| Title |
X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis
|
|---|---|
| Published in |
Journal of Clinical Immunology, August 2016
|
| DOI | 10.1007/s10875-016-0320-3 |
| Pubmed ID | |
| Authors |
Cathal L. Steele, Matthew Doré, Sandra Ammann, Maurice Loughrey, Angeles Montero, Siobhan O. Burns, Emma C. Morris, Bobby Gaspar, Kimberly Gilmour, Shahnaz Bibi, Hiba Shendi, Lisa Devlin, Carsten Speckmann, David M. Edgar |
| Abstract |
The X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency characterized by Epstein-Barr virus (EBV)-driven hemophagocytic lymphohistiocytosis (HLH), splenomegaly, and colitis. Here, we present, for the first time, granulomatous hepatitis and granulomatous and lymphocytic interstitial lung disease (GLILD) as manifestations of XIAP deficiency. We report successful treatment of GLILD in XIAP deficiency with rituximab and azathioprine and discuss the role of XIAP deficiency in immune dysregulation. |
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 % |
|---|---|---|
| Members of the public | 1 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 36 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United Kingdom | 1 | 3% |
| Unknown | 35 | 97% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Researcher | 7 | 19% |
| Student > Master | 4 | 11% |
| Student > Ph. D. Student | 4 | 11% |
| Student > Doctoral Student | 3 | 8% |
| Other | 3 | 8% |
| Other | 7 | 19% |
| Unknown | 8 | 22% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 11 | 31% |
| Immunology and Microbiology | 5 | 14% |
| Pharmacology, Toxicology and Pharmaceutical Science | 2 | 6% |
| Biochemistry, Genetics and Molecular Biology | 2 | 6% |
| Nursing and Health Professions | 2 | 6% |
| Other | 2 | 6% |
| Unknown | 12 | 33% |
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 16 March 2018.
All research outputs
#18,541,268
of 22,963,381 outputs
Outputs from Journal of Clinical Immunology
#1,137
of 1,570 outputs
Outputs of similar age
#284,710
of 367,538 outputs
Outputs of similar age from Journal of Clinical Immunology
#15
of 23 outputs
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