Abstract
We report adjunct treatment of (interferon) IFN-alpha2b (Intron-A) in a patient with complete interferon-gamma receptor R1 (IFNGR1) deficiency suffering from disseminated infection with Mycobacterium avium complex (MAC) resistant to multiple anti-mycobacterial agents. A low dose of IFN-alpha2b (3 x 10(6) units/m(2) three times weekly subcutaneously) successfully attenuated progressive hepatosplenomegaly and abdominal/retroperitoneal/pelvic lymphadenopathy, although the patient continued to be mycobacteremic. This is the first report of a complete IFNGR1 deficiency treated with adjuvant IFN-alpha2b for disseminated MAC infection.
MeSH terms
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Child, Preschool
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Drug Resistance, Multiple, Bacterial / immunology
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Female
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Humans
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Immunologic Factors / therapeutic use*
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Infant
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Interferon alpha-2
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Interferon gamma Receptor
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Interferon-alpha / therapeutic use*
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Mycobacterium avium Complex / immunology*
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Mycobacterium avium Complex / pathogenicity
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Mycobacterium avium-intracellulare Infection / drug therapy*
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Mycobacterium avium-intracellulare Infection / immunology*
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Receptors, Interferon / deficiency*
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Recombinant Proteins
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STAT1 Transcription Factor / immunology
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Signal Transduction / immunology
Substances
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Immunologic Factors
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Interferon alpha-2
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Interferon-alpha
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Receptors, Interferon
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Recombinant Proteins
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STAT1 Transcription Factor
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STAT1 protein, human