PMID- 31348545
OWN - NLM
STAT- MEDLINE
DCOM- 20200908
LR  - 20200908
IS  - 1365-2265 (Electronic)
IS  - 0300-0664 (Linking)
VI  - 91
IP  - 5
DP  - 2019 Nov
TI  - Paediatric and young adult manifestations and outcomes of multiple endocrine 
      neoplasia type 1.
PG  - 633-638
LID - 10.1111/cen.14067 [doi]
AB  - CONTEXT: Multiple endocrine neoplasia 1 (MEN 1) is an autosomal dominant disease 
      presenting as hyperplasia and neoplasia of parathyroid, pituitary and 
      enteropancreatic tissues. Over 90% of gene carriers develop phenotypic disease by 
      age 30 years, potentially with onset of asymptomatic disease during childhood and 
      adolescence. OBJECTIVE: To describe the paediatric and young adult manifestations 
      of MEN 1. DESIGN: Descriptive retrospective study of 180 patients with a common 
      MEN1 genotype. The paediatric and young adult (age <22 years) manifestations were 
      determined using hospital records and disease surveillance data. RESULTS: Primary 
      hyperparathyroidism (PHPT) was identified in 42 patients (mean age 
      17.2 +/- 3.3 years). Parathyroidectomy was performed in 16 (38.1%; mean age 
      17.8 +/- 3.2). Four patients experienced recurrent PHPT (25%), and six (37.5%) 
      developed permanent hypoparathyroidism. Pituitary disease was identified in 13 
      patients. Prolactinoma was found in nine patients (mean age 16.6 +/- 2.6 years) of 
      whom four (44.4%) had macroprolactinoma. Two patients required surgical 
      intervention; dopamine agonists showed efficacy in six patients. Two patients 
      with Cushing's disease were successfully treated surgically. Three patients with 
      nonfunctioning pituitary microadenoma managed conservatively. Pancreatic 
      neuroendocrine neoplasms (pNENs) were diagnosed in 12 patients (mean age 
      17.0 +/- 2.6 years): three patients with insulinoma successfully resected (two 
      resected and one exhibiting perineural invasion) and nine patients with 
      nonfunctioning adenomas (NFAs). CONCLUSION: Pituitary adenomas, PHPT and pNENs 
      are encountered in the paediatric and young adult MEN 1 population. Successful 
      outcomes are typically achieved using standard medical and surgical paradigms; 
      however, parathyroidectomy was associated with a substantial complication rate.
CI  - (c) 2019 John Wiley & Sons Ltd.
FAU - Herath, Madhuni
AU  - Herath M
AUID- ORCID: 0000-0003-2776-2083
AD  - Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart, TAS, 
      Australia.
FAU - Parameswaran, Venkat
AU  - Parameswaran V
AD  - Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart, TAS, 
      Australia.
AD  - School of Medicine, University of Tasmania, Hobart, TAS, Australia.
FAU - Thompson, Michael
AU  - Thompson M
AUID- ORCID: 0000-0003-1425-5808
AD  - Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart, TAS, 
      Australia.
AD  - School of Medicine, University of Tasmania, Hobart, TAS, Australia.
FAU - Williams, Michelle
AU  - Williams M
AD  - School of Medicine, University of Tasmania, Hobart, TAS, Australia.
AD  - Department of Paediatrics, Royal Hobart Hospital, Hobart, TAS, Australia.
FAU - Burgess, John
AU  - Burgess J
AD  - Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart, TAS, 
      Australia.
AD  - School of Medicine, University of Tasmania, Hobart, TAS, Australia.
LA  - eng
PT  - Journal Article
DEP - 20190814
PL  - England
TA  - Clin Endocrinol (Oxf)
JT  - Clinical endocrinology
JID - 0346653
RN  - 0 (Dopamine Agonists)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Dopamine Agonists/therapeutic use
MH  - Female
MH  - Genotype
MH  - Humans
MH  - Male
MH  - Multiple Endocrine Neoplasia Type 1/drug therapy/metabolism/*pathology
MH  - Parathyroid Diseases/metabolism/pathology
MH  - Phenotype
MH  - Pituitary Diseases/drug therapy/metabolism/pathology
MH  - Prolactinoma/metabolism/pathology
MH  - Retrospective Studies
MH  - Survival Analysis
MH  - Young Adult
OTO - NOTNLM
OT  - MEN 1
OT  - clinical management
OT  - multiple endocrine neoplasia type 1
OT  - paediatric
OT  - parathyroidectomy
OT  - phenotype
OT  - young adult
EDAT- 2019/07/28 06:00
MHDA- 2020/09/09 06:00
CRDT- 2019/07/27 06:00
PHST- 2019/06/04 00:00 [received]
PHST- 2019/07/14 00:00 [revised]
PHST- 2019/07/23 00:00 [accepted]
PHST- 2019/07/28 06:00 [pubmed]
PHST- 2020/09/09 06:00 [medline]
PHST- 2019/07/27 06:00 [entrez]
AID - 10.1111/cen.14067 [doi]
PST - ppublish
SO  - Clin Endocrinol (Oxf). 2019 Nov;91(5):633-638. doi: 10.1111/cen.14067. Epub 2019 
      Aug 14.