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.
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Acknowledgements
SHIRE, pharmaceutical company.
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All data analyzed during this study are included in this published article.
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This study was funded by Shire, a pharmaceutical company.
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MLB was the coordinator of the project and designed the study. All authors contributed to the collection of clinical data, and the paper was drafted by GM, LC and MLB. SP was responsible for statistical analysis of the data. All authors revised the paper critically for intellectual content and approved the final version. All authors agree to be accountable for the work and to ensure that any questions relating to the accuracy and integrity of the paper are investigated and properly resolved.
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G. Mantovani: member of advisory boards for Shire; C. Marcocci: consultant for Shire Italia. M.L. Brandi: is a consultant for Alexion, Bruno Farmaceutici, Shire, Servier, Kyowa Kirin; academic grants and/or speaker: Abiogen, Alexion, Amgen, Bruno Farmaceutici, Eli Lilly, Kyowa Kirin, MSD, NPS, Servier, Shire, SPA; she has received honoraria from: Amgen, Bruno Farmaceutici, Kyowa Kirin. The other authors declare that they have no competing interests that could be perceived as prejudicing the impartiality of the research reported.
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The study was approved by the Institutional Review Board (Comitato Etico Area Vasta Centro, AUOC, Florence, Italy); Number: 10641_oss.
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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study formal consent is not required.
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Marcucci, G., Cianferotti, L., Parri, S. et al. HypoparaNet: A Database of Chronic Hypoparathyroidism Based on Expert Medical-Surgical Centers in Italy. Calcif Tissue Int 103, 151–163 (2018). https://doi.org/10.1007/s00223-018-0411-7
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DOI: https://doi.org/10.1007/s00223-018-0411-7