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The 11q Terminal Deletion Disorder Jacobsen Syndrome is a Syndromic Primary Immunodeficiency.

https://arctichealth.org/en/permalink/ahliterature275933
Source
J Clin Immunol. 2015 Nov;35(8):761-8
Publication Type
Article
Date
Nov-2015
Author
Virgil A S H Dalm
Gertjan J A Driessen
Barbara H Barendregt
Petrus M van Hagen
Mirjam van der Burg
Source
J Clin Immunol. 2015 Nov;35(8):761-8
Date
Nov-2015
Language
English
Publication Type
Article
Keywords
Adolescent
Adult
B-Lymphocytes - immunology
Child
Chromosome Deletion
Chromosomes, Human, Pair 11 - genetics
Denmark
Female
Germinal Center - immunology
Humans
Immunologic Deficiency Syndromes - epidemiology - immunology
Infection - epidemiology - immunology
Jacobsen Distal 11q Deletion Syndrome - epidemiology - immunology
Male
Young Adult
Abstract
Jacobsen syndrome (JS) is a rare contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. Clinical features include physical and mental growth retardation, facial dysmorphism, thrombocytopenia, impaired platelet function and pancytopenia. In case reports, recurrent infections and impaired immune cell function compatible with immunodeficiency were described. However, Jacobsen syndrome has not been recognized as an established syndromic primary immunodeficiency.
To evaluate the presence of immunodeficiency in a series of 6 patients with JS.
Medical history of 6 patients with JS was evaluated for recurrent infections. IgG, IgA, IgM and specific antibodies against S. pneumoniae were measured. Response to immunization with a polysaccharide vaccine (Pneumovax) was measured and B and T lymphocyte subset analyses were performed using flowcytometry.
Five out of 6 patients suffered from recurrent infections. These patients had low IgG levels and impaired response to S. pneumoniae polysaccharide vaccination. Moreover, we also found a significant decrease in the absolute number of memory B cells, suggesting a defective germinal center function. In a number of patients, low numbers of T lymphocytes and NK cells were found.
Most patients with JS suffer from combined immunodeficiency in the presence of recurrent infections. Therefore, we consider JS a syndromic primary immunodeficiency. Early detection of immunodeficiency may reduce the frequency and severity of infections. All JS patients should therefore undergo immunological evaluation. Future studies in a larger cohort of patients will more precisely define the pathophysiology of the immunodeficiency in JS.
Notes
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PubMed ID
26566921 View in PubMed
Less detail

Abnormalities within CD4 and CD8 T lymphocytes subsets in type 1 (insulin-dependent) diabetes.

https://arctichealth.org/en/permalink/ahliterature37074
Source
Clin Exp Immunol. 1991 Aug;85(2):278-81
Publication Type
Article
Date
Aug-1991
Author
J. Ilonen
H M Surcel
M L Käär
Author Affiliation
Department of Medical Microbiology, University of Oulu, Finland.
Source
Clin Exp Immunol. 1991 Aug;85(2):278-81
Date
Aug-1991
Language
English
Publication Type
Article
Keywords
Adolescent
Antigens, CD
Antigens, CD4
Antigens, CD45
Antigens, CD8
Antigens, Differentiation, T-Lymphocyte
CD4-Positive T-Lymphocytes - immunology
Child
Diabetes Mellitus, Type 1 - immunology
Female
Histocompatibility Antigens
Humans
Macrophage-1 Antigen
Male
Research Support, Non-U.S. Gov't
T-Lymphocyte Subsets - immunology
Abstract
Abnormalities in the proportions of various T lymphocyte subpopulations have been found in a number of autoimmune diseases. Monoclonal antibodies labelled with various fluorochromes were used here to define the percentages of subsets, and especially to divide CD4+ (helper/inducer) and CD8+ (suppressor/cytotoxic) cells into phenotypic subgroups. Blood samples were analysed from 25 patients (age 10.1 +/- 3.7 years) with recently diagnosed insulin-dependent diabetes mellitus (IDDM) and 25 age- and sex-matched control subjects. The percentages of CD4+ cells and CD4+CD45RA+ cells described as naive T helper cells or suppressor/inducers were increased in the IDDM patients (P less than 0.05 and P less than 0.05. Student's t-test, respectively), whereas the percentage of CD4+CD45RA- cells (memory T-helper cells, helper/inducers) was similar in the patients and controls. The percentage of CD8+CD11b+ cells containing suppressor/effector lymphocytes was decreased in the IDDM patients as compared with the controls (P less than 0.01) but no significant difference was seen in total CD8+ cells. The percentages of CD3+ cells and the proportions of these simultaneously positive for HLA-DR antigen (activated T cells) were also increased in the recent IDDM patients (P less than 0.001 and P less than 0.05, respectively), while the proportion of CD20+ B cells was decreased (P less than 0.05). The findings support the view that disturbed immune regulation occurs in IDDM and indicate that further division of T cell subpopulations may clarify our understanding of the disease process.
PubMed ID
1677834 View in PubMed
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Acquired angioedema--occurrence, clinical features and associated disorders in a Danish nationwide patient cohort.

