Adrenal cortical diseases

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Adrenal Cortex - Anatomy & Steroidogenesis

Adrenal Gland Anatomy: Cortex, Medulla, and Zones

  • Cortex Layers (superficial to deep): Zona Glomerulosa, Fasciculata, Reticularis.
  • 📌 Mnemonic: Go Find Rex (GFR) - Makes Sweet Sex (Salt, Sugar, Sex).
    • Glomerulosa → Salt (Aldosterone)
    • Fasciculata → Sugar (Cortisol)
    • Reticularis → Sex (Androgens)

⭐ The rate-limiting enzyme for steroidogenesis is cholesterol desmolase, which converts cholesterol to pregnenolone. It is stimulated by ACTH.

Cushing Syndrome - Cortisol Overload

  • Pathophysiology: Excess cortisol from any cause. Cushing disease is specifically due to a pituitary adenoma.
  • Etiology:
    • Exogenous: Most common cause; iatrogenic steroids.
    • Endogenous:
      • ACTH-dependent: Cushing disease (~70%), ectopic ACTH (e.g., small cell lung cancer).
      • ACTH-independent: Adrenal adenoma, carcinoma, or hyperplasia.

Cushing's Syndrome: Clinical Features Diagram

  • Clinical Features: Central obesity, moon facies, buffalo hump, purple striae, hypertension, hyperglycemia, osteoporosis, immunosuppression.

  • Diagnosis Flowchart:

Exam Favorite: In ACTH-dependent Cushing's, a high-dose dexamethasone test differentiates causes. Cortisol suppression suggests a pituitary adenoma (Cushing's disease), whereas a lack of suppression points to an ectopic source like small cell lung cancer.

Hyperaldosteronism - The Salt & Pressure Show

  • Primary (Conn's Syndrome): Caused by adrenal adenoma or hyperplasia.
  • Secondary: Driven by high renin (e.g., renal artery stenosis, diuretics).
  • Clinical Picture: Hypertension, hypokalemia (muscle weakness, paresthesias), and metabolic alkalosis.
  • Diagnosis:
    • Primary: ↑ Aldosterone, ↓ Renin. High aldosterone-to-renin ratio.
    • Secondary: ↑ Aldosterone, ↑ Renin.

⭐ Despite ↑Na+ reabsorption, significant hypernatremia and edema are rare due to the 'aldosterone escape' mechanism (pressure natriuresis).

Adrenal Insufficiency - Powering Down

  • Primary (Addison's Disease): Adrenal gland destruction (autoimmune, TB). Results in ↓ Cortisol, ↓ Aldosterone, but ↑ ACTH.
    • Presents with hypotension, hyperkalemia, metabolic acidosis, and skin/mucosal hyperpigmentation.
  • Secondary/Tertiary: Pituitary/hypothalamic failure (e.g., chronic steroid use). Causes ↓ Cortisol, ↓ ACTH, but normal Aldosterone.
    • No hyperpigmentation or significant electrolyte shifts.
  • Acute Adrenal Crisis: Life-threatening shock from stressors (infection, surgery). Requires immediate fluid and steroid resuscitation.

Waterhouse-Friderichsen Syndrome: Acute, massive hemorrhagic adrenalitis, classically due to Neisseria meningitidis septicemia, leading to adrenal crisis.

Hyperpigmentation of gums and buccal mucosa in Addison's

Adrenocortical Neoplasms - Lumps & Bumps

  • Adrenocortical Adenoma:
    • Benign, well-circumscribed tumor. Most are non-functional incidentalomas.
    • Functional types can cause Cushing's syndrome (cortisol) or Conn's syndrome (aldosterone).
  • Adrenocortical Carcinoma:
    • Rare, aggressive malignancy; often large (>5 cm) with necrosis & hemorrhage.
    • Frequently functional, causing rapid virilization or Cushing's.
    • Associated with Li-Fraumeni syndrome (TP53 mutation).

⭐ Most adrenal "incidentalomas" (asymptomatic masses) are benign, non-functioning adenomas.

High‑Yield Points - ⚡ Biggest Takeaways

  • Cushing's syndrome is most commonly due to exogenous steroids. Use the dexamethasone suppression test for endogenous causes.
  • Addison's disease (primary adrenal insufficiency) presents with hypotension, hyperpigmentation, and hyperkalemia; often autoimmune.
  • Conn's syndrome (primary hyperaldosteronism) causes hypertension, hypokalemia, and low plasma renin, typically from an adenoma.
  • 21-hydroxylase deficiency is the most common Congenital Adrenal Hyperplasia (CAH), causing virilization and salt wasting.
  • Waterhouse-Friderichsen syndrome is acute adrenal hemorrhage from sepsis (classically meningococcemia), causing shock.

Practice Questions: Adrenal cortical diseases

Test your understanding with these related questions

A 33-year-old woman presents to the physician because of abdominal discomfort, weakness, and fever. She has had a significant weight loss of 15 kg (33.1 lb) over the past 2 months. She has no history of medical illness and is not on any medications. Her pulse is 96/min, the blood pressure is 167/92 mm Hg, the respiratory rate is 20/min, and the temperature is 37.7°C (99.8°F). Her weight is 67 kg (147.71 lb), height is 160 cm (5 ft 3 in), and BMI is 26.17 kg/m2. Abdominal examination shows purple striae and a vaguely palpable mass in the left upper quadrant of the abdomen, which does not move with respirations. She has coarse facial hair and a buffalo hump along with central obesity. Her extremities have poor muscle bulk, and muscle weakness is noted on examination. An ultrasound of the abdomen demonstrates an adrenal mass with para-aortic lymphadenopathy. Which of the following is the most likely laboratory profile in this patient?

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Flashcards: Adrenal cortical diseases

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Pituitary _____ is a benign tumor of the anterior pituitary cells

TAP TO REVEAL ANSWER

Pituitary _____ is a benign tumor of the anterior pituitary cells

adenoma

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