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Body composition, muscle strength and hormonal status in patients with ataxia telangiectasia: a cohort study

Overview of attention for article published in Orphanet Journal of Rare Diseases, December 2015
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Title
Body composition, muscle strength and hormonal status in patients with ataxia telangiectasia: a cohort study
Published in
Orphanet Journal of Rare Diseases, December 2015
DOI 10.1186/s13023-015-0373-z
Pubmed ID
Authors

H. Pommerening, S. van Dullemen, M. Kieslich, R. Schubert, S. Zielen, S. Voss

Abstract

Ataxia-telangiectasia (A-T) is a devastating human autosomal recessive disorder that causes progressive cerebellar ataxia, immunodeficiency, premature aging, chromosomal instability and increased cancer risk. Affected patients show growth failure, poor weight gain, low body mass index (BMI), myopenia and increased fatigue during adolescence. The prevalence of alterations in body composition, muscle strength and hormonal status has not been well described in classical A-T patients. Additionally, no current guidelines are available for the assessment and management of these changes. We analyzed body composition, manual muscle strength and hormonal status in 25 A-T patients and 26 age-matched, healthy controls. Bioelectrical impedance analysis (BIA) was performed to evaluate the body composition, fat-free mass (FFM), body cell mass (BCM), extracellular matrix (ECM), phase angle (PhA), fat mass (FM) and ECM to BCM ratio. Manual muscle strength was measured using a hydraulic hand dynamometer. The BMI, FFM and PhA were significantly lower in A-T patients than in controls (BMI 16.56 ± 3.52 kg/m(2) vs. 19.86 ± 3.54 kg/m(2); Z-Score: -1.24 ± 1.29 vs. 0.05 ± 0.92, p <0.001; FFM 25.4 ± 10.03 kg vs. 41.77 ± 18.25 kg, p < 0.001; PhA: 4.6 ± 0.58° vs. 6.15 ± 0.88°, p < 0.001). Manual muscle strength was significantly impaired in A-T patients compared with controls (10.65 ± 10.97 kg vs. 26.8 ± 30.39 kg, p < 0.0001). In addition, cortisol and dehydroepiandrosterone sulfate (DHEAS) levels were significantly lower in A-T patients than in controls. Altered body composition, characterized by depleted BMI, PhA and BCM; by the need to sit in a wheelchair; by altered hormone levels; and by poor muscle strength, is a major factor underlying disease progression and increased fatigue in A-T patients. ClinicalTrials.gov NCT02345200.

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The data shown below were collected from the profiles of 3 X users who shared this research output. Click here to find out more about how the information was compiled.
Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 76 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Libya 1 1%
Unknown 75 99%

Demographic breakdown

Readers by professional status Count As %
Student > Doctoral Student 11 14%
Student > Bachelor 11 14%
Student > Master 10 13%
Student > Ph. D. Student 7 9%
Researcher 6 8%
Other 12 16%
Unknown 19 25%
Readers by discipline Count As %
Medicine and Dentistry 20 26%
Nursing and Health Professions 10 13%
Sports and Recreations 8 11%
Biochemistry, Genetics and Molecular Biology 5 7%
Agricultural and Biological Sciences 4 5%
Other 10 13%
Unknown 19 25%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 2. 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 08 July 2016.
All research outputs
#13,757,736
of 22,835,198 outputs
Outputs from Orphanet Journal of Rare Diseases
#1,474
of 2,620 outputs
Outputs of similar age
#193,477
of 389,038 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#51
of 82 outputs
Altmetric has tracked 22,835,198 research outputs across all sources so far. This one is in the 38th percentile – i.e., 38% of other outputs scored the same or lower than it.
So far Altmetric has tracked 2,620 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.5. This one is in the 42nd percentile – i.e., 42% 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 389,038 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 82 others from the same source and published within six weeks on either side of this one. This one is in the 37th percentile – i.e., 37% of its contemporaries scored the same or lower than it.