synthetic ACTH?

    <p>Cosyntropin Stimulation Test</p> Signup and view all the answers

    What is the recommended treatment for a patient with a pituitary adenoma causing Cushing disease?

    <p>Transsphenoidal resection</p> Signup and view all the answers

    What does an AM serum cortisol level of less than 3 mcg/dl indicate in a patient?

    <p>Adrenal insufficiency</p> Signup and view all the answers

    Which lab result is likely to be found in a patient with Addison's disease?

    <p>Increased plasma renin activity</p> Signup and view all the answers

    Which of the following medications is used as part of medical therapy for Cushing's disease?

    <p>Mitotane</p> Signup and view all the answers

    In a patient with Addison's disease, what is a possible neuropsychological symptom?

    <p>Confusion</p> Signup and view all the answers

    What is the main risk associated with acute stress in a patient with Addison's disease?

    <p>Life-threatening vascular collapse</p> Signup and view all the answers

    Which type of adrenal insufficiency is characterized by a decrease in ACTH due to a pituitary issue?

    <p>Secondary adrenal insufficiency</p> Signup and view all the answers

    What is a common consequence of untreated Addison's disease?

    <p>Life-threatening adrenal crisis</p> Signup and view all the answers

    What percentage of females is typically affected by Addison's disease?

    <p>90%</p> Signup and view all the answers

    In the context of adrenal insufficiency, what laboratory finding is indicative when 90% of the adrenal cortex is destroyed?

    <p>Decreased DHEA levels</p> Signup and view all the answers

    What condition may result from rapid discontinuation of chronic steroid use?

    <p>Addison's disease</p> Signup and view all the answers

    Which adrenal tumor is characterized by being common but only mildly functional in a small percentage of cases?

    <p>Adrenocortical adenoma</p> Signup and view all the answers

    What is a common presentation of neuroblastoma?

    <p>Firm, irregular abdominal mass</p> Signup and view all the answers

    Which clinical symptom is most likely associated with primary hyperaldosteronism?

    <p>Hypokalemic hypertension</p> Signup and view all the answers

    For adrenal incidentalomas, what is the common approach for tumors under 3 cm?

    <p>Regular monitoring without treatment</p> Signup and view all the answers

    What is the primary mechanism behind the decreased renin production in primary hyperaldosteronism?

    <p>Increased blood volume from aldosterone</p> Signup and view all the answers

    Which demographic most commonly experiences bilateral adrenal cortical hyperplasia causing primary hyperaldosteronism?

    <p>Individuals aged 50-60</p> Signup and view all the answers

    Which of the following symptoms is least likely to occur in patients with primary hyperaldosteronism?

    <p>Weight gain</p> Signup and view all the answers

    Which treatment is commonly recommended for neuroblastoma with metastatic disease?

    <p>Surgery and radiation therapy</p> Signup and view all the answers

    Which laboratory finding is most characteristic of Addison's disease?

    <p>Hyperkalemia</p> Signup and view all the answers

    Which of the following tests is considered a confirmatory diagnostic test for Addison's disease?

    <p>Cosyntropin (ACTH) stimulation test</p> Signup and view all the answers

    In patients with Addison's disease, what is the typical effect of stress on cortisol levels?

    <p>Decreased significantly</p> Signup and view all the answers

    Which of the following conditions could lead to bilateral adrenal hemorrhage?

    <p>Sepsis</p> Signup and view all the answers

    Which of these is a primary adrenal hormone that would be expected to be suppressed in a patient with Addison's disease?

    <p>Aldosterone</p> Signup and view all the answers

    What is the primary route of action for medications like mitotane in Cushing's disease treatment?

    <p>Suppress adrenal enzyme function</p> Signup and view all the answers

    Which of the following is a significant risk factor for the development of adrenal suppression due to steroid therapy?

    <p>Prolonged use of glucocorticoids</p> Signup and view all the answers

    In Cushing's disease, what does an elevated level of both ACTH and DHEAS suggest?

    <p>Pituitary or ectopic source of hypercortisolism</p> Signup and view all the answers

    What is the most frequent cause of adrenal insufficiency in developed countries?

    <p>Autoimmune polyglandular syndromes</p> Signup and view all the answers

    Which physical symptom is associated with hyperpigmentation in Addison's disease?

    <p>Darkening of knuckles</p> Signup and view all the answers

    Which of the following best describes primary adrenal insufficiency?

    <p>Decreased levels of adrenocortical hormones after substantial adrenal cortex damage</p> Signup and view all the answers

    What is a common consequence of untreated Addison's disease?

    <p>Life-threatening adrenal crisis</p> Signup and view all the answers

    How does secondary adrenal insufficiency primarily differ from primary adrenal insufficiency?

    <p>It results from decreased ACTH levels from the pituitary gland</p> Signup and view all the answers

    Which laboratory finding is typically observed in primary adrenal insufficiency?

    <p>Elevated serum ACTH levels</p> Signup and view all the answers

    What is a characteristic demographic feature of Addison's disease?

    <p>Predominantly affects females</p> Signup and view all the answers

    What is the most common cause of primary hyperaldosteronism?

    <p>Bilateral adrenal cortical hyperplasia</p> Signup and view all the answers

    Which of the following symptoms is most characteristic of primary hyperaldosteronism?

    <p>Hypertension with hypokalemia</p> Signup and view all the answers

    What defines an adrenal incidentaloma?

    <p>Unexpected adrenal tumor noticed during imaging</p> Signup and view all the answers

    In neuroblastoma, which of the following features is most likely observed?

    <p>Ability to cross the midline on imaging</p> Signup and view all the answers

    Which of these treatments is commonly applied for metastatic neuroblastoma?

    <p>Chemotherapy, radiation therapy, and surgery</p> Signup and view all the answers

    What is a primary characteristic of adrenal cortical carcinoma?

    <p>It often requires surgical intervention</p> Signup and view all the answers

    Which clinical feature indicates a potential diagnosis of hyperaldosteronism?

    <p>Hypokalemic hypertension with muscle symptoms</p> Signup and view all the answers

    What is the typical demographic for unilateral aldosterone-producing adrenocortical adenoma?

    <p>Middle-aged individuals peaking between ages 30-50</p> Signup and view all the answers

    What is the primary cause of adrenal insufficiency in developed countries?

    <p>Addison's Disease</p> Signup and view all the answers

    Which diagnostic test is specifically used to confirm adrenal insufficiency?

    <p>Cosyntropin (ACTH) Stimulation Test</p> Signup and view all the answers

    Which symptom is indicative of Addison's Disease related to neuropsychological conditions?

    <p>Irritability</p> Signup and view all the answers

    What abnormal lab finding is associated with Addison's Disease?

    <p>Hypoglycemia</p> Signup and view all the answers

    In the context of Addison's Disease, what does a serum cortisol level less than 3 mcg/dl usually indicate?

    <p>Primary adrenal insufficiency</p> Signup and view all the answers

    What role does cosyntropin play in diagnosing adrenal insufficiency?

    <p>It promotes cortisol release from the adrenal gland</p> Signup and view all the answers

    What is a major risk factor for the persistence of adrenal suppression following steroid therapy?

    <p>Duration of steroid therapy</p> Signup and view all the answers

    Which condition may contribute to atrophy of the adrenal cortex?

