PMID- 14747767
OWN - NLM
STAT- MEDLINE
DCOM- 20040224
LR  - 20220409
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 83
IP  - 1
DP  - 2004 Jan
TI  - Multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome: a prospective 
      study of 107 cases and comparison with 1009 cases from the literature.
PG  - 43-83
LID - 10.1097/01.md.0000112297.72510.32 [doi]
AB  - In patients with multiple endocrine neoplasia type 1 (MEN1), the most common 
      functional pancreatic endocrine tumor (PET) syndrome is Zollinger-Ellison 
      syndrome (ZES). ZES has been well studied in its sporadic form (that is, without 
      MEN1); however, there are limited data on patients with MEN1 and ZES (MEN1/ZES), 
      and the long-term natural history is largely unknown. To address this issue we 
      report the results of a prospective long-term National Institutes of Health (NIH) 
      study of 107 MEN1/ZES patients and compare our results with those of 1009 
      MEN1/ZES patients in 278 case reports and small series in the literature. 
      Patients were clinically, radiologically, and biochemically evaluated yearly for 
      all MEN1 manifestations (mean follow-up, 10 yr; range, 0.1-31 yr). Compared with 
      patients from the literature, the NIH MEN1/ZES patients more frequently had 
      pituitary (60%) and adrenal (45%) disease and carcinoid tumors (30%), but had 
      equal frequency of hyperparathyroidism (94%), thyroid disease (6%), or lipomas 
      (5%). Twenty-five percent of both the NIH and the literature patients lacked a 
      family history of MEN1; ZES was the initial clinical manifestation of MEN1 in 
      40%. ZES onset preceded the diagnosis of hyperparathyroidism in 45%. However, ZES 
      was rarely (8%) the only initial manifestation of MEN1 if careful testing was 
      done. ZES occurred before age 40 years in 50%-60% of the current patients, in 
      contrast to older studies. The diagnosis of ZES is delayed 3-5 years from its 
      onset and is delayed as long as in sporadic ZES cases. Pituitary disease and 
      carcinoid tumors (gastric > bronchial, thymic) are more frequent than generally 
      reported, whereas a second functional PET is uncommon. In patients with MEN1/ZES 
      without a family history of MEN1, the MEN1 manifestations are not as severe. This 
      study shows that MEN1/ZES patients differ in many aspects from those commonly 
      reported in older studies involving few MEN1/ZES patients. In this study we have 
      identified a number of important clinical and laboratory features of MEN1/ZES 
      that were not previously appreciated, which should contribute to earlier 
      diagnosis and improve both short- and long-term management.
FAU - Gibril, Fathia
AU  - Gibril F
AD  - From Digestive Diseases Branch, National Institute of Diabetes and Digestive and 
      Kidney Diseases, National Institutes of Health, Bethesda, Maryland.
FAU - Schumann, Michael
AU  - Schumann M
FAU - Pace, Andrea
AU  - Pace A
FAU - Jensen, Robert T
AU  - Jensen RT
LA  - eng
PT  - Case Reports
PT  - Comparative Study
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Hormones)
SB  - IM
EIN - Medicine (Baltimore). 2004 May;83(3):175
MH  - Adult
MH  - Age Distribution
MH  - Age of Onset
MH  - Aged
MH  - Diagnosis, Differential
MH  - Disease Progression
MH  - Female
MH  - Follow-Up Studies
MH  - Gastrinoma/blood/*diagnosis
MH  - Genetic Testing
MH  - Hormones/blood
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*diagnosis/epidemiology/therapy
MH  - Prospective Studies
MH  - Survival Analysis
MH  - Tomography, X-Ray Computed
MH  - Treatment Outcome
MH  - Zollinger-Ellison Syndrome/blood/*diagnosis/epidemiology
RF  - 505
EDAT- 2004/01/30 05:00
MHDA- 2004/02/26 05:00
CRDT- 2004/01/30 05:00
PHST- 2004/01/30 05:00 [pubmed]
PHST- 2004/02/26 05:00 [medline]
PHST- 2004/01/30 05:00 [entrez]
AID - 00005792-200401000-00004 [pii]
AID - 10.1097/01.md.0000112297.72510.32 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2004 Jan;83(1):43-83. doi: 
      10.1097/01.md.0000112297.72510.32.