title: Treatment of disseminated mycobacterial infection with high-dose IFN-γ in a patient with IL-12Rβ1 deficiency.
abstract: IFN-γ has been used in the treatment of IL-12Rβ1 deficiency patients with disseminated BCG infection (BCGosis), but the optimal dose to reach efficacy is not clear. We used IFN-γ in the treatment of a 2.7-year-old patient with IL-12Rβ1 deficiency and refractory BCG-osis. IFNγ was started at a dose of 50 μg/m² 3 times per week. The dose was upgraded to 100 mcg/m² after 3 months, then to 200 mcg/m² 6 months afterwards. Serum mycobactericidal activity and lymphocytes number and function were evaluated throughout the study. There was no clinical response to IFN-γ with 50 or 100 μg/m² doses. However, there was some response to the 200 μg/m² dose with no additional adverse effects. The serum mycobactericidal activity was not significantly different during the whole treatment period. Lymphocytes proliferation in response to PHA was significantly higher after 3 months of using the highest dose as compared to the lowest dose. The tuberculin skin test reaction remained persistently negative. We conclude that in a patient with IL-12Rβ1 deficiency, IFN-γ at a dose of 200 μg/m², but not at lower dosages, was found to have a noticeable clinical effect with no additional adverse effects.
authors.affiliation: Department of Pediatrics, College of Medicine, King Saud University, P.O. Box 2925, Riyadh 11461, Saudi Arabia. aangari@ksu.edu.sa
authors.forename: Abdullah A
authors.initials: AA
authors.lastname: Alangari
authors.forename: Fahad
authors.initials: F
authors.lastname: Al-Zamil
authors.forename: Abdulrahman
authors.initials: A
authors.lastname: Al-Mazrou
authors.forename: Saleh
authors.initials: S
authors.lastname: Al-Muhsen
authors.forename: Stéphanie
authors.initials: S
authors.lastname: Boisson-Dupuis
authors.forename: Sitalbanat
authors.initials: S
authors.lastname: Awadallah
authors.forename: Abdelmageed
authors.initials: A
authors.lastname: Kambal
authors.forename: Jean-Laurent
authors.initials: JL
authors.lastname: Casanova
mesh_terms: D001500:BCG Vaccine / Q000009:adverse effects*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007371:Interferon-gamma / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D008213:Lymphocyte Activation; D009164:Mycobacterium Infections / Q000188:drug therapy* / Q000276:immunology / Q000382:microbiology; D009163:Mycobacterium bovis / Q000187:drug effects* / Q000276:immunology; D053707:Receptors, Interleukin-12 / Q000172:deficiency* / Q000235:genetics; D016896:Treatment Outcome
chemical_list: D001500:BCG Vaccine; C508438:IL12RB1 protein, human; D053707:Receptors, Interleukin-12; D007371:Interferon-gamma