Hypoparathyroidism is a disease characterized by inadequately low circulating concentrations of parathyroid hormone (PTH) resulting in low calcium levels and increased phosphate levels in the blood. Symptoms of the disease result from increased neuromuscular irritability caused by hypocalcaemia and include tingling, muscle cramps and seizures. The most common cause of the disease is inadvertent removal of, or injury to, the parathyroid glands during neck surgery, followed by genetic, idiopathic and autoimmune aetiologies. Conventional treatment includes activated vitamin D and/or calcium supplements, but this treatment does not fully replace the functions of PTH and can lead to short-term problems (such as hypocalcaemia, hypercalcaemia and increased urinary calcium excretion) and long-term complications (which include nephrocalcinosis, kidney stones and brain calcifications). PTH replacement has emerged as a new treatment option. Clinical trials using human PTH(1–34) and PTH(1–84) showed that this treatment was safe and effective in studies lasting up to 6 years. Recombinant human PTH(1–84) has been approved in the United States and Europe for the management of hypoparathyroidism; however, its effect on long-term complications is still being evaluated. Clinical practice guidelines, which describe the consensus of experts in the field, have been published and recognize the need for more research to optimize care. In this Primer, we summarize current knowledge of the prevalence, pathophysiology, clinical presentation and management of hypoparathyroidism.
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The authors thank J. Warshauer (Division of Endocrinology, Metabolism, and Diabetes, University of California, San Francisco, USA) for providing Figure 5a,b, and H. Jüppner (Endocrine Unit and Pediatric Nephrology, Massachusetts General Hospital, Boston, Massachusetts, USA) for comments.
M.M. has received consulting fees and a research grant from Shire Pharmaceuticals. J.P.B. is a consultant for Shire Pharmaceuticals. R.V.T. has received grant funding from NPS/Shire Pharmaceuticals, GlaxoSmithKline, Novartis Pharma AG and the Marshall Smith Syndrome Foundation, and he is a medical adviser for the patient charity HypoPara UK. F.M.H. has received grant funding from NPS/Shire Pharmaceuticals and GlaxoSmithKline. B.L.C. has received research grants from Shire Pharmaceuticals and has received honoraria from Amgen. L.R. has received honoraria and speaker fees from Amgen, Eli Lilly, Novo Nordic, Takeda Pharmaceuticals, NPS Pharmaceuticals, Shire, Bristol-Myers Squibb, Abbott, and Boehringer Ingelheim Denmark. T.J.V. is a consultant and investigator for Shire Pharmaceuticals. D.M.S. is a consultant for Shire Pharmaceuticals and Ascendis Pharmaceuticals. D.M.M. and K.K.W. declare no competing interests.
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Mannstadt, M., Bilezikian, J., Thakker, R. et al. Hypoparathyroidism. Nat Rev Dis Primers 3, 17055 (2017). https://doi.org/10.1038/nrdp.2017.55
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