Abstract:
OBJECTIVE:Different sets of warning signs can be used if primary immunodeficiency (PID) is suspected: those of the Jeffrey Modell Foundation (JMF), the German Patients' Organisation for Primary Immunodeficiencies (DSAI) and the Association of the Scientific Medical Societies in Germany (AWMF). A few studies have tested the JMF criteria, with unconvincing results, but the diagnostic models of the DSAI and AWMF have not been tested at all. We set out to establish the utility of these three scoring systems and compare them with our own set of five warning signs (Duesseldorf criteria). DESIGN:Prospective study. PATIENTS:Two hundred ten patients admitted to our hospital between 2010 and 2012 with suspected PID. RESULTS:PID were found in 36 (17 %) of the patients admitted. Of the established sets of warning signs, the JMF and the DSAI had inadequate sensitivity, while the DSAI and the AWMF showed insufficient specificity. Our own criteria were analyzed with regard to maximal specificity and sensitivity (Youden Index) and sensitivity and yielded NPV of 0.89 and 0.91 respectively. Youden index revealed combination of five signs and symptoms: lymphopenia, otitis media >7, failure to thrive, failure to grow normally, pneumonia >1. For maximum negative predictive value the following set was found: lymphopenia, hypogammglobulinemia, failure to thrive, growth disorders, iv antibiotics and abscesses. CONCLUSION:In contrast to the new, evaluated Duesseldorf criteria, all three established sets of warning signs proved inadequate for preselection of patients for admission to specialized PID centers. The Duesseldorf criteria should now being tested in further studies.
journal_name
J Clin Immunoljournal_title
Journal of clinical immunologyauthors
Lankisch P,Schiffner J,Ghosh S,Babor F,Borkhardt A,Laws HJdoi
10.1007/s10875-015-0149-1subject
Has Abstractpub_date
2015-04-01 00:00:00pages
273-9issue
3eissn
0271-9142issn
1573-2592journal_volume
35pub_type
杂志文章abstract::A patient affected with multiple myeloma displayed in the serum, urine, and cerebrospinal fluid a paraprotein with identical electrophoretic mobility. The paraprotein, which was polymeric, appeared in the serum and cerebrospinal fluid mainly as the dimer and tetramer, whereas in the urine the tetramer was predominant....
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00917333
更新日期:1986-07-01 00:00:00
abstract::Cytotoxic lymphocytes are critical in the control of HIV replication, it has been shown that perforin is the key effector of killing machinery for CTLs and NK cells, so we investigated the circulating levels of perforin in CD8+ T cells and NK cells by flow cytometry intracellular stain in Chinese HIV infected individu...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-006-9027-1
更新日期:2006-07-01 00:00:00
abstract::A molecular analysis of complement components (C3, C4, and factor B) in human saliva was performed by SDS-PAGE and immunoblotting. Complement C3 was detected as a molecule composed of a 115-kDa alpha-chain linked to a 70-kDa beta chain by disulfide bonds, and C3 levels ranged from 0.52 to 15.0 micrograms/ml (n = 15). ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1027320425291
更新日期:1997-09-01 00:00:00
abstract::Severe congenital neutropenia (SCN), originally described by the Swedish pediatrician Rolf Kostmann, constitutes a heterogeneous disorder associated with a dramatic decrease of peripheral neutrophil granulocytes. Patients suffer from life-threatening bacterial infections unless treated by recombinant human granulocyte...
journal_title:Journal of clinical immunology
pub_type: 历史文章,杂志文章,评审
doi:10.1007/s10875-016-0358-2
更新日期:2017-02-01 00:00:00
abstract::Oligoclonal free light chains (FLC) banding has been described in multiple sclerosis (MS) and should be correlated with disease activity. However, discrepancies between studies have been reported because of differences in methods. A new quantitative, rapid, and automated method using nephelometry is now available. Our...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-005-5371-9
更新日期:2005-07-01 00:00:00
abstract::The sensitivity of tumor cells to lysis by natural killer (NK) and interleukin-2 (IL-2)-activated killer (LAK) cells was studied in three ovarian carcinoma cell lines (2780.9S, SKOV-3, and CHOAUXB1), four multidrug-resistant (MDR) variants, and a melphalan-resistant line. The antitumor activity of LAK cells was evalua...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF01541671
更新日期:1996-11-01 00:00:00
abstract::Using an enzyme-linked immunoassay we tested the sera of 71 patients with digestive system cancer, 35 patients with various nonmalignant disorders, and 28 normal individuals for anti-CEA activity. Antibodies were found in the sera of 51% of the patients. Most of the patients positive for the antibodies (70%) had no ev...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF01540984
更新日期:1994-09-01 00:00:00
abstract:OBJECTIVES:Gastrointestinal manifestations are frequent in patients with common variable immunodeficiency (CVID), and some of the patients present with celiac-like features. Diagnosing celiac disease (CD) in CVID however is challenging, as autoantibody detection and histopathology of the small intestine cannot reliably...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-013-9892-3
更新日期:2013-07-01 00:00:00
abstract::We examined sera from 160 HIV-infected individuals for antibodies reactive to HIV-1 gp160 epitopes defined by seven synthetic peptides. Seropositive individuals were placed into three groups based upon levels of circulating CD4+ cells. These groups consisted of individuals with (1) more than 400 CD4+ cells, (2) 200-40...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00918790
更新日期:1991-01-01 00:00:00
abstract:PURPOSE:Poikiloderma with neutropenia (PN) is a genodermatosis currently described in 77 patients, all presenting with early-onset poikiloderma, neutropenia, and several additional signs. Biallelic loss-of-function mutations in USB1 gene are detected in all molecularly tested patients but genotype-phenotype correlation...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-018-0508-9
