CD4+CD25highFoxp3+ Treg deficiency in a Brazilian patient with Gaucher disease and lupus nephritis
Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We...
Ausführliche Beschreibung
Autor*in: |
Matta, Marina Cadena [verfasserIn] |
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E-Artikel |
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Sprache: |
Englisch |
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2016transfer abstract |
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Umfang: |
5 |
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Übergeordnetes Werk: |
Enthalten in: Towards a Dynamic Understanding of Cadherin-Based Mechanobiology - Hoffman, Brenton D. ELSEVIER, 2015, official journal of the American Society for Histocompatibility and Immunogenetics (ASHI), Amsterdam [u.a.] |
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Übergeordnetes Werk: |
volume:77 ; year:2016 ; number:2 ; pages:196-200 ; extent:5 |
Links: |
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DOI / URN: |
10.1016/j.humimm.2015.11.018 |
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ELV024372536 |
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520 | |a Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. | ||
520 | |a Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. | ||
650 | 7 | |a Invariant natural killer T cells |2 Elsevier | |
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700 | 1 | |a Kerstenetzky, Marcelo Soares |4 oth | |
700 | 1 | |a Freitas, Augustus Cesar Pinto |4 oth | |
700 | 1 | |a Kim, Chong Ae |4 oth | |
700 | 1 | |a Torres, Leuridan Cavalcante |4 oth | |
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10.1016/j.humimm.2015.11.018 doi GBVA2016008000029.pica (DE-627)ELV024372536 (ELSEVIER)S0198-8859(15)00579-0 DE-627 ger DE-627 rakwb eng 610 610 DE-600 570 VZ BIODIV DE-30 fid Matta, Marina Cadena verfasserin aut CD4+CD25highFoxp3+ Treg deficiency in a Brazilian patient with Gaucher disease and lupus nephritis 2016transfer abstract 5 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. Invariant natural killer T cells Elsevier Gaucher disease Elsevier Lupus nephritis Elsevier Regulatory T cells Elsevier Soares, Diogo Cordeiro oth Kerstenetzky, Marcelo Soares oth Freitas, Augustus Cesar Pinto oth Kim, Chong Ae oth Torres, Leuridan Cavalcante oth Enthalten in Elsevier Science Hoffman, Brenton D. ELSEVIER Towards a Dynamic Understanding of Cadherin-Based Mechanobiology 2015 official journal of the American Society for Histocompatibility and Immunogenetics (ASHI) Amsterdam [u.a.] (DE-627)ELV000456578 volume:77 year:2016 number:2 pages:196-200 extent:5 https://doi.org/10.1016/j.humimm.2015.11.018 Volltext GBV_USEFLAG_U GBV_ELV SYSFLAG_U FID-BIODIV SSG-OLC-PHA AR 77 2016 2 196-200 5 045F 610 |
spelling |
10.1016/j.humimm.2015.11.018 doi GBVA2016008000029.pica (DE-627)ELV024372536 (ELSEVIER)S0198-8859(15)00579-0 DE-627 ger DE-627 rakwb eng 610 610 DE-600 570 VZ BIODIV DE-30 fid Matta, Marina Cadena verfasserin aut CD4+CD25highFoxp3+ Treg deficiency in a Brazilian patient with Gaucher disease and lupus nephritis 2016transfer abstract 5 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. Invariant natural killer T cells Elsevier Gaucher disease Elsevier Lupus nephritis Elsevier Regulatory T cells Elsevier Soares, Diogo Cordeiro oth Kerstenetzky, Marcelo Soares oth Freitas, Augustus Cesar Pinto oth Kim, Chong Ae oth Torres, Leuridan Cavalcante oth Enthalten in Elsevier Science Hoffman, Brenton D. ELSEVIER Towards a Dynamic Understanding of Cadherin-Based Mechanobiology 2015 official journal of the American Society for Histocompatibility and Immunogenetics (ASHI) Amsterdam [u.a.] (DE-627)ELV000456578 volume:77 year:2016 number:2 pages:196-200 extent:5 https://doi.org/10.1016/j.humimm.2015.11.018 Volltext GBV_USEFLAG_U GBV_ELV SYSFLAG_U FID-BIODIV SSG-OLC-PHA AR 77 2016 2 196-200 5 045F 610 |
allfields_unstemmed |
10.1016/j.humimm.2015.11.018 doi GBVA2016008000029.pica (DE-627)ELV024372536 (ELSEVIER)S0198-8859(15)00579-0 DE-627 ger DE-627 rakwb eng 610 610 DE-600 570 VZ BIODIV DE-30 fid Matta, Marina Cadena verfasserin aut CD4+CD25highFoxp3+ Treg deficiency in a Brazilian patient with Gaucher disease and lupus nephritis 2016transfer abstract 5 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. Invariant natural killer T cells Elsevier Gaucher disease Elsevier Lupus nephritis Elsevier Regulatory T cells Elsevier Soares, Diogo Cordeiro oth Kerstenetzky, Marcelo Soares oth Freitas, Augustus Cesar Pinto oth Kim, Chong Ae oth Torres, Leuridan Cavalcante oth Enthalten in Elsevier Science Hoffman, Brenton D. ELSEVIER Towards a Dynamic Understanding of Cadherin-Based Mechanobiology 2015 official journal of the American Society for Histocompatibility and Immunogenetics (ASHI) Amsterdam [u.a.] (DE-627)ELV000456578 volume:77 year:2016 number:2 pages:196-200 extent:5 https://doi.org/10.1016/j.humimm.2015.11.018 Volltext GBV_USEFLAG_U GBV_ELV SYSFLAG_U FID-BIODIV SSG-OLC-PHA AR 77 2016 2 196-200 5 045F 610 |
