30-Year Review of Pediatric- and Adult-Onset CVID: Clinical Correlates and Prognostic Indicators.

Abstract:

PURPOSE:To evaluate mortality risk factors in pediatric-onset common variable immunodeficiency disorders (CVID), we evaluated the largest single-institution cohort of pediatric-onset CVID patients. Previous publications on CVID have provided valuable descriptive data, but lack risk stratification to guide physicians in management of these patients. METHODS:Retrospective chart review of 198 subjects with CVID at a single institution, of whom 91 had disease onset at a pediatric age. Clinical and laboratory data were collected at diagnosis and in follow-up. Odds ratios and Fisher tests were utilized to examine trends. This study was approved by an institutional review board. RESULTS:Clinical features and laboratory results for subjects diagnosed with CVID at a pediatric age are similar to those who had adult-onset CVID. However, majority of the deceased subjects (13/18) were at a pediatric age at CVID symptom onset. These subjects had a lower age at mortality, multiple comorbidities, and often depression. The most common cause of death was infection. Lung disease (OR 5, p < 0.05) and infection with severe/opportunistic organisms (OR 9, p < 0.05) are directly related to increased mortality. Delay in diagnosis of CVID is also correlated with mortality. Intermediary markers correlating with mortality include anemia, GERD, and depression. CONCLUSIONS:There are many similarities between patients with pediatric- and adult-onset CVID; however, the mortality of pediatric CVID in our cohort is striking. This is the first study to identify specific factors correlated with mortality in pediatric-onset CVID to guide pediatricians and subspecialists in managing these immunodeficient patients.

journal_name

J Clin Immunol

authors

Baloh C,Reddy A,Henson M,Prince K,Buckley R,Lugar P

doi

10.1007/s10875-019-00674-9

subject

Has Abstract

pub_date

2019-10-01 00:00:00

pages

678-687

issue

7

eissn

0271-9142

issn

1573-2592

pii

10.1007/s10875-019-00674-9

journal_volume

39

pub_type

杂志文章
  • Anti-ribosomal ribonucleoprotein autoantibodies in systemic lupus erythematosus.

    abstract::Sera from systemic lupus erythematosus (SLE) patients giving a fluorescent ribosomal pattern on tissue and cell preparations also showed precipitating autoantibodies against purified rat liver ribosomes. Ribosomal antigen is also present in rabbit thymus cellular extract (RTE), since the same sera gave precipitin line...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00915287

    authors: Meroni PL,de Bartolo G,Barcellini W,Riboldi PS,Basile R,Betterle C,Zanussi C

    更新日期:1984-01-01 00:00:00

  • A role of fas-associated death domain (FADD) in increased apoptosis in aged humans.

    abstract::Fas-associated death domain (FADD) is an adapter protein that is recruited to the death-inducing signaling complex (DISC) during signaling via death receptors. FADD is a common conduit in both CD95-mediated and TNF-R-mediated apoptosis. In aging, T cells show increased susceptibility to death receptor-induced apoptosi...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1023/B:JOCI.0000018059.56924.99

    authors: Gupta S,Kim C,Yel L,Gollapudi S

    更新日期:2004-01-01 00:00:00

  • Early systemic sclerosis: serum profiling of factors involved in endothelial, T-cell, and fibroblast interplay is marked by elevated interleukin-33 levels.

    abstract:PURPOSE:To assess the serum profile of factors involved in endothelial, T-cell, and fibroblast interplay in patients with Raynaud's phenomenon (RP) associated with nailfold vodeocapillaroscopy (NVC) scleroderma findings and/or systemic sclerosis (SSc) marker autoantibodies, recently labeled as early SSc patients. METH...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-014-0037-0

    authors: Vettori S,Cuomo G,Iudici M,D'Abrosca V,Giacco V,Barra G,De Palma R,Valentini G

    更新日期:2014-08-01 00:00:00

  • Lymphoma Secondary to Congenital and Acquired Immunodeficiency Syndromes at a Turkish Pediatric Oncology Center.

    abstract::The prevalence of lymphoma in primary immunodeficiency cases and autoimmune diseases, as well as on a background of immunodeficiency following organ transplants, is increasing. The lymphoma treatment success rate is known to be a low prognosis. Our study aimed to emphasize the low survival rates in immunodeficient vs....

