Primary hyperparathyroidism

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Primary hyperparathyroidism
Classifications and external resources
ICD-10 E21.
ICD-9 252.01
DiseasesDB 6283
eMedicine radio/355 
MeSH D049950

Primary hyperparathyroidism causes hypercalcemia (elevated blood calcium levels) through the excessive secretion of parathyroid hormone (PTH), usually by an adenoma (benign tumors) of the parathyroid glands. Its incidence is approximately 42 per 100,000 people. It is roughly two to three times as common in women as men. complications. The classic bone disease in hyperparathyroidism is osteitis fibrosa cystica, which results in pain and sometimes pathological fractures. Other bone diseases associated with hyperparathyroidism are osteoporosis, osteomalacia, and arthritis.

Other signs include proximal muscle weakness, itching, and band keratopathy of the eyes.

Contents

[edit] Diagnosis

The diagnosis of primary hyperparathyroidism is made by blood tests. Serum calcium levels are elevated. Intact PTH levels are also elevated. Urinary cAMP is occasionally measured; this is generally elevated.

[edit] Causes

The most common cause of primary hyperparathyroidism is a sporadic, single parathyroid adenoma resulting from a clonal mutation (~97%). Less common are hyperplasia of all parathyroid glands (~2.5%), parathyroid carcinoma (malignant tumor), and adenomas in more than one gland (together ~0.5%). Primary hyperparathyroidism is also a feature of several familial endocrine disorders: Multiple endocrine neoplasia type 1 and type 2A (MEN type 1 and MEN type 2A), and familial hyperparathyroidism.

In all cases, the disease is idiopathic, but is thought to involve inactivation of tumor suppression genes.

[edit] Treatment

Treatment is usually surgical removal of the gland(s) containing adenomas. Medications (such as estrogen replacement therapy in postmenopausal women and bisphosphonates) thus far have not been very effective. Future developments such as calcimemetic agents (e.g. cinacalcet) which activate the parathyroid calcium-sensing receptor may offer a good alternative to surgery.

[edit] See also

[edit] References

  • Greenspan FS, Gardner DG. Basic & Clinical Endocrinology 7th ed. McGraw-Hill Companies: 2004.
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