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X Demographics
Mendeley readers
Attention Score in Context
| Title |
Biochemical response to substrate reduction therapy versus enzyme replacement therapy in Gaucher disease type 1 patients
|
|---|---|
| Published in |
Orphanet Journal of Rare Diseases, March 2016
|
| DOI | 10.1186/s13023-016-0413-3 |
| Pubmed ID | |
| Authors |
Bouwien E. Smid, Maria J. Ferraz, Marri Verhoek, Mina Mirzaian, Patrick Wisse, Herman S. Overkleeft, Carla E. Hollak, Johannes M. Aerts |
| Abstract |
We retrospectively compared biochemical responses in type 1 Gaucher disease patients to treatment with glycosphingolipid synthesis inhibitors miglustat and eliglustat and ERT. Seventeen GD1 patients were included (n = 6 eliglustat, (two switched from ERT), n = 9 miglustat (seven switchers), n = 4 ERT (median dose 60U/kg/m). Plasma protein markers reflecting disease burden (chitotriosidase, CCL18) and lipids reflecting substrate accumulation (glucosylsphingosine, glucosylceramide) were determined. Also, liver and spleen volumes, hemoglobin, platelets, and fat fraction were measured. In patients naïve to treatment, chitotriosidase, CCL18 and glucosylsphingosine decreased comparably upon eliglustat and ERT treatment, while the response to miglustat was less. After 2 years, median decrease of chitotriosidase was 89 % (range 77-98), 88 % (78-92) and 37 % (29-46) for eliglustat, ERT and miglustat naïve patients respectively; decrease of CCL18 was 73 % (63-78), 54 % (43-86), and 10 % (3-18); decrease of glucosylsphingosine was 86 % (78-93), 78 % (65-91), 48 % (46-50). Plasma glucosylceramide in eliglustat treated patients (n = 4) reached values below the normal range (n = 20 healthy controls). Biochemical markers decreased or stabilized in switchers from ERT to eliglustat (n = 2), but less in miglustat switchers (n = 7). Clinical parameters responded comparably upon eliglustat and ERT treatment. Our explorative study provides evidence that biochemical markers respond comparably in patients receiving eliglustat treatment and ERT, while the corresponding response to miglustat treatment is less. |
X Demographics
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.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United Kingdom | 2 | 67% |
| Unknown | 1 | 33% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Scientists | 1 | 33% |
| Members of the public | 1 | 33% |
| Science communicators (journalists, bloggers, editors) | 1 | 33% |
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 % |
|---|---|---|
| Spain | 1 | 1% |
| Unknown | 75 | 99% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Researcher | 13 | 17% |
| Student > Ph. D. Student | 12 | 16% |
| Other | 9 | 12% |
| Student > Doctoral Student | 8 | 11% |
| Student > Bachelor | 8 | 11% |
| Other | 10 | 13% |
| Unknown | 16 | 21% |
| Readers by discipline | Count | As % |
|---|---|---|
| Biochemistry, Genetics and Molecular Biology | 19 | 25% |
| Medicine and Dentistry | 15 | 20% |
| Pharmacology, Toxicology and Pharmaceutical Science | 8 | 11% |
| Agricultural and Biological Sciences | 4 | 5% |
| Chemistry | 4 | 5% |
| Other | 6 | 8% |
| Unknown | 20 | 26% |
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 28 March 2016.
All research outputs
#15,220,418
of 24,164,942 outputs
Outputs from Orphanet Journal of Rare Diseases
#1,664
of 2,840 outputs
Outputs of similar age
#164,779
of 305,022 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#26
of 30 outputs
Altmetric has tracked 24,164,942 research outputs across all sources so far. This one is in the 34th percentile – i.e., 34% of other outputs scored the same or lower than it.
So far Altmetric has tracked 2,840 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 7.9. This one is in the 37th percentile – i.e., 37% 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 305,022 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 43rd percentile – i.e., 43% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 30 others from the same source and published within six weeks on either side of this one. This one is in the 16th percentile – i.e., 16% of its contemporaries scored the same or lower than it.