title: Hepatoportal venopathy due to disseminated Mycobacterium avium complex infection in a child with IFN-gamma receptor 2 deficiency.
abstract: The control of intracellular microorganisms such as mycobacteria is largely dependent on the adaptive immune response, specifically the interaction of T helper cells and antigen presenting cells such as macrophages. The interferon gamma (IFN-gamma) pathway activation is crucial for containment and killing of mycobacteria, as evidenced by the fact that defects in this pathway often result in profound infections with both tuberculous and non-tuberculous mycobacteria. We herein report a case of a child with autosomal recessive IFN-gamma receptor 2 (IFN-gammaR2) deficiency who developed hepatic venopathy secondary to disseminated Mycobacterium avium complex (MAC) infection.
authors.affiliation: Laboratory of Pathology, National Cancer Institute, National Institute of Health, Bethesda, MD, USA. Rapkia01@med.nyu.edu
authors.forename: Amy V
authors.initials: AV
authors.lastname: Rapkiewicz
authors.forename: Smita Y
authors.initials: SY
authors.lastname: Patel
authors.forename: Steven M
authors.initials: SM
authors.lastname: Holland
authors.forename: David E
authors.initials: DE
authors.lastname: Kleiner
mesh_terms: D001344:Autopsy; D001706:Biopsy; D002648:Child; D006801:Humans; D007371:Interferon-gamma / Q000494:pharmacology; D008099:Liver / Q000473:pathology*; D008297:Male; D015270:