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Slow, progressive myopathy in neonatally treated patients with infantile-onset Pompe disease: a muscle magnetic resonance imaging study

Overview of attention for article published in Orphanet Journal of Rare Diseases, January 2016
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About this Attention Score

  • Above-average Attention Score compared to outputs of the same age (53rd percentile)

Mentioned by

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4 tweeters

Citations

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13 Dimensions

Readers on

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30 Mendeley
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Title
Slow, progressive myopathy in neonatally treated patients with infantile-onset Pompe disease: a muscle magnetic resonance imaging study
Published in
Orphanet Journal of Rare Diseases, January 2016
DOI 10.1186/s13023-016-0446-7
Pubmed ID
Authors

Steven Shinn-Forng Peng, Wuh-Liang Hwu, Ni-Chung Lee, Fuu-Jen Tsai, Wen-Hui Tsai, Yin-Hsiu Chien

Abstract

Patients with infantile-onset Pompe disease (IOPD) can be identified through newborn screening, and the subsequent immediate initiation of enzyme replacement therapy significantly improves the prognosis of these patients. However, they still present residual muscle weakness. In the present study, we used longitudinal muscle magnetic resonance imaging (MRI) to determine whether this condition is progressive. A cohort of classic IOPD patients who were diagnosed through newborn screening were treated with recombinant human acid α-glucosidase (rhGAA) and followed prospectively from birth. The trunk (and abdominal wall), pelvis and upper thighs were scanned for muscle MRI every 2-3 years. Seven groups of muscles were individually scored from 0 to 4 based on the extent of their involvement, and the sum was correlated to the clinical manifestations. Twenty-four MRI scans from a total of 12 neonatally treated IOPD patients were analyzed in the present study. The median age at the time of MRI scanning was 4.2 years (13 days to 9 years). High intensity over the quadriceps on T2-weighted and short-tau inversion recovery images was observed in all scans and was followed by a decrease in muscle mass. Trunk muscle involvement was slower, except in one patient who exhibited progressive psoas atrophy. Among the 10 patients for whom follow-up scans were repeated more than 2 years after the first scan, four patients (40 %) showed increased myopathy severity. This prospective muscle MRI study provides evidence for the occurrence of slow, progressive muscle damage in neonatally treated IOPD patients during childhood. New treatment strategies are necessary to improve outcomes in these patients.

Twitter Demographics

The data shown below were collected from the profiles of 4 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 30 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 30 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 7 23%
Student > Ph. D. Student 5 17%
Other 4 13%
Student > Doctoral Student 2 7%
Student > Bachelor 2 7%
Other 3 10%
Unknown 7 23%
Readers by discipline Count As %
Medicine and Dentistry 11 37%
Biochemistry, Genetics and Molecular Biology 3 10%
Neuroscience 3 10%
Nursing and Health Professions 2 7%
Agricultural and Biological Sciences 2 7%
Other 1 3%
Unknown 8 27%

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 05 December 2017.
All research outputs
#6,888,922
of 12,253,439 outputs
Outputs from Orphanet Journal of Rare Diseases
#758
of 1,328 outputs
Outputs of similar age
#122,746
of 276,207 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#42
of 58 outputs
Altmetric has tracked 12,253,439 research outputs across all sources so far. This one is in the 42nd percentile – i.e., 42% of other outputs scored the same or lower than it.
So far Altmetric has tracked 1,328 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 6.9. This one is in the 39th percentile – i.e., 39% 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 276,207 tracked outputs that were published within six weeks on either side of this one in any source. This one has gotten more attention than average, scoring higher than 53% of its contemporaries.
We're also able to compare this research output to 58 others from the same source and published within six weeks on either side of this one. This one is in the 24th percentile – i.e., 24% of its contemporaries scored the same or lower than it.