title: Adjunctive treatment of disseminated Mycobacterium avium complex infection with interferon alpha-2b in a patient with complete interferon-gamma receptor R1 deficiency.
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.
authors.affiliation: Division of Pulmonology, Allergy/Immunology, and Infectious Diseases, Department of Pediatrics, New Jersey Medical School/UMDNJ, 185 South Orange Avenue, Newark, NJ 07101-1709, USA.
authors.forename: Carla M
authors.initials: CM
authors.lastname: Ward
authors.forename: Harumi
authors.initials: H
authors.lastname: Jyonouchi
authors.forename: Sergei V
authors.initials: SV
authors.lastname: Kotenko
authors.forename: Sergey V
authors.initials: SV
authors.lastname: Smirnov
authors.forename: Rakesh
authors.initials: R
authors.lastname: Patel
authors.forename: Helen
authors.initials: H
authors.lastname: Aguila
authors.forename: George
authors.initials: G
authors.lastname: McSherry
authors.forename: Barry
authors.initials: B
authors.lastname: Dashefsky
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
mesh_terms: D002675:Child, Preschool; D024901:Drug Resistance, Multiple, Bacterial / Q000276:immunology; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007223:Infant; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D015269:Mycobacterium avium Complex / Q000276:immunology* / Q000472:pathogenicity; D015270:Mycobacterium avium-intracellulare Infection / Q000188:drug therapy* / Q000276:immunology*; D017471:Receptors, Interferon / Q000172:deficiency*; D011994:Recombinant Proteins; D050794:STAT1 Transcription Factor / Q000276:immunology; D015398:Signal Transduction / Q000276:immunology; D000096743:Interferon gamma Receptor
chemical_list: D007155:Immunologic Factors; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D017471:Receptors, Interferon; D011994:Recombinant Proteins; D050794:STAT1 Transcription Factor; D000096743:Interferon gamma Receptor; C494079:STAT1 protein, human