allfieldsGer |
10.1016/j.humimm.2015.11.018 doi GBVA2016008000029.pica (DE-627)ELV024372536 (ELSEVIER)S0198-8859(15)00579-0 DE-627 ger DE-627 rakwb eng 610 610 DE-600 570 VZ BIODIV DE-30 fid Matta, Marina Cadena verfasserin aut CD4+CD25highFoxp3+ Treg deficiency in a Brazilian patient with Gaucher disease and lupus nephritis 2016transfer abstract 5 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. Invariant natural killer T cells Elsevier Gaucher disease Elsevier Lupus nephritis Elsevier Regulatory T cells Elsevier Soares, Diogo Cordeiro oth Kerstenetzky, Marcelo Soares oth Freitas, Augustus Cesar Pinto oth Kim, Chong Ae oth Torres, Leuridan Cavalcante oth Enthalten in Elsevier Science Hoffman, Brenton D. ELSEVIER Towards a Dynamic Understanding of Cadherin-Based Mechanobiology 2015 official journal of the American Society for Histocompatibility and Immunogenetics (ASHI) Amsterdam [u.a.] (DE-627)ELV000456578 volume:77 year:2016 number:2 pages:196-200 extent:5 https://doi.org/10.1016/j.humimm.2015.11.018 Volltext GBV_USEFLAG_U GBV_ELV SYSFLAG_U FID-BIODIV SSG-OLC-PHA AR 77 2016 2 196-200 5 045F 610 |
allfieldsSound |
10.1016/j.humimm.2015.11.018 doi GBVA2016008000029.pica (DE-627)ELV024372536 (ELSEVIER)S0198-8859(15)00579-0 DE-627 ger DE-627 rakwb eng 610 610 DE-600 570 VZ BIODIV DE-30 fid Matta, Marina Cadena verfasserin aut CD4+CD25highFoxp3+ Treg deficiency in a Brazilian patient with Gaucher disease and lupus nephritis 2016transfer abstract 5 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. Invariant natural killer T cells Elsevier Gaucher disease Elsevier Lupus nephritis Elsevier Regulatory T cells Elsevier Soares, Diogo Cordeiro oth Kerstenetzky, Marcelo Soares oth Freitas, Augustus Cesar Pinto oth Kim, Chong Ae oth Torres, Leuridan Cavalcante oth Enthalten in Elsevier Science Hoffman, Brenton D. ELSEVIER Towards a Dynamic Understanding of Cadherin-Based Mechanobiology 2015 official journal of the American Society for Histocompatibility and Immunogenetics (ASHI) Amsterdam [u.a.] (DE-627)ELV000456578 volume:77 year:2016 number:2 pages:196-200 extent:5 https://doi.org/10.1016/j.humimm.2015.11.018 Volltext GBV_USEFLAG_U GBV_ELV SYSFLAG_U FID-BIODIV SSG-OLC-PHA AR 77 2016 2 196-200 5 045F 610 |
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Enthalten in Towards a Dynamic Understanding of Cadherin-Based Mechanobiology Amsterdam [u.a.] volume:77 year:2016 number:2 pages:196-200 extent:5 |
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Enthalten in Towards a Dynamic Understanding of Cadherin-Based Mechanobiology Amsterdam [u.a.] volume:77 year:2016 number:2 pages:196-200 extent:5 |
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CD4+CD25highFoxp3+ Treg deficiency in a Brazilian patient with Gaucher disease and lupus nephritis |
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Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. |
abstractGer |
Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. |
abstract_unstemmed |
Gaucher Disease (GD) is a rare autosomal recessive disorder caused by the deficient activity of beta-glucocerebrosidase. GD is one of the lysosomal storage diseases with the most remarkable alterations in the immune system, and that may manifest clinically as autoimmune disorders and malignancy. We reported the immunological evaluation of a patient with GD and lupus nephritis. Decreased absolute values of T, and NK, and an inversion of CD4+/CD8+ ratio, low levels of IgM and normal B cells were found when compared to reference values in a Brazilian population. Absence ofCD4+CD25highFoxp3+ Treg and high levels of total NKT, iNKT cells and CD8+ iNKT subsets were also observed when compared to the healthy control and GD patient without lupus nephritis. Treg subset and CD8+ iNKT abnormalities might be involved in severe lupus nephritis in a GD patient. We conclude by emphasizing the importance of the immunological evaluation on early diagnosis of autoimmunity contributing to reduce mortality and morbidity of these patients. |
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