NDLI: Killer cell immunoglobulin-like receptor gene polymorphisms predispose susceptibility to Epstein-Barr virus associated hemophagocytic lymphohistiocytosis in Chinese children.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Qiang, Qin Zhengde, Xie Chunyan, Liu Zhizhuo, Huang Junmei, Xu Junhong, Ai Zheng, Chengyun Henter, Jan-Inge Kunling, Shen
Spatial Coverage	China
Description	Country affiliation: China Author Affiliation: Qiang Q ( Key Laboratory of Major Diseases in Child and National Key Discipline of Pediatrics, Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, Beijing, China.)
Abstract	Epstein-Barr virus associated hemophagocytic lymphohistiocytosis (EBV-HLH) has a high mortality rate among children. The pathogenesis of, and underlying predisposing factors for, EBV-HLH are as yet unclear; however, natural killer cells may play a key role in progression of the disease. This study attempted to determine whether killer cell immunoglobulin-like receptor (KIR) gene polymorphisms are responsible for susceptibility to EBV-HLH. Of the 125 children with EBV infection studied, 59 had EBV-HLH and 66 patients had EBV associated infectious mononucleosis (IM) without HLH. The control group was 146 normal children without immune deficiency. KIR polymorphisms were determined by polymerase chain reaction with sequence-specific primers. KIR polymorphism data were analyzed using the X(2) test or Fisher's exact test. The overall observed carrier frequency (OF) of KIR2DS5 was significantly higher in EBV-HLH patients than in IM patients and normal controls (49.2% versus 31.8%, P = 0.048; 49.2% versus 31.5%, P = 0.018, respectively), and the odds ratios (95% confidence interval) were 2.071 (1.001-4.286) and 2.101(1.132-3.900) respectively. The OF of KIR3DS1 was significantly higher in the EBV-HLH patients than in the IM patients (47.4% versus 24.6%, P = 0.012), but not different from normal controls. In summary, KIR polymorphisms may be involved in the development of EBV-HLH, with KIR2DS5 promoting susceptibility to this disease. The obtained KIR data will enrich the understanding of genetic relationships among diseases associated with EBV infection in children.
File Format	HTM / HTML
ISSN	03855600
Issue Number	6
Volume Number	56
e-ISSN	13480421
Journal	Microbiology and Immunology
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2012-06-01
Publisher Place	Australia
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Microbiology Discipline Immunology Epstein-barr Virus Infections Complications Genetic Predisposition To Disease Lymphohistiocytosis, Hemophagocytic Genetics Polymorphism, Genetic Receptors, Kir Adolescent Asian Continental Ancestry Group Child Child, Preschool China Dna Primers Female Gene Frequency Humans Infant Male Polymerase Chain Reaction Journal Article Research Support, Non-u.s. Gov't
Content Type	Text
Resource Type	Article
Subject	Virology Immunology Microbiology

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