Hypoparathyroidism is a rare endocrine disorder characterized by low or insufficient parathyroid hormone (PTH) concentrations leading to hypocalcemia, hyperphosphatemia, and markedly reduced bone turnover. Despite being a rare disease, hypoparathyroidism has a profound impact on affected patients.
Recent epidemiologic surveys demonstrate a prevalence of between 5.3 and 40/100 000, confirming the orphan status of this disease. There is a female predominance, and anterior neck surgery is the most common etiology. Recent studies have better elucidated the chronic manifestations of the disease, impacting quality of life and multiple organ systems including the renal, cardiovascular, and skeletal systems. There are now data on longer term use of parathyroid hormone (PTH) and PTH analogs.
This review focuses on recent contributions to the literature on the prevalence and epidemiology of the disease, risk of chronic manifestations, and treatment with PTH(1-34) and rhPTH(1-84). Further research is needed to determine the pathophysiology of complications in hypoparathyroidism and whether interventions can decrease future risk of these complications. In addition, further data are needed with regards to more physiologic dosing regimens and long-term treatment with PTH and PTH analogs.
aBone Metabolism Program, Division of Endocrinology, Department of Medicine, Lenox Hill Hospital and Zucker School of Medicine at Hofstra/Northwell
bInternational Education and Research, Emeritus, Division of Endocrinology, Department of Medicine, Columbia University College of Physicians & Surgeons, New York, New York, USA
Correspondence to John P. Bilezikian, MD, PhD, Professor of Pharmacology, Vice Chair for International Education and Research, Chief, Emeritus, Division of Endocrinology, Department of Medicine, Columbia University College of Physicians & Surgeons, 630 West 168th Street, PH 8E 105G, New York, NY 10032, USA. Tel: +1 212 305 6257; fax: +1 212 305 8466; e-mail: email@example.com