HypoparaNet: A Database of Chronic Hypoparathyroidism Based on Expert Medical-Surgical Centers in Italy

Abstract

Hypoparathyroidism is a rare disease characterized by low serum calcium levels and absent or deficient parathyroid hormone level. Regarding the epidemiology of chronic hypoparathyroidism, there are limited data in Italy and worldwide. Therefore, the purpose of this study was to build a unique database of patients with chronic hypoparathyroidism, derived from the databases of 16 referral centers for endocrinological diseases, affiliated with the Italian Society of Endocrinology, and four centers for endocrine surgery with expertise in hypoparathyroidism, to conduct an epidemiological analysis of chronic hypoparathyroidism in Italy. The study was approved by the Institutional Review Board. A total of 537 patients with chronic hypoparathyroidism were identified. The leading etiology was represented by postsurgical hypoparathyroidism (67.6%), followed by idiopathic hypoparathyroidism (14.6%), syndromic forms of genetic hypoparathyroidism (11%), forms of defective PTH action (5.2%), non-syndromic forms of genetic hypoparathyroidism (0.9%), and, finally, other forms of acquired hypoparathyroidism, due to infiltrative diseases, copper or iron overload, or ionizing radiation exposure (0.7%). This study represents one of the first large-scale epidemiological assessments of chronic hypoparathyroidism based on data collected at medical and/or surgical centers with expertise in hypoparathyroidism in Italy. Although the study presents some limitations, it introduces the possibility of a large-scale national survey, with the final aim of defining not only the prevalence of chronic hypoparathyroidism in Italy, but also standards for clinical and therapeutic approaches.

A registry for patients with chronic hypoparathyroidism in Russian adults

“…This is the first report of clinical presentations and treatment approaches in Russian patients with hypoparathyroidism referred to specialized care. The analysis has shown that the most frequent cause of hypoparathyroidism was neck surgery, which made middle-aged females the most common patients with hypoparathyroidism, as described in literature (3,5,7,8,10,11). Postsurgical hypoparathyroidism occurred after a total thyroidectomy in most cases, following by a parathyroidectomy and then a partial thyroidectomy.…”

“…In cases where this data were available, magnesium deficiency was confirmed in 50% of cases (magnesium levels below the reference range) and vitamin D deficiency in 37.5% of all cases (serum 25 hydroxyvitamin D concentration was under 30 ng/dL). Hypoparathyroidism is characterized by low bone metabolism, explaining why bone mineral density (BMD) is usually normal or higher than normal, except after a parathyroidectomy for PHPT (8,12,23,24). A DXA exam was carried out at baseline evaluation for half of the patients and showed normal to high values and a low bone turnover profile typical of the disease.…”

“…The estimated prevalence of chronic hypoparathyroidism ranges significantly: 5.3–58 per 100,000 in recent studies ( 1 , 2 , 3 , 4 , 5 , 6 ). The most common cause is post-surgical parathyroid damage which causes chronic hypoparathyroidism when PTH is consistently inappropriately low 6 months after surgery ( 2 , 7 , 8 , 9 , 10 ). However, due to the absence of a standard definition of hypocalcemia, the incidence of postsurgical hypoparathyroidism varies 0–20.2% ( 3 , 8 , 9 , 10 , 11 , 12 ).…”

“…The most common cause is post-surgical parathyroid damage which causes chronic hypoparathyroidism when PTH is consistently inappropriately low 6 months after surgery ( 2 , 7 , 8 , 9 , 10 ). However, due to the absence of a standard definition of hypocalcemia, the incidence of postsurgical hypoparathyroidism varies 0–20.2% ( 3 , 8 , 9 , 10 , 11 , 12 ). There are other less-frequent etiologies which may be autoimmune, genetic, infiltrative, or idiopathic hypoparathyroidism ( 5 , 8 , 13 ).…”

Hypoparathyroidism and Pseudohypoparathyroidism in Pregnancy: An Italian Retrospective Observational Study

“…This project involved nine Italian referral centers for endocrine diseases, a liated with the Italian Society of Endocrinology (Società Italiana di Endocrinologia, S.I.E.) and involved in the "HypoparaNet" research group (HypoPT working group) (27,28).…”