https://arctichealth.org/en/permalink/ahliterature108201
Source
Int Arch Allergy Immunol. 2013;162(2):149-55
Publication Type
Article
Date
2013
Author
Anette Bygum
Hanne Vestergaard
Author Affiliation
Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.
Source
Int Arch Allergy Immunol. 2013;162(2):149-55
Date
2013
Language
English
Publication Type
Article
Keywords
Adrenergic beta-Antagonists - therapeutic use
Aged
Aged, 80 and over
Angioedema - diagnosis - drug therapy - epidemiology
Antibodies, Monoclonal, Murine-Derived - therapeutic use
Autoantibodies - immunology
B-Lymphocytes - immunology
Bradykinin - analogs & derivatives - therapeutic use
Cohort Studies
Complement C1 Inactivator Proteins - immunology - metabolism
Denmark - epidemiology
Female
Humans
Lymphocytosis - immunology
Male
Middle Aged
Abstract
The prevalence of acquired angioedema (AAE) is hitherto unknown and, to date, less than 200 patients have been reported worldwide. AAE is associated with lymphoproliferative conditions and autoantibodies against C1 inhibitor (C1INH). Rituximab (RTX) is increasingly used in the treatment of AAE patients.
A nationwide study of AAE patients was performed in Denmark. Clinical features, associated disorders, treatments and outcomes were registered.
Eight AAE patients were identified. The diagnostic delay was on average 1 year and 8 months. Patients were treated with C1INH concentrate or icatibant on demand. Six patients were diagnosed with a clonal B-cell disorder during follow-up, on average 2.5 years after the first swelling. Two patients had monoclonal B-cell lymphocytosis (MBL). Two patients received RTX.
AAE is a rare condition occurring in less than 10% of patients with C1INH deficiency in Denmark. AAE is highly associated with haematologic disorders, and we recommend yearly follow-up visits with clinical examination and blood tests including flow cytometry to diagnose B-cell conditions at an early stage. We report 2 patients with AAE and associated MBL, which is a benign expansion of clonal B lymphocytes. MBL can be the precursor of chronic lymphocytic leukaemia or is associated with non-Hodgkin's lymphoma. If angioedema is poorly controlled with standard treatment regimens, we suggest treatment of the associated haematologic disorder. Based on a review of the literature and our own data, we recommend therapy with RTX, especially in patients with anti-C1INH autoantibodies.
PubMed ID
23921495 View in PubMed
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[Acute and chronic alcoholic intoxication as a cause of modified immunological reactivity in patients with generalized peritonitis]

https://arctichealth.org/en/permalink/ahliterature94540
Source
Vestn Ross Akad Med Nauk. 2009;(6):19-23
Publication Type
Article
Date
2009
Author
Loktin E M
Firsov S A
Pliaskin A E
Shpagina L A
Source
Vestn Ross Akad Med Nauk. 2009;(6):19-23
Date
2009
Language
Russian
Publication Type
Article
Keywords
Acute Disease
Alcoholic Intoxication - complications - epidemiology - immunology
Chronic Disease
Follow-Up Studies
Humans
Immunity, Cellular
Incidence
Male
Middle Aged
Peritonitis - epidemiology - etiology - immunology
Retrospective Studies
Severity of Illness Index
Siberia - epidemiology
T-Lymphocytes - immunology
Abstract
The importance of assessment of immunological reactivity in patients with generalized peritonitis suffering alcoholic intoxication ensues from the possibility to use it for the prediction of abdominal inflammation dynamics and thereby for the optimization of organ-protective therapy in critical situations.
PubMed ID
19645100 View in PubMed
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Acute laryngotracheitis in the rat induced by Sendai virus: the influx of six different types of immunocompetent cells into the laryngeal mucosa differs strongly between the subglottic and the glottic compartment.

