PMID- 1362398
OWN - NLM
STAT- MEDLINE
DCOM- 19930217
LR  - 20151119
IS  - 0018-0416 (Print)
IS  - 0018-0416 (Linking)
VI  - 40
IP  - 3-4
DP  - 1992
TI  - Prospective screening in multiple endocrine neoplasia type 1.
PG  - 167-70
AB  - To assess the age of clinically detectable onset of multiple endocrine neoplasia 
      type 1 (MEN 1), 88 members of four families were invited to participate in a 
      ten-year biochemical screening program. Evidence for clinically detectable MEN 1 
      was found in adolescence. Pancreatic endocrine dysfunction constituted the 
      presenting lesion in a majority of these individuals. The age at diagnosis of 
      pancreatic endocrine tumors averaged 25 years and was lowered by almost two 
      decades by prospective investigation. Furthermore, the penetrance of the 
      pancreatic endocrine and parathyroid lesions equaled the penetrance found in 
      autopsy studies. The use of a standardized meal stimulation test with the 
      measurement of serum pancreatic polypeptide (PP) and gastrin responses resulted 
      in diagnostic sensitivities of 75% and 100%, respectively. In addition to basal 
      serum PP and insulin values, the proinsulin level was predictive for early 
      pancreatic involvement in MEN 1. Serum gastrin was another useful tumor marker 
      but only in the patients with pancreatic tumors diagnosed outside the prospective 
      investigation. Two of the four MEN 1 kindreds selected for the screening 
      investigation displayed homogeneity within families with respect to the profile 
      of peptide excess and malignant potential of the pancreatic endocrine lesion, 
      while the remaining kindreds demonstrated variable MEN 1 traits.
FAU - Skogseid, B
AU  - Skogseid B
AD  - Department of Internal Medicine, University Hospital, Uppsala, Sweden.
FAU - Oberg, K
AU  - Oberg K
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Henry Ford Hosp Med J
JT  - Henry Ford Hospital medical journal
JID - 0122304
RN  - 0 (Biomarkers, Tumor)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Age Factors
MH  - Aged
MH  - Biomarkers, Tumor/analysis
MH  - Child
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia/*diagnosis/genetics
MH  - Pancreatic Neoplasms/diagnosis
MH  - Pituitary Neoplasms/diagnosis
MH  - Prospective Studies
EDAT- 1992/01/01 00:00
MHDA- 1992/01/01 00:01
CRDT- 1992/01/01 00:00
PHST- 1992/01/01 00:00 [pubmed]
PHST- 1992/01/01 00:01 [medline]
PHST- 1992/01/01 00:00 [entrez]
PST - ppublish
SO  - Henry Ford Hosp Med J. 1992;40(3-4):167-70.