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Immunodeficiency for Severe Epstein-Barr Virus Infection

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ClinicalTrials.gov Identifier: NCT03374566
Recruitment Status : Recruiting
First Posted : December 15, 2017
Last Update Posted : December 24, 2018
Sponsor:
Information provided by (Responsible Party):
Jinqiao Sun, Children's Hospital of Fudan University

Study Description
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Brief Summary:
The purpose of this study is to investigate the immune responses associated with Epstein-Barr virus infections, and to find out the possible immunodeficiency that may be linked to severe Epstein-Barr virus infections.

Condition or disease
Epstein-Barr Virus Infections Immunodeficiency

Detailed Description:

Epstein-Barr virus (EBV) belongs to herpesviridae family, which infects more than 90% of the population. EBV infection is usually asymptomatic and establishes lifelong persistence in the host, although primary infection later than adolescence frequently results in infectious mononucleosis (IM). Rarely, individuals may develop a subgroup of EBV-associated life threatening complications (including liver dysfunction, haemophagocytosis and malignancy).

Although EBV-infected B cells have the potential for proliferation, they are effectively removed by the EBV-specific cytotoxic T cells (CTL). In the immunocompetent hosts, natural killer (NK) cells and antigen-specific cluster designation 8 (CD8+) T-cells play an important role in inhibiting progression of primary EBV infection by granule-mediated cytotoxicity. The immune system is necessary to control the virus-induced transformation and the B-cell unlimited proliferation.

Primary immunodeficiency are a heterogeneous group of hereditary diseases that are associated with compromised immune responses. There are a number of immunodeficiency resulting in inability of immune system to control EBV infection, for example X-linked Lymphoproliferative disease (XLP)/signaling lymphocyte activation molecule (SLAM)-associated protein (SAP) deficiency, X-linked inhibitor of apoptosis (XIAP) deficiency, cluster of differentiation antigen 27 (CD27) deficiency, interleukin-2-inducible T-cell kinase (ITK) deficiency, and so on. Whereas, some other clinical states associated with EBV-susceptibility remain largely unknown. Rare EBV-infected individuals without apparent immunodeficiency also present with persistent IM-like symptoms, hepatosplenomegaly, liver dysfunction, lymphadenopathy and haemophagocytic lymphohistiocytosis.

Patients presenting with severe EBV infections should be focused on early identification of a possible primary immunodeficiency or a chronic active EBV infection clinical condition (CAEBV) and haemophagocytic lymphohistiocytosis(HLH). Immunological phenotyping of NK-, T- and B-cell differentiation, and functional assays including cytotoxic cell killing function and cytotoxic granule release, provide a useful identification for clinical conditions inability to control EBV infections. Genomic DNA is isolated from peripheral blood mononuclear cells and will be amplified to screen for possible immunodeficiency.

The reasons for those patients inability to control the EBV infection are still unknown. However no effective treatment is currently available, those patients might benefit from early hematopoietic stem cell transplantation (HSCT). Through this study, we hope to identify the unknown immune immunodeficiency and pathophysiology of those EBV-associated conditions. The investigators propose to help make early diagnosis and develop effective therapies.


Study Design
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Study Type : Observational
Estimated Enrollment : 100 participants
Observational Model: Cohort
Time Perspective: Prospective
Official Title: Screening for Immunodeficiency Diseases in Patients With Severe Epstein-Barr Virus Infection
Actual Study Start Date : December 1, 2017
Estimated Primary Completion Date : November 30, 2022
Estimated Study Completion Date : November 30, 2022

Resource links provided by the National Library of Medicine


Groups and Cohorts
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Group/Cohort
Screened patients
Immunodeficiency screening: Heparinized peripheral blood is obtained from patients with severe EBV infections for immunological function assays and genetic analysis, when current screening is performed after parents' information and consent.


Outcome Measures
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Primary Outcome Measures :
  1. Immunodeficiency incidence in patients with severe EBV infection [ Time Frame: 5 years ]
    We will investigate immunodeficiency incidence in patients with severe EBV infection.


Biospecimen Retention:   Samples With DNA
Heparinized peripheral blood is obtained from patients with severe EBV infections. Samples is performed after parents' information and consent.

Eligibility Criteria
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Ages Eligible for Study:   up to 18 Years   (Child, Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
We will evaluate immunodeficiency incidence in patients with severe EBV infection
Criteria

Inclusion Criteria:

  • 1. Age：birth to 18 years 2. Severe Epstein-Barr Virus infection

Exclusion Criteria:

  • 1. Lack of parental consent
Contacts and Locations
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Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03374566


Contacts
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Contact: Jinqiao Sun, Ph.D.,M.D 86-21-64932909 jinqiaosun@sina.com
Contact: Weili Yan, Ph.D. 86-21-64931913 yanwl@fudan.edu.cn

Locations
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China, Shanghai
Children's Hospital of Fudan University Recruiting
Shanghai, Shanghai, China
Sponsors and Collaborators
Children's Hospital of Fudan University
Investigators
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Principal Investigator: Jinqiao Sun, Ph.D.,M.D Children's Hospital of Fudan University
More Information
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Publications:

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Responsible Party: Jinqiao Sun, Professor, Children's Hospital of Fudan University
ClinicalTrials.gov Identifier: NCT03374566     History of Changes
Other Study ID Numbers: EBV
First Posted: December 15, 2017    Key Record Dates
Last Update Posted: December 24, 2018
Last Verified: December 2018
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No

Keywords provided by Jinqiao Sun, Children's Hospital of Fudan University:
Epstein-Barr Virus Infections
Immunodeficiency
Screening
Immunologic Function

Additional relevant MeSH terms:
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Infection
Communicable Diseases
Immunologic Deficiency Syndromes
Virus Diseases
Epstein-Barr Virus Infections
 Immune System Diseases
Herpesviridae Infections
DNA Virus Infections
Tumor Virus Infections