    <p>Autoimmune polyglandular syndromes</p> Signup and view all the answers

    Which treatment is commonly employed for patients with a pituitary adenoma causing Cushing's Disease?

    <p>Transsphenoidal resection</p> Signup and view all the answers

    What is a common long-term management strategy following surgical treatment of Cushing's disease?

    <p>Replacement therapy with glucocorticoids and mineralocorticoids</p> Signup and view all the answers

    What is the primary difference between primary and secondary adrenal insufficiency?

    <p>Primary adrenal insufficiency results from an adrenal gland defect, while secondary results from a pituitary deficiency.</p> Signup and view all the answers

    In cases of Addison's disease, which hormonal levels would primarily be expected to decrease?

    <p>Cortisol and aldosterone levels will both decrease.</p> Signup and view all the answers

    What is the typical clinical consequence of untreated primary adrenal insufficiency?

    <p>Acute adrenal crisis can occur, which is life-threatening.</p> Signup and view all the answers

    Which demographic is primarily affected by Addison’s disease?

    <p>Women, with at least 90% prevalence.</p> Signup and view all the answers

    What role does the hypothalamus play in tertiary adrenal insufficiency?

    <p>It is responsible for the secretion of CRH, which affects ACTH levels.</p> Signup and view all the answers

    What is the primary cause of primary hyperaldosteronism in most cases?

    <p>Bilateral adrenal cortical hyperplasia</p> Signup and view all the answers

    Which of the following clinical features is NOT typically associated with hyperaldosteronism?

    <p>Severe abdominal distention</p> Signup and view all the answers

    Which treatment modality is most commonly used for metastatic neuroblastoma?

    <p>Chemotherapy</p> Signup and view all the answers

    What is a common outcome of excess aldosterone in primary hyperaldosteronism?

    <p>Hypokalemia</p> Signup and view all the answers

    Which characteristic differentiates adrenocortical carcinoma from adrenocortical adenoma?

    <p>Increased aggressiveness and potential for large size</p> Signup and view all the answers

    What is the primary clinical manifestation of neuroblastoma related to its production of vasoactive intestinal peptide?

    <p>Severe watery diarrhea</p> Signup and view all the answers

    Which age group is most likely to experience the peak incidence of unilateral aldosterone-producing adrenocortical adenoma?

    <p>Ages 30 to 50</p> Signup and view all the answers

    What is the expected change in renin production due to increased aldosterone in primary hyperaldosteronism?

    <p>Decreased renin production</p> Signup and view all the answers

    What is a key aspect of the diagnostic testing for Addison's Disease involving cosyntropin?

    <p>It measures serum cortisol response after administering synthetic ACTH.</p> Signup and view all the answers

    Which of the following is a potential cause of bilateral adrenal hemorrhage leading to adrenal insufficiency?

    <p>Antiphospholipid syndrome.</p> Signup and view all the answers

    What is the effect of an elevated serum ACTH in a patient suspected of having adrenal insufficiency?

    <p>Suggests Addison's disease.</p> Signup and view all the answers

    Which of the following statements about the symptoms of Addison's disease is true?

    <p>Hyperpigmentation is common around scars and pressure areas.</p> Signup and view all the answers

    In the context of treating Cushing's disease, when surgical therapy is initiated for a pituitary adenoma, what is the goal?

    <p>To perform a transsphenoidal resection.</p> Signup and view all the answers

    What is an important consideration regarding the management of secondary adrenal insufficiency?

    <p>Long-term steroid use can lead to adrenal suppression.</p> Signup and view all the answers

    What do high doses of dexamethasone indicate in the context of Cushing's Disease diagnostic testing?

    <p>Pituitary etiology in Cushing's disease.</p> Signup and view all the answers

    Which laboratory finding typically accompanies Addison's disease due to its impact on sodium reabsorption?

    <p>Hyponatremia.</p> Signup and view all the answers

    What distinguishes Cushing syndrome treatment when addressing adrenal tumors?

    <p>Acknowledgment of benign and malignant tumor variations.</p> Signup and view all the answers

    Which consequence might be expected when adrenal suppression follows steroid therapy for an extended period?

    <p>Development of Addison’s disease.</p> Signup and view all the answers

    What is the main characteristic of primary adrenal insufficiency in relation to adrenal hormones?

    <p>Decreased levels of cortisol, aldosterone, and DHEA</p> Signup and view all the answers

    Which of the following statements about the incidence of Addison's disease is correct?

    <p>It occurs in approximately 5 cases per million in the U.S.</p> Signup and view all the answers

    What is the crucial physiological factor necessary for the onset of adrenal insufficiency?

    <p>Destruction of 90% of adrenal cortex</p> Signup and view all the answers

    Which condition is most commonly associated with adrenal crisis?

    <p>Untreated Addison's disease</p> Signup and view all the answers

    What should be expected in terms of aldosterone synthesis in primary adrenal insufficiency?

    <p>Aldosterone synthesis is unaffected due to intact adrenal glands.</p> Signup and view all the answers

    What lab finding indicates Addison's disease when obtained after an ACTH stimulation test?

    <p>Serum cortisol &lt; 20 mcg/dL</p> Signup and view all the answers

    Which medication is associated with adrenal suppression following steroid therapy for an extended period?

    <p>Dexamethasone</p> Signup and view all the answers

    What type of adrenal insufficiency is characterized by elevated ACTH levels?

    <p>Addison's disease</p> Signup and view all the answers

    In patients with Addison's disease, which symptom is most likely to exacerbate during periods of stress?

    <p>Neurological confusion</p> Signup and view all the answers

    Which diagnostic test is used to measure the response to metyrapone stimulation?

    <p>Overnight metyrapone test</p> Signup and view all the answers

    What is the immediate consequence of adrenal hemorrhage, particularly in the context of sepsis?

    <p>Acute adrenal crisis</p> Signup and view all the answers

    Which therapy is considered the definitive treatment for refractory Cushing's disease?

    <p>Bilateral adrenalectomy</p> Signup and view all the answers

    In the diagnosis of adrenal insufficiency, what does an elevated serum ACTH level indicate?

    <p>Primary adrenal insufficiency</p> Signup and view all the answers

    What clinical symptom can be observed in Addison's disease due to a decrease in sodium reabsorption?

    <p>Hypovolemia</p> Signup and view all the answers

    Which test is primarily used for distinguishing between primary and secondary adrenal insufficiency?

    <p>Plasma renin activity test</p> Signup and view all the answers

    What is the primary cause of unilateral aldosterone-producing adrenocortical adenoma?

    <p>Adrenal cortical hyperplasia</p> Signup and view all the answers

    Which clinical feature is least likely associated with neuroblastoma?

    <p>Hypertension</p> Signup and view all the answers

    What is the most common unexpected finding on a CT scan related to adrenal conditions?

    <p>Incidentaloma</p> Signup and view all the answers

    What electrolyte disturbance is a hallmark of primary hyperaldosteronism?

    <p>Hypokalemia</p> Signup and view all the answers

    What surgical approach is commonly utilized for the treatment of adrenocortical carcinoma?

    <p>Laparoscopic adrenal surgery</p> Signup and view all the answers

    How does primary hyperaldosteronism affect renin production in the body?

    <p>Reduces renin production</p> Signup and view all the answers

    In which demographic is bilateral adrenal cortical hyperplasia most prevalent?

    <p>Males aged 50-60</p> Signup and view all the answers

    What is a common consequence of the excessive aldosterone production in primary hyperaldosteronism?

