title: Cinacalcet therapy in patients affected by primary hyperparathyroidism associated to Multiple Endocrine Neoplasia Syndrome type 1 (MEN1).
abstract: Primary hyperparathyroidism is the main endocrinopathy associated with Multiple Endocrine Neoplasia type 1 syndrome. Cinacalcet is a calcimimetic agent licensed for the treatment of secondary hyperparathyroidism in patients with end-stage renal disease, and for the reduction of marked hypercalcemia in patients with parathyroid carcinoma and sporadic hyperparathyroidism requiring surgery but for whom parathyroidectomy is contraindicated. It may provide a medical alternative for the management of primary hyperparathyroidism in subjects affected by Multiple Endocrine Neoplasia type 1. In this longitudinal, intervention study, 33 MEN1 patients had been enrolled, 10 males and 23 females with a mean age of 40 ± 11.9 years, range 20-63. Primary hyperparathyroidism was the first clinical manifestation in 12 patients. All subjects commenced with Cinacalcet 30 mg/day, 22 patients starting therapy with calcimimetics as an alternative to surgery, and 11 patients opting for the medication after the onset of persistent post-surgical primary hyperparathyroidism. Duration of follow-up was 12 months. The results of this study show significant reductions in serum calcium. The changes in hormonal secretions of pituitary and gastroenteropancreatic glands were not significant, demonstrating the overall safety of this drug in this disease. Cinacalcet has been well tolerated by 28 patients, whereas five individuals complained of heartburn and grade 1 nausea, which did not prevent the completion of the study. In conclusion, Cinacalcet has resulted to be well tolerated and safe in patients with MEN1 syndrome and the calcium homeostasis was stabilized.
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Palagi 1, 50139, Florence, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Giusti
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Palagi 1, 50139, Florence, Italy.
authors.forename: Luisella
authors.initials: L
authors.lastname: Cianferotti
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Palagi 1, 50139, Florence, Italy.
authors.forename: Giorgio
authors.initials: G
authors.lastname: Gronchi
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Palagi 1, 50139, Florence, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Cioppi
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Palagi 1, 50139, Florence, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Masi
authors.affiliation: Department of Clinical Medicine and Surgery, Federico II University, Naples, Italy.
authors.forename: Antongiulio
authors.initials: A
authors.lastname: Faggiano
authors.affiliation: Department of Clinical Medicine and Surgery, Federico II University, Naples, Italy.
authors.forename: Annamaria
authors.initials: A
authors.lastname: Colao
authors.affiliation: Department of Internal Medicine and Endocrine Sciences, University of Perugia, Perugia, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Ferolla
authors.affiliation: Department of Surgery and Translational Medicine, University of Florence, Largo Palagi 1, 50139, Florence, Italy. marialuisa.brandi@unifi.it.
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Brandi
mesh_terms: D000236:Adenoma / Q000097:blood / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism; D000328:Adult; D002118:Calcium / Q000097:blood; D000069449:Cinacalcet / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D049950:Hyperparathyroidism, Primary / Q000097:blood / Q000188:drug therapy* / Q000209:etiology; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000097:blood / Q000150:complications / Q000188:drug therapy*; D010281:Parathyroid Hormone / Q000097:blood; D010282:Parathyroid Neoplasms / Q000097:blood / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism; D016105:Parathyroidectomy; D055815:Young Adult
chemical_list: D010281:Parathyroid Hormone; D002118:Calcium; D000069449:Cinacalcet
keywords: Calcimimetic; Neuroendocrine tumor; PHPT; Pituitary tumor