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Validity and interexaminer reliability of a new method to quantify skin neurofibromas of neurofibromatosis 1 using paper frames

Overview of attention for article published in Orphanet Journal of Rare Diseases, December 2014
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Title
Validity and interexaminer reliability of a new method to quantify skin neurofibromas of neurofibromatosis 1 using paper frames
Published in
Orphanet Journal of Rare Diseases, December 2014
DOI 10.1186/s13023-014-0202-9
Pubmed ID
Authors

Karin SG Cunha, Rafaela E Rozza-de-Menezes, Raquel M Andrade, Amy Theos, Ronir R Luiz, Bruce Korf, Mauro Geller

Abstract

BackgroundSkin neurofibromas represent one of the main clinical manifestations of neurofibromatosis 1, and their number varies greatly between individuals. Quantifying their number is an important step in the methodology of many clinical studies, but counting neurofibromas one by one in individuals with thousands of tumors is arduous, time-consuming, and subject to intra and interexaminer variability. We aimed to evaluate the efficacy of a new methodology for skin neurofibromas quantification using paper frames.MethodsThe sample comprised 92 individuals with NF1. Paper frames, with a central square measuring 100 cm2, were placed on the back, abdomen and thigh. Images were taken, transferred to a computer and two independent examiners counted the neurofibromas. The average number of neurofibromas/100 cm2 of skin was obtained from the mean of the three values. The differences in the quantity of neurofibromas counted by two examiners were evaluated with Intraclass correlation coefficient (ICC), paired t-test, Bland-Altman and survival-agreement plots. To evaluate the predictive value of the method in obtaining the total number of neurofibromas, 49 participants also had their tumors counted one by one. Reproducibility was assessed with Pearson¿s correlation coefficients and simple linear regression model.ResultsThere was excellent agreement between examiners (ICC range 0.992-0.997) and the total number of skin neurofibromas could be predicted by the adhesive frames technique using a specific formula (P¿<¿0.0001).ConclusionsIn this article we describe a reliable, easy and rapid technique using paper frames to quantify skin neurofibromas that accurately predicts the total number of these tumors in patients with NF1. This method may be a useful tool in clinical practice and clinical research to help achieve an accurate quantitative phenotype of NF1.

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Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 13 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 13 100%

Demographic breakdown

Readers by professional status Count As %
Student > Ph. D. Student 3 23%
Student > Master 3 23%
Student > Doctoral Student 2 15%
Professor 1 8%
Professor > Associate Professor 1 8%
Other 0 0%
Unknown 3 23%
Readers by discipline Count As %
Biochemistry, Genetics and Molecular Biology 3 23%
Agricultural and Biological Sciences 2 15%
Medicine and Dentistry 2 15%
Psychology 1 8%
Nursing and Health Professions 1 8%
Other 0 0%
Unknown 4 31%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 1. 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 05 December 2014.
All research outputs
#18,385,510
of 22,772,779 outputs
Outputs from Orphanet Journal of Rare Diseases
#2,132
of 2,613 outputs
Outputs of similar age
#260,671
of 359,774 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#73
of 97 outputs
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