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-016-0324-z

    authors: Tanyildiz HG,Dincaslan H,Yavuz G,Unal E,Ikinciogulları A,Dogu F,Tacyildiz N

    更新日期:2016-10-01 00:00:00

  • Autoimmunity and Inflammation in CVID: a Possible Crosstalk between Immune Activation, Gut Microbiota, and Epigenetic Modifications.

    abstract::Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency among adults and is characterized by a B cell dysfunction and increased risk of respiratory tract infections with encapsulated bacteria. However, a large proportion of patients also has inflammatory and autoimmune complicat...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审

    doi:10.1007/s10875-018-0574-z

    authors: Jørgensen SF,Fevang B,Aukrust P

    更新日期:2019-01-01 00:00:00

  • Infectious and immunologic phenotype of MECP2 duplication syndrome.

    abstract::MECP2 (methyl CpG binding protein 2) duplication causes syndromic intellectual disability. Patients often suffer from life-threatening infections, suggesting an additional immunodeficiency. We describe for the first time the detailed infectious and immunological phenotype of MECP2 duplication syndrome. 17/27 analyzed ...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-015-0129-5

    authors: Bauer M,Kölsch U,Krüger R,Unterwalder N,Hameister K,Kaiser FM,Vignoli A,Rossi R,Botella MP,Budisteanu M,Rosello M,Orellana C,Tejada MI,Papuc SM,Patat O,Julia S,Touraine R,Gomes T,Wenner K,Xu X,Afenjar A,Toutain

    更新日期:2015-02-01 00:00:00

  • Life-Threatening Infections Due to Live-Attenuated Vaccines: Early Manifestations of Inborn Errors of Immunity.

    abstract::Live-attenuated vaccines (LAVs) can protect humans against 12 viral and three bacterial diseases. By definition, any clinical infection caused by a LAV that is sufficiently severe to require medical intervention attests to an inherited or acquired immunodeficiency that must be diagnosed or identified. Self-healing inf...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审

    doi:10.1007/s10875-019-00642-3

    authors: Pöyhönen L,Bustamante J,Casanova JL,Jouanguy E,Zhang Q

    更新日期:2019-05-01 00:00:00

  • A portrait of the Bcl-2 protein family: life, death, and the whole picture.

    abstract::The Bcl-2 family of proteins are important regulators of cell death. They are comprised of two opposing factions, the proapoptotic versus the antiapoptotic members. Both are required for normal development and cellular homeostasis of the immune system and other tissues. However, in certain circumstances they may parti...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审

    doi:10.1023/a:1020598632068

    authors: Pellegrini M,Strasser A

    更新日期:1999-11-01 00:00:00

  • Efficacy and safety of subcutaneous vivaglobin® replacement therapy in previously untreated patients with primary immunodeficiency: a prospective, multicenter study.

    abstract::Treatment of primary immunodeficiency (PI) is typically initiated with intravenous immunoglobulin (IVIG) loading and then continued with IVIG or subcutaneous IgG (SCIG). This prospective, open-label, multicenter, 6-month study evaluated a new regimen of initiating IgG therapy with SCIG in 18 previously untreated patie...

    journal_title:Journal of clinical immunology

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1007/s10875-011-9588-5

    authors: Borte M,Quinti I,Soresina A,Fernández-Cruz E,Ritchie B,Schmidt DS,McCusker C

    更新日期:2011-12-01 00:00:00

  • Identification of patients with RAG mutations previously diagnosed with common variable immunodeficiency disorders.

    abstract:PURPOSE:Combined immunodeficiency (CID) presents a unique challenge to clinicians. Two patients presented with the prior clinical diagnosis of common variable immunodeficiency (CVID) disorder marked by an early age of presentation, opportunistic infections, and persistent lymphopenia. Due to the presence of atypical cl...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-014-0121-5

    authors: Buchbinder D,Baker R,Lee YN,Ravell J,Zhang Y,McElwee J,Nugent D,Coonrod EM,Durtschi JD,Augustine NH,Voelkerding KV,Csomos K,Rosen L,Browne S,Walter JE,Notarangelo LD,Hill HR,Kumánovics A

