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Hematopoietic Stem Cell Transplantation for XIAP Deficiency in Japan

Overview of attention for article published in Journal of Clinical Immunology, November 2016
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Title
Hematopoietic Stem Cell Transplantation for XIAP Deficiency in Japan
Published in
Journal of Clinical Immunology, November 2016
DOI 10.1007/s10875-016-0348-4
Pubmed ID
Authors

Shintaro Ono, Tsubasa Okano, Akihiro Hoshino, Masakatsu Yanagimachi, Kazuko Hamamoto, Yozo Nakazawa, Toshihiko Imamura, Masaei Onuma, Hidetaka Niizuma, Yoji Sasahara, Hiroshi Tsujimoto, Taizo Wada, Reiko Kunisaki, Masatoshi Takagi, Kohsuke Imai, Tomohiro Morio, Hirokazu Kanegane

Abstract

X-linked inhibitor of apoptosis protein (XIAP) deficiency is a rare immunodeficiency that is characterized by recurrent hemophagocytic lymphohistiocytosis (HLH) and splenomegaly and sometimes associated with refractory inflammatory bowel disease (IBD). Although hematopoietic stem cell transplantation (HSCT) is the only curative therapy, the outcomes of HSCT for XIAP deficiency remain unsatisfactory compared with those for SLAM-associated protein deficiency and familial HLH. To investigate the outcomes and adverse events of HSCT for patients with XIAP deficiency, a national survey was conducted. A spreadsheet questionnaire was sent to physicians who had provided HSCT treatment for patients with XIAP deficiency in Japan. Up to the end of September 2016, 10 patients with XIAP deficiency had undergone HSCT in Japan, 9 of whom (90%) had survived. All surviving patients had received a fludarabine-based reduced intensity conditioning (RIC) regimen. Although 5 patients developed post-HSCT HLH, 4 of them survived after etoposide administration. In addition, the IBD associated with XIAP deficiency improved remarkably after HSCT in all affected cases. The RIC regimen and HLH control might be important factors for successful HSCT outcomes, with improved IBD, in patients with XIAP deficiency.

Mendeley readers

The data shown below were compiled from readership statistics for 27 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 27 100%

Demographic breakdown

Readers by professional status Count As %
Unspecified 8 30%
Student > Ph. D. Student 7 26%
Student > Postgraduate 3 11%
Other 3 11%
Researcher 1 4%
Other 5 19%
Readers by discipline Count As %
Medicine and Dentistry 11 41%
Unspecified 9 33%
Agricultural and Biological Sciences 3 11%
Immunology and Microbiology 3 11%
Biochemistry, Genetics and Molecular Biology 1 4%
Other 0 0%

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 05 November 2016.
All research outputs
#7,451,497
of 8,620,037 outputs
Outputs from Journal of Clinical Immunology
#413
of 600 outputs
Outputs of similar age
#213,087
of 256,007 outputs
Outputs of similar age from Journal of Clinical Immunology
#15
of 19 outputs
Altmetric has tracked 8,620,037 research outputs across all sources so far. This one is in the 1st percentile – i.e., 1% of other outputs scored the same or lower than it.
So far Altmetric has tracked 600 research outputs from this source. They typically receive a little more attention than average, with a mean Attention Score of 6.1. This one is in the 1st percentile – i.e., 1% of its peers scored the same or lower than it.
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We're also able to compare this research output to 19 others from the same source and published within six weeks on either side of this one. This one is in the 1st percentile – i.e., 1% of its contemporaries scored the same or lower than it.