PMID- 8917740
OWN - NLM
STAT- MEDLINE
DCOM- 19961210
LR  - 20230411
IS  - 1460-2725 (Print)
IS  - 1460-2393 (Linking)
VI  - 89
IP  - 9
DP  - 1996 Sep
TI  - Clinical studies of multiple endocrine neoplasia type 1 (MEN1).
PG  - 653-69
AB  - Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder 
      characterized by the combined occurrence of parathyroid, pancreatic islet and 
      anterior pituitary tumours. To facilitate a screening programme for MEN1, we 
      investigated 709 people (364 males and 345 females, age range 1-84 years) from 62 
      MEN1 families, and 36 non-familial MEN1 patients. Of those investigated, 220 (95 
      males and 125 females, age range 8-79 years) suffered from MEN1. Parathyroid, 
      pancreatic and pituitary tumours occurred in 95%, 41% and 30% of the patients, 
      respectively. Parathyroid tumours were the first manifestation of MEN1 in 87% of 
      patients, and amongst the pituitary and pancreatic tumours, somatotrophinomas and 
      gastrinomas were more common in patients above the age of 40 years, whilst 
      insulinomas occurred more frequently in patients below the age of 40 years. 
      Biochemical screening indicated that the penetrance of MEN1 by the ages of 20, 35 
      and 50 years was 43%, 85% and 94%, respectively, and that the development of MEN1 
      was confined to first-degree relatives in 91% of patients and to second-degree 
      relatives in 9% of patients. These findings have helped to define a proposed 
      screening programme for MEN1.
FAU - Trump, D
AU  - Trump D
AD  - MRC Molecular Endocrinology Group, Royal Postgraduate Medical School, Hammersmith 
      Hospital, London, UK.
FAU - Farren, B
AU  - Farren B
FAU - Wooding, C
AU  - Wooding C
FAU - Pang, J T
AU  - Pang JT
FAU - Besser, G M
AU  - Besser GM
FAU - Buchanan, K D
AU  - Buchanan KD
FAU - Edwards, C R
AU  - Edwards CR
FAU - Heath, D A
AU  - Heath DA
FAU - Jackson, C E
AU  - Jackson CE
FAU - Jansen, S
AU  - Jansen S
FAU - Lips, K
AU  - Lips K
FAU - Monson, J P
AU  - Monson JP
FAU - O'Halloran, D
AU  - O'Halloran D
FAU - Sampson, J
AU  - Sampson J
FAU - Shalet, S M
AU  - Shalet SM
FAU - Wheeler, M H
AU  - Wheeler MH
FAU - Zink, A
AU  - Zink A
FAU - Thakker, R V
AU  - Thakker RV
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - QJM
JT  - QJM : monthly journal of the Association of Physicians
JID - 9438285
SB  - IM
EIN - QJM 1996 Dec;89(12):957-8
MH  - Adolescent
MH  - Adult
MH  - Age Factors
MH  - Aged
MH  - Aged, 80 and over
MH  - Child
MH  - Child, Preschool
MH  - Female
MH  - Humans
MH  - Hypercalcemia/complications
MH  - Hyperparathyroidism/complications
MH  - Infant
MH  - Male
MH  - Middle Aged
MH  - Models, Genetic
MH  - Multiple Endocrine Neoplasia Type 1/*diagnosis
MH  - Mutation
MH  - Pancreatic Neoplasms/complications
MH  - Parathyroid Neoplasms/complications
MH  - Pedigree
MH  - Pituitary Neoplasms/complications
EDAT- 1996/09/01 00:00
MHDA- 1996/09/01 00:01
CRDT- 1996/09/01 00:00
PHST- 1996/09/01 00:00 [pubmed]
PHST- 1996/09/01 00:01 [medline]
PHST- 1996/09/01 00:00 [entrez]
AID - 10.1093/qjmed/89.9.653 [doi]
PST - ppublish
SO  - QJM. 1996 Sep;89(9):653-69. doi: 10.1093/qjmed/89.9.653.