Summary of Primary Hyperparathyroidism (PHPT)

  • Primary hyperparathyroidism (PHPT):
    • Is a relatively common disorder:
      • That affects 0.3% of the human population:
        • Most commonly women:
        • 75% of the cases
  • The exact cause of PHPT:
    • Is unknown
  • In 85% to 90% of patients:
    • Only a single adenoma is present
  • Multiple adenomas or hyperplasia:
    • Can be present in up to 15% to 20% of the cases:
      • Doble adenomas:
        • 6% to 9% of the cases
      • Triple adenomas:
        • Less than 0.3% of the cases
      • Hyperplasia:
        • 3% of the cases
  • Patients with PHPT:
    • Can have symptomatic disease:
      • 95% of the cases
    • “Asymptomatic” disease:
      • 5% of the cases
  • Some degree of renal dysfunction:
    • Is present in up to 80% of patients
    • Nephrolithiasis, however, is far less common:
      • With an incidence of approximately 15% to 25%
  • The clinical manifestations of PHPT:
    • Vary widely across patients:
      • But if a detailed history is taken:
        • Many will complain of polydipsia and polyuria:
          • From the calciuresis associated with the disease
  • Although PHPT occurs sporadically in the majority of patients:
    • In a small percentage it is part of a familial syndrome:
      • Multiple endocrine neoplasia type I (MEN-I / Werner Syndrome):
        • Results from a germline mutation in the Menin gene:
          • Located on chromosome 11q12-13
        • Patients with MEN-I are susceptible to the development of:
          • Pancreatic neuroendocrine tumors
          • Pituitary adenomas
          • PHPT
      • MEN-IIA (Sipple Syndrome):
        • Is an autosomal dominantly inherited condition:
          • Caused by a germline mutation on chromosome 11:
            • That is associated with:
              • PHPT
              • Pheochromocytoma
              • Medullary thyroid cancer
      • Patients with familial jaw tumor syndrome:
        • Have a higher risk for the development of:
          • Parathyroid carcinoma
      • Familial hypercalcemic hypocalciuria:
        • Is associated with an:
          • Elevated calcium levels and low urinary excretion of calcium
        • The primary defect is the abnormal sensing of calcium in blood:
          • By the calcium sensing receptor (CaSR):
            • In the the parathyroid glands (chief cells) and the renal tubules:
              • Which causes:
                • Inappropriate secretion of PTH
                • Excessive renal reabsorption of calcium

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