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| Kokron,Cristina M.; Errante,Paolo R.; Barros,Myrthes T.; Baracho,Gisele V.; Camargo,Maristela M.; Kalil,Jorge; Rizzo,Luiz V.. |
| Common variable immunodeficiency (CVID) is an immunological disorder characterized by defective antibody production, recurrent infections, most notably of the respiratory tract, autoimmune phenomena and cancer. Some CVID patients may also present disturbances of the cellular immune response such as a decrease in the number and proportion of different lymphocyte populations, diminished lymphoproliferative response to mitogens and antigens, altered production of cytokines, and deficient expression of cell-surface molecules. Most Brazilian CVID patients included in this study show a decrease in T and B lymphocyte counts in the peripheral blood. Furthermore, their lymphocytes are more susceptible to apoptosis following activation than normal individuals, and... |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Immunodeficiency; Humans; Antibody; Cytokines; Clinical features. |
| Ano: 2004 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0001-37652004000400007 |
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| Meschede,I.P.; Santos,T.O.; Izidoro-Toledo,T.C.; Gurgel-Gianetti,J.; Espreafico,E.M.. |
| Griscelli syndrome (GS) is a rare autosomal recessive disorder caused by mutation in the MYO5A (GS1, Elejalde), RAB27A (GS2) or MLPH (GS3) genes. Typical features of all three subtypes of this disease include pigmentary dilution of the hair and skin and silvery-gray hair. Whereas the GS3 phenotype is restricted to the pigmentation dysfunction, GS1 patients also show primary neurological impairment and GS2 patients have severe immunological deficiencies that lead to recurrent infections and hemophagocytic syndrome. We report here the diagnosis of GS2 in 3-year-old twin siblings, with silvery-gray hair, immunodeficiency, hepatosplenomegaly and secondary severe neurological symptoms that culminated in multiple organ failure and death. Light microscopy... |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Griscelli syndrome; RAB27A; Hemophagocytic lymphohistiocytosis; Cerebellitis; Immunodeficiency. |
| Ano: 2008 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2008001000002 |
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| Morawetz,R.A.; Giese,N.A.; Gabriele,L.; Rothman,P.; Horak,I.; Ozato,K.; Morse III,H.C.. |
| The contributions of cytokines to the development and progression of disease in a mouse model of retrovirus-induced immunodeficiency (MAIDS) are controversial. Some studies have indicated an etiologic role for type 2 cytokines, while others have emphasized the importance of type 1 cytokines. We have used mice deficient in expression of IL-4, IL-10, IL-4 and IL-10, IFN-<FONT FACE="Symbol">g</font>, or ICSBP - a transcriptional protein involved in IFN signaling - to examine their contributions to this disorder. Our results demonstrate that expression of type 2 cytokines is an epiphenomenon of infection and that IFN-<FONT FACE="Symbol">g</font> is a driving force in disease progression. In addition, exogenously administered IL-12... |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: ICSBP; Immunodeficiency; Interferon; MAIDS. |
| Ano: 1998 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X1998000100008 |
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