PMID- 31557724 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200928 IS - 2049-3614 (Print) IS - 2049-3614 (Electronic) IS - 2049-3614 (Linking) VI - 8 IP - 10 DP - 2019 Oct TI - MEN1-associated primary hyperparathyroidism in the Spanish Registry: clinical characterictics and surgical outcomes. PG - 1416-1424 LID - 10.1530/EC-19-0321 [doi] AB - 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. Sixty-four 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 hypoparathyroidism and patients with different mutated exons, but a second surgery was more frequent after a less than subtotal parathyroidectomy. FAU - Lamas, Cristina AU - Lamas C AD - Department of Endocrinology and Nutrition, Complejo Hospitalario Universitario de Albacete, Albacete, Spain. FAU - Navarro, Elena AU - Navarro E AD - Department of Endocrinology and Nutrition, Hospital Universitario Virgen del Rocio, Sevilla, Spain. FAU - Casteras, Anna AU - Casteras A AD - Department of Endocrinology and Nutrition, Hospital Vall d'Hebron, Barcelona, Spain. FAU - Portillo, Paloma AU - Portillo P AD - Department of Endocrinology and Nutrition, Hospital Clinico Universitario Virgen de la Arrixaca, Murcia, Spain. FAU - Alcazar, Victoria AU - Alcazar V AD - Department of Endocrinology and Nutrition, Hospital Universitario Severo Ochoa, Leganes, Spain. FAU - Calatayud, Maria AU - Calatayud M AD - Department of Endocrinology and Nutrition, Hospital Univeristario Doce de Octubre, Madrid, Spain. FAU - Alvarez-Escola, Cristina AU - Alvarez-Escola C AD - Department of Endocrinology and Nutrition, Hospital Universitario La Paz, Madrid, Spain. FAU - Sastre, Julia AU - Sastre J AD - Department of Endocrinology and Nutrition, Complejo Hospitalario de Toledo, Hospital Virgen de la Salud, Toledo, Spain. FAU - Boix, Evangelina AU - Boix E AD - Department of Endocrinology and Nutrition, Hospital General Universitario de Elche, Elche, Spain. FAU - Forga, Lluis AU - Forga L AD - Department of Endocrinology and Nutrition, Complejo Hospitalario de Navarra, Hospital de Navarra, Pamplona, Spain. FAU - Vicente, Almudena AU - Vicente A AD - Department of Endocrinology and Nutrition, Complejo Hospitalario de Toledo, Hospital Virgen de la Salud, Toledo, Spain. FAU - Oriola, Josep AU - Oriola J AD - Biochemistry and Molecular Genetics Department, Hospital Clinic i Universitari de Barcelona, Barcelona, Spain. FAU - Mesa, Jordi AU - Mesa J AD - Department of Endocrinology and Nutrition, Hospital Vall d'Hebron, Barcelona, Spain. FAU - Valdes, Nuria AU - Valdes N AD - Department of Endocrinology and Nutrition, Hospital Universitario Central de Asturias, Oviedo, Spain. LA - eng PT - Journal Article PL - England TA - Endocr Connect JT - Endocrine connections JID - 101598413 PMC - PMC6826168 OTO - NOTNLM OT - MEN1 gene OT - hypoparathyroidism OT - multiple endocrine neoplasia type 1 OT - parathyroidectomy OT - primary hyperparathyroidism EDAT- 2019/09/27 06:00 MHDA- 2019/09/27 06:01 PMCR- 2019/09/23 CRDT- 2019/09/27 06:00 PHST- 2019/09/15 00:00 [received] PHST- 2019/09/23 00:00 [accepted] PHST- 2019/09/27 06:00 [pubmed] PHST- 2019/09/27 06:01 [medline] PHST- 2019/09/27 06:00 [entrez] PHST- 2019/09/23 00:00 [pmc-release] AID - EC-19-0321.R2 [pii] AID - EC-19-0321 [pii] AID - 10.1530/EC-19-0321 [doi] PST - ppublish SO - Endocr Connect. 2019 Oct;8(10):1416-1424. doi: 10.1530/EC-19-0321.