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- W3037112686 abstract "Abstract Primary hyperparathyroidism (PHPT) resulting from multiple endocrine neoplasia (MEN) type 1 or MEN 2A is a relatively uncommon cause of PHPT. However, it is important that the physicians treating patients with inherited PHPT be well versed in the management of this condition. The diagnosis of MEN 1 should be considered in patients with two or more MEN 1–related tumors or a clinical history otherwise suggestive of MEN 1. PHPT is often the first clinical manifestation of disease in the proband of an MEN 1 family or in at-risk members of a known MEN 1 kindred. The diagnosis of MEN 2A is typically made before the onset of PHPT because of the near 100% penetrance of medullary thyroid cancer (MTC). Genetic testing (before surgery on the neck) is particularly important in patients with MEN 2A because of the strong genotype-phenotype correlation of the specific rearranged during transfection (RET) mutation identified. We prefer to know the RET status of the patient before thyroid/parathyroid surgery because it will indicate the location of a parathyroid autograft if one is performed. Indications for surgery in MEN–associated PHPT are no different than for those with sporadic PHPT. The goals of initial parathyroidectomy in patients with MEN–associated PHPT are to achieve eucalcemia and minimize the risk of immediate hypoparathyroidism. Forearm autografting of remnant parathyroid tissue should be considered for all patients with MEN 2A–associated PHPT and invasive MTC as well as all patients with inherited PHPT who undergo reoperative neck surgery." @default.
- W3037112686 created "2020-07-02" @default.
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- W3037112686 date "2021-01-01" @default.
- W3037112686 modified "2023-09-24" @default.
- W3037112686 title "Parathyroid Management in the MEN Syndromes" @default.
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- W3037112686 doi "https://doi.org/10.1016/b978-0-323-66127-0.00062-4" @default.
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