https://arctichealth.org/en/permalink/ahliterature31970
Source
Laryngoscope. 2001 Sep;111(9):1645-51
Publication Type
Article
Date
Sep-2001
Author
P. Jecker
A. McWilliam
A. Marsh
P G Holt
W J Mann
R. Pabst
J. Westermann
Author Affiliation
Department of Otolaryngology, Mainz Medical School, Mainz, Germany.
Source
Laryngoscope. 2001 Sep;111(9):1645-51
Date
Sep-2001
Language
English
Publication Type
Article
Keywords
Acute Disease
Animals
B-Lymphocytes - immunology
Dendritic Cells - cytology - immunology
Disease Models, Animal
Gene Expression Regulation, Viral - immunology
Genes, MHC Class II - immunology
Glottis - cytology - immunology
Immunity, Cellular - immunology
Immunity, Mucosal - immunology
Immunocompetence - immunology
Immunohistochemistry
Killer Cells, Natural - immunology
Laryngeal Mucosa - cytology - immunology
Laryngitis - immunology - virology
Leukocyte Count
Macrophages - immunology
Neutrophils - immunology
Rats
Respirovirus
Respirovirus Infections - complications
T-Lymphocytes - immunology
Tracheitis - immunology - virology
Abstract
OBJECTIVES: Acute laryngotracheitis is a disease in which mainly the subglottic area is infected, whereas adjacent parts of the larynx, especially the narrow glottic fold, remain unaffected. The reason for the difference between these two directly adjacent regions is unknown. Therefore, in the present study the influx of dendritic cells, neutrophils, T and B lymphocytes, natural killer cells, and macrophages into the mucosa of different laryngeal compartments was investigated after Sendai virus infection in the rat. The aims were to study both the influx of immunocompetent cells and the adhesion of the pathogen and to correlate them to the different reactions of the laryngeal areas during pseudocroup. METHODS: Acute laryngotracheitis was induced by intranasal application of Sendai virus in brown Norway rats. This virus is exclusively pneumotropic in rodents and belongs to the parainfluenza virus type 1, the main pathogen of acute laryngotracheitis in children. The numbers of dendritic cells, neutrophils, T and B lymphocytes, natural killer cells, and macrophages were determined in the supraglottic, glottic, subglottic, and tracheal mucosa on days 2, 5, 7, and 14 after virus application. Furthermore, the nucleoprotein of the virus and major histocompatibility complex (MHC) Class II expression were detected immunohistologically on the laryngeal epithelium. RESULTS: All cell subsets entered the laryngeal mucosa during inflammation. The highest influx was detected among dendritic cells subglottically. This was accompanied by a strong virus adhesion and MHC Class II expression on the subglottic epithelium. In contrast, only a few immunocompetent cells entered the adjacent glottic mucosa, and on the glottic epithelium staining for virus nucleoprotein and MHC Class II expression was weak. CONCLUSIONS: The inflammatory response of the laryngeal mucosa shows great regional differences in this animal model during experimental viral infection. The response was characterized by a strong subglottic and a weak glottic reaction. A possible reason for this difference might be region-specific viral adhesion on the epithelium of the laryngeal areas, as well as differences in MHC Class II expression. Thus, these data agree with the clinical observation during acute laryngotracheitis and may explain why the subglottic part of the larynx is affected preferentially during pseudocroup. The molecular mechanisms mediating the different reactions await clarification.
PubMed ID
11568621 View in PubMed
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[Acute lymphoblastic leukemia in adults. Treatment results and prognosis]

https://arctichealth.org/en/permalink/ahliterature26024
Source
Ugeskr Laeger. 1987 Dec 14;149(51):3460-4
Publication Type
Article
Date
Dec-14-1987

[A deficient T-cell count and its elimination during the treatment and prevention of infections].

