↓ Skip to main content

A conceptual disease model for adult Pompe disease

Overview of attention for article published in Orphanet Journal of Rare Diseases, January 2015
Altmetric Badge

About this Attention Score

  • In the top 25% of all research outputs scored by Altmetric
  • Good Attention Score compared to outputs of the same age (79th percentile)
  • Good Attention Score compared to outputs of the same age and source (77th percentile)

Mentioned by

twitter
8 tweeters
facebook
2 Facebook pages

Citations

dimensions_citation
5 Dimensions

Readers on

mendeley
9 Mendeley
You are seeing a free-to-access but limited selection of the activity Altmetric has collected about this research output. Click here to find out more.
Title
A conceptual disease model for adult Pompe disease
Published in
Orphanet Journal of Rare Diseases, January 2015
DOI 10.1186/s13023-015-0334-6
Pubmed ID
Authors

Tim A Kanters, W. K Redekop, Maureen PMH Rutten-Van Mölken, Michelle E Kruijshaar, Deniz Güngör, Ans T van der Ploeg, Leona Hakkaart, Kanters, Tim A, Redekop, W Ken, Rutten-Van Mölken, Maureen P M H, Kruijshaar, Michelle E, Güngör, Deniz, van der Ploeg, Ans T, Hakkaart, Leona, Tim A. Kanters, W. Ken Redekop, Maureen P.M.H. Rutten-Van Mölken, Michelle E. Kruijshaar, Ans T. van der Ploeg

Abstract

Studies in orphan diseases are, by nature, confronted with small patient populations, meaning that randomized controlled trials will have limited statistical power. In order to estimate the effectiveness of treatments in orphan diseases and extrapolate effects into the future, alternative models might be needed. The purpose of this study is to develop a conceptual disease model for Pompe disease in adults (an orphan disease). This conceptual model describes the associations between the most important levels of health concepts for Pompe disease in adults, from biological parameters via physiological parameters, symptoms and functional indicators to health perceptions and final health outcomes as measured in terms of health-related quality of life. The structure of the Wilson-Cleary health outcomes model was used as a blueprint, and filled with clinically relevant aspects for Pompe disease based on literature and expert opinion. Multiple observations per patient from a Dutch cohort study in untreated patients were used to quantify the relationships between the different levels of health concepts in the model by means of regression analyses. Enzyme activity, muscle strength, respiratory function, fatigue, level of handicap, general health perceptions, mental and physical component scales and utility described the different levels of health concepts in the Wilson-Cleary model for Pompe disease. Regression analyses showed that functional status was affected by fatigue, muscle strength and respiratory function. Health perceptions were affected by handicap. In turn, self-reported quality of life was affected by health perceptions. We conceptualized a disease model that incorporated the mechanisms believed to be responsible for impaired quality of life in Pompe disease. The model provides a comprehensive overview of various aspects of Pompe disease in adults, which can be useful for both clinicians and policymakers to support their multi-faceted decision making.

Twitter Demographics

The data shown below were collected from the profiles of 8 tweeters who shared this research output. Click here to find out more about how the information was compiled.

Mendeley readers

The data shown below were compiled from readership statistics for 9 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 9 100%

Demographic breakdown

Readers by professional status Count As %
Student > Master 3 33%
Student > Ph. D. Student 1 11%
Student > Doctoral Student 1 11%
Other 1 11%
Researcher 1 11%
Other 1 11%
Unknown 1 11%
Readers by discipline Count As %
Pharmacology, Toxicology and Pharmaceutical Science 2 22%
Business, Management and Accounting 2 22%
Sports and Recreations 2 22%
Medicine and Dentistry 1 11%
Engineering 1 11%
Other 0 0%
Unknown 1 11%

Attention Score in Context

This research output has an Altmetric Attention Score of 6. 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 28 May 2016.
All research outputs
#1,308,738
of 7,822,782 outputs
Outputs from Orphanet Journal of Rare Diseases
#174
of 1,089 outputs
Outputs of similar age
#46,729
of 231,523 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#10
of 45 outputs
Altmetric has tracked 7,822,782 research outputs across all sources so far. Compared to these this one has done well and is in the 83rd percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 1,089 research outputs from this source. They receive a mean Attention Score of 4.8. This one has done well, scoring higher than 83% of its peers.
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 231,523 tracked outputs that were published within six weeks on either side of this one in any source. This one has done well, scoring higher than 79% of its contemporaries.
We're also able to compare this research output to 45 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 77% of its contemporaries.