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Parathyroid surgery for inherited syndromes

Authors
Tina WF Yen, MD, MS
Tracy S Wang, MD, MPH
Section Editor
Sally E Carty, MD, FACS
Deputy Editor
Wenliang Chen, MD, PhD

INTRODUCTION

Primary hyperparathyroidism (HPT) is a disorder of the parathyroid glands in which one or more of the glands secrete an excess amount of parathyroid hormone. Although most commonly a sporadic disease, primary HPT can be a part of an inherited genetic syndrome in a small subset of patients. These inherited syndromes include multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2A (MEN2A), hyperparathyroid-jaw tumor syndrome (HPT-JT), and familial isolated hyperparathyroidism (FIHPT). An inherited form of HPT should be suspected in younger patients, especially those with multiglandular disease, and those with a family history of an endocrinopathy that is known to be associated with HPT [1].

Parathyroid surgery for the treatment of inherited syndromes will be reviewed here. The inherited syndromes are discussed in detail elsewhere. (See "Multiple endocrine neoplasia type 1: Treatment" and "Approach to therapy in multiple endocrine neoplasia type 2" and "Pathogenesis and etiology of primary hyperparathyroidism", section on 'Familial hyperparathyroidism'.)

INDICATIONS

The indications for parathyroidectomy are the same for patients with either sporadic or inherited forms of primary hyperparathyroidism (HPT) [2-16]. Parathyroidectomy is required to treat HPT in symptomatic patients (eg, nephrolithiasis) and is the preferred treatment in asymptomatic patients who meet the Fourth International Workshop on Asymptomatic Primary Hyperparathyroidism criteria (table 1). (See "Primary hyperparathyroidism: Management", section on 'Candidates for surgery'.)

In addition, the potential benefits of parathyroidectomy should be discussed with MEN1 patients who have severe, medically refractory peptic ulcer disease or other symptoms due to a gastrinoma (ie, the Zollinger-Ellison syndrome). Hypercalcemia worsens hypergastrinemia, which can exacerbate peptic ulcer disease. Successful parathyroidectomy may reduce gastrin secretion [14,17]. (See "Multiple endocrine neoplasia type 1: Treatment", section on 'Indications for surgery' and "Zollinger-Ellison syndrome (gastrinoma): Clinical manifestations and diagnosis".)

SURGICAL APPROACHES

Surgical approaches to patients with inherited hyperparathyroidism (HPT) vary depending upon their individual syndromes [8]. The principles of parathyroid surgery, however, remain the same.

                                 

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Literature review current through: Nov 2016. | This topic last updated: Mon Apr 11 00:00:00 GMT 2016.
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