Hypoparathyroidism

Source: Wikipedia, the free encyclopedia.
Hypoparathyroidism
SpecialtyEndocrinology
Symptomshypocalcemia
MedicationRecombinant human parathyroid hormone, Palopegteriparatide

Hypoparathyroidism is decreased function of the

kidney stones and chronic kidney disease.[1] Additionally, medications such as recombinant human parathyroid hormone or teriparatide may be given by injection to replace the missing hormone.[2]

Signs and symptoms

The main symptoms of hypoparathyroidism are the result of the

Chvostek's sign) or by using the cuff of a sphygmomanometer to temporarily obstruct the blood flow to the arm (a phenomenon known as Trousseau's sign of latent tetany).[4]

A number of

Related conditions

Condition Appearance PTH levels Calcitriol Calcium
Phosphates
Imprinting
Hypoparathyroidism Normal Low Low Low High Not applicable
Pseudohypoparathyroidism Type 1A Skeletal defects High Low Low High Gene defect from mother (
GNAS1
)
Type 1B Normal High Low Low High Gene defect from mother (
GNAS1 and STX16
)
Type 2 Normal High Low Low High ?
Pseudopseudohypoparathyroidism Skeletal defects Normal Normal Normal[6] Normal gene defect from father

Causes

Hypoparathyroidism can have the following causes:[1]

Mechanism

The parathyroid glands are so named because they are usually located behind the

fetal development from structures known as the third and fourth pharyngeal pouch. The glands, usually four in number, contain the parathyroid chief cells that sense the level of calcium in the blood through the calcium-sensing receptor and secrete parathyroid hormone. Magnesium is required for PTH secretion. Under normal circumstances, the parathyroid glands secrete PTH to maintain a calcium level within normal limits, as calcium is required for adequate muscle and nerve function (including the autonomic nervous system
).

PTH acts on several organs to regulate calcium levels and phosphorus levels. PTH acts on the kidneys to increase calcium reabsorption into the blood, and to inhibit phosphorus reabsorption (which causes phosphorus to be lost in the urine).[5] It increases calcium and phosphorus absorption in the bowel indirectly by stimulating the kidneys to produce vitamin D which then acts on the gut.[5] PTH also causes increase bone resorption which leads to the releases calcium and phosphorus into the blood.[5]

Diagnosis

Diagnosis is by measurement of calcium, serum albumin (for correction) and PTH in blood. If necessary, measuring cAMP (cyclic AMP) in the urine after an intravenous dose of PTH can help in the distinction between hypoparathyroidism and other causes.[citation needed]

Differential diagnoses are:

Other tests include

ECG for abnormal heart rhythms, and measurement of blood magnesium levels.[citation needed
]

Treatment

Severe hypocalcaemia, a potentially life-threatening condition, is treated as soon as possible with

Long-term treatment of hypoparathyroidism is with

hypercalcemia and hypercalciuria (elevated calcium in the urine) which may lead to kidney calcification (nephrocalcinosis) and chronic kidney disease.[5][8] Calcium levels in the blood and urine (along with other electrolytes) must be monitored during long-term treatment of hypoparathyroidism and blood calcium levels are intentionally kept at the lower limits of normal, or mildly low, specifically to avoid hypercalciuria, kidney calcification and kidney damage.[5]

N-terminal 34 amino acids of parathyroid hormone, the bioactive portion of the hormone)(PTH 1-34) may be used as a second line therapy in those that have not responded to conventional therapy.[5] Both medication may be given via subcutaneous injections, but the use of pump delivery of synthetic PTH 1-34 provides the closest approach to physiologic PTH replacement therapy.[9] Recombinant human parathyroid hormone and teriparatide are also associated with a risk of hypercalcemia, hypercalciuria with associated kidney calcification and kidney damage.[5] If these medications are discontinued, they should be tapered and calcium levels should be closely monitored as the transient PTH depletion after stopping the medications can lead to bone leaching of calcium as a compensatory mechanism to increase calcium levels.[5]

A 2019 systematic review has highlighted that there is a lack of high-quality evidence for the use of vitamin D, calcium, or recombinant parathyroid hormone in the management of both temporary and long-term hypoparathyroidism following thyroidectomy.[10]

Kidney ultrasound may be considered periodically to assess for any nephrocalcinosis for those on long term therapy for hypoparathyroidism.[5]

See also

References

  1. ^
    PMID 21812031
    .
  2. .
  3. .
  4. ^ .
  5. ^ .
  6. . Retrieved 30 October 2010.
  7. .
  8. ^ Winer KK, Yanovski JA, Cutler GB Jr. Synthetic human parathyroid hormone 1-34 vs calcitriol and calcium in the treatment of hypoparathyroidism: Results of a randomized crossover trial" JAMA 1996;276:631-636
  9. ^ Winer KK, Zhang B, Shrader J, et al. Synthetic human parathyroid hormone 1-34 replacement therapy: A randomized crossover trial comparing pump versus injections in the treatment of chronic hypoparathyroidism. J Clin Endocrinol Metab. Nov.2011.
  10. PMID 31116878
    .

External links