Hypoparathyroidism
Hypoparathyroidism | |
---|---|
Specialty | Endocrinology |
Symptoms | hypocalcemia |
Medication | Recombinant human parathyroid hormone, Palopegteriparatide |
Hypoparathyroidism is decreased function of the
Signs and symptoms
The main symptoms of hypoparathyroidism are the result of the
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]
- Removal of, or trauma to, the parathyroid glands due to anterior neck surgery (including thyroid surgery) (thyroidectomy), parathyroid surgery (parathyroidectomy). This is the most common cause of hypoparathyroidism, with 78% of cases of hypoparathyroidism arising due to a complication of anterior neck surgery.[5] Although surgeons generally make attempts to spare normal parathyroid glands at surgery, inadvertent injury to the glands or their blood supply is still common. When this happens, the parathyroids may cease functioning. This is usually temporary but occasionally long term (permanent). Hypoparathyroidism may occur as a complication in up to 8% of anterior neck surgeries with 75% of those resolving by 6 months, and 25% of those having permanent hypoparathyroidism.[5]
- Kenny-Caffey Syndrome
- Autoimmune invasion and destruction is the most common non-surgical cause. It can occur as part of autoimmune polyendocrine syndromes.
- Hemochromatosiscan lead to iron accumulation and consequent dysfunction of a number of endocrine organs, including the parathyroids.
- Absence or dysfunction of the parathyroid glands is one of the components of chromosome 22q11 microdeletion syndrome(other names: DiGeorge syndrome, Schprintzen syndrome, velocardiofacial syndrome).
- Magnesium deficiency
- A defect in the calcium receptor leads to a rare congenital form of the disease
- Idiopathic (of unknown cause)
- Occasionally due to other hereditary causes (e.g. Barakat syndrome (HDR syndrome) a genetic development disorder resulting in hypoparathyroidism, sensorineural deafness, and kidney disease)
Mechanism
The parathyroid glands are so named because they are usually located behind the
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:
- Pseudohypoparathyroidism (normal PTH levels but tissue insensitivity to the hormone, associated with intellectual disability and skeletal deformities) and pseudopseudohypoparathyroidism.[7]
- Vitamin D deficiency or hereditary insensitivity to this vitamin (X-linked dominant).
- Malabsorption
- Kidney disease
- Medication: antiepileptics.
Other tests include
Treatment
Severe hypocalcaemia, a potentially life-threatening condition, is treated as soon as possible with
Long-term treatment of hypoparathyroidism is with
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]
See also
References
- ^ PMID 21812031.
- hdl:1805/22077.
- PMID 18650515.
- ^ ISBN 978-0-07-139140-5.
- ^ .
- ISBN 978-1-84076-120-7. Retrieved 30 October 2010.
- ISSN 1677-9487.
- ^ 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
- ^ 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.
- PMID 31116878.