    <p>Hypertensive crisis</p> Signup and view all the answers

    What is a significant characteristic of adrenal crisis in untreated Addison's disease?

    <p>Severe drop in blood pressure</p> Signup and view all the answers

    Which factor is primarily responsible for the development of secondary adrenal insufficiency?

    <p>Lower levels of ACTH due to pituitary dysfunction</p> Signup and view all the answers

    What is the impact of abrupt withdrawal from chronic steroid therapy on adrenal insufficiency?

    <p>It can precipitate adrenal crisis</p> Signup and view all the answers

    In which population is Addison's disease most prevalent?

    <p>Females aged 20-30</p> Signup and view all the answers

    What percentage of adrenal cortex destruction is required to manifest symptoms of adrenal insufficiency?

    <p>90%</p> Signup and view all the answers

    What is the primary reason for adrenal cortex atrophy in developed countries?

    <p>Autoimmune disorders</p> Signup and view all the answers

    Which laboratory finding would most likely indicate adrenal insufficiency in Addison's disease?

    <p>Hyponatremia</p> Signup and view all the answers

    In the management of Cushing's disease, what role does pituitary irradiation serve?

    <p>Reserves fertility concerns</p> Signup and view all the answers

    When performing an ACTH stimulation test, which serum cortisol level indicates adrenal insufficiency?

    <p>&lt; 20 mcg/dL</p> Signup and view all the answers

    Which of the following conditions is most commonly associated with bilateral adrenal hemorrhage?

    <p>Anticoagulation therapy</p> Signup and view all the answers

    What is a key diagnostic test that differentiates between Addison’s disease and secondary adrenal insufficiency?

    <p>Cosyntropin stimulation test</p> Signup and view all the answers

    In Addison's disease, which symptom is least likely to be observed?

    <p>Weight gain</p> Signup and view all the answers

    What is the most common cause of primary hyperaldosteronism?

    <p>Adrenal adenoma</p> Signup and view all the answers

    Which adrenal function test measures the serum cortisol response to metyrapone stimulation?

    <p>Overnight metyrapone test</p> Signup and view all the answers

    What is the most common cause of primary hyperaldosteronism?

    <p>Bilateral adrenal cortical hyperplasia</p> Signup and view all the answers

    What is the survival rate for patients undergoing surgical treatment for pituitary adenoma?

    <p>90%</p> Signup and view all the answers

    Which symptom is least likely to be associated with primary hyperaldosteronism?

    <p>Hypercalcemia</p> Signup and view all the answers

    What is the typical age peak for unilateral aldosterone-producing adrenocortical adenoma?

    <p>30 to 50 years</p> Signup and view all the answers

    Which of the following is a common treatment option for neuroblastoma?

    <p>Surgery and radiation therapy</p> Signup and view all the answers

    Which of the following statements is true regarding adrenal incidentalomas?

    <p>They are commonly detected during imaging for unrelated reasons.</p> Signup and view all the answers

    Which laboratory finding is most characteristic of primary hyperaldosteronism?

    <p>Reduced renin activity</p> Signup and view all the answers

    What is the hallmark clinical feature of hyperaldosteronism?

    <p>Hypokalemic hypertension</p> Signup and view all the answers

    Which symptom is most closely associated with neuroblastoma?

    <p>Severe abdominal distention</p> Signup and view all the answers

    Study Notes

    Adrenal Gland Disorders

    • Addison Disease and Cushing Syndrome are contrasting conditions affecting the adrenal glands.
    • Causes of Adrenal Diseases include:
      • Autoimmunity (90% of Addison's Disease in developed countries)
      • Bilateral Adrenal Hemorrhage (sepsis, HIT, anticoagulation)
      • Genetic Conditions
      • Medications (mitotane, abiraterone acetate)
      • Tuberculosis (global leading cause)
    • Signs and Symptoms of Adrenal Diseases
      • Addison Disease:
        • Hyperpigmentation: knuckles, elbows, nipple area, pressure areas, skin folds, scars
        • Neuro/Psych: anxiety, irritability, depression, confusion.
        • Endo: Hypoglycemia
        • Hypovolemia: Decreased cardiac output and increased renal blood flow with azotemia.
        • Intolerance to Stress: Unable to increase cortisol levels during stress, leading to life-threatening vascular collapse.
      • Cushing Syndrome:
        • Hypertension
        • Weight gain
        • Muscle weakness
        • Purple striae
        • Moon face
        • Buffalo hump
        • Hirsutism
        • Acne
        • Menstrual irregularities
        • Osteoporosis
    • Abnormal Laboratory and Diagnostic Findings for Adrenal Diseases:
      • Addison Disease:
        • Basic Metabolic Panel: Hyponatremia, Hypoglycemia, Hyperkalemia.
        • CBC: Moderate neutropenia, lymphocytosis, & eosinophilia.
        • AM Serum Cortisol (test of exclusion): < 3 mcg/dl = Adrenal insufficiency (Addison Disease).
        • ACTH Stimulation Test (confirmatory): Normal → serum cortisol ≥ 20 mcg/dL, Adrenal insufficiency → Serum cortisol < 20 mcg/dL.
        • Serum ACTH: ↑ = Addison Disease, ↓ = Secondary adrenal insufficiency.
      • Cushing Syndrome:
        • Plasma ACTH & DHEAS: High ACTH & high DHEAS = Pituitary or ectopic sources, Low ACTH & low DHEAS = Probable adrenal tumor.
        • High-dose dexamethasone suppression test: Suppression of cortisol and ACTH secretion = Pituitary etiology.
        • Imaging:
          • MRI head → Pituitary lesion (more common).
          • CT abdomen → Adrenal lesion (less common).
          • Whole body imaging (68Ga-somatostatin receptor-PET/CT) → For ectopic causes.
    • Treatment Options for Adrenal Diseases:
      • Addison Disease:
        • Lifelong hormone replacement therapy: Glucocorticoids & Mineralocorticoids.
      • Cushing Disease:
        • Surgical Therapy:
          • Pituitary adenoma: Transsphenoidal resection.
          • Ectopic ACTH-secreting tumors: Resection of tumor & metastases +/- medical therapy of adrenal enzyme inhibitors.
        • Medical Therapy:
          • Adrenal enzyme inhibitors (Mitotane, ketoconazole, metyrapone).
          • Pituitary irradiation (for patients with fertility concerns).
          • Nonresectable tumor → Adrenal enzyme inhibitors.
          • Bilateral adrenalectomy PLUS lifelong glucocorticoid & mineralocorticoid replacement therapy.
      • Cushing Syndrome:
        • Surgical therapy:
          • Benign adrenal adenomas: Laparoscopic resection (if < 6 cm)
          • Adrenocortical carcinomas: Surgical resection & post-operative mitotane for 2-5 years.
        • Medical Therapy:
          • Mineralocorticoid hypertension: Spironolactone, eplerenone, dihydropyridine calcium channel blockers.
          • Hyperandrogenism: Flutamide.
          • Cabergoline.
    • Adrenal Tumors:
      • Adrenocortical adenoma:
        • Most common adrenal tumor.
        • Only 15% are "functional" (hormone producing) and usually treated surgically.
      • Adrenocortical carcinoma:
        • Rare, highly aggressive cancer.
        • Can grow large and be "functional."
        • Treated surgically.
      • Incidentaloma:
        • Common unexpected finding on CT scan or MRI.
        • Tumors under 3 cm are usually benign and only treated if Cushing's syndrome or pheochromocytoma are present.
      • Neuroblastoma:
        • Aggressive cancer of immature neuroblastic cells.
        • One of the most common pediatric cancers.
        • Elevated catecholamine hormone metabolites.
        • Presentation: Abdominal distention, firm irregular mass, severe watery diarrhea, opsoclonus-myoclonus syndrome.
        • Treatment: Surgery, radiation therapy, chemotherapy.
    • Hyperaldosteronism:
      • Excessive production of aldosterone.
      • Causes:
        • Bilateral adrenal cortical hyperplasia (75%): M:F → 4:1, peaking between ages 50 and 60.
        • Unilateral aldosterone-producing adrenocortical adenoma (Conn Syndrome, 25%): M:F → 1:2, peaking between ages 30 & 50.
      • Clinical Features:
        • Hypokalemic hypertension: Sodium retention → Blood pressure elevation, Potassium loss → Hypokalemia → muscle weakness, paresthesias, tetany, headache, polyuria, metabolic alkalosis.
      • Renin levels: Renin production is decreased in primary hyperaldosteronism due to volume expansion caused by increased aldosterone production.

