Abstract:
PURPOSE:Patients with primary immunodeficiency (PID) are at risk of serious complications. However, data on the incidence and causes of emergency hospital admissions are scarce. The primary objective of the present study was to describe emergency hospital admissions among patients with PID, with a view to identifying "at-risk" patient profiles. METHODS:We performed a prospective observational 12-month multicenter study in France via the CEREDIH network of regional PID reference centers from November 2010 to October 2011. All patients with PIDs requiring emergency hospital admission were included. RESULTS:A total of 200 admissions concerned 137 patients (73 adults and 64 children, 53% of whom had antibody deficiencies). Thirty admissions were reported for 16 hematopoietic stem cell transplantation recipients. When considering the 170 admissions of non-transplant patients, 149 (85%) were related to acute infections (respiratory tract infections and gastrointestinal tract infections in 72 (36%) and 34 (17%) of cases, respectively). Seventy-seven percent of the admissions occurred during winter or spring (December to May). The in-hospital mortality rate was 8.8% (12 patients); death was related to a severe infection in 11 cases (8%) and Epstein-Barr virus-induced lymphoma in 1 case. Patients with a central venous catheter (n = 19, 13.9%) were significantly more hospitalized for an infection (94.7%) than for a non-infectious reason (5.3%) (p = 0.04). CONCLUSION:Our data showed that the annual incidence of emergency hospital admission among patients with PID is 3.4%. The leading cause of emergency hospital admission was an acute infection, and having a central venous catheter was associated with a significantly greater risk of admission for an infectious episode.
journal_name
J Clin Immunoljournal_title
Journal of clinical immunologyauthors
Coignard-Biehler H,Mahlaoui N,Pilmis B,Barlogis V,Brosselin P,De Vergnes N,Debré M,Malphettes M,Frange P,Catherinot E,Pellier I,Durieu I,Perlat A,Royer B,Le Quellec A,Jeziorski E,Fischer A,Lortholary O,CEREDIH Frenchdoi
10.1007/s10875-019-00658-9subject
Has Abstractpub_date
2019-10-01 00:00:00pages
702-712issue
7eissn
0271-9142issn
1573-2592pii
10.1007/s10875-019-00658-9journal_volume
39pub_type
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-014-0037-0
更新日期:2014-08-01 00:00:00
abstract::Unusual susceptibility to mycobacterial infections can be caused by deleterious mutations in genes that encode the interferon-gamma receptor 1 chain. Such mutations hamper the activation of macrophages by a type 1 immune response and result in enhanced survival of intracellular pathogens. We here report two patients w...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-007-9097-8
更新日期:2007-09-01 00:00:00
abstract::Natural killer (NK) cell deficiency (NKD) is a subset of primary immunodeficiency disorders (PID) in which an abnormality of NK cells represents a major immunological defect resulting in the patient's clinical immunodeficiency. This is distinct from a much larger group of PIDs that include an NK cell abnormality as a ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
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更新日期:2020-01-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-014-0004-9
更新日期:2014-04-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-014-0115-3
更新日期:2015-01-01 00:00:00
abstract:OBJECTIVES:Mutations affecting the TMEM173 gene cause STING-associated vasculopathy with onset in infancy (SAVI). No standard immunosuppressive treatment approach is able to control disease progression in patients with SAVI. We studied the efficacy and safety of targeting type I IFN signaling with the Janus kinase inhi...
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doi:10.1007/s10875-019-00645-0
更新日期:2019-07-01 00:00:00
abstract::Common variable immune deficiency (CVID) B cells have impaired responses to TLR7 and TLR9 agonists including poor cell proliferation, loss of cytokine production, and failure to produce IgG or IgA. We show that TLR7- or 9-activated B cells from CVID subjects with >0.5% peripheral isotype-switched CD27(+) B cells (grou...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-011-9602-y
更新日期:2012-02-01 00:00:00
abstract::The original version of this article contained an error in Fig. 1. The incomplete heredogram mistakenly appeared in panel d. ...
journal_title:Journal of clinical immunology
pub_type: 已发布勘误
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更新日期:2019-11-01 00:00:00
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更新日期:2012-06-01 00:00:00
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journal_title:Journal of clinical immunology
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更新日期:1987-03-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
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更新日期:2010-01-01 00:00:00
abstract:INTRODUCTION:Major histocompatibility complex class II (MHC-II) expression deficiency is a combined primary immunodeficiency leading to the impairment of the cellular and humoral immune responses. A majority of affected patients belong to consanguineous families particularly from the Maghreb, where a founder effect for...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-013-9863-8
更新日期:2013-05-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-009-9285-9
更新日期:2009-07-01 00:00:00
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pub_type: 杂志文章
doi:10.1007/s10875-018-0567-y
更新日期:2018-11-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
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更新日期:2010-09-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-017-0423-5
更新日期:2017-08-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-010-9447-9
更新日期:2010-11-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00917916
更新日期:1990-05-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9800-2
更新日期:2013-01-01 00:00:00
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pub_type: 杂志文章
doi:10.1007/s10875-012-9703-2
更新日期:2012-10-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
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更新日期:1988-11-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-013-9891-4
更新日期:2013-07-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
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更新日期:2012-09-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-019-0594-3
更新日期:2019-02-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章,多中心研究
doi:10.1007/s10875-014-0001-z
更新日期:2014-05-01 00:00:00
abstract::Murine hematopoietic tissues contain cells which, upon injection into lethally irradiated mice, produce nodules on the surface of their spleen (colony-forming unit--spleen; CFU-S). The exact hierarchical level of the hematopoietic progenitors which give rise to CFU-S is not fully established; however, cell populations...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1027302310505
更新日期:1997-05-01 00:00:00
abstract:INTRODUCTION:The molecular and cellular interactions between the immune system and bone tissue have been established. Sex hormone deficiency after menopause has multifunctional role by influencing growth, differentiation, and metabolism of the skeletal and the immune system. DISCUSSION:We have used nonparametric and m...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-009-9321-9
更新日期:2009-11-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1010987426835
更新日期:2001-07-01 00:00:00
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journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00916947
更新日期:1988-11-01 00:00:00
abstract::The cellular and molecular basis of immune senescence is unclear. A number of mechanisms have been proposed. In this issue of the Journal of Clinical Immunology, some of the mechanisms for various immunologic abnormalities in aging are presented. In this article, various molecular steps of both death receptor and mito...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1023/a:1006653917314
更新日期:2000-07-01 00:00:00