| Title |
Teenagers and young adults with nephropathic cystinosis display significant bone disease and cortical impairment
|
|---|---|
| Published in |
Pediatric Nephrology, February 2018
|
| DOI | 10.1007/s00467-018-3902-x |
| Pubmed ID | |
| Authors |
Aurélia Bertholet-Thomas, Debora Claramunt-Taberner, Ségolène Gaillard, Georges Deschênes, Elisabeth Sornay-Rendu, Pawel Szulc, Martine Cohen-Solal, Solenne Pelletier, Marie-Christine Carlier, Pierre Cochat, Justine Bacchetta |
| Abstract |
Bone impairment appears to be a novel complication of nephropathic cystinosis despite cysteamine therapy. Its exact underlying pathophysiology is nevertheless unclear. The objective of this study was to evaluate bone status among patients included in the French Crystobs study. In addition to clinical data, bone status was evaluated using biomarkers (ALP, PTH, 25-D, 1-25D, FGF23), DXA (spine and total body), and high-resolution peripheral quantitative computed tomography (HR-pQCT) at the tibia and radius. Results were compared to age- and gender-matched healthy controls (1:2 basis) from the local reference cohorts. At a median age of 22.5 (10.2-34.6) years, 10 patients with nephropathic cystinosis were included (2 receiving conservative therapies, 2 undergoing hemodialysis, 6 with a past of renal transplantation); 7 out of 10 patients complained of a bone symptom (past of fracture, bone deformations, and/or bone pain). Biochemicals and spine DXA did not show any significant abnormalities. Using HR-pQCT, significant decreases in cortical parameters (e.g., cortical thickness 850 (520-1100) versus 1225 (480-1680) μm; p < 0.05) and total volumetric bone mineral density (290 (233-360) versus 323 (232-406) mg/cm3; p < 0.05) were observed in cystinotic patients in comparison to controls at the tibia. There were no differences for trabecular parameters. Similar results were observed at the radius. In this pilot study, bone impairment (rather cortical than trabecular) is a significant clinical problem in nephropathic cystinosis; 70% of patients displayed significant bone symptoms, during teenage or young adulthood. This new complication should be known by physicians because of its potential dramatic impact on quality of life. |
X Demographics
The data shown below were collected from the profiles of 11 X users who shared this research output. Click here to find out more about how the information was compiled.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Brazil | 1 | 9% |
| Mexico | 1 | 9% |
| Canada | 1 | 9% |
| Unknown | 8 | 73% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 9 | 82% |
| Science communicators (journalists, bloggers, editors) | 2 | 18% |
Mendeley readers
The data shown below were compiled from readership statistics for 58 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 58 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Bachelor | 8 | 14% |
| Student > Master | 8 | 14% |
| Student > Ph. D. Student | 6 | 10% |
| Student > Postgraduate | 4 | 7% |
| Other | 4 | 7% |
| Other | 7 | 12% |
| Unknown | 21 | 36% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 20 | 34% |
| Nursing and Health Professions | 8 | 14% |
| Biochemistry, Genetics and Molecular Biology | 3 | 5% |
| Sports and Recreations | 3 | 5% |
| Psychology | 1 | 2% |
| Other | 2 | 3% |
| Unknown | 21 | 36% |
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 15 June 2018.
All research outputs
#5,715,264
of 23,023,224 outputs
Outputs from Pediatric Nephrology
#934
of 3,589 outputs
Outputs of similar age
#117,757
of 445,207 outputs
Outputs of similar age from Pediatric Nephrology
#20
of 74 outputs
Altmetric has tracked 23,023,224 research outputs across all sources so far. Compared to these this one has done well and is in the 75th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 3,589 research outputs from this source. They receive a mean Attention Score of 4.9. This one has gotten more attention than average, scoring higher than 73% 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 445,207 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 73% of its contemporaries.
We're also able to compare this research output to 74 others from the same source and published within six weeks on either side of this one. This one has gotten more attention than average, scoring higher than 72% of its contemporaries.