更新日期:2018-05-01 00:00:00
abstract::These studies report findings that demonstrate that human alpha lymphotoxin (LT) induces local, visible, and microscopic inflammatory reactions in normal skin. Skin sites in rabbits, when inoculated with a single injection of native or recombinant human alpha lymphotoxin, demonstrated erythema, swelling, and warmth wi...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00915556
更新日期:1987-07-01 00:00:00
abstract::Although CpG methylation is thought to be a negative regulator of gene transcription, its relationship with cytokine expression remains unclear. Interleukin (IL)-4 and interferon (IFN)-gamma are major cytokines that affect the differentiation of naïve CD4+ T lymphocytes into the Th1 and Th2 lineage. We used bisulfite ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-007-9148-1
更新日期:2008-03-01 00:00:00
abstract:INTRODUCTION:Rheumatic fever (RF) is an autoimmune disease caused by the gram-positive bacteria Streptococcus pyogenes that follows a nontreated throat infection in susceptible children. The disease manifests as polyarthritis, carditis, chorea, erythema marginatum, and/or subcutaneous nodules. Carditis, the most seriou...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-009-9332-6
更新日期:2010-01-01 00:00:00
abstract:PURPOSE:Chronic granulomatous disease (CGD) is an inherited disorder, with phagocytes failing to produce antimicrobial superoxide due to deficient NADPH oxidase activity. Mutations in the gene encoding CYBB are responsible for the majority of the CGD cases. To date, there have been no reports on large samples of childr...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-014-0061-0
更新日期:2014-08-01 00:00:00
abstract::A monoclonal antibody produced by hybridomas obtained from a mouse immunized with drug-resistant cells recognizes certain hamster and human drug-resistant cell lines but not their drug-sensitive parental lines. Preliminary characterization of the antigen indicates that it is a molecule of approximately 180,000 daltons...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00917144
更新日期:1984-09-01 00:00:00
abstract::Adoptive immunotherapy with in vitro expanded cytotoxic T lymphocytes specific for Epstein-Barr virus (EBV) can successfully treat post-transplant lymphoproliferative disease (PTLD). However, extension of a similar strategy to Hodgkin's disease (HD) and nasopharyngeal carcinoma (NPC) is limited by the poor immunogenic...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-006-6532-1
更新日期:2006-01-01 00:00:00
abstract:OBJECTIVE:Dynamic cytokine profiles from endogenously activated T cells in transit from lymph node to the infected sites via the blood compartment after recent exposure to Mycobacterium tuberculosis may differentiate disease progressors from non-disease progressors in a BCG-vaccinated population. METHODS:Household con...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-011-9566-y
更新日期:2011-10-01 00:00:00
abstract:PURPOSE:Autosomal recessive hyper-IgE syndrome is a rare combined immunodeficiency characterized by susceptibility to viral infections, atopic eczema, high serum IgE and defective T cell activation. The genetic etiologies are diverse. Null mutations in DOCK8 and TYK2 are responsible for many cases. This study aims to p...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-012-9769-x
更新日期:2013-01-01 00:00:00
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
更新日期:2019-02-01 00:00:00
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
更新日期:1981-01-01 00:00:00
abstract::Antibodies to certain self-antigens are detected in normal individuals as well as in patients with autoimmune diseases. Natural autoantibodies found in normal human sera are thought to act as an immune regulator, a functional controller of specific proteins, or the first-line defense against pathogenic microorganisms....
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1023/a:1020599526249
更新日期:1999-05-01 00:00:00
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
更新日期:2009-07-01 00:00:00
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
更新日期:2009-01-01 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
abstract::Thymomas can present with a variety of paraneoplastic manifestations, mostly of autoimmune origin, including Good's syndrome when there is associated hypogammaglobulinemia. Although pure red cell aplasia is a recognised complication of thymoma, selective white cell aplasia is very rare, particularly in Good's syndrome...
journal_title:Journal of clinical immunology
pub_type: 杂志文章,评审
doi:10.1007/s10875-014-0014-7
更新日期:2014-04-01 00:00:00
abstract::Early treatment intervention during human immunodeficiency virus (HIV) infection is a strategy aimed to preserve and/or enhance the developing anti-HIV immune responses. We report the effect of highly active antiretroviral therapy (HAART) combined with intermittent subcutaneous doses of Interleukin 2 (IL-2) on CD8(+) ...
journal_title:Journal of clinical immunology
pub_type: 临床试验,杂志文章
doi:10.1023/B:JOCI.0000019778.96564.26
更新日期:2004-03-01 00:00:00
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
更新日期:2015-07-01 00:00:00
abstract:PURPOSE:The interaction of Shiga toxin (Stx) and/or lipopolysaccharide (LPS) with monocytes (Mo) may be central to the pathogenesis of hemolytic uremic syndrome (HUS), providing the cytokines necessary to sensitize endothelial cells to Stx action. We have previously demonstrated phenotypical alterations in Mo from HUS ...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/s10875-011-9646-z
更新日期:2012-06-01 00:00:00
abstract::An absolute requirement for monocytes was demonstrated in the T-cell proliferative response to tetanus toxoid (TT) antigen. Antigen-pulsed monocytes were shown to be effective in triggering T-cell proliferation. Using 125I-radiolabeled TT antigen, uptake by monocytes increased progressively over an 18-hr period, at wh...
journal_title:Journal of clinical immunology
pub_type: 杂志文章
doi:10.1007/BF00915473
更新日期:1981-01-01 00:00:00
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
更新日期:2019-05-01 00:00:00