    更新日期:2015-02-01 00:00:00

  • In vitro differentiation of human monocytes into dendritic cells by peptic-tryptic digest of gliadin is independent of genetic predisposition and the presence of celiac disease.

    abstract:INTRODUCTION:This study was done to further reveal the role of the innate immune system in celiac disease. METHODS:Dendritic cells were matured from venous blood of patients with active or treated celiac disease and DQ2-DQ8-positive or negative controls. Dendritic cells were treated with a peptic-tryptic digest of gli...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-008-9228-x

    authors: Rakhimova M,Esslinger B,Schulze-Krebs A,Hahn EG,Schuppan D,Dieterich W

    更新日期:2009-01-01 00:00:00

  • The Elements Steering Pathogenesis in IgG-Mediated Alloimmune Diseases.

    abstract::Alloimmune diseases can occur in pregnancy and after blood transfusions, where antibodies are formed, targeting foreign cells and tissues for destruction by myeloid cells through IgG Fc-receptors (FcγR). In pregnancy, antibodies against human blood group or platelet antigens (e.g. HPA1-a) cause life-threatening anemia...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审

    doi:10.1007/s10875-016-0253-x

    authors: Sonneveld ME,van der Schoot CE,Vidarsson G

    更新日期:2016-05-01 00:00:00

  • Effect of palifermin in a murine model of graft-versus-host disease (GVHD) associated with Th2 cytokine production, autoantibody production, and glomerulonephritis.

    abstract::Palifermin (recombinant human keratinocyte growth factor) prevents the development of acute, lethal graft-versus-host disease (GVHD). It does so, at least in part, by protecting cells from injury. Another property of Palifermin is immune regulation. How the latter influences the evolution of GVHD remains uncertain. We...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-006-9039-x

    authors: Ellison CA,Gibson IW,Hayglass KT,Gartner JG

    更新日期:2006-09-01 00:00:00

  • Disseminated Mycobacterium kansasii disease in complete DiGeorge syndrome.

    abstract:PURPOSE:Complete DiGeorge syndrome (cDGS) describes a subset of patients with DiGeorge syndrome that have thymic aplasia, and thus are at risk for severe opportunistic infections. Patients with cDGS and mycobacterial infection have not previously been described. We present this case to illustrate that patients with cDG...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-015-0171-3

    authors: Yin SM,Ferdman RM,Wang L,Markert ML,Tam JS

    更新日期:2015-07-01 00:00:00

  • High frequency of immune dysfunctions in asbestos workers and in patients with malignant mesothelioma.

    abstract::We have examined the primary immune responses, the numbers of total T (T11+) cells, T-helper (T4+) cells, T-suppressor (T8+) cells, and natural killer (NK) (Leu7+) cells, in 118 healthy control subjects and compared the data to those obtained from 20 patients with clinically diagnosed malignant mesothelioma and 375 lo...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00918702

    authors: Lew F,Tsang P,Holland JF,Warner N,Selikoff IJ,Bekesi JG

    更新日期:1986-05-01 00:00:00

  • Molecular steps of cell suicide: an insight into immune senescence.

    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

    authors: Gupta S

    更新日期:2000-07-01 00:00:00

  • Glucocorticoid regulates interleukin-37 in systemic lupus erythematosus.

    abstract::The aim of this study was to research the expression of IL-37 in systemic lupus erythematosus (SLE) patients and the effect of glucocorticoid on IL-37. Thirty newly diagnosed severe SLE patients receiving prednisone 1 mg/kg/day for 14 consecutive days and 30 healthy subjects were enrolled into this study. The plasma l...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-012-9791-z

    authors: Song L,Qiu F,Fan Y,Ding F,Liu H,Shu Q,Liu W,Li X

    更新日期:2013-01-01 00:00:00

  • Intravenous immunoglobulin: an update on the clinical use and mechanisms of action.