    Adrenal Gland Disorders

    • Addison Disease is a condition of adrenal insufficiency caused by the destruction of the adrenal gland.
    • Addison Disease typically affects women, occurring around 5 cases per million in the US.
    • Addison Disease is most often caused by autoimmune processes.
    • Addison Disease can be triggered by medications, tuberculosis, or adrenal hemorrhage.
    • Addison Disease can lead to hyperpigmentation of the skin.
    • Addison Disease can result in hypoglycemia and hypovolemia.
    • Addison Disease leads to intolerance to stress, meaning an inability to increase cortisol levels in times of stress.
    • Addison Disease may result in life-threatening vascular collapse.
    • Addison Disease can be diagnosed by low serum cortisol and high serum ACTH levels.
    • Addison Disease is treated with glucocorticoids, mineralocorticoids, and hormone replacement therapies.
    • Cushing Syndrome is a condition of hypercortisolism, defined as excess cortisol production from the adrenal glands.
    • Cushing Syndrome is a constellation of symptoms that include hyperglycemia, hyperpigmentation, and weight gain.
    • Cushing Syndrome can be caused by pituitary, adrenal, or ectopic tumors.
    • Cushing Syndrome can be treated with surgical removal of a tumor, radiation therapy, or medications.
    • Hyperaldosteronism is a condition of excess aldosterone production from the adrenal glands.
    • Hyperaldosteronism is characterized by hypokalemic hypertension and metabolic alkalosis.

    Adrenal Gland Anatomy

    • Adrenal glands are located on top of the kidneys.
    • The adrenal gland is made up of the outer cortex and the inner medulla.
    • The adrenal cortex produces cortisol, aldosterone, and DHEA.
    • The adrenal medulla produces catecholamines.

    Aldosterone

    • Aldosterone is responsible for regulating blood pressure and fluid balance.
    • Aldosterone acts on the kidneys to promote sodium reabsorption and potassium excretion.

    Cortisol

    • Cortisol is a steroid hormone that regulates the body's stress response.
    • Cortisol helps to regulate blood sugar, immune function, and metabolism.

    Adrenal Insufficiently

    • The adrenal glands are unable to produce sufficient amounts of hormones.
    • Primary Adrenal Insufficiency (Addison Disease) is caused by adrenal gland dysfunction.
    • Secondary Adrenal Insufficiency is caused by the pituitary gland's inability to produce ACTH.
    • Tertiary Adrenal Insufficiency is caused by dysfunction of the hypothalamus.
    • Tertiary Adrenal Insufficiency is caused by chronic exogenous steroid use or abrupt steroid withdrawal.

    Adrenal Tumors

    • Adrenocortical adenomas are benign tumors of the adrenal cortex that are often asymptomatic.
    • Adrenocortical carcinomas are malignant tumors of the adrenal cortex that can be life-threatening.
    • Incidentalomas are tumors of the adrenal glands that are discovered incidentally during imaging studies.

    Neuroblastoma

    • Neuroblastoma is a pediatric cancer that arises from immature neuroblastic cells.
    • Neuroblastoma often presents with abdominal distention and a firm, irregular mass.
    • Neuroblastoma is often treated with surgery, radiation therapy, and chemotherapy.

    Hyperaldosteronism

    • Primary Hyperaldosteronism is caused by excess aldosterone production from the adrenal glands.
    • Primary Hyperaldosteronism is often caused by bilateral adrenal hyperplasia or an aldosterone-producing adenoma (Conn syndrome).
    • Primary Hyperaldosteronism is characterized by hypokalemia, hypertension, and metabolic alkalosis.
    • Primary Hyperaldosteronism causes decreased renin activity.

    Treatment Options

    • Primary Hyperaldosteronism is treated with adrenalectomy, aldosterone antagonists, or spironolactone.

