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11 Cards in this Set
- Front
- Back
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What is lacking in Chronic Granulomatous Disease, and what does it lead to?
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NADPH Oxidase - leads to impotent Phagocytes
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What are people with Chronic Granulomatous Disease susceptible to?
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Organisms with catalase
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How is CDG diagnosed?
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NEGATIVE nitrobule terazolium, it doesn't turn blue-black from the original yellow color
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What are the two treatments for CGD?
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Prophylactic TMP-SMX
INFy |
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What is defective in Chediak-Higashi Disease, and what is the result?
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Defective LYST gene, which is the gene for lysosomal transport
No phagocyte lysosome leads to giant cytoplasmic granules in PMN's |
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What is the presentation triad for Chediak-Higashi?
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Partial albinism
Recurent respiratory tract and skin infections Neurologic disorders |
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What is increased in Job Syndrome, and what is deficient?
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Increased IgE
Deficient INFy |
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What does deficient INFy cause?
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PMN's fail to respond to chemotactic stimuli
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What is the presentation triad for Job Syndrome?
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Exzema
Recurrent colds: s. aureus abscesses Course facial features: broad nose, prominent forehead, deep set eyes, doughy skin, 2 rows of teeth |
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What is abnormal in Leukocyte Adhesion Deficiency Syndrome, and what does this cause?
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Integrins - inability of phagocytes to exit circulation
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What is a typical indicator of infants with Leukocyte Adhesion Deficiency Syndrome?
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Delayed separation of the umbilicus
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