    abstract::Initially used as a replacement therapy for immunodeficiency diseases, intravenous immunoglobulin (IVIg) is now widely used for a number of autoimmune and inflammatory diseases. Considerable progress has been made in understanding the mechanisms by which IVIg exerts immunomodulatory effects in autoimmune and inflammat...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审

    doi:10.1007/s10875-007-9088-9

    authors: Negi VS,Elluru S,Sibéril S,Graff-Dubois S,Mouthon L,Kazatchkine MD,Lacroix-Desmazes S,Bayry J,Kaveri SV

    更新日期:2007-05-01 00:00:00

  • Frequent anti-V-region immune response to mouse B72.3 monoclonal antibody.

    abstract::The immune response of 56 colorectal cancer patients to a single infusion of 1 mg of radiolabeled (111In) mouse B72.3-GYK-DTPA immunoconjugate was examined using a double-antigen radiometric assay system. The incidence of antibody response was 48% to polyclonal mouse IgG, 71% to mouse B72.3, and 62% to chimeric B72.3....

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00918141

    authors: Khazaeli MB,Saleh MN,Liu T,Kaladas PM,Gilman SC,LoBuglio AF

    更新日期:1992-03-01 00:00:00

  • The influence of recombinant human erythropoietin on apoptosis and cytokine production of CD4+ lymphocytes from hemodialyzed patients.

    abstract::Recombinant human erythropoietin (rhEPO) treatment of hemodialyzed (HD) patients normalizes the altered phenotype of CD4(+) lymphocytes and restores the balance of Th1/Th2 cytokines. We decided to test how the presence of rhEPO in cell culture modulates cytokine production of CD4(+) lymphocytes in HD patients with sta...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-012-9835-4

    authors: Lisowska KA,Dębska-Ślizień A,Jasiulewicz A,Daca A,Bryl E,Witkowski JM

    更新日期:2013-04-01 00:00:00

  • Neurological Involvement in Childhood Evans Syndrome.

    abstract:PURPOSE:Immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) are associated in the definition of Evans syndrome (ES). The occurrence of neurological involvement in this population is poorly described and suggests an underlying primary immunodeficiency (PID). We aimed to describe the clinical man...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-019-0594-3

    authors: Pincez T,Neven B,Le Pointe HD,Varlet P,Fernandes H,Gareton A,Leverger G,Leblanc T,Chambost H,Michel G,Pasquet M,Millot F,Hermine O,Mathian A,Hully M,Zephir H,Hamidou M,Durand JM,Perel Y,Landman-Parker J,Rieux-Lauc

    更新日期:2019-02-01 00:00:00

  • Chronic arsenic exposure impairs macrophage functions in the exposed individuals.

    abstract:INTRODUCTION:Owing to the established roles of human macrophages in immune defense, we investigated the effect of chronic arsenic exposure upon these major hematopoietic cells in 70 arsenic-exposed individuals with skin lesions and 64 unexposed individuals. METHODS:Human monocyte-derived macrophages were prepared from...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-009-9304-x

    authors: Banerjee N,Banerjee S,Sen R,Bandyopadhyay A,Sarma N,Majumder P,Das JK,Chatterjee M,Kabir SN,Giri AK

    更新日期:2009-09-01 00:00:00

  • Anti-SM and anti-U1-RNP lupus antibody fine specificities.

    abstract::Evidence for the presence of multiple shared epitopes on the target cellular antigens was found when 290 anti-Sm and anti-U1-RNP lupus sera were analyzed by immunoblotting. Forty-eight different immunoblot patterns were observed with the sera. Studies with selected antigen-affinity-purified antibodies confirmed the pr...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00916822

    authors: Francoeur AM

    更新日期:1989-05-01 00:00:00

  • Elevated serum interleukin-6 levels in patients with acute hepatitis.

