PMID- 1677252
OWN - NLM
STAT- MEDLINE
DCOM- 19910826
LR  - 20190902
IS  - 0284-186X (Print)
IS  - 0284-186X (Linking)
VI  - 30
IP  - 4
DP  - 1991
TI  - Genetic and clinical characteristics of multiple endocrine neoplasia type 1.
PG  - 485-8
AB  - Multiple endocrine neoplasia type 1 (MEN 1) is an inherited disorder of 
      autosomal-dominant type encompassing tumors of the parathyroid glands, anterior 
      pituitary and endocrine pancreas. The genetic defect responsible for MEN 1 maps 
      to the long arm of chromosome 11 (11q13). Constitutional heterozygosity for the 
      MEN 1 region is lost in proliferating pancreatic endocrine and parathyroid 
      tissue, which suggests that the MEN 1 gene belongs to the group of tumor 
      suppressor genes (antioncogenes). Genetic screening by linkage analysis using 
      RFLP markers allows selection of gene carriers with high degree of accuracy and 
      makes it possible to concentrate the laborious biochemical screening efforts to 
      family members at risk of developing the MEN 1 syndrome. Biochemical screening of 
      the MEN 1 lesions in young asymptomatic individuals decreases the age at 
      diagnosis with many years (decades) and therapeutic intervention can be 
      instituted early. Although it still remains to be established whether early 
      intervention will have an impact on mortality, our long-term experience with 20 
      MEN 1 kindreds suggests a reduced morbidity as a result of thorough biochemical 
      screening.
FAU - Skogseid, B
AU  - Skogseid B
AD  - Department of Internal Medicine, University Hospital, Ludwig Institute for Cancer 
      Research, Uppsala, Sweden.
FAU - Larsson, C
AU  - Larsson C
FAU - Oberg, K
AU  - Oberg K
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - Sweden
TA  - Acta Oncol
JT  - Acta oncologica (Stockholm, Sweden)
JID - 8709065
SB  - IM
MH  - Adult
MH  - Age Factors
MH  - *Chromosome Deletion
MH  - *Chromosomes, Human, Pair 11
MH  - Humans
MH  - Male
MH  - Multiple Endocrine Neoplasia/complications/*genetics
MH  - Pancreatic Neoplasms/complications/genetics
MH  - Polymorphism, Restriction Fragment Length
MH  - Prognosis
EDAT- 1991/01/01 00:00
MHDA- 1991/01/01 00:01
CRDT- 1991/01/01 00:00
PHST- 1991/01/01 00:00 [pubmed]
PHST- 1991/01/01 00:01 [medline]
PHST- 1991/01/01 00:00 [entrez]
AID - 10.3109/02841869109092405 [doi]
PST - ppublish
SO  - Acta Oncol. 1991;30(4):485-8. doi: 10.3109/02841869109092405.