https://arctichealth.org/en/permalink/ahliterature214429
Source
Zh Mikrobiol Epidemiol Immunobiol. 1995 Sep-Oct;(5):68-71
Publication Type
Article
Author
A M Zemskov
V M Zemskov
V A Platonova
E D Chertok
A M Tupkalova
A T Vysotskaia
T A Zemskova
A A Degtiarev
S E Furgal
Iu A Evstratov
Source
Zh Mikrobiol Epidemiol Immunobiol. 1995 Sep-Oct;(5):68-71
Language
Russian
Publication Type
Article
Keywords
Acute Disease
Adjuvants, Immunologic - administration & dosage
Adolescent
Adult
Bacterial Infections - drug therapy - immunology - prevention & control
Biological Markers
Carrier State - drug therapy - immunology - prevention & control
Child
Child, Preschool
Chronic Disease
Female
Humans
Infant
Lymphocyte Count
Male
Middle Aged
Military Personnel
Nucleic Acids - administration & dosage
Russia
T-Lymphocytes - immunology
Abstract
The results of the study of the parameters of the immune system in persons suffering from frequent infections, bacteriocarriers and persons with nonspecific infections are presented. The study revealed that T-cell deficiency of the 2nd and 3rd degrees could be regarded as the universal marker of decreased immune reactiveness. Sodium nucleinate was found to be capable of stimulating the T-cell element of immunity and antibody formation, which made it possible to achieve a considerable decrease in morbidity rate. Sodium nucleinate was shown to be highly effective in the prophylaxis of acute respiratory viral infections, carrier state and in the sanitation of purulent foci of infection.
PubMed ID
8525738 View in PubMed
Less detail

Adjuvant-induced arthritis in four inbred strains of rats. An in vitro study of peripheral T and B lymphocytes.

https://arctichealth.org/en/permalink/ahliterature14754
Source
Agents Actions. 1976 Feb;6(1-3):219-27
Publication Type
Article
Date
Feb-1976
Author
A. Kahan
F. Perlik
A. Le Go
F. Delbarre
J P Giroud
Source
Agents Actions. 1976 Feb;6(1-3):219-27
Date
Feb-1976
Language
English
Publication Type
Article
Keywords
Animals
Arthritis, Rheumatoid - immunology
B-Lymphocytes - immunology
Freund's Adjuvant
Lymphocyte Activation - drug effects
Mitogens - pharmacology
Rats
Rats, Inbred Strains
Skin Tests
Species Specificity
T-Lymphocytes - immunology
Abstract
The lymphoblastic response (LTT) to non-specific mitogens (PHA, PWM and ConA) of peripheral lymphocytes was investigated at days 0, 7, 14, 21 and 28 after adjuvant injection in four strains of inbred rats: Wistar (WAG), Long Evans (LE), Lewis (LEW) and Brown Norway (BN). LTT was assessed by using 18 hours H3 TdR incorporation in 5 days cultures of whole blood (micromethod). The statistical treatment of data, using principal components multifactorial analysis and analysis of variance showed a striking difference between strains. In control animals the responses to PHA and PWM were correlated and were higher in LE and WAG than in LEW and BN (BN=LEW less than LE=WAG). The response to ConA was independent of that to the other mitogens. It was generally low, but significantly higher in LEW and BN than in WAG and LE. In adjuvant-injected animals the responses to PHA and PWM were still correlated, but modified compared to control: in LE and LEW, but not in WAG and BN, a marked decrease of the response was found, reaching a minimum value within days 7 and 14. In the same time the response to ConA increased in the four strains, later in LE than in the others. However the intensity of the ConA response varied from one strain to another: it was constantly low in LE and WAG compared to LEW and BN. So the most striking modification of LTT were observed in LE and LEW, which both developed the most severe arthritis. However these different behaviours after adjuvant injection were not explained by the initial level of LTT to the different mitogens. These data suggest that the development of intense arthritis is associated with the proliferation and the release into the blood stream of a lymphocyte subpopulation, which exhibits a low response to PHA and PWM and a high response to ConA. These LTT modifications are not paralleled by quantitative variations of B-cells assessed by surface Ig immunofluorescent staining and EAC rosetting.
PubMed ID
1085095 View in PubMed
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Adoptively transferred late allergic airway responses are associated with Th2-type cytokines in the rat.

