MEN1-associated primary hyperparathyroidism in the Spanish Registry: Clinical characterictics and surgical outcomes
Entity
UAM. Departamento de Medicina; Instituto de Investigación Sanitaria Hospital Universitario de La Paz (IdiPAZ)Publisher
BioScientifica Ltd.Date
2019Citation
10.1530/EC-19-0321
Endocrine Connections 8 (2019): 1416–1424
ISSN
2049-3614DOI
10.1530/EC-19-0321Funded by
The Spanish Registry of Multiple Endocrine Neoplasia, Pheochromocytmas and Paragangliomas is supported by IPSEN Pharmaceutical.Editor's Version
https://doi.org/10.1530/EC-19-0321Subjects
Multiple endocrine neoplasia type 1; MEN1 gene; Primary hyperparathyroidism; Parathyroidectomy; Hypoparathyroidism; MedicinaRights
© 2019 The authorsAbstract
Primary hyperparathyroidism is the most frequent manifestation of multiple endocrine
neoplasia type 1 (MEN1) syndrome. Bone and renal complications are common. Surgery
is the treatment of choice, but the best timing for surgery is controversial and predictors
of persistence and recurrence are not well known. Our study describes the clinical
characteristics and the surgical outcomes, after surgery and in the long term, of the
patients with MEN1 and primary hyperparathyroidism included in the Spanish Registry
of Multiple Endocrine Neoplasia, Pheochromocytomas and Paragangliomas (REGMEN).
Eighty-nine patients (49 men and 40 women, 34.2 ± 13 years old) were included. Sixtyfour
out of the 89 underwent surgery: a total parathyroidectomy was done in 13 patients,
a subtotal parathyroidectomy in 34 and a less than subtotal parathyroidectomy in 15.
Remission rates were higher after a total or a subtotal parathyroidectomy than after a less
than subtotal (3/4 and 20/22 vs 7/12, P < 0.05), without significant differences in permanent
hypoparathyroidism (1/5, 9/23 and 0/11, N.S.). After a median follow-up of 111 months,
20 of the 41 operated patients with long-term follow-up had persistent or recurrent
hyperparathyroidism. We did not find differences in disease-free survival rates between
different techniques, patients with or without permanent hypopar athyroidism and patients
with different mutated exons, but a second surgery was more freq uent after a less than
subtotal parathyroidectomy
Files in this item
Google Scholar:Lamas, Cristina
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Navarro, Elena
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Casterás, Anna
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Portillo, Paloma
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Alcázar, Victoria
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Calatayud, María
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Álvarez-Escolá, Cristina
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Sastre, Julia
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Boix, Evangelina
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Forga, Lluis
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Vicente, Almudena
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Oriola, Josep
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Mesa, Jordi
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Valdés, Nuria
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