کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
3349562 1216357 2016 5 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
CD4+CD25highFoxp3+ Treg deficiency in a Brazilian patient with Gaucher disease and lupus nephritis
موضوعات مرتبط
علوم زیستی و بیوفناوری ایمنی شناسی و میکروب شناسی ایمونولوژی
پیش نمایش صفحه اول مقاله
CD4+CD25highFoxp3+ Treg deficiency in a Brazilian patient with Gaucher disease and lupus nephritis
چکیده انگلیسی

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.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Human Immunology - Volume 77, Issue 2, February 2016, Pages 196–200
نویسندگان
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