    Adrenal Gland Disorders

    • Addison disease is primary adrenal insufficiency, caused by destruction of adrenal cortex.
    • Causes of primary adrenal insufficiency:
      • Autoimmunity: Most common cause (90% of cases).
        • Autoimmune polyglandular syndromes can affect other organs as well.
      • Bilateral adrenal hemorrhage can be caused by sepsis, heparin-induced thrombocytopenia, anticoagulation, or antiphospholipid syndrome.
      • Genetic conditions can also lead to adrenal insufficiency.
      • Medication side effects: Mitotane and abiraterone acetate can cause adrenal insufficiency.
      • Steroid therapy: Abrupt steroid withdrawal can lead to adrenal insufficiency.
      • Tuberculosis: Globally, tuberculosis is the most common cause of adrenal insufficiency.
    • Secondary adrenal insufficiency is caused by decreased ACTH production by the pituitary gland.
    • Tertiary adrenal insufficiency is caused by decreased CRH production by the hypothalamus.
    • Signs and symptoms of Addison disease:
      • Gradual onset, most often.
      • Neuro/psych: Anxiety, irritability, depression, confusion.
      • Hyperpigmentation: Knuckles, elbows, nipple area, pressure areas, skin folds, scars.
      • Endo: Hypoglycemia.
      • Hypovolemia: Due to decreased cardiac output and increased renal blood flow with azotemia.
      • Intolerance to stress: Patients can't increase cortisol levels during stressful situations, leading to life-threatening vascular collapse.
    • Diagnosing Addison disease:
      • Basic metabolic panel: Hyponatremia, hypoglycemia, hyperkalemia.
      • CBC: May show moderate neutropenia, lymphocytosis, and eosinophilia.
      • AM serum cortisol: Level below 3 mcg/dl suggests adrenal insufficiency.
      • ACTH stimulation test: Confirms adrenal insufficiency, with cortisol levels below 20 mcg/dL after ACTH administration.
      • Serum ACTH: Elevated ACTH indicates Addison disease, low ACTH suggests secondary adrenal insufficiency.
    • Other tests for Addison disease:
      • Overnight metyrapone test: Measures cortisol response to metyrapone stimulation.
      • Plasma renin activity: Helps distinguish Addison disease from secondary adrenal insufficiency.
      • DHEA and DHEA-S: Suppressed levels confirm Addison disease.
    • Cushing syndrome caused by excess cortisol production, due to adrenal tumor or pituitary gland malfunction.
    • Causes of Cushing syndrome:
      • Pituitary adenoma (ACTH-secreting): The most common cause.
      • Ectopic ACTH-secreting tumors: Can occur outside the pituitary gland.
      • Adrenal tumor: Less common cause.
    • Cushing disease is a specific type of Cushing syndrome caused by a pituitary adenoma (ACTH-secreting).
    • Diagnosing Cushing syndrome:
      • Plasma ACTH: High ACTH indicates pituitary or ectopic sources, low ACTH suggests adrenal tumor.
      • DHEAS: High DHEAS indicates pituitary or ectopic sources, low DHEAS suggests adrenal tumor.
      • High-dose dexamethasone suppression test: Suppression of cortisol and ACTH secretion suggests pituitary origin.
      • Imaging: MRI head to identify pituitary lesion, CT abdomen for adrenal lesion, whole-body imaging (68Ga-somatostatin receptor-PET/CT) for ectopic causes.
    • Treating Cushing disease:
      • Surgical therapy: Transsphenoidal resection of pituitary adenoma, resection of ectopic ACTH-secreting tumors, and adrenal enzyme inhibitors.
      • Medical therapy: Adrenal enzyme inhibitors (mitotane, ketoconazole, metyrapone), pituitary irradiation, and adrenal enzyme inhibitors for nonresectable tumors.
      • Bilateral adrenalectomy: For refractory cases, involves lifelong glucocorticoid and mineralocorticoid replacement therapy.
    • Treating Cushing syndrome:
      • Surgical therapy: Resection of adrenal adenomas, surgical resection and mitotane treatment for adrenocortical carcinomas.
      • Medical therapy: Spironolactone, eplerenone, dihydropyridine calcium channel blockers for mineralocorticoid hypertension, flutamide for hyperandrogenism.
    • Transsphenoidal surgery: A surgical technique to access tumors in the pituitary gland.
    • Adrenal tumors:
      • Adrenocortical adenoma: Most common, often treated surgically.
      • Adrenocortical carcinoma: Rare, highly aggressive cancer, treated with surgery.
      • Incidentaloma: Common, benign tumors under 3cm, only treated with evidence of Cushing's syndrome or pheochromocytoma.
      • Neuroblastoma: Aggressive cancer of immature neuroblastic cells, may produce elevated levels of catecholamine hormone metabolites.
      • Pheochromocytoma: Tumor of adrenal medulla, secretes catecholamines.
    • Neuroblastoma presentation:
      • Abdominal distention and firm, irregular mass.
      • Severe watery diarrhea due to vasoactive intestinal peptide production.
      • Less likely to cause hypertension than pheochromocytoma.
      • Opsoclonus-myoclonus syndrome.
      • Treatment: Surgery, radiation therapy, and chemotherapy for metastatic disease.
    • Hyperaldosteronism: Excessive aldosterone production.
      • Causes: Bilateral adrenal cortical hyperplasia (most common) and unilateral aldosterone-producing adrenocortical adenoma (Conn syndrome).
      • Aldosterone actions: Promote sodium and water retention, potassium excretion.
    • Hyperaldosteronism clinical features:
      • Hypokalemic hypertension: Hallmark of hyperaldosteronism.
      • Sodium retention: Leads to blood pressure elevation.
      • Potassium loss: Leads to hypokalemia with muscle weakness, paresthesias, tetany, headache, and polyuria.
      • Metabolic alkalosis: Result of renal potassium and hydrogen ion depletion.
      • Renin activity: Decreased in primary hyperaldosteronism due to volume expansion.

    Adrenal Gland Disorders

    • Primary Adrenal Insufficiency (Addison’s Disease)
      • Adrenal gland abnormality = ↓cortisol, ↓aldosterone, ↓dehydroepiandrosterone (DHEA)
      • 90% of adrenal cortex must be destroyed to cause adrenal insufficiency
      • Acute Addison disease = Adrenal Crisis
      • Addison disease untreated = life-threatening
      • 90% female
      • Incidence of 5 cases per million in the U.S.
      • Etiology
        • In developed countries: 90% → Autoimmunity = Most common cause
          • Autoimmune polyglandular syndromes
          • Leading to atrophy of the adrenal cortex
        • The remaining 10%:
          • Bilateral adrenal hemorrhage: Sepsis, HIT, anticoagulation, Antiphospholipid syndrome
          • Genetic conditions
          • Medications – mitotane, abiraterone acetate (chemotherapy drugs)
          • Adrenal suppression following steroid therapy can persist up to a year (more so in SECONDARY)
          • Tuberculosis - #1 in the world-may involve the adrenals & destroy them

    Addison’s Disease: Signs & Symptoms

    • Usually gradual
    • Neuro/Psych: Anxiety, irritability, depression, confusion
    • Endo: Hypoglycemic
    • Hypovolemia: Due to decreased cardiac output & increased renal blood flow with azotemia
    • Intolerance to stress: In times of stress they cannot increase cortisol levels→ can result in life-threatening vascular collapse
    • Hyperpigmentation:
      • Knuckles, elbows, nipple area, around pressure areas, skin folds, scars

    Addison’s Labs & Diagnostics

    • Basic Metabolic Panel
      • Hyponatremia: Due to reduced aldosterone-mediated reabsorption of sodium in the distal renal tubules
      • Hypoglycemia
      • Hyperkalemia
    • CBC
      • +/- moderate neutropenia, lymphocytosis, & eosinophilia
    • AM Serum Cortisol (test of exclusion)
      • < 3 mcg/dl = Adrenal insufficiency (Addison Disease)
        • Diagnostic if accompanied by elevation of plasma ACTH
      • 3-18 mcg/dl → perform ACTH Stimulation Test

      • 18 mcg/dl = Excludes Addison Disease

    Addison’s Labs & Diagnostics (cont.)

    • Cosyntropin (ACTH) Stimulation Test – confirmatory test
      • 1.) Synthetic ACTH (cosyntropin) is given IM/IV
      • 2.) Serum cortisol is obtained 45 minutes after
        • Normal: serum cortisol ≥ 20 mcg/dL
        • Adrenal insufficiency: Serum cortisol < 20 mcg/d
    • Serum ACTH
      • Perform to determine type of adrenal insufficiency
      • If ↑ = Addison disease
      • If ↓ = Secondary adrenal insufficiency

    Additional Addison's Labs

    • Overnight Metyrapone Test
      • Measure cortisol response to metyrapone stimulation
    • Plasma Renin Activity
      • May distinguish Addison Disease & secondary adrenal insufficiency
    • DHEA & DHEA-S
      • Suppressed ( ↓)

    Cushing Syndrome

    • Definition
      • Excess cortisol
    • Causes
      • Causes of hypercortisolism (Cushing’s Syndrome): - Pituitary adenoma (Cushing’s disease) - Ectopic ACTH-secreting tumors (rare) - Adrenal adenoma (benign) - Adrenocortical carcinoma (malignant) - Others: Iatrogenic Cushing Syndrome, secondary Cushing Syndrome (exogenous cortisol administration)