https://arctichealth.org/en/permalink/ahliterature57629
Source
Am J Respir Cell Mol Biol. 1997 Jan;16(1):69-74
Publication Type
Article
Date
Jan-1997
Author
A. Watanabe
H. Mishima
T C Kotsimbos
M. Hojo
P M Renzi
J G Martin
Q A Hamid
Author Affiliation
Meakins-Christie Laboratories, McGill University, Montreal, Quebec, Canada.
Source
Am J Respir Cell Mol Biol. 1997 Jan;16(1):69-74
Date
Jan-1997
Language
English
Publication Type
Article
Keywords
Adoptive Transfer
Airway Resistance
Animals
Bronchoalveolar Lavage Fluid - cytology - immunology
CD4-Positive T-Lymphocytes - immunology - transplantation
CD8-Positive T-Lymphocytes - immunology - transplantation
Interferon Type II - biosynthesis
Interleukin-2 - biosynthesis
Interleukin-4 - biosynthesis
Interleukin-5 - biosynthesis
Interleukins - biosynthesis
Ovalbumin - immunology
Rats
Research Support, Non-U.S. Gov't
Respiratory Hypersensitivity - immunology
Th2 Cells - immunology
Abstract
Late allergic airway responses can be transferred by CD4+ T cells in the rat. To investigate the role of T-cell cytokines in these responses, we examined the expression of mRNA for Th2 (interleukin [IL]-4 and IL-5) and Th1 (IL-2 and interferon gamma [INF-gamma])-type cytokines in Brown Norway rats that were administered either antigen-primed W3/25(CD4)+ or OX8(CD8)+ T cells. Donors were actively sensitized by subcutaneous injection of ovalbumin (OVA) in the neck and T cells were obtained from the cervical lymph nodes by immunomagnetic cell sorting for administration to unsensitized rats. Control rats received bovine serum albumin (BSA)-primed CD4+ and CD8+ T cells. Two days later, recipient rats were challenged with aerosolized OVA, and bronchoalveolar lavage (BAL) was performed 8 h after challenge. BAL cells expressing mRNA for IL-2, IL-4, IL-5, and INF-gamma were analyzed using the technique of in situ hybridization. Recipients of OVA-primed CD4+ T cells had an increase in the fraction of BAL cells expressing mRNA for IL-4 and IL-5 compared with BSA-primed CD4+ or OVA-primed CD8+ cells (P
PubMed ID
8998081 View in PubMed
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Adoptive transfer of allergen-specific CD4+ T cells induces airway inflammation and hyperresponsiveness in brown-Norway rats.

https://arctichealth.org/en/permalink/ahliterature57604
Source
Immunology. 1997 Jun;91(2):176-85
Publication Type
Article
Date
Jun-1997
Author
A. Haczku
P. Macary
T J Huang
H. Tsukagoshi
P J Barnes
A B Kay
D M Kemeny
K F Chung
R. Moqbel
Author Affiliation
Department of Allergy and Clinical Immunology, Guy's Hospital, London, UK.
Source
Immunology. 1997 Jun;91(2):176-85
Date
Jun-1997
Language
English
Publication Type
Article
Keywords
Adoptive Transfer
Animals
Bronchial Hyperreactivity - immunology
Bronchoalveolar Lavage Fluid - immunology
CD4-Positive T-Lymphocytes - immunology - transplantation
CD8-Positive T-Lymphocytes - immunology - transplantation
Cell Culture Techniques
Cell Division - immunology
Eosinophils - immunology
Leukocyte Count
Lymphocyte Transfusion
Male
Rats
Rats, Inbred BN
Research Support, Non-U.S. Gov't
Spleen - immunology
Abstract
Following allergen exposure, sensitized Brown-Norway rats develop airway hyperresponsiveness (AHR) and eosinophilic inflammation together with an increase in activated T cells (CD25+) in the airways. We tested the hypothesis that CD4+ T cells are involved directly in the acquisition of AHR. Spleen T cells from animals that were injected intraperitoneally on three consecutive days with ovalbumin/Al(OH)3, showed a dose-dependent proliferative response in vitro to ovalbumin, but not to bovine serum albumin, as measured by [3H]thymidine uptake. For total T-cell transfer, spleen cells obtained from donor rats 4 days after sensitization were depleted of adherent cells by a nylon wool column separation. CD4+ and CD8+ T cells were purified by immunomagnetic beads cell separation. Recipient naive rats were injected intravenously with 50 x 10(6) total T cells, 20 x 10(6) and 5 x 10(6) CD4+ cells, and 5 x 10(6) CD8+ cells, and were exposed to ovalbumin aerosol 24 hr afterwards. After a further 24 hr, airway responsiveness to acetylcholine (ACh) was measured and provocative concentration (PC) values PC100, PC200 and PC300) (the ACh concentration needed to achieve 100, 200 and 300% increase in lung resistance above baseline) were calculated. Airway responsiveness was significantly increased in recipients of sensitized total T cells compared with recipients of cells from saline-injected donor rats (P
PubMed ID
9227314 View in PubMed
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380 records – page 1 of 38.