Severe Combined Immunodeficiency Disease with Adenosine Deaminase Deficiency
نویسندگان
چکیده
منابع مشابه
Adenosine deaminase deficiency and severe combined immunodeficiency disease.
The nature of the association of adenosine deaminase deficiency and severe combined immunodeficiency disease is reviewed . The basis for the molecular heterogeneity exhibited by adenosine deaminase in human tissue and the mechanisms whereby a deficiency of this activity results in the extreme perturbation of the immune system as observed in severe combined immunodeficiency are critically discus...
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Enzyme replacement therapy was performed for a 1-year and 5-month old boy with adenosine deaminase deficiency disease, the first case in Japan. Irradiated fresh red blood cells were administered without any clinical improvement, but there was an increase in the peripheral lymphocytes from 300/mm3 to 1849/mm3, of which 88% had T cell marker. B lymphocytes did not bear any classes of surface immu...
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mice are discussed by Alfred S. Steinberg who presents data in support of the idea that early loss of a thymic regulatory function in these mice may account for a breakdown in tolerance to "self" and certain other antigens, leading to excessive antibody responses with immune complex formation. The last two chapters, one by Stanley A. Plotkin, the other by Frautisek Sokol, deal, respectively, wi...
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Deficiency of erythrocytic and lymphocytic adenosine deaminase (ADA) occurs in some patients with severe combined immunodeficiency disease (SCID). SCID with ADA deficiency is inherited as an autosomal recessive trait. ADA is markedly reduced or undetectable in affected patients (homozygotes), and approximately one-half normal levels are found in individuals heterozygous for ADA deficiency. The ...
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Adenosine deaminase deficiency is a type of severe combined immunodeficiency that involves about 15% of all SCID cases. This form of SCID has low B cells, T cells, and NK cells. This condition is complex with many possible complications including neurological abnormalities. Guillainbarré syndrome is an acute inflammatory disease of the peripheral nerves that involves an autoimmune attack on mye...
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ژورنال
عنوان ژورنال: The Tohoku Journal of Experimental Medicine
سال: 1979
ISSN: 0040-8727,1349-3329
DOI: 10.1620/tjem.129.197