PMID- 1677362
OWN - NLM
STAT- MEDLINE
DCOM- 19910827
LR  - 20061115
IS  - 0021-972X (Print)
IS  - 0021-972X (Linking)
VI  - 73
IP  - 2
DP  - 1991 Aug
TI  - Multiple endocrine neoplasia type 1: a 10-year prospective screening study in 
      four kindreds.
PG  - 281-7
AB  - A total of 80 individuals in 4 kindreds with multiple endocrine neoplasia type 1 
      (MEN 1) have been subjected to repeated biochemical screening during a 10-yr 
      period with the principal aim being to analyze characteristics of the developing 
      pancreatic lesion. Age at presentation of the MEN 1 trait averaged 18 yr in 7 
      previously unaffected individuals, and this effect of the screening procedure 
      represented a lowering by almost 2 decades. Pancreatic endocrine involvement was 
      recognized at a mean age of 25 yr and constituted the presenting lesion in a 
      majority of the patients. A standardized meal test and basal values of serum 
      pancreatic polypeptide, insulin, proinsulin, and gastrin were the most efficient 
      markers for the pancreatic lesion and preceded signs of pancreatic tumors upon 
      radiological examinations by a mean of 3.5 yr. A 75% penetrance of the islet cell 
      disease and 90% for primary hyperparathyroidism within the affected individuals 
      equalled the prevalences reported in autopsy studies. Two of the kindreds showed 
      signs of intrafamilial homogeneity with respect to the profile of peptide excess 
      (P less than 0.05) and considerable discrepancy in the malignant potential of the 
      pancreatic lesions. The results of early detection and surgical intervention of 
      the pancreatic tumors in MEN 1 suggested an impact on morbidity, while any effect 
      on the mortality of these individuals remains to be clarified.
FAU - Skogseid, B
AU  - Skogseid B
AD  - Department of Internal Medicine, University Hospital, Uppsala, Sweden.
FAU - Eriksson, B
AU  - Eriksson B
FAU - Lundqvist, G
AU  - Lundqvist G
FAU - Lorelius, L E
AU  - Lorelius LE
FAU - Rastad, J
AU  - Rastad J
FAU - Wide, L
AU  - Wide L
FAU - Akerstrom, G
AU  - Akerstrom G
FAU - Oberg, K
AU  - Oberg K
LA  - eng
PT  - Case Reports
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Clin Endocrinol Metab
JT  - The Journal of clinical endocrinology and metabolism
JID - 0375362
RN  - 0 (Hormones)
SB  - IM
MH  - Adult
MH  - Female
MH  - Hormones/blood
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia/blood/diagnosis/*genetics
MH  - Pancreatic Neoplasms/blood/genetics
MH  - Pedigree
MH  - Pituitary Neoplasms/blood/genetics
MH  - Prospective Studies
EDAT- 1991/08/01 00:00
MHDA- 1991/08/01 00:01
CRDT- 1991/08/01 00:00
PHST- 1991/08/01 00:00 [pubmed]
PHST- 1991/08/01 00:01 [medline]
PHST- 1991/08/01 00:00 [entrez]
AID - 10.1210/jcem-73-2-281 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 1991 Aug;73(2):281-7. doi: 10.1210/jcem-73-2-281.