Phenotype and Therapeutic Approach to Primary Hyperparathyroidism (PHPT) in Italy: The Multicentre Experience from “Hyperparanet”

Presentation Number: MON 352
Date of Presentation: April 3rd, 2017

Federica Saponaro*1, Filomena Cetani1, Andrea Repaci2, Valentina Camozzi3, Antonella D'Angelo4, Salvatore Minisola4, Alfredo Scillitani5, Claudia Cipri6, Serena Palmieri7, Iacopo Chiodini7, Francesco Romanelli8, Bruno Madeo9, Elena Castellano10, Laura Gianotti10, Antongiulio Faggiano11, Luisella Cianferotti12, Maria Luisa Brandi12, Sabrina Corbetta13, Maria Laura De Feo14, Andrea Palermo15, Paolo Pozzilli15 and Claudio Marcocci1
1Endocrine Unit 2, University of Pisa, Pisa-ITALY, Pisa, 2Endocrine Unit, Sant'Orsola Malpighi, Bologna, Italy, Bologna, 3Endocrine Unit, University of Padova, Padova, Italy, Padova, 4Department of Internal Medicine and Medical Disciplines'Sapienza' University, Rome, Italy., 5‘‘Casa Sollievo della Sofferenza,’’ IRCCS, San Giovanni Rotondo, Italy, 6Endocrinology and Metabolism Unit University-Hospital of Udine, Udine, Italy, 7Unit of Endocrinology and Metabolic DiseasesFondazione IRCCS Cà Granda-Ospedale Maggiore Policlinico, Milan, Italy, 8Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy, Roma, ITALY, 9Unit of Endocrinology, Modena, Italy, 10Division of Endocrinology, Diabetes and Metabolism, Santa Croce and Carle Hospital, 12100, Cuneo, Italy, 11Endocrinology, Federico II University of Naples, Napoli, Italy, 12Department of Internal Medicine, University of Florence, Largo Palagi 1, 50139, Florence, Italy, 13Area di Endocrinologia e Malattie Metaboliche, IRCCS Policlinico San Donato , Piazza Edmondo Malan, 1, 20097 San Donato Milanese , Milano , Italy., Monticello Brianza, Italy, 14Endocrinology Unit, Careggi Hospital and University of Florence, Florence, Italy., 15Department of Endocrinology and Diabetes, University Campus Bio-Medico, Rome, Italy

Abstract

The aim of this study was to evaluate the phenotype of Primary Hyperparathyroidism PHPT, the adherence to International Guidelines and the rate of surgical cure of PHPT in Italy.

From January 2014–January 2016, we conducted a prospective, multicenter (n=29 endocrine tertiary referral centers) study on patients with PHPT.

Clinical features, biochemical (serum and urinary), parathyroid imaging data at baseline and during follow-up, etiological diagnosis of PHPT and the therapeutic choice (surgery, surveillance) were recorded (www.hyperparanet.org). The study group included 604 patients, with a mean age of 61±14 yrs (range 15-88), mostly women (n=502, 83%). Sporadic patients were 566 (93.7%), mean age 63±13 yrs; the remaining 38 (6.3%, mean age 41±17yrs) were familial forms (23 MEN1, 6 FIHP, 3 HPT-JT, and 6 FHH). At least one of the following features was present in 246 (41%) patients: history of nephrolithiasis (n=277, 29%) or clinical fragility fractures (n=79, 12%), and symptoms of hypercalcemia (n=34.6%). 308 (59) were asymptomatic. Osteoporosis, (T score <-2.5 at any skeletal site by DXA), was detected in 264 (44%). PTx was advised in 281 (46.5%).

Follow up data were available in 158 symptomatic (45.8%) and 187 asymptomatic patients (54.2%). Eighty-six patients (54.4%) of the former group underwent PTx, mainly for symptomatic nephrolithiasis (n=71, 82.6%).

One hundred twenty-one asymptomatic patients (64.7%) met at least one criterion for PTx according to the 2008 International guidelines and 65 patients (53.7%) underwent surgery. Criteria for PTX were: serum calcium levels >1 mg upper normal limit (n=37, 56.9%), osteoporosis (n=35, 53.8%) and age <50 yrs (n=14, 21.5%). PTx was not performed in the remaining patients (n=56, 46.3%) despite the presence of serum calcium levels > 1 mg upper normal limit (n=12, 21.4%), osteoporosis (n=44, 78.6%) and age <50 yrs (n=11, 19.6%).

PTx was also performed in 17 of the 66 (25.7%) patients who did not meet the criteria for surgery.

A total of 168 patients underwent PTX. The histological diagnosis was single adenoma in 89% of cases, hyperplasia in 9%, atypical adenoma in 1%, and carcinoma in 1%. The majority (n=158, 94%) of patients were cured. Persistence of PHPT was observed in 10 patients, with apparently sporadic PHPT: 7 single adenomas, 2 hyperplasia and 1 double adenoma.

Patients followed without PTx showed a stable clinical and biochemical disease, after one year of follow-up.

This is the first multicenter Italian study with the aim of evaluating the current phenotype and therapeutic approach of patients with PHPT. Sporadic patients had classical symptoms in about 40%. Most patients with criteria for PTx did not undergo surgery; on the other hand about 25% of those who did not met any surgical criterion underwent PTX. These data indicate that a large proportion of endocrinologist in Italy do not follow International guidelinesfor the management of PHPT

 

Nothing to Disclose: FS, FC, AR, VC, AD, SM, AS, CC, SP, IC, FR, BM, EC, LG, AF, LC, MLB, SC, MLD, AP, PP, CM