Adrenal Cortical Disorders

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Adrenal Cortex: Basics - Gland Essentials

Adrenal Cortex Layers and Hormone Production

  • Adrenal Cortex Layers (Outer to Inner) & Primary Products:
    • Zona Glomerulosa (ZG): Mineralocorticoids (e.g., Aldosterone - salt balance).
    • Zona Fasciculata (ZF): Glucocorticoids (e.g., Cortisol - sugar metabolism).
    • Zona Reticularis (ZR): Androgens (e.g., DHEA - sex characteristics).
  • 📌 Mnemonic (GFR → Hormones): Glomerulosa, Fasciculata, Reticularis → Salt, Sugar, Sex.

⭐ Embryological Origin: Adrenal cortex develops from mesoderm; adrenal medulla from neural crest cells.

Cushing's Syndrome - Moon Face Mayhem

⭐ Most common cause of Cushing's syndrome is exogenous glucocorticoid administration; most common endogenous cause is Cushing's disease (pituitary adenoma).

  • Chronic cortisol excess. 📌 CUSHINGOID features: Central obesity, Moon facies, Buffalo hump, Purple striae, Hypertension, Hyperglycemia, Osteoporosis, Amenorrhea, Immunosuppression.
  • Clinical features of Cushing syndrome
  • Diagnosis:
    • Screening: 24-hr Urine Free Cortisol (UFC), Late-night salivary cortisol, Low-dose Dexamethasone Suppression Test (LDDST - 1mg).
    • Localization:
      • Plasma ACTH: If low (e.g., <5 pg/mL) → Adrenal cause (ACTH-independent). Proceed to adrenal imaging.
      • If normal/high (e.g., ≥5 pg/mL) → ACTH-dependent. Differentiate pituitary (Cushing's disease) vs. ectopic ACTH with High-dose DST (HDDST - 8mg; pituitary source typically suppresses) and/or CRH stimulation test.

Hyperaldosteronism & CAH - Salty Surges & Enzyme Snags

  • Hyperaldosteronism (Conn's Syndrome):

    • Primary: ↑Aldosterone, ↓Renin.
    • Causes: Adrenal adenoma (most common), bilateral adrenal hyperplasia.
    • Sx: Resistant HTN, unexplained ↓K+, metabolic alkalosis. 📌 Conn's: Salty (HTN), Low K+, High Aldo.
    • Dx: Aldosterone:Renin Ratio (ARR) > 20-30 (ng/dL per ng/mL/hr); confirmatory tests (e.g., saline infusion).
    • Rx: Spironolactone/Eplerenone; surgery for adenoma.
  • Congenital Adrenal Hyperplasia (CAH):

    • Autosomal recessive enzyme defects in cortisol synthesis → ↓Cortisol → ↑ACTH → adrenal hyperplasia & precursor shunting.
    • Steroidogenesis pathway & enzyme deficiencies
    • ⭐ > 21-hydroxylase deficiency is the most common cause of Congenital Adrenal Hyperplasia.
    • Key Types & Features:

Adrenal Insufficiency - Energy Crisis Central

FeaturePrimary (Addison's)Secondary Adrenal Insufficiency
ACTH↑↑ High↓ Low / Normal
Cortisol↓ Low↓ Low
Aldosterone↓ LowNormal
K+↑ High (Hyperkalemia)Normal
Pigmentation↑ Hyperpigmentation (↑MSH)Normal
CausesAutoimmune, TB, Waterhouse-Friderichsen (acute)Pituitary/Hypothalamic failure, Chronic steroids

Oral hyperpigmentation in Addison's disease

⭐ Hyperpigmentation and hyperkalemia are characteristic of primary (Addison's) but not secondary adrenal insufficiency.

  • Acute Crisis: Hypotension, shock. IV hydrocortisone.
  • ACTH Stim Test: Primary: Cortisol no rise > 20 µg/dL.

Adrenal Cortical Neoplasms - Cortical Growth Alarms

Adrenocortical Adenoma vs Carcinoma Microscopy

FeatureAdrenocortical AdenomaAdrenocortical Carcinoma
SizeUsually <4 cm, well-circumscribedOften >6 cm, large, poorly-defined
FunctionalityMay be functional (Cushing's, Conn's) or non-functionalFrequently functional (virilization, Cushing's, mixed)
Weiss CriteriaLow score (<3), benign featuresHigh score (≥3) indicates malignancy
PrognosisExcellentPoor, aggressive, high recurrence

High‑Yield Points - ⚡ Biggest Takeaways

  • Cushing's syndrome: Excess cortisol from pituitary adenomas (Cushing's disease), adrenal tumors, or ectopic ACTH.
  • Conn's syndrome: Primary hyperaldosteronism causing hypertension, hypokalemia, low renin.
  • Addison's disease: Primary adrenal insufficiency with ↓cortisol/aldosterone, ↑ACTH, hyperpigmentation, hypotension.
  • Waterhouse-Friderichsen syndrome: Acute adrenal hemorrhage, classically with N. meningitidis sepsis.
  • CAH: Most common is 21-hydroxylase deficiency leading to virilization, salt wasting.
  • Adrenocortical carcinoma: Differentiated from adenoma by large size, necrosis, invasion.

Practice Questions: Adrenal Cortical Disorders

Test your understanding with these related questions

A patient presents with headaches, palpitations, hypertension, and urine VMA positivity. The biopsy findings are shown in the image. Which of the following statements is correct?

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Flashcards: Adrenal Cortical Disorders

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Depletion of cortical cell _____ is seen in adrenal gland in shock

TAP TO REVEAL ANSWER

Depletion of cortical cell _____ is seen in adrenal gland in shock

lipids

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