Hypoparathyroidism

Hypoparathyroidism is a rare condition caused by the body’s not making or releasing enough parathyroid hormone (PTH).

PTH maintains normal blood levels of calcium, phosphorus, and magnesium through its actions on the bone and kidney.

About Hypoparathyroidism

Hypoparathyroidism is a rare disorder of calcium metabolism.1

The body has four parathyroid glands, which are pea-sized and located in the neck behind the thyroid gland.2 The parathyroid glands make parathyroid hormone (PTH) and are part of the endocrine system.

PTH regulates the amount of calcium and phosphorus in the blood through its direct effects on the kidney and bone, where the body stores most of its calcium.

Decreased PTH levels lead to low calcium levels and high phosphorus levels in the blood. This imbalance can lead to problems with muscles, teeth, and nerve endings.1

Citations

  1. Society for Endocrinology. (2018). Endocrine conditions: Hypoparathyroidism. Retrieved October 2, 2018, from http://www.yourhormones.info/endocrine-conditions/hypoparathyroidism/ 
  2. Hormone Health Network (Endocrine Society). (2018). Hypoparathyroidism. Retrieved October 2, 2018, from https://www.hormone.org/diseases-and-conditions/bone-health/hypoparathyroidism 

What are the symptoms of hypoparathyroidism?

People with hypoparathyroidism have low or undetectable levels of parathyroid hormone (PTH) and low levels of calcium in the blood. Chronic symptoms may include:1

  • Tingling in the lips, fingers, and toes
  • Muscle cramps and spasms (called tetany) that cause pain in the face, hands, legs, and feet
  • Muscle weakness and generalized fatigue
  • Problems with the teeth, including weakened enamel
  • Calcium deposits in the brain or kidney
  • Seizures
  • Cataracts
  • Increased risk of kidney problems

Citations

  1. Mannstadt, M., Bilezikian, J. P., Thakker, R. V., Hannan, F. M., Clarke, B. L., Rejnmark, L., et al. (2017). Hypoparathyroidism. Nature Reviews Disease Primers, 3, 17055. Retrieved May 24, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/28857066

What causes hypoparathyroidism?

The most common cause of hypoparathyroidism is injury to the parathyroid glands during head and neck surgery. For example, thyroid surgery can damage the parathyroid glands or the surrounding tissues.1

In adults, the disorder usually is a complication of neck surgery. In children, it is most often due to an inherited disorder. Congenital hypoparathyroidism, which can be present at birth or appear in early childhood, may be caused by a genetic variant or mutation. Some cases of congenital hypoparathyroidism may be part of a syndrome, meaning it is one of several symptoms, while in other instances the problem occurs by itself.1

Autoimmune polyglandular failure type 1 (APS-1) is a rare inherited autoimmune disease including hypoparathyroidism. APS-1 is usually diagnosed in early childhood, and hypoparathyroidism is usually the first of several hormonal deficiencies to appear.2,3

Citations

  1. Mannstadt, M., Bilezikian, J. P., Thakker, R. V., Hannan, F. M., Clarke, B. L., Rejnmark, L., et al. (2017). Hypoparathyroidism. Nature Reviews Disease Primers, 3, 17055. Retrieved May 24, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/28857066
  2. Husebye, E. S., Perheentupa, J., Rautemaa, R., & Kämpe, O. (2009). Clinical manifestations and management of patients with autoimmune polyendocrine syndrome type I. Journal of Internal Medicine, 265(5), 514–529. Retrieved May 24, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/19382991
  3. Ferre, E. M., Rose, S. R., Rosenzweig, S. D., Burbelo, P. D., Romito, K. R., Niemela, J. E.,et al. (2016).Redefined clinical features and diagnostic criteria in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy. JCI Insight, 1(13), pii: e88782. Retrieved May 24, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/27588307

How do healthcare providers diagnose hypoparathyroidism?

To diagnose hypoparathyroidism, a healthcare provider will usually start with a blood test to determine the levels of the following:1,2

  • Parathyroid hormone
  • Calcium
  • Phosphorus
  • Creatinine
  • Magnesium

A urine test determines how much calcium the kidneys are releasing.

Citations

  1. MedlinePlus. (2018). Hypoparathyroidism. Retrieved May 24, 2019, from https://medlineplus.gov/ency/article/000385.htm
  2. National Organization for Rare Disorders. (2017). Hypoparathyroidism. Retrieved May 24, 2019, from https://rarediseases.org/rare-diseases/hypoparathyroidism 

How is hypoparathyroidism treated?

Unlike most other hormonal deficits, hypoparathyroidism is not treated with replacement of the missing hormone, parathyroid hormone (PTH).

Currently, the standard treatment for hypoparathyroidism consists of activated vitamin D (calcitriol) and calcium supplements. Some people may also need magnesium supplementation. Conventional therapy requires many pills taken throughout the day.1,2

Diet recommendations usually include eating foods high in calcium, such as dairy products, breakfast cereals, fortified orange juice, and green, leafy vegetables, or avoiding foods high in phosphorus, such as meat, poultry, fish, nuts, whole grains, and beans.3,4

Conventional therapy with vitamin D and calcium may lead to a buildup of calcium in the kidneys. This buildup may lead to problems, including kidney stones and deposits of calcium, reduced kidney function, tissue damage, or even kidney failure.

The development of PTH as a replacement therapy in patients with hypoparathyroidism was first explored by NICHD investigators beginning in 1992. Since then, NICHD researchers have conducted a series of classic studies evaluating PTH 1-34 regimens, including once-daily and twice-daily PTH injections without concurrent use of calcitriol or calcium supplements in adults and children. Recently, these NICHD researchers introduced PTH delivery by an insulin pump in two landmark studies in adults5 and in children.6,7,8,9,10 In January 2015, the U.S. Food and Drug Administration approved PTH 1-84 for adult hypoparathyroidism patients who do not respond well to standard treatment, as an add-on to that treatment.11

Citations

  1. National Library of Medicine. (2016). Hypoparathyroidism. Retrieved October 10, 2018, from http://www.nlm.nih.gov/medlineplus/ency/article/000385.htm
  2. Bollerslev, J., Rejnmark, L., Marcocci, C., Shoback, D. M., Sitges-Serra, A., van Biesen, W., & Dekkers, O. M.; European Society of Endocrinology. (2015). European Society of Endocrinology Clinical Guideline: Treatment of chronic hypoparathyroidism in adults. European Journal of Endocrinology, 173(2), G1–G20. Retrieved May 24, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/26160136
  3. Hormone Health Network (Endocrine Society). (2018). Hypoparathyroidism. Retrieved October 2, 2018, from https://www.hormone.org/diseases-and-conditions/bone-health/hypoparathyroidism  
  4. U.S. Department of Agriculture. (2018). Non-dairy sources of calcium. Retrieved May 29, 2019, from https://www.choosemyplate.gov/dairy-calcium-sources
  5. Winer, K. K., Zhang, B., Shrader, J. A., Peterson, D., Smith, M., Albert, P. S., & Cutler, G. B. Jr. (2012). Synthetic human parathyroid hormone 1-34 replacement therapy: A randomized crossover trial comparing pump versus injections in the treatment of chronic hypoparathyroidism. Journal of Clinical Endocrinology and Metabolism, 97(2), 391–399. Retrieved May 3, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/22090268
  6. Winer, K. K., Fulton, K. A., Albert, P. S., & Cutler, G. B. Jr. (2014). Effects of pump versus twice-daily injection delivery of synthetic parathyroid hormone 1-34 in children with severe congenital hypoparathyroidism. Journal of Pediatrics, 165(3), 556–563.e1. Retrieved May 3, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/24948345
  7. Winer, K. K. (2019). Advances in the treatment of hypoparathyroidism with PTH 1-34. Bone, 120, 535–541. Retrieved March 19, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/30243992
  8. Saraff, V., Rothenbuhler, A., Högler, W., & Linglart, A. (2018). Continuous subcutaneous recombinant parathyroid hormone (1-34) infusion in the management of childhood hypoparathyroidism associated with malabsorption. Hormone Research in Paediatrics, 89(4), 271–277. Retrieved May 24, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/28926829
  9. Mannstadt, M., Clarke, B. L., Vokes, T., Brandi, M. L., Ranganath, L., Fraser, W. D., et al. (2013). Efficacy and safety of recombinant human parathyroid hormone (1-84) in hypoparathyroidism (REPLACE): A double-blind, placebo-controlled, randomised phase 3 study. The Lancet Diabetes & Endocrinology, 1(4), 275–283. Retrieved May 24, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/24622413
  10. Rejnmark, L., Sikjaer, T., Underbjerg, L., & Mosekilde, L. (2013). PTH replacement therapy of hypoparathyroidism. Osteoporosis International, 24(5), 1529–1536. Retrieved May 24, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/23184178
  11. U.S. Food and Drug Administration. (2018). Novel drug approvals for 2015. Retrieved May 28, 2019, from https://www.fda.gov/drugs/new-drugs-fda-cders-new-molecular-entities-and-new-therapeutic-biological-products/novel-drug-approvals-2015
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