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MEN1 redefined, a clinical comparison of mutation-positive and mutation-negative patients

Overview of attention for article published in BMC Medicine, November 2016
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Title
MEN1 redefined, a clinical comparison of mutation-positive and mutation-negative patients
Published in
BMC Medicine, November 2016
DOI 10.1186/s12916-016-0708-1
Pubmed ID
Authors

Joanne M. de Laat, Rob B. van der Luijt, Carolina R. C. Pieterman, Maria P. Oostveen, Ad R. Hermus, Olaf M. Dekkers, Wouter W. de Herder, Anouk N. van der Horst-Schrivers, Madeleine L. Drent, Peter H. Bisschop, Bas Havekes, Menno R. Vriens, Gerlof D. Valk

Abstract

Multiple Endocrine Neoplasia type 1 (MEN1) is diagnosed when two out of the three primary MEN1-associated endocrine tumors occur in a patient. Up to 10-30 % of those patients have no mutation in the MEN1 gene. It is unclear if the phenotype and course of the disease of mutation-negative patients is comparable with mutation-positive patients and if these patients have true MEN1. The present study aims to describe and compare the clinical course of MEN1 mutation-negative patients with two out of the three main MEN1 manifestations and mutation-positive patients during long-term follow-up. This is a cohort study performed using the Dutch MEN1 database, including > 90 % of the Dutch MEN1 population. A total of 293 (90.7 %) mutation-positive and 30 (9.3 %) mutation-negative MEN1 patients were included. Median age of developing the first main MEN1 manifestation was higher in mutation-negative patients (46 vs. 33 years) (P = 0.007). Mutation-negative patients did not develop a third main MEN1 manifestation in the course of follow-up compared to 48.3 % of mutation-positive patients (P < 0.001). Median survival in mutation-positive patients was estimated at 73.0 years (95 % CI, 69.5-76.5) compared to 87.0 years (95 % CI not available) in mutation-negative patients (P = 0.001). Mutation-positive and mutation-negative MEN1 patients have a different phenotype and clinical course. Mutation-negative patients develop MEN1 manifestations at higher age and have a life expectancy comparable with the general population. The apparent differences in clinical course suggest that MEN1 mutation-negative patients do not have true MEN1, but another MEN1-like syndrome or sporadic co-incidence of two neuro-endocrine tumors.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 57 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 57 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 10 18%
Researcher 6 11%
Student > Bachelor 5 9%
Student > Doctoral Student 5 9%
Student > Postgraduate 5 9%
Other 12 21%
Unknown 14 25%
Readers by discipline Count As %
Medicine and Dentistry 30 53%
Biochemistry, Genetics and Molecular Biology 4 7%
Nursing and Health Professions 2 4%
Immunology and Microbiology 2 4%
Engineering 1 2%
Other 0 0%
Unknown 18 32%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 3. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 09 January 2017.
All research outputs
#12,912,964
of 22,901,818 outputs
Outputs from BMC Medicine
#2,719
of 3,443 outputs
Outputs of similar age
#152,340
of 306,450 outputs
Outputs of similar age from BMC Medicine
#54
of 67 outputs
Altmetric has tracked 22,901,818 research outputs across all sources so far. This one is in the 43rd percentile – i.e., 43% of other outputs scored the same or lower than it.
So far Altmetric has tracked 3,443 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 43.6. This one is in the 20th percentile – i.e., 20% of its peers scored the same or lower than it.
Older research outputs will score higher simply because they've had more time to accumulate mentions. To account for age we can compare this Altmetric Attention Score to the 306,450 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 49th percentile – i.e., 49% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 67 others from the same source and published within six weeks on either side of this one. This one is in the 19th percentile – i.e., 19% of its contemporaries scored the same or lower than it.