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1.
Invest. clín ; 51(4): 561-571, dic. 2010. ilus
Article in Spanish | LILACS | ID: lil-630913

ABSTRACT

La adenosin deaminasa (ADA), es una enzima del metabolismo de las purinas que ha sido objeto de mucho interés debido a que el defecto congénito de esta enzima causa el síndrome de inmunodeficiencia combinada severa. Una de las tres isoformas de la enzima (ecto-ADA) es capaz de unirse a la glicoproteína CD26 y a los receptores de adenosina A1 y A2B. La interacción ADA-CD26 produce una señal coestimuladora en los eventos de activación de las células T y en la secreción de IFN-g, TNF-a e IL-6. Durante dicha activación la actividad de la enzima está regulada de manera positiva por IL-2 e IL-12 y negativamente por IL-4, basado en un mecanismo de translocación. Diversos estudios señalan que los niveles séricos y plasmáticos de ADA se elevan en algunas enfermedades causadas por microorganismos que infectan principalmente a los macrófagos; así como en trastornos hipertensivos, lo cual podría representar un mecanismo compensatorio como consecuencia de la elevación de los niveles de adenosina y la liberación de mediadores hormonales e inflamatorios estimulados por la hipoxia.


Adenosine deaminase (ADA) is an enzyme of purine metabolism which has been the subject of much interest because the congenital defect of this enzyme causes severe combined immunodeficiency syndrome. One of the three isoforms of the enzyme (ecto-ADA) is capable of binding to the glycoprotein CD26 and adenosine receptors A1 and A2B. ADA-CD26 interaction produces a costimulatory signal in the events of T cell activation and secretion of IFN-g, TNF-a and IL-6. During this activation, the enzyme activity is regulated positively by IL-2 and IL-12 and negatively by IL-4, based on the mechanism of translocation. Diverse studies suggest that seric and plasmatic levels of ADA rise in some diseases caused by microorganisms infecting mainly the macrophages and in hypertensive disorders, which may represent a compensatory mechanism resulting from increased adenosine levels and the release of hormones and inflammatory mediators estimulated by hipoxia.


Subject(s)
Female , Humans , Pregnancy , Adenosine Deaminase/physiology , Immunity, Cellular , Adenosine Deaminase/blood , Adenosine Deaminase/deficiency , Adenosine Deaminase/genetics , Adenosine Deaminase/immunology , Adenosine/physiology , Agammaglobulinemia/genetics , Agammaglobulinemia/immunology , Cell Hypoxia , Communicable Diseases/enzymology , Communicable Diseases/immunology , Dendritic Cells/enzymology , Dendritic Cells/immunology , /physiology , Enzyme Induction , Hepatitis, Viral, Human/enzymology , Hepatitis, Viral, Human/immunology , Hypertension, Pregnancy-Induced/enzymology , Hypertension, Pregnancy-Induced/physiopathology , Immunological Synapses , Inflammation Mediators/metabolism , Interferon-gamma , Interleukins , Isoenzymes/physiology , Lymphocyte Activation , Receptors, Purinergic P1/physiology , Severe Combined Immunodeficiency/genetics , Severe Combined Immunodeficiency/immunology , T-Lymphocytes/immunology , T-Lymphocytes , Tumor Necrosis Factor-alpha
2.
Ceylon Med J ; 2007 Sep; 52(3): 83-6
Article in English | IMSEAR | ID: sea-49254

ABSTRACT

OBJECTIVES: Primary immune deficiency is relatively rare. Patients present with recurrent or persistent infections or infections with opportunistic pathogens. We investigated patients who presented during the years 2005-7 with recurrent or persistent infections or infections with opportunistic organisms, for underlying immune deficiency. DESIGN: Descriptive study. SETTING: Department of Immunology, Medical Research Institute, Colombo. STUDY POPULATION: 257 patients referred to the Department of Immunology, Medical Research Institute, Colombo, with a history of recurrent infections, for evaluation of possible immune deficiency. MEASUREMENTS: Appropriate evaluation of immunological competence of the humoral and cell mediated immune systems. RESULTS: There were 8 patients with agammaglobulinaemia (X linked agammaglobulinaemia and autosomal recessive agammaglobulinaemia), 2 patients each with ataxia telangiectasia, IgA deficiency and hyper-IgE syndrome, 3 patients with common variable immune deficiency (CVID), and 1 patient each with Griscelli syndrome, hyper-IgM syndrome and X linked severe combined immune deficiency (SCID). CONCLUSIONS: Primary immune deficiency must be included in the evaluation of patients with recurrent infections, and timely intervention can prevent morbidity and mortality.


Subject(s)
Ataxia Telangiectasia , Autoimmune Diseases/immunology , Child , Child, Preschool , Common Variable Immunodeficiency/immunology , Female , Humans , IgA Deficiency , Immunologic Deficiency Syndromes/diagnosis , Infant , Infant, Newborn , Male , Pilot Projects , Recurrence , Severe Combined Immunodeficiency/immunology
4.
Article in English | WPRIM | ID: wpr-138960

ABSTRACT

Disseminated mycobacterial infection after bacillus Calmette-Guerin (BCG)vaccination is a very rare disorder, occurring mostly in patients with immunologic deficiency. We report a case of disseminated BCG infection in a 16-month-old girl with severe combined immunodeficiency. Plain radiographs showed multiple osteolytic lesions in the femora, tibiae, humerus, and phalanges. Abdominal sonography and CT scanning revealed multiple nodules in the spleen, and porto-caval lymphadenopathy.


Subject(s)
BCG Vaccine , Female , Humans , Infant , Mycobacterium bovis , Severe Combined Immunodeficiency/immunology , Tomography, X-Ray Computed , Tuberculosis/immunology
5.
Article in English | WPRIM | ID: wpr-138958

ABSTRACT

Disseminated mycobacterial infection after bacillus Calmette-Guerin (BCG)vaccination is a very rare disorder, occurring mostly in patients with immunologic deficiency. We report a case of disseminated BCG infection in a 16-month-old girl with severe combined immunodeficiency. Plain radiographs showed multiple osteolytic lesions in the femora, tibiae, humerus, and phalanges. Abdominal sonography and CT scanning revealed multiple nodules in the spleen, and porto-caval lymphadenopathy.


Subject(s)
BCG Vaccine , Female , Humans , Infant , Mycobacterium bovis , Severe Combined Immunodeficiency/immunology , Tomography, X-Ray Computed , Tuberculosis/immunology
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