    Cushing Disease Diagnostic Testing

    • To determine source of hypercortisolism
      • Plasma ACTH & DHEAS
        • High ACTH & high DHEAS = Pituitary or ectopic sources
        • Low ACTH & low DHEAS = Probable adrenal tumor
      • High-dose dexamethasone suppression test
        • Suppression of cortisol and ACTH secretion = Pituitary etiology
      • Imaging
        • MRI head → Pituitary lesion (more common)
        • CT abdomen → Adrenal lesion (less common)
        • Whole body imaging (68Ga-somatostatin receptor-PET/CT) → For ectopic causes

    Cushing Disease Treatment

    • Surgical Therapy
      • Pituitary Adenoma (ACTH-secreting) - transsphenoidal resection
      • Ectopic ACTH-secreting tumors - resection of tumor & metastases +/- medical therapy of Adrenal enzyme inhibitors (Mitotane, ketoconazole, metyrapone)
    • Medical therapy: Often required when surgery is delayed, contraindicated, unsuccessful or the tumor cannot be located or resected
      • Adrenal enzyme inhibitors are the most commonly used drugs
      • Pituitary irradiation — For patients in whom fertility is an important concern
      • Nonresectable tumor → Adrenal enzyme inhibitors
    • If treatment continues to be refractory, the final definitive cure:
      • Bilateral Adrenalectomy PLUS
      • Lifelong daily glucocorticoid & mineralocorticoid replacement therapy
      • May be preferred by some patients instead of radiation therapy

    Cushing Syndrome Treatment

    • Surgical Therapy
      • Benign adrenal adenomas
        • Are usually resected laparoscopically if smaller than 6 cm
      • Adrenocortical carcinomas
        • Usually resected surgically and treated with mitotane postoperatively for 2-5 years
      • Medical Therapy
        • Mineralocorticoid Hypertension: Spironolactone, eplerenone, dihydropyridine calcium channel blockers
        • Hyperandrogenism: Flutamide
        • Other agents: Cabergoline

    Patient Education: Cushing Syndrome

    • Surgical treatment has an approximate 90% 10-year survival
    • Complete remission after surgery has about a 15-20% chance of recurrence over the next 10 years
    • Patients should be referred to Endocrinology with an abnormal dexamethasone suppression test

    Adrenal Tumors

    • Tumors of the Adrenal Cortex
      • Adrenocortical adenoma (MC)
      • Adrenocortical carcinoma (v.rare, highly aggressive)
    • Tumors of the Adrenal Medulla
      • Neuroblastoma
      • Pheochromocytoma
      • Incidentalomas

    Adrenal Tumors (cont.)

    • Adrenocortical adenoma
      • Common, 1-10% of persons at autopsy
      • Only 15% are “functional” (hormone producing)
        • These are often treated surgically
    • Adrenocortical carcinoma
      • Rare, highly aggressive cancer
      • These may grow quite large and may be “functional”
      • Often treated with surgery

    Adrenal Tumors (cont.)

    • Incidentaloma
      • One of the more common unexpected findings on a CT scan or MRI
      • Tumors under 3 cm are generally considered benign & are only treated if there is evidence of Cushing’s syndrome or pheochromocytoma

    Adrenal Tumors (cont.)

    • Neuroblastoma
      • Aggressive cancer of immature neuroblastic cells
      • One of the most common pediatric cancers
      • Often produce elevated levels of catecholamine hormone metabolites

    Neuroblastoma Presentation

    • Most common: Abdominal distention and a firm, irregular mass that can cross the midline
      • Severe watery diarrhea through production of vasoactive intestinal peptide
      • Less likely to develop hypertension than with pheochromocytoma (Neuroblastoma is normotensive)
      • Opsoclonus-myoclonus syndrome ("dancing eyes-dancing feet")
    • Treatment: Surgery & radiation therapy (& chemotherapy for metastatic disease)
      • Laproscopic Adrenal surgery

    Hyperaldosteronism

    • Definition = Excessive Production of aldosterone
      • MC cause: Bilateral adrenal cortical hyperplasia (75%)
        • M:F→ 4:1
        • Peaking between ages 50 and 60
      • Unilateral aldosterone-producing adrenocortical adenoma (aka Conn syndrome, 25%)
        • M:F→ 1:2
        • Peaking between ages 30 & 50

    Renin & Primary Hyperaldosteronism

    • In primary hyperaldosteronism, the increased aldosterone production is caused by an adrenal abnormality
    • The volume expansion that results decreases renin production
      • Primary Hyperaldosteronism causes increased aldosterone production & reduced renin activity

    Hyperaldosteronism Clinical Features

    • Hallmark = Hypokalemic hypertension
      • Sodium retention → Blood pressure elevation
      • Potassium loss → Hypokalemia → Symptoms include muscle weakness, paresthesias, & tetany, headache, polyuria
      • Metabolic Alkalosis: Result of renal potassium & hydrogen ion depletion

    Adrenal Gland Disorders

    • Adrenal insufficiency involves decreased function of the adrenal cortex, leading to reduced production of cortisol, aldosterone, and DHEA.
    • Primary Adrenal Insufficiency (Addison's Disease) is characterized by adrenal gland abnormality resulting in decreased cortisol, aldosterone, and dehydroepiandrosterone (DHEA).
      • 90% of the adrenal cortex must be destroyed for adrenal insufficiency to occur.
      • 90% of cases in developed countries are caused by autoimmunity.
      • Other causes include:
        • Bilateral adrenal hemorrhage due to sepsis, heparin-induced thrombocytopenia (HIT), anticoagulation, antiphospholipid syndrome, and genetic conditions.
        • Medications such as mitotane and abiraterone acetate.
        • Adrenal suppression following steroid therapy.
        • Tuberculosis, which is the leading cause globally.
    • Secondary Adrenal Insufficiency occurs when the pituitary gland fails to produce enough ACTH, leading to decreased cortisol production by the adrenal glands.
    • Tertiary Adrenal Insufficiency occurs when the hypothalamus fails to produce enough CRH, leading to decreased ACTH production by the pituitary and, subsequently, decreased cortisol production by the adrenal glands.
    • Addison's Disease can be acute (Adrenal Crisis) or chronic.
    • Adrenal Crisis is a life-threatening condition that occurs in untreated Addison's Disease.
    • Addison's Disease is more common in women (90% incidence).
    • Hyperpigmentation is a hallmark sign of Addison's Disease, affecting knuckles, elbows, nipple area, pressure areas, skin folds, and scars.
    • Key symptoms of Addison's Disease:
      • Neuropsychiatric: Anxiety, irritability, depression, confusion.
      • Endocrine: Hypoglycemia.
      • Hypovolemia: Decreased cardiac output, increased renal blood flow, and azotemia.
      • Intolerance to stress: Inability to increase cortisol levels during stress, leading to vascular collapse.
    • Laboratory findings in Addison's Disease:
      • Basic Metabolic Panel: Hyponatremia, hypoglycemia, hyperkalemia.
      • Complete Blood Count (CBC): Moderate neutropenia, lymphocytosis, and eosinophilia.
      • AM Serum Cortisol:
        • < 3 mcg/dl: Suggests adrenal insufficiency but requires confirmation.
        • 3-18 mcg/dl: Further testing (ACTH Stimulation Test) is required.

        • 18 mcg/dl: Excludes Addison's Disease.

