Skip to main content

CASE REPORT article

Front. Endocrinol.
Sec. Bone Research
Volume 15 - 2024 | doi: 10.3389/fendo.2024.1496386
This article is part of the Research Topic Phosphate handling: from cells to human View all 3 articles

Prolonged and severe hungry bone syndrome after parathyroidectomy in X-linked hypophosphatemia

Provisionally accepted
Giulia Puliani Giulia Puliani 1Valeria Hasenmajer Valeria Hasenmajer 2Matteo Spaziani Matteo Spaziani 2Federico Frusone Federico Frusone 2Chiara Tarantino Chiara Tarantino 2Francesco Angelini Francesco Angelini 2Ludovica Vincenzi Ludovica Vincenzi 2Riccardo Lubrano Riccardo Lubrano 2Alessia Marcellino Alessia Marcellino 2Marco Biffoni Marco Biffoni 2Andrea M. Isidori Andrea M. Isidori 2*
  • 1 Regina Elena National Cancer Institute, Hospital Physiotherapy Institutes (IRCCS), Rome, Lazio, Italy
  • 2 Sapienza University of Rome, Rome, Italy

The final, formatted version of the article will be published soon.

    Tertiary hyperparathyroidism is characterized by hypercalcemia resulting from autonomous parathyroid hormone production and usually occurs after a prolonged period of secondary hyperparathyroidism. This condition can be a complication of X-linked hypophosphatemia (XLH), a rare genetic disease characterized by renal phosphate loss and consequent hypophosphatemia. Parathyroidectomy is considered the first-line therapy but surgical intervention can be complicated by hungry bone syndrome.A male Caucasian patient presented with XLH, diagnosed at the age of 3 years. At the age of 21, tertiary hyperparathyroidism occurred. Neck ultrasonography, neck magnetic resonance imaging, and 99 Tc-sestamibi parathyroid scintigraphy revealed two hyperplastic parathyroid glands. To minimize the risk of hypercalcemia calcimimetic therapy was initiated. After 6 months and preparation with 1α-hydroxyvitamin D, the patient underwent total parathyroidectomy with autotransplantation of half of a parathyroid gland into the sternocleidomastoid muscle. Histopathological examination revealed diffuse microscopical hyperplasia of the parathyroid glands. Despite oral supplementation with calcium carbonate and calcitriol, severe hypocalcemia developed on the second postoperative day, attributable to hungry bone syndrome. This finding was confirmed by an increase in bone turnover markers and a reduction in urinary calcium excretion. Hypocalcemia correction required continuous infusion of calcium gluconate for over 2 months. After approval, the patient began burosumab therapy with significant benefits. This case illustrates the complexity of treating tertiary hyperparathyroidism and mineral metabolism in patients with XLH. The hungry bone syndrome can complicate parathyroidectomy, exposing the patients to life-threatening risks. Burosumab therapy may reduce the risk of tertiary hyperparathyroidism developing in these patients.

    Keywords: tertiary hyperparathyroidism, Hungry bone syndrome, X-linked hypophosphatemia, Burosumab, FGF23, Parathyroidectomy

    Received: 14 Sep 2024; Accepted: 02 Dec 2024.

    Copyright: © 2024 Puliani, Hasenmajer, Spaziani, Frusone, Tarantino, Angelini, Vincenzi, Lubrano, Marcellino, Biffoni and Isidori. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence: Andrea M. Isidori, Sapienza University of Rome, Rome, Italy

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.