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Update on Chronic Granulomatous Diseases of ChildhoodImmunotherapy and Potential for Gene Therapy
John I. Gallin, MD;
Harry L. Malech, MD
JAMA. 1990;263(11):1533-1537.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings. |
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SELECTED CASE
A 19-YEAR-OLD black man presented with an autosomal recessive form of chronic granulomatous disease (CGD) of childhood (47-kd cytosol protein deficiency). At age 5 years, he was evaluated for a prolonged febrile illness and was found to have a positive tuberculin skin test. He responded to therapy with isoniazid and ethambutol for presumptive tuberculosis. He did well until age 8 years, when he presented with malaise, fever, and an infiltrate in the upper part of his right lung. Bronchoscopy did not yield a diagnosis. He was treated with broad-spectrum antibacterial therapy without altering his signs and symptoms. An immunologic workup that included a nitroblue tetrazolium dye reduction test indicated a failure of his blood neutrophils to produce superoxide. Subsequent studies confirmed the diagnosis of CGD, as indicated by a failure of his stimulated neutrophils to consume oxygen or produce hydrogen peroxide and by defective in vitro killing of
. . . [Full Text PDF of this Article]
Author Affiliations
From the Bacterial Diseases Section, Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, Bethesda, Md.
Footnotes
Reprint requests to Bacterial Diseases Section, Laboratory of Clinical Investigation, National Institute of Allergy and Infectious Diseases, Bldg 10, Room 11C103, Bethesda, MD 20892 (Dr Gallin).
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