    • Cosyntropin (ACTH) Stimulation Test:
      • Confirms the diagnosis of Addison's Disease.
      • Synthetic ACTH (cosyntropin) is given intravenously or intramuscularly.
      • Serum cortisol is measured 45 minutes after injection.
        • Normal: Serum cortisol 20 mcg/dL or higher.
        • Adrenal insufficiency: Serum cortisol less than 20 mcg/dL.
    • Serum ACTH levels:
      • Elevated levels indicate Addison's Disease.
      • Decreased ACTH levels indicate secondary adrenal insufficiency.
    • Other laboratory tests for Addison's Disease:
      • Overnight Metyrapone Test: Measures cortisol response to metyrapone stimulation.
      • Plasma Renin Activity: May distinguish Addison's Disease from secondary adrenal insufficiency.
      • DHEA & DHEA-S: Suppressed levels.
    • Cushing Syndrome is characterized by excess cortisol production, resulting from either endogenous or exogenous sources.
    • Cushing Disease is caused by an ACTH-secreting pituitary adenoma.
    • Cushing Syndrome can be caused by an ACTH-secreting tumor in the pituitary gland (Cushing Disease), ectopic ACTH production from non-pituitary sources, or adrenal tumors.
    • Key signs and symptoms of Cushing Syndrome:
      • Metabolic: Hyperglycemia, weight gain (especially central obesity), insulin resistance, hyperlipidemia.
      • Endocrine: Irregular menstruation, hirsutism, acne, skin thinning, easy bruising, muscle weakness, osteoporosis.
      • Cardiovascular: Hypertension, hypertrophic cardiomyopathy, hypercholesterolemia, arrhythmias.
      • Psychomotor: Anxiety, irritability, depression, mood swings, psychosis.
    • Cushing Disease Diagnostic Testing:
      • Plasma ACTH and DHEAS:
        • High ACTH and high DHEAS: Pituitary or ectopic sources.
        • Low ACTH and low DHEAS: Possible adrenal tumor.
      • High-dose dexamethasone suppression test:
        • Suppression of cortisol and ACTH: Pituitary etiology.
      • Imaging studies:
        • MRI of the head: Pituitary lesion.
        • CT scan of the abdomen: Adrenal lesion.
        • Whole-body imaging (68Ga-somatostatin receptor-PET/CT): For ectopic causes.
    • Cushing Disease treatment:
      • Surgical therapy:
        • For ACTH-secreting pituitary adenomas, transsphenoidal resection is performed.
        • For ectopic ACTH-secreting tumors: Tumor resection and metastases removal, along with adrenal enzyme inhibitors.
      • Medical therapy:
        • Adrenal enzyme inhibitors (mitotane, ketoconazole, metyrapone).
        • Pituitary irradiation.
    • Bilateral Adrenalectomy: Required if treatment remains refractory.
    • Cushing Syndrome treatment:
      • Surgical therapy:
        • Benign adrenal adenomas are usually resected laparoscopically if smaller than 6 cm.
        • Adrenocortical carcinomas are resected surgically and treated with mitotane postoperatively.
      • Medical therapy:
        • Mineralocorticoid hypertension: Spironolactone, eplerenone, dihydropyridine calcium channel blockers.
        • Hyperandrogenism: Flutamide.
        • Other agents: Cabergoline.

    Hyperaldosteronism

    • Primary Hyperaldosteronism is characterized by excessive aldosterone production.
      • Most common cause: Bilateral adrenal cortical hyperplasia (75%).
      • Unilateral aldosterone-producing adrenocortical adenoma (Conn syndrome, 25%).
    • Clinical Features of Hyperaldosteronism:
      • Hypokalemic hypertension.
      • Sodium retention leading to elevated blood pressure.
      • Potassium loss leading to muscle weakness, paresthesias, tetany, headache, and polyuria.
      • Metabolic alkalosis due to renal potassium and hydrogen ion depletion.

    Adrenal Gland Disorders

    • Adrenal insufficiency is caused by a decrease in the production of adrenal hormones, leading to a deficiency in cortisol, aldosterone, and DHEA.

    • Primary adrenal insufficiency (Addison's disease) is caused by adrenal gland abnormalities, resulting in decreased cortisol, aldosterone, and dehydroepiandrosterone (DHEA) production.

    • Secondary adrenal insufficiency occurs when the pituitary gland fails to produce enough ACTH, leading to reduced cortisol production.

    • Tertiary adrenal insufficiency is a result of hypothalamic dysfunction, leading to reduced CRH production and subsequent decline in ACTH and cortisol levels.

    • Addison's disease affects 90% females and has an incidence of 5 cases per million in the US.

    • Addison's disease etiology:

      • Autoimmunity is the most common cause.
      • Leading to atrophy of the adrenal cortex.
      • Bilateral adrenal hemorrhage: Often associated with sepsis, HIT, anticoagulation, antiphospholipid syndrome, and genetic conditions.
      • Medications: Medications such as mitotane and abiraterone acetate (chemotherapy drugs) can cause adrenal insufficiency.
      • Adrenal suppression: Steroid therapy can persist for up to a year, particularly in secondary adrenal insufficiency.
      • Tuberculosis: The leading cause globally, involving the adrenals.

    • Addison disease symptoms are usually gradual and include:

      • Hyperpigmentation: Commonly observed on knuckles, elbows, nipples, pressure areas, skin folds, and scars.
      • Neuro/psych: Anxiety, irritability, depression, and confusion.
      • Endo: Hypoglycemia.
      • Hypovolemia: Due to decreased cardiac output and increased renal blood flow with azotemia.
      • Intolerance to stress: Individuals with Addison's cannot increase cortisol levels during stress, potentially leading to life-threatening vascular collapse.

    • Addison disease labs & diagnostics:

      • Basic metabolic panel:
        • Hyponatremia.
        • Hypoglycemia.
        • Hyperkalemia.
      • CBC: May reveal moderate neutropenia, lymphocytosis, and eosinophilia.
      • AM serum cortisol:
        • Less than 3 mcg/dL suggests adrenal insufficiency.
        • 3-18 mcg/dL requires an ACTH stimulation test.
        • Greater than 18 mcg/dL excludes Addison disease.
      • Cosyntropin (ACTH) stimulation test:
        • Normal: Serum cortisol greater than or equal to 20 mcg/dL.
        • Adrenal insufficiency: Serum cortisol less than 20 mcg/dL.
      • Serum ACTH:
        • Elevated ACTH suggests Addison's disease.
        • Decreased ACTH suggests secondary adrenal insufficiency.

    • Additional Addison's labs:

      • Overnight metyrapone test: Measures cortisol response to metyrapone stimulation.
      • Plasma renin activity: May distinguish Addison's disease from secondary adrenal insufficiency.
      • DHEA & DHEA-S: Suppressed.

    ### Cushing Syndrome

    • Cushing syndrome: characterized by prolonged exposure to high levels of the hormone cortisol, resulting from various causes, including pituitary adenomas, ectopic ACTH-producing tumors, or adrenal tumors.

    Cushing Syndrome Causes

    • Causes of Cushing syndrome:
      • Pituitary adenomas are the most common cause, leading to excessive ACTH production.
      • Ectopic ACTH-producing tumors: May originate from various locations like the lung, pancreas, or thymus.
      • Adrenal tumors: Benign adenomas or malignant carcinomas can cause excessive cortisol production.
      • Exogenous steroid use: Long-term administration of steroids can lead to Cushing-like symptoms.

