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Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1

Overview of attention for article published in Orphanet Journal of Rare Diseases, July 2014
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Title
Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1
Published in
Orphanet Journal of Rare Diseases, July 2014
DOI 10.1186/1750-1172-9-89
Pubmed ID
Authors

Yasuhiro Hasegawa, Hisamitsu Hayashi, Sotaro Naoi, Hiroki Kondou, Kazuhiko Bessho, Koji Igarashi, Kentaro Hanada, Kie Nakao, Takeshi Kimura, Akiko Konishi, Hironori Nagasaka, Yoko Miyoshi, Keiichi Ozono, Hiroyuki Kusuhara

Abstract

Progressive familial intrahepatic cholestasis type 1 (PFIC1), an inherited liver disease caused by mutations in ATP8B1, progresses to severe cholestasis with a sustained intractable itch. Currently, no effective therapy has been established for PFIC1. Decreased function of the bile salt export pump (BSEP) in hepatocytes is suggested to be responsible for the severe cholestasis observed in PFIC1. We found a previously unidentified pharmacological effect of 4-phenylbutyrate (4PB) that increases the expression and function of BSEP. Here, we tested 4PB therapy in three patients with PFIC1.

X Demographics

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.
Mendeley readers

Mendeley readers

The data shown below were compiled from readership statistics for 41 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Japan 1 2%
Unknown 40 98%

Demographic breakdown

Readers by professional status Count As %
Researcher 6 15%
Student > Master 5 12%
Student > Bachelor 4 10%
Other 3 7%
Student > Ph. D. Student 3 7%
Other 10 24%
Unknown 10 24%
Readers by discipline Count As %
Medicine and Dentistry 12 29%
Biochemistry, Genetics and Molecular Biology 6 15%
Agricultural and Biological Sciences 4 10%
Pharmacology, Toxicology and Pharmaceutical Science 3 7%
Psychology 3 7%
Other 3 7%
Unknown 10 24%
Attention Score in Context

Attention Score in Context

This research output has an Altmetric Attention Score of 1. 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 24 July 2014.
All research outputs
#15,303,056
of 22,758,963 outputs
Outputs from Orphanet Journal of Rare Diseases
#1,785
of 2,611 outputs
Outputs of similar age
#131,964
of 226,959 outputs
Outputs of similar age from Orphanet Journal of Rare Diseases
#29
of 42 outputs
Altmetric has tracked 22,758,963 research outputs across all sources so far. This one is in the 22nd percentile – i.e., 22% of other outputs scored the same or lower than it.
So far Altmetric has tracked 2,611 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 23rd percentile – i.e., 23% 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 226,959 tracked outputs that were published within six weeks on either side of this one in any source. This one is in the 32nd percentile – i.e., 32% of its contemporaries scored the same or lower than it.
We're also able to compare this research output to 42 others from the same source and published within six weeks on either side of this one. This one is in the 23rd percentile – i.e., 23% of its contemporaries scored the same or lower than it.