Pathophysiology

Pathophysiology

I-28. Hormonal regulation of calcium & phosphate metabolism

カルシウム・リン代謝のホルモン調節

Calcium Overview

  • Plasma total Ca²⁺: 2.2–2.6 mmol/L.
  • Needed for: bone structure, neurotransmitter release, muscle contraction, cardiac conduction, clotting.

Key Regulating Hormones

  • PTH (parathyroid glands), Calcitriol/vitamin D (skin-liver-kidney axis), FGF23 (bone), Calcitonin (thyroid, mostly childhood).

Parathyroid Hormone (PTH)

  • Serum 10–65 pg/ml; regulated by serum Ca²⁺ (Ca²⁺ binds parathyroid → inhibits PTH; ↓Ca²⁺ → ↑PTH).
  • Functions (↑serum Ca²⁺): osteoblasts express RANKL → osteoclast differentiation; ↑renal Ca²⁺ reabsorption; ↑renal vitamin D activation; inhibits osteogenesis.
  • Continuous high PTH → cardiac (LV) hypertrophy.

Calcitriol (Vitamin D)

  • Regulates gene expression via VDR.
  • Intestine: ↑Ca²⁺ uptake. Kidney: limits its own activation (24-hydroxylase), ↓renin (protective vs kidney fibrosis). Parathyroid: ↓PTH. Bone: activates osteoclasts + osteoblasts → remodels weak → stronger bone.

FGF23

  • From osteocytes; regulates PO₄³⁻ (stimulated by hyperphosphatemia).
  • Kidney: → phosphaturia (normalizes phosphate); inhibits calcitriol synthesis.
  • Also: inhibits PTH secretion, induces cardiac hypertrophy.

Hyperparathyroidism

Primary

  • PTH made independent of Ca²⁺; most common cause of outpatient hypercalcemia (1:500, females 3×).
  • Causes: parathyroid adenoma, PTH-producing tumor.
  • Labs: ↑Ca²⁺, ↓PO₄³⁻ (hypercalcemia).
  • Symptoms: often asymptomatic; bone fractures (osteoporosis), kidney stones, gastritis/constipation/abdominal cramps, ectopic calcifications (lung, heart, pancreas → pancreatitis). (“Stones, bones, groans.”)

Secondary

  • PTH overproduction responding to chronic hypocalcemia.
  • Causes:
    • Vitamin D deficiency (↓sunlight/diet) → ↓intestinal Ca²⁺, ↓PTH-gene inhibition.
    • Chronic kidney disease: ↓phosphate excretion → phosphate binds Ca²⁺ (↓free Ca²⁺); ↓vitamin D activation → ↓Ca²⁺ absorption → hypocalcemia → ↑PTH.
  • Labs: ↑Ca²⁺ and ↑PO₄³⁻. Symptoms: ↓intestinal Ca²⁺, bone resorption, vascular/soft-tissue calcification.

Hypo- vs Hypercalcemia

Hypocalcemia

  • ↓threshold potential → ↑excitability: muscle spasms, longer QT (arrhythmogenic), tremor/tetany (~1.5 mM), laryngospasm → suffocation (~1 mM); general weakness.

Hypercalcemia

  • Muscle weakness; very high Ca²⁺ blocks voltage-gated Na⁺ channels → ↓depolarization → cardiac arrest.

一問一答

How is PTH secretion regulated by calcium?

Serum Ca²⁺ binds the parathyroid calcium-sensing receptor and inhibits PTH; a fall in Ca²⁺ increases PTH release.

What is the normal plasma total calcium range, and why is calcium essential?

2.2–2.6 mmol/L; needed for bone structure, neurotransmitter release, muscle contraction, cardiac conduction, and clotting.

What are the actions of calcitriol (active vitamin D)?

Intestine: ↑Ca²⁺ uptake. Kidney: limits its own activation and ↓renin. Parathyroid: ↓PTH. Bone: activates osteoclasts + osteoblasts to remodel weak into stronger bone.

What are the four key hormones regulating calcium/phosphate, and their sources?

PTH (parathyroid glands), calcitriol/vitamin D (skin-liver-kidney axis), FGF23 (bone osteocytes), and calcitonin (thyroid, mainly in childhood).

How does PTH raise serum calcium?

Osteoblasts express RANKL → osteoclast differentiation/bone resorption; ↑renal Ca²⁺ reabsorption; ↑renal vitamin D activation; and inhibition of osteogenesis.

What is the role of FGF23?

Secreted by osteocytes and stimulated by hyperphosphatemia, it promotes renal phosphate excretion (phosphaturia), inhibits calcitriol synthesis and PTH secretion, and induces cardiac hypertrophy.

What is primary hyperparathyroidism and its biochemistry?

PTH produced independently of calcium (e.g., parathyroid adenoma); the most common cause of outpatient hypercalcemia, with ↑Ca²⁺ and ↓PO₄³⁻.

What is secondary hyperparathyroidism?

PTH overproduction responding to chronic hypocalcemia (e.g., vitamin D deficiency or chronic kidney disease), characterized by ↑Ca²⁺ effort but ↑PO₄³⁻.

What are the classic symptoms of primary hyperparathyroidism?

"Stones, bones, groans": kidney stones, bone fractures/osteoporosis, gastritis/constipation/abdominal cramps, and ectopic calcifications (lung, heart, pancreas → pancreatitis); often asymptomatic.

How does chronic kidney disease cause secondary hyperparathyroidism?

↓Phosphate excretion → phosphate binds Ca²⁺ (↓free Ca²⁺), and ↓vitamin D activation → ↓Ca²⁺ absorption → hypocalcemia → ↑PTH.

Why does hypocalcemia increase neuromuscular excitability?

Low Ca²⁺ lowers the threshold potential, raising excitability → muscle spasms, prolonged QT (arrhythmias), tremor/tetany (~1.5 mM), and laryngospasm (~1 mM).

What are the effects of hypercalcemia?

Muscle weakness; very high Ca²⁺ blocks voltage-gated Na⁺ channels → ↓depolarization → cardiac arrest.

How does FGF23 regulate phosphate in the kidney?

It promotes renal phosphate excretion (phosphaturia) to normalize phosphate and inhibits calcitriol synthesis.

How does vitamin D deficiency lead to secondary hyperparathyroidism?

↓Intestinal Ca²⁺ absorption and reduced inhibition of the PTH gene → hypocalcemia → compensatory ↑PTH.

Why does continuous high PTH cause cardiac hypertrophy?

Persistently elevated PTH has direct effects promoting left ventricular hypertrophy.

What labs distinguish primary from secondary hyperparathyroidism?

Primary: ↑Ca²⁺, ↓PO₄³⁻. Secondary: ↑PO₄³⁻ with low/normal Ca²⁺ driving ↑PTH.

How does calcitriol act at the cellular level?

It binds the vitamin D receptor (VDR) and regulates gene expression in target tissues.

Why is renin reduced by calcitriol clinically relevant?

Calcitriol's suppression of renin is protective against kidney fibrosis.

Why does primary hyperparathyroidism cause kidney stones?

Excess bone resorption raises serum and filtered calcium, leading to hypercalciuria and calcium stone formation.

How does PTH increase active vitamin D?

PTH stimulates renal 1α-hydroxylase, increasing conversion of vitamin D to active calcitriol → ↑intestinal Ca²⁺ absorption.