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An immunodeficiency characterized by defective signal transduction in T lymphocytes

Article Abstract:

T lymphocytes are a type of white blood cell responsible for controlling the immune response and fighting disease. The activation of T cells is prompted by the stimulation of specific sites on the cell surface. This case study examines a 9-year-old boy with severe chronic infections, anemia, persistent cough, and Hodgkin's disease (a cancer of the lymph nodes). Although the boy had a normal amount of blood lymphocytes, T cell activation by the infections appeared abnormal. Prior to his birth, his mother had been taking two drugs during pregnancy that suppress the immune system, azathioprine and prednisone. The lack of T cell activation in the patient's immune response was traced to a defect in the cell surface receptors' ability to signal certain transducer proteins in his T lymphocytes.

Author: Chatila, Talal, Young, Michael, Miller, Richard, Wong, Richard, Terhorst, Cox, Geha, Raif S.
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 1989
Analysis, Pediatric diseases, Immunologic deficiency syndromes, Lymphocyte transformation, T cells, Interleukin-2, Immunological deficiency syndromes in children, Drug receptors

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A novel subtype of type 1 diabetes mellitus characterized by a rapid onset and an absence of diabetes-related antibodies

Article Abstract:

There appears to be a variant of type 1, or juvenile onset, diabetes that does not involve autoantibodies or destruction of the pancreas. Type 1 diabetes is normally an autoimmune disease in which the body produces antibodies against pancreatic tissue, where insulin is produced. Researchers describe 11 patients with elevated blood sugar levels and a complication of diabetes called ketoacidosis. However, they did not have elevated blood levels of glycosylated hemoglobin, another marker of diabetes. Biopsies of the pancreas revealed no pancreatic inflammation or destruction of any kind and no autoantibodies were found in blood samples.

Author: Imagawa, Akihisa, Hanafusa, Toshiaki, Miyagawa, Jun-ichiro, Matsuzawa, Yuji
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 2000

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Rapid-onset type 1 diabetes with pancreatic exocrine dysfunction

Article Abstract:

There appears to be a variant of type 1 diabetes that can occur rapidly without any evidence of autoantibodies against the pancreas. Autoantibodies are antibodies produced against the body's own tissues. Type 1 diabetes is believed to be an autoimmune disease caused by the production of autoantibodies against the pancreas. Since the pancreas produces insulin, destruction of the pancreas results in a decrease in insulin production. Researchers published a study of 11 Japanese patients who had severe hypoglycemia and evidence of an immune reaction against the pancreas, but no sign of autoantibodies in their blood.

Author: Lernmark, Ake
Publisher: Massachusetts Medical Society
Publication Name: The New England Journal of Medicine
Subject: Health
ISSN: 0028-4793
Year: 2000
Editorial

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Subjects list: Development and progression, Type 1 diabetes
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