PMID- 18847656
OWN - NLM
STAT- MEDLINE
DCOM- 20081104
LR  - 20081013
IS  - 1532-7361 (Electronic)
IS  - 0039-6060 (Linking)
VI  - 144
IP  - 4
DP  - 2008 Oct
TI  - The influence of surgery in MEN-1 syndrome: observations over 150 years.
PG  - 695-701; discussion 701-2
LID - 10.1016/j.surg.2008.06.015 [doi]
AB  - BACKGROUND: Efficacy and timing of operative intervention in patients with 
      multiple endocrine neoplasia type 1 (MEN-1) syndrome remains controversial. This 
      report utilizes a novel approach to evaluate the influence of evolving operative 
      interventions for patients with MEN-1 syndrome. METHODS: Six generations from a 
      large MEN-1 family pedigree were studied. The number of operations for MEN-1 
      related pathology was recorded according to birth eras over 150 years. Length of 
      life was a primary outcome measurement. RESULTS: Inheritance of the MEN-1 trait 
      was near 50%. There were no instances of a skipped generation. Affected 
      individuals born before 1900 died from gastrointestinal hemorrhage and without 
      any surgical intervention. After 1900, there were increasing numbers of gastric, 
      parathyroid, and pancreatic operations in successive eras. Death occurred >20 
      years earlier in MEN-1 individuals than unaffected family members in eras 1 and 
      2. Family members with MEN-1 lived longer in succeeding eras with increasing 
      number of operative and pharmacologic interventions. CONCLUSION: MEN-1 family 
      members invariably have pathologic changes in pituitary, parathyroid, and 
      pancreatic islets when long lived, the "all-or-none" phenomenon. Patients are not 
      cured with operative interventions, although they may live longer and without 
      symptoms with a good quality of life. This model may allow better comparisons 
      with other MEN-1 patients when evaluating outcomes of new medical and operative 
      management schemes and long-term follow-up.
FAU - Wilson, Stuart D
AU  - Wilson SD
AD  - Endocrine Surgery Study Group, Department of Surgery, Medical College of 
      Wisconsin and Froedtert Hospital, Milwaukee, WI 53226, USA. rmccoy@mcw.edu
FAU - Krzywda, Elizabeth A
AU  - Krzywda EA
FAU - Zhu, Yong-ran
AU  - Zhu YR
FAU - Yen, Tina W F
AU  - Yen TW
FAU - Wang, Tracy S
AU  - Wang TS
FAU - Sugg, Sonia L
AU  - Sugg SL
FAU - Pappas, Sam G
AU  - Pappas SG
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Surgery
JT  - Surgery
JID - 0417347
SB  - IM
MH  - Adult
MH  - Age Distribution
MH  - Aged
MH  - Aged, 80 and over
MH  - *Cause of Death
MH  - Cohort Studies
MH  - Female
MH  - Follow-Up Studies
MH  - Genetic Predisposition to Disease/*epidemiology
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/genetics/*mortality/*surgery
MH  - Pancreatic Neoplasms/genetics/mortality/surgery
MH  - Parathyroid Neoplasms/genetics/mortality/surgery
MH  - Pedigree
MH  - Pituitary Neoplasms/genetics/mortality/surgery
MH  - Registries
MH  - Retrospective Studies
MH  - Risk Assessment
MH  - Sex Distribution
MH  - Surgical Procedures, Operative/*methods/mortality
MH  - Survival Analysis
MH  - Syndrome
MH  - Time Factors
MH  - Treatment Outcome
EDAT- 2008/10/14 09:00
MHDA- 2008/11/05 09:00
CRDT- 2008/10/14 09:00
PHST- 2008/03/19 00:00 [received]
PHST- 2008/06/21 00:00 [accepted]
PHST- 2008/10/14 09:00 [pubmed]
PHST- 2008/11/05 09:00 [medline]
PHST- 2008/10/14 09:00 [entrez]
AID - S0039-6060(08)00417-0 [pii]
AID - 10.1016/j.surg.2008.06.015 [doi]
PST - ppublish
SO  - Surgery. 2008 Oct;144(4):695-701; discussion 701-2. doi: 
      10.1016/j.surg.2008.06.015.