PMID- 30364322
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20191120
IS  - 1664-2392 (Print)
IS  - 1664-2392 (Electronic)
IS  - 1664-2392 (Linking)
VI  - 9
DP  - 2018
TI  - When Parathyroidectomy Should Be Indicated or Postponed in Adolescents With 
      MEN1-Related Primary Hyperparathyroidism.
PG  - 597
LID - 10.3389/fendo.2018.00597 [doi]
LID - 597
AB  - Multiple Endocrine Neoplasia Type 1 (MEN1) is a rare inherited endocrine tumor 
      syndrome principally affecting parathyroid glands, neuroendocrine tissues of the 
      gastro-entero-pancreatic and thoracic tracts, and anterior pituitary, caused by 
      germline inactivating mutations of the MEN1 tumor suppressor gene. Primary 
      hyperparathyroidism (PHPT) is usually the first clinical manifestation of the 
      syndrome, normally manifesting during the third decade of life. Cases of affected 
      children and adolescents have been described by the age of 5. Clinical 
      characteristics and therapeutic management of MEN1 in adolescents have been 
      described mainly by case reports. Only two studies on MEN1 patient series under 
      the age of 22 years have recently been published. Given the scarcity of data and 
      the lack of a consistent number of targeted studies, there are currently no 
      specific guidelines available for children and adolescents with MEN1; diagnostic 
      and therapeutic management is, thus, usually the same as for adult patients. 
      Here, we report our experience with 19 adolescent MEN1 patients, developing 
      MEN1-associated PHPT before the age of 20. Fourteen of them, manifesting 
      hypercalcemic PHPT before the age of 20 underwent parathyroidectomy before the 
      age of 25 to control calcemia. Parathyroid surgery restored normal calcemia in 
      all the operated patients. No post-surgical nephrolithiasis has been reported 
      after a mean of 12.0 +/- 5.8 years of follow-up. Comparison between pre-surgical 
      and post-surgical values of bone mineral density (BMD) in 2 patients evidenced an 
      improvement of bone mass after parathyroid adenoma ablation. Two patients 
      (14.28%) developed permanent post-surgical hypoparathyroidism.
FAU - Marini, Francesca
AU  - Marini F
AD  - Department of Surgery and Translational Medicine, University of Florence, 
      Florence, Italy.
FAU - Giusti, Francesca
AU  - Giusti F
AD  - Department of Surgery and Translational Medicine, University of Florence, 
      Florence, Italy.
FAU - Tonelli, Francesco
AU  - Tonelli F
AD  - Department of Surgery and Translational Medicine, University of Florence, 
      Florence, Italy.
FAU - Brandi, Maria Luisa
AU  - Brandi ML
AD  - Department of Surgery and Translational Medicine, University of Florence, 
      Florence, Italy.
LA  - eng
PT  - Journal Article
DEP - 20181005
PL  - Switzerland
TA  - Front Endocrinol (Lausanne)
JT  - Frontiers in endocrinology
JID - 101555782
PMC - PMC6193525
OTO - NOTNLM
OT  - MEN1 adolescent patients
OT  - Multiple Endocrine Neoplasia Type 1
OT  - parathyroid adenomas
OT  - parathyroidectomy
OT  - primary hyperparathyroidism
EDAT- 2018/10/27 06:00
MHDA- 2018/10/27 06:01
PMCR- 2018/01/01
CRDT- 2018/10/27 06:00
PHST- 2018/06/26 00:00 [received]
PHST- 2018/09/19 00:00 [accepted]
PHST- 2018/10/27 06:00 [entrez]
PHST- 2018/10/27 06:00 [pubmed]
PHST- 2018/10/27 06:01 [medline]
PHST- 2018/01/01 00:00 [pmc-release]
AID - 10.3389/fendo.2018.00597 [doi]
PST - epublish
SO  - Front Endocrinol (Lausanne). 2018 Oct 5;9:597. doi: 10.3389/fendo.2018.00597. 
      eCollection 2018.