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X Demographics
Mendeley readers
Attention Score in Context
| Title |
Ammonia control and neurocognitive outcome among urea cycle disorder patients treated with glycerol phenylbutyrate
|
|---|---|
| Published in |
Hepatology, January 2013
|
| DOI | 10.1002/hep.26058 |
| Pubmed ID | |
| Authors |
George A. Diaz, Lauren S. Krivitzky, Masoud Mokhtarani, William Rhead, James Bartley, Annette Feigenbaum, Nicola Longo, William Berquist, Susan A. Berry, Renata Gallagher, Uta Lichter‐Konecki, Dennis Bartholomew, Cary O. Harding, Stephen Cederbaum, Shawn E. McCandless, Wendy Smith, Gerald Vockley, Stephen A., Mark S. Korson, David Kronn, Roberto Zori, J. Lawrence Merritt, Sandesh C.S. Nagamani, Joseph Mauney, Cynthia LeMons, Klara Dickinson, Tristen L. Moors, Dion F. Coakley, Bruce F. Scharschmidt, Brendan Lee |
| Abstract |
Glycerol phenylbutyrate is under development for treatment of urea cycle disorders (UCDs), rare inherited metabolic disorders manifested by hyperammonemia and neurological impairment. We report the results of a pivotal Phase 3, randomized, double-blind, crossover trial comparing ammonia control, assessed as 24-hour area under the curve (NH3 -AUC0-24hr ), and pharmacokinetics during treatment with glycerol phenylbutyrate versus sodium phenylbutyrate (NaPBA) in adult UCD patients and the combined results of four studies involving short- and long-term glycerol phenylbutyrate treatment of UCD patients ages 6 and above. Glycerol phenylbutyrate was noninferior to NaPBA with respect to ammonia control in the pivotal study, with mean (standard deviation, SD) NH3 -AUC0-24hr of 866 (661) versus 977 (865) μmol·h/L for glycerol phenylbutyrate and NaPBA, respectively. Among 65 adult and pediatric patients completing three similarly designed short-term comparisons of glycerol phenylbutyrate versus NaPBA, NH3 -AUC0-24hr was directionally lower on glycerol phenylbutyrate in each study, similar among all subgroups, and significantly lower (P < 0.05) in the pooled analysis, as was plasma glutamine. The 24-hour ammonia profiles were consistent with the slow-release behavior of glycerol phenylbutyrate and better overnight ammonia control. During 12 months of open-label glycerol phenylbutyrate treatment, average ammonia was normal in adult and pediatric patients and executive function among pediatric patients, including behavioral regulation, goal setting, planning, and self-monitoring, was significantly improved. Conclusion: Glycerol phenylbutyrate exhibits favorable pharmacokinetics and ammonia control relative to NaPBA in UCD patients, and long-term glycerol phenylbutyrate treatment in pediatric UCD patients was associated with improved executive function (ClinicalTrials.gov NCT00551200, NCT00947544, NCT00992459, NCT00947297). (HEPATOLOGY 2012). |
X Demographics
The data shown below were collected from the profile of 1 X user who shared this research output. Click here to find out more about how the information was compiled.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 1 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Practitioners (doctors, other healthcare professionals) | 1 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 100 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United Kingdom | 1 | 1% |
| Japan | 1 | 1% |
| Spain | 1 | 1% |
| Unknown | 97 | 97% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Master | 14 | 14% |
| Other | 12 | 12% |
| Student > Ph. D. Student | 11 | 11% |
| Researcher | 10 | 10% |
| Professor | 7 | 7% |
| Other | 24 | 24% |
| Unknown | 22 | 22% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 30 | 30% |
| Agricultural and Biological Sciences | 10 | 10% |
| Biochemistry, Genetics and Molecular Biology | 8 | 8% |
| Psychology | 8 | 8% |
| Pharmacology, Toxicology and Pharmaceutical Science | 4 | 4% |
| Other | 15 | 15% |
| Unknown | 25 | 25% |
Attention Score in Context
This research output has an Altmetric Attention Score of 13. 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 19 December 2023.
All research outputs
#2,721,824
of 25,374,917 outputs
Outputs from Hepatology
#1,185
of 9,093 outputs
Outputs of similar age
#26,293
of 289,017 outputs
Outputs of similar age from Hepatology
#19
of 153 outputs
Altmetric has tracked 25,374,917 research outputs across all sources so far. Compared to these this one has done well and is in the 89th percentile: it's in the top 25% of all research outputs ever tracked by Altmetric.
So far Altmetric has tracked 9,093 research outputs from this source. They typically receive a lot more attention than average, with a mean Attention Score of 10.4. This one has done well, scoring higher than 86% 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 289,017 tracked outputs that were published within six weeks on either side of this one in any source. This one has done particularly well, scoring higher than 90% of its contemporaries.
We're also able to compare this research output to 153 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 87% of its contemporaries.