    Cushing Syndrome Signs and Symptoms

    • Signs and symptoms:
      • Weight gain: Central obesity.
      • Moon facies: Round, full face with flushed cheeks.
      • Buffalo hump: Fat deposition on the upper back.
      • Striae: Purple or pink stretch marks on the abdomen, thighs, and arms.
      • Thinning skin: Fragile, easily bruised skin.
      • Muscle weakness: Proximal muscle weakness, especially in the arms and legs.
      • Hirsutism: Excessive body hair growth, particularly in women.
      • Hypertension: High blood pressure.
      • Hyperglycemia: Elevated blood sugar levels.
      • Osteoporosis: Thinning and weakening of the bones.
      • Depression: Mood swings and psychological changes.
      • Menstrual irregularities: In women, irregular periods or amenorrhea.

    Cushing Syndrome Diagnosis

    • Diagnostic testing:
      • Plasma ACTH and DHEAS:
        • Elevated ACTH and DHEAS suggest pituitary or ectopic sources.
        • Low ACTH and DHEAS suggest an adrenal tumor.
      • High-dose dexamethasone suppression test:
        • Suppression of cortisol and ACTH levels suggests a pituitary etiology.
      • Imaging:
        • MRI of the head to assess for a pituitary lesion.
        • CT scan of the abdomen to evaluate for an adrenal lesion.
        • Whole-body imaging (68Ga-somatostatin receptor-PET/CT) to investigate for ectopic causes.

    Cushing Syndrome Treatment

    • Treatment:
      • Surgical therapy:
        • Pituitary adenoma: Transphenoidal resection.
        • Ectopic ACTH-secreting tumors: Resection of the tumor and metastases, followed by medical therapy with adrenal enzyme inhibitors (mitotane, ketoconazole, metyrapone).
      • Medical therapy:
        • Adrenal enzyme inhibitors: Most commonly used drugs to suppress cortisol production.
        • Pituitary irradiation: For patients concerned about fertility.
        • Non-resectable tumor: Requires long-term medical treatment with adrenal enzyme inhibitors.
      • Adrenalectomy:
        • Bilateral adrenalectomy: Considered for refractory cases, requiring lifelong replacement therapy.
        • Surgical therapy for adrenal tumors:
          • Benign adrenal adenomas: Resected laparoscopically.
          • Adrenocortical carcinomas: Resected surgically and treated with mitotane postoperatively.
      • Medical therapy for Cushing syndrome:
        • Mineralocorticoid hypertension: Spironolactone, eplerenone, dihydropyridine calcium channel blockers.
        • Hyperandrogenism: Flutamide.
        • Other agents: Cabergoline.

    Patient Education

    • Patient education:
      • Surgical treatment: Has an approximate 90% 10-year survival rate.
      • Complete remission: After surgery, there is a 15-20% chance of recurrence within 10 years.
      • Referral to Endocrinology: Patients with abnormal dexamethasone suppression tests should be referred to an endocrinologist.

    Transsphenoidal Surgery

    • Transsphenoidal surgery: A minimally invasive surgical approach to access the pituitary gland through the nasal cavity.

    Adrenal Tumors

    • Adrenal tumors: Can arise from the adrenal cortex or medulla.

    Adrenal Tumors Types

    • Adrenal cortex tumors:

      • Adrenocortical adenoma: The most common, with only 15% being functional (hormone-producing).
      • Adrenocortical carcinoma: A rare, highly aggressive cancer, often large and functional.

    • Adrenal medulla tumors:

      • Neuroblastoma: Aggressive cancer of immature neuroblastic cells, commonly seen in children.
      • Pheochromocytoma: A rare, usually benign tumor of the adrenal medulla, secreting catecholamines.
      • Incidentalomas: Unintentional findings on imaging studies, generally benign and treated only if functional.

    Adrenal Tumor Characteristics

    • Adrenocortical adenoma:

      • Common: Found in 1-10% of people at autopsy.
      • Functional: Only 15% produce hormones.
      • Treatment: Surgical removal for functional tumors.

    • Adrenocortical carcinoma:

      • Rare: A rare, highly aggressive cancer.
      • Functional: May produce hormones.
      • Treatment: Surgical removal.
      • Large size: Tumors can grow large.

    Incidentaloma

    • Incidentaloma:
      • Common findings: Often found incidentally on CT scans or MRIs.
      • Size: Tumors under 3 cm are generally considered benign.
      • Treatment: Only treated if there is evidence of Cushing's syndrome or pheochromocytoma.

    Neuroblastoma

    • Neuroblastoma:
      • Aggressive cancer: Involves immature neuroblastic cells.
      • Pediatric cancer: One of the most common cancers in children.
      • Catecholamine production: Often releases elevated levels of catecholamine hormone metabolites.

    Neuroblastoma Presentation

    • Presentation:
      • Abdominal distention and a firm, irregular mass that can cross the midline.
      • Severe watery diarrhea: Due to the production of vasoactive intestinal peptide.
      • Normotensive: Less likely to have hypertension compared to pheochromocytoma.
      • Opsoclonus-myoclonus syndrome ("dancing eyes-dancing feet"): A rare neurological disorder characterized by rapid, involuntary eye movements (opsoclonus) and jerky muscle contractions (myoclonus).

    Neuroblastoma Treatment

    • Treatment:
      • Surgery: Primary mode of treatment.
      • Radiation therapy: For localized disease.
      • Chemotherapy: For metastatic disease.
      • Laparoscopic adrenal surgery: Minimally invasive surgical approach to remove adrenal tumors.

    Hyperaldosteronism

    • Hyperaldosteronism: Characterized by excessive aldosterone production.

    Aldosterone Actions

    • Aldosterone actions:
      • Sodium retention: Promotes sodium reabsorption in the kidneys.
      • Potassium excretion: Increases potassium excretion in the kidneys.
      • Hydrogen ion excretion: Increases hydrogen ion excretion, leading to a metabolic alkalosis.

    Primary & Secondary Hyperaldosteronism

    • Primary hyperaldosteronism:

      • Excessive aldosterone production: Caused by an adrenal abnormality.
      • Causes:
        • Bilateral adrenal cortical hyperplasia: Most common cause, affecting 75% of cases.
        • Unilateral aldosterone-producing adrenocortical adenoma (Conn syndrome): Affects 25% of cases.
      • Renin: Decreased renin activity due to volume expansion.

    • Secondary hyperaldosteronism:

      • Caused by: Renin-angiotensin-aldosterone system (RAAS) activation.
      • Conditions:
        • Renal artery stenosis: Narrowing of the renal artery.
        • Heart failure: Reduced cardiac output.
        • Liver cirrhosis: Reduced albumin production.
      • Renin: Elevated renin levels.

    Hyperaldosteronism Clinical Features

    • Clinical features:
      • Hypokalemic hypertension: A hallmark of the condition.
      • Hypokalemia: Caused by increased potassium excretion.
      • Symptoms:
        • Muscle weakness.
        • Paresthesias: Tingling or numbness in the extremities.
        • Tetany: Muscle spasms.
        • Headache: Especially in the mornings.
        • Polyuria: Frequent urination.
      • Metabolic alkalosis: Due to increased hydrogen ion excretion.

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    Description

    This quiz focuses on the contrasting conditions affecting the adrenal glands, namely Addison Disease and Cushing Syndrome. Explore the causes, signs, and symptoms of these adrenal disorders, and understand the implications of hormone imbalances in the body.

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