    abstract::To study the mechanisms of hepatocyte injury, we examined serum interleukin-6 (IL-6) level in acute hepatitis patients. Based on their clinical features, these patients were divided into three groups, acute hepatitis (AH), severe acute hepatitis, and fulminant hepatic failure (FHF). The present study demonstrated that...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00918089

    authors: Sun Y,Tokushige K,Isono E,Yamauchi K,Obata H

    更新日期:1992-05-01 00:00:00

  • Clinical and molecular characteristics of 35 Chinese children with Wiskott-Aldrich syndrome.

    abstract:BACKGROUND:Wiskott-Aldrich syndrome (WAS) is a rare primary immunodeficiency disease, with an incidence of 4/1,000,000 live male births. In China, an estimated number of 35 babies with WAS are born each year, but likely many remain undiagnosed. OBJECTIVES:The objectives of study were to review the clinical and molecul...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/s10875-009-9285-9

    authors: Lee PP,Chen TX,Jiang LP,Chen J,Chan KW,Lee TL,Ho MH,Nong SH,Yang Y,Fang YJ,Li Q,Wang XC,Yang XQ,Lau YL

    更新日期:2009-07-01 00:00:00

  • Spontaneous human lymphocyte-mediated cytotoxicity against tumor target cells. IX. The quantitation of natural killer cell activity.

    abstract::On analysis of in vitro assays of human natural killer (NK) cell function the inadequacy of commonly used methods of expressing lytic activity was apparent. A comparison was made of the data obtained using modifications of two equations-the simple exponential fit and the von Krogh equations. Both of these equations we...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00915477

    authors: Pross HF,Baines MG,Rubin P,Shragge P,Patterson MS

    更新日期:1981-01-01 00:00:00

  • Abnormal antibody responses in patients with persistent generalized lymphadenopathy.

    abstract::Persistent, generalized lymphadenopathy (PGL) is a recognized component of human immunodeficiency virus (HIV) infection. We conducted longitudinal studies of B and T cell function in seven homosexual men with HIV infection and PGL. All seven had abnormal antibody-mediated immunity as studied by sequential assessment o...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00915157

    authors: Ochs HD,Junker AK,Collier AC,Virant FS,Handsfield HH,Wedgwood RJ

    更新日期:1988-01-01 00:00:00

  • Fatty acid chain is a critical epitope for antiphospholipid antibody.

    abstract::To explore the role of phospholipid fatty acids in binding of antiphospholipid antibody (aPL) in ELISA, we tested aPL binding to phospholipids containing fatty acids of varying chain length and degree of saturation using direct ELISA and inhibition methods. Polyclonal IgG and IgM human aPL's bind to C18:1 phosphatidyl...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章

    doi:10.1007/BF00917913

    authors: Levy RA,Gharavi AE,Sammaritano LR,Habina L,Lockshin MD

    更新日期:1990-05-01 00:00:00

  • Monitoring of CD4+ and CD8+ T-cell responses after dendritic cell-based immunotherapy using CFSE dye dilution analysis.

    abstract::CFSE dye dilution analysis and [3H] thymidine incorporation were used side by side to assess proliferative responses of peripheral blood mononuclear cells (PBMCs) after vaccination of renal cell carcinoma patients (n=6) with antigen-loaded dendritic cells. Immune responses against the control antigen keyhole limpet he...

    journal_title:Journal of clinical immunology

    pub_type: 临床试验,杂志文章

    doi:10.1007/s10875-004-6237-2

    authors: Putz T,Ramoner R,Gander H,Rahm A,Bartsch G,Höltl L,Thurnher M

    更新日期:2004-11-01 00:00:00

  • RAG Deficiency: Two Genes, Many Diseases.

    abstract:PURPOSE:To review the clinical and laboratory spectrum of RAG gene defects in humans, and discuss the mechanisms underlying phenotypic heterogeneity, the basis of immune dysregulation, and the current and perspective treatment modalities. METHODS:Literature review and analysis of medical records RESULTS: RAG gene defe...

    journal_title:Journal of clinical immunology

    pub_type: 杂志文章,评审

    doi:10.1007/s10875-018-0537-4

    authors: Delmonte OM,Schuetz C,Notarangelo LD

    更新日期:2018-08-01 00:00:00