Adrenal Pathologies BMS 200

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Questions and Answers

Which condition is primarily characterized by excessive cortisol production resulting from a pituitary adenoma?

  • Conn's disease
  • Addison's disease
  • Cushing's disease (correct)
  • Pheochromocytoma

What characterizes primary adrenal insufficiency compared to secondary adrenal insufficiency?

  • It leads to an increase in ACTH levels.
  • It is primarily caused by a pituitary tumor.
  • It originates from dysfunction at the level of the adrenal glands. (correct)
  • It typically presents with elevated aldosterone levels.

Which clinical feature is associated with Conn's disease?

  • Hypotension
  • Hypoglycemia
  • Hyperkalemia
  • Hypertension (correct)

Which diagnostic test would be most informative for confirming the presence of congenital adrenal hyperplasia?

<p>17-OH progesterone testing (D)</p> Signup and view all the answers

What is a common complication associated with pheochromocytoma?

<p>Severe hypertension (B)</p> Signup and view all the answers

Which of the following is NOT a source of excess cortisol associated with Cushing Syndrome?

<p>Hypoaldosteronism (C)</p> Signup and view all the answers

What is the most common source of endogenous hypercortisolism?

<p>Pituitary ACTH-producing microadenoma (C)</p> Signup and view all the answers

Which pathological condition is characterized by decreased levels of glucocorticoids?

<p>Adrenocortical insufficiency (D)</p> Signup and view all the answers

Which condition may cause a significant hypersecretion of ACTH leading to Cushing Syndrome?

<p>Ectopic ACTH secretion from small cell lung cancer (B)</p> Signup and view all the answers

In the majority of cases of endogenous Cushing syndrome, what is typically noted in the pituitary gland?

<p>Presence of an ACTH-producing microadenoma (A)</p> Signup and view all the answers

What percentage of Cushing syndrome cases are attributed to ectopic ACTH production?

<p>5 to 10% (A)</p> Signup and view all the answers

In which demographic is Cushing Disease more prevalent?

<p>Women in their twenties and thirties (B)</p> Signup and view all the answers

What is the consequence of an adrenal adenoma found in one adrenal gland in the case of Cushing syndrome?

<p>Atrophy of the unaffected adrenal gland (A)</p> Signup and view all the answers

What characterizes primary hyperaldosteronism?

<p>Excess aldosterone secretion (D)</p> Signup and view all the answers

Which condition is associated with secondary hyperaldosteronism?

<p>Decreased renal perfusion (A)</p> Signup and view all the answers

What is the expected result of an ACTH stimulation test in primary adrenal insufficiency?

<p>No change in cortisol levels (B)</p> Signup and view all the answers

What is a common enzyme deficiency in Congenital Adrenal Hyperplasia (CAH)?

<p>21-Hydroxylase (B)</p> Signup and view all the answers

What would represent a typical finding in a patient with primary hyperaldosteronism?

<p>High serum aldosterone levels (B)</p> Signup and view all the answers

Which of the following conditions can lead to secondary hyperaldosteronism?

<p>Cirrhosis (A)</p> Signup and view all the answers

What is the role of ACTH in adrenal function testing?

<p>Stimulates cortisol production (D)</p> Signup and view all the answers

What effect does hyperaldosteronism typically have on potassium levels?

<p>Decreased potassium levels (B)</p> Signup and view all the answers

What characterizes the salt-wasting form of classic Congenital Adrenal Hyperplasia (CAH)?

<p>Complete inactivation of 21-hydroxylase (D)</p> Signup and view all the answers

In females with classic CAH, which condition is commonly observed at birth?

<p>Ambiguous genitalia (B)</p> Signup and view all the answers

Which statement best describes the late-onset form of CAH?

<p>It is often misdiagnosed as polycystic ovary syndrome in women. (B)</p> Signup and view all the answers

Which of the following is a characteristic clinical feature of pheochromocytoma?

<p>Persistent hypertension (D)</p> Signup and view all the answers

What laboratory result is likely in individuals suspected of having late-onset CAH?

<p>Increased 17 hydroxyprogesterone (17-OHP) levels (C)</p> Signup and view all the answers

What potential complication can arise from the paroxysmal hypertension associated with pheochromocytoma?

<p>Hypertrophy of the heart (B)</p> Signup and view all the answers

What is the most common etiology of pheochromocytoma?

<p>Sporadic occurrence (D)</p> Signup and view all the answers

Which treatment is considered curative for the hypertension associated with pheochromocytoma?

<p>Surgical excision of the tumor (B)</p> Signup and view all the answers

What is the most common cause of adrenal insufficiency globally?

<p>Tuberculosis (A)</p> Signup and view all the answers

Which statement about Addison's disease is true?

<p>It can cause sudden adrenal crisis due to stress. (B)</p> Signup and view all the answers

Which feature is typically absent in secondary adrenocortical insufficiency compared to primary adrenal insufficiency?

<p>Hyperpigmentation (C)</p> Signup and view all the answers

What condition can precipitate primary acute adrenocortical insufficiency?

<p>Surgical recovery without medication adjustments (C)</p> Signup and view all the answers

Which of the following is a potential cause of adrenal hemorrhage?

<p>Anticoagulant therapy (B)</p> Signup and view all the answers

What is the effect of the insulin tolerance test in diagnosing secondary adrenal insufficiency?

<p>Cortisol and ACTH secretion should increase. (C)</p> Signup and view all the answers

In which scenario is acute adrenal insufficiency most likely to occur?

<p>High stress without increased steroid doses (A)</p> Signup and view all the answers

What is a common clinical presentation of primary chronic adrenal insufficiency?

<p>Hypotension (D)</p> Signup and view all the answers

What does an elevated level of ACTH indicate in a patient with suspected Cushing's syndrome?

<p>Ectopic ACTH syndrome (A)</p> Signup and view all the answers

Which of the following diseases is categorized as an autoimmune cause of Addison's disease?

<p>Autoimmune polyendocrine syndrome type 1 (D)</p> Signup and view all the answers

Which condition could be a result of adrenal insufficiency during times of stress?

<p>Adrenal crisis (C)</p> Signup and view all the answers

What is a common sign in patients suffering from Addison's disease?

<p>Hypoglycemia (B)</p> Signup and view all the answers

What physiological effect does the withdrawal of glucocorticoids too rapidly cause?

<p>Adrenal crisis due to inability to produce glucocorticoids (B)</p> Signup and view all the answers

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Study Notes

Adrenal Pathologies Overview

  • Adrenal disorders can manifest as hyperfunction (excess hormone production) or hypofunction (insufficient hormone production).
  • Hyperfunction includes conditions such as Cushing's syndrome, hyperaldosteronism, and pheochromocytoma.
  • Hypofunction primarily involves adrenal insufficiency and congenital adrenal hyperplasia.

Cushing's Syndrome and Disease

  • Cushing's syndrome results from elevated glucocorticoid levels, often due to factors such as iatrogenic causes, hypothalamic-pituitary diseases, adrenal adenomas, or ectopic ACTH production.
  • Hypersecretion of ACTH accounts for 70-80% of Cushing's syndrome cases, predominantly affecting women in their twenties and thirties.
  • Cushing's Disease specifically refers to pituitary adenoma producing ACTH; adrenal glands may exhibit changes due to cortisol overproduction.
  • Ectopic ACTH production, typically from tumors like small cell lung cancer, accounts for about 10% of cases.
  • Adrenocortical carcinoma is a rare cause of primary Cushing's syndrome and often presents with severe symptoms.

Adrenal Insufficiency

  • Adrenal insufficiency can be classified as primary (Addison's disease) or secondary (decreased ACTH stimulation).
  • Acute adrenal insufficiency may arise from rapid glucocorticoid withdrawal, adrenal hemorrhage, or stress in chronic insufficiency cases.
  • Addison's disease results from 90% destruction of the adrenal cortex, often due to autoimmune processes, tuberculosis, or malignancy.

Clinical Features of Addison’s Disease

  • Initial symptoms include fatigue, gastrointestinal disturbances, and hyperpigmentation.
  • Patients may experience hypotension, hypokalemia, and can develop acute crises during stress without adequate corticosteroid therapy.
  • If untreated, adrenal crises can be fatal.

Secondary Adrenocortical Insufficiency

  • Caused by hypothalamic or pituitary disorders leading to reduced ACTH output; characterized by deficient cortisol with normal aldosterone levels.
  • Patients do not exhibit hyperpigmentation typical of primary insufficiency.

Hyperaldosteronism

  • Primary hyperaldosteronism (Conn's syndrome) involves autonomous aldosterone overproduction, leading to hypertension and hypokalemia.
  • Secondary hyperaldosteronism results from increased renin levels due to conditions like renal perfusion issues or heart failure.

Primary Hyperaldosteronism Clinical Features

  • Hypertension results from sodium retention, while hypokalemia causes neuromuscular symptoms.
  • Diagnosis can involve measuring serum aldosterone and renin levels for aldosterone: renin ratio.

Congenital Adrenal Hyperplasia (CAH)

  • CAH is an autosomal recessive disorder, often due to a deficiency in 21-hydroxylase, leading to cortisol production deficits.
  • Classic forms include salt-wasting conditions that can be fatal without treatment, and simple virilizing forms that cause ambiguous genitalia in females at birth.
  • Non-classic CAH presents later in life, often mimicking polycystic ovary syndrome in females.

Pheochromocytoma

  • Pheochromocytoma arises from chromaffin cells in the adrenal medulla, producing excess catecholamines.
  • Clinical features prominently include hypertension, which can be paroxysmal; episodes may lead to serious cardiovascular complications.
  • Surgical resection can effectively treat the resulting hypertension.
  • Insulin tolerance tests, CRH tests, and ACTH stimulation tests help differentiate between adrenal insufficiency types and assess adrenal response.
  • Specific hormones measured include cortisol, ACTH, aldosterone, and 17-hydroxyprogesterone, contributing to diagnosis and treatment plans.

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