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Hypoparathyreoïdie

  • Article
  • 2021-02-28

Hypoparathyroidism is a rare disorder of calcium metabolism. 1

The body has four parathyroid glands, the size of a pea 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 kidneys and bone, where the body stores most of the calcium.

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

Symptoms

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

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

Therapy

Unlike most other hormonal deficiencies, 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 to be taken during the day. 1, 2

Diet recommendations usually include eating foods high in calcium, such as dairy products, 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 can lead to a build-up of calcium in the kidneys. This build-up can lead to problems including kidney stones and calcium deposits, decreased 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 researchers starting 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 concomitant use of calcitriol or calcium supplements in adults and children. Recently, these NICHD researchers introduced PTH delivery from an insulin pump in two groundbreaking studies in adults 5 and in children. 6, 7, 8, 9, 10 In January 2015, the United States Food and Drug Administration approved PTH 1-84 for adult patients with hypoparathyroidism who do not respond well to standard treatment, in addition to that treatment. 11

  1. National Library of Medicine. (2016). Hypoparathyroidism . Retrieved on October 10, 2018, from http://www.nlm.nih.gov/medlineplus/ency/article/000385.htm
  2. Bollerslev, J., Rejnmark, L., Marcocci, C., Shoback, DM, Sitges-Serra, A., van Biesen, W., & Dekkers, OM; European Society of Endocrinology. (2015). Clinical guideline European Society of Endocrinology: 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 on October 2, 2018, from https://www.hormone.org/diseases-and-conditions/bone-health/hypoparathyroidism
  4. United States Department of Agriculture. (2018). Non-dairy sources of calcium. Retrieved on May 29, 2019, from https://www.choosemyplate.gov/dairy-calcium-sources
  5. Winer, KK, Zhang, B., Shrader, JA, Peterson, D., Smith, M., Albert, PS, and Cutler, GB Jr. (2012). Synthetic human parathyroid hormone 1-34 replacement therapy: a randomized crossover study comparing pump versus injections in the treatment of chronic hypoparathyroidism. Journal of Clinical Endocrinology and Metabolism, 97 (2), 391-399. Retrieved on May 3, 2019, fromn https://www.ncbi.nlm.nih.gov/pubmed/22090268
  6. Winer, KK, Fulton, KA, Albert, PS, and Cutler, GB Jr. (2014). Effects of pump versus twice daily injection of synthetic parathyroid hormone 1-34 in children with severe congenital hypoparathyroidism. Journal of Pediatrics, 165 (3), 556-563.e1. Retrieved on May 3, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/24948345
  7. Winer, KK (2019). Advances in the Treatment of Hypoparathyroidism with PTH 1-34. Bot, 120, 535-541. Retrieved on March 19, 2019, from https://www.ncbi.nlm.nih.gov/pubmed/30243992
  8. Saraff, V., Rothenbuhler, A., Högler, W., & Linglart, A. (2018). Continuous subcutaneous infusion of recombinant parathyroid hormone (1-34) in the treatment of hypoparathyroidism in children 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, BL, Vokes, T., Brandi, ML, Ranganath, L., Fraser, WD, et al. (2013). Efficacy and safety of recombinant human parathyroid hormone (1-84) in hypoparathyroidism (REPLACE): a double-blind, placebo-controlled, randomized 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., and Mosekilde, L. (2013). PTH replacement therapy for hypoparathyroidism. Osteoporosis International , 24 (5), 1529-1536. Retrieved May 24, 2019 from https://www.ncbi.nlm.nih.gov/pubmed/23184178
  11. United States Food and Drug Administration. (2018). New drug approvals for 2015. Retrieved on 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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