PMID- 35407574
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20220419
IS  - 2077-0383 (Print)
IS  - 2077-0383 (Electronic)
IS  - 2077-0383 (Linking)
VI  - 11
IP  - 7
DP  - 2022 Apr 1
TI  - Management and Long-Term Follow-Up of Hyperparathyroidism in Multiple Endocrine 
      Neoplasia Type 1: Single Center Experience.
LID - 10.3390/jcm11071967 [doi]
LID - 1967
AB  - BACKGROUND: Primary hyperparathyroidism (PHPT) in the most common and earliest 
      manifestation of multiple endocrine neoplasia type-1 (MEN1). Epidemiological data 
      have been reported in MEN1 patients but data on long-term follow-up focusing on 
      PHPT are scarce. METHODS: In this retrospective cohort study, we included 
      patients diagnosed with MEN1-related PHPT that were under regular follow-up in 
      our institution. RESULTS: Data on 68 patients (39 males), with a mean age at 
      MEN1-diagnosis of 39 +/- 13.06 years, were analyzed. Pancreatic neuroendocrine 
      tumors were encountered in 82% (71% nonsecreting) followed by pituitary adenomas 
      in 66% (49% nonsecreting). Mean age at PHPT diagnosis was 35.2 +/- 4.0 years. 
      Parathyroidectomy was performed in 57 patients (82.3%), of whom 56% achieved 
      long-term remission, while 12.2% and 31.5% had persistent and recurrent disease, 
      respectively (median follow-up of 4 years; range 1-21 years). Cinacalcet restored 
      serum calcium levels in 33.8%, both as first and as a second line treatment. 
      Permanent hypoparathyroidism occurred in 19.2%. MEN1 pathogenic variants were 
      identified in 77.2% of the tested individuals, but no genotype-phenotype 
      associations were reported. CONCLUSIONS: MEN1-related PHPT involves a 
      multiglandular disease and its management remains a therapeutic challenge, as 
      recurrent disease can develop even after 20 years of follow-up. Prolonged 
      follow-up of these patients at referral centers is critical for their optimal 
      management.
FAU - Yavropoulou, Maria P
AU  - Yavropoulou MP
AUID- ORCID: 0000-0002-6501-3692
AD  - Endocrinology Unit, First Department of Propaedeutic and Internal Medicine, 
      Centre of Expertise for Rare Endocrine Disease (C.E.R.E.D.), Medical School, 
      National and Kapodistrian University of Athens, 11527 Athens, Greece.
FAU - Vlachou, Sofia
AU  - Vlachou S
AD  - Endocrinology Unit, First Department of Propaedeutic and Internal Medicine, 
      Centre of Expertise for Rare Endocrine Disease (C.E.R.E.D.), Medical School, 
      National and Kapodistrian University of Athens, 11527 Athens, Greece.
FAU - Tsoli, Marina
AU  - Tsoli M
AUID- ORCID: 0000-0002-6013-5893
AD  - Endocrinology Unit, First Department of Propaedeutic and Internal Medicine, 
      Centre of Expertise for Rare Endocrine Disease (C.E.R.E.D.), Medical School, 
      National and Kapodistrian University of Athens, 11527 Athens, Greece.
FAU - Fostira, Florentia
AU  - Fostira F
AUID- ORCID: 0000-0003-2751-2332
AD  - Molecular Diagnostics Laboratory, National Center for Scientific Research 
      "Demokritos", Institute of Nuclear & Radiological Sciences & Technology, Energy & 
      Safety, 15341 Athens, Greece.
FAU - Kaltsas, Gregory
AU  - Kaltsas G
AD  - Endocrinology Unit, First Department of Propaedeutic and Internal Medicine, 
      Centre of Expertise for Rare Endocrine Disease (C.E.R.E.D.), Medical School, 
      National and Kapodistrian University of Athens, 11527 Athens, Greece.
FAU - Kassi, Eva
AU  - Kassi E
AD  - Endocrinology Unit, First Department of Propaedeutic and Internal Medicine, 
      Centre of Expertise for Rare Endocrine Disease (C.E.R.E.D.), Medical School, 
      National and Kapodistrian University of Athens, 11527 Athens, Greece.
LA  - eng
PT  - Journal Article
DEP - 20220401
PL  - Switzerland
TA  - J Clin Med
JT  - Journal of clinical medicine
JID - 101606588
PMC - PMC8999236
OTO - NOTNLM
OT  - MEN1
OT  - cinacalcet
OT  - long-term follow-up
OT  - permanent hypoparathyroidism
OT  - primary hyperparathyroidism
COIS- The authors declare no conflict of interest.
EDAT- 2022/04/13 06:00
MHDA- 2022/04/13 06:01
PMCR- 2022/04/01
CRDT- 2022/04/12 01:07
PHST- 2022/02/22 00:00 [received]
PHST- 2022/03/13 00:00 [revised]
PHST- 2022/03/28 00:00 [accepted]
PHST- 2022/04/12 01:07 [entrez]
PHST- 2022/04/13 06:00 [pubmed]
PHST- 2022/04/13 06:01 [medline]
PHST- 2022/04/01 00:00 [pmc-release]
AID - jcm11071967 [pii]
AID - jcm-11-01967 [pii]
AID - 10.3390/jcm11071967 [doi]
PST - epublish
SO  - J Clin Med. 2022 Apr 1;11(7):1967. doi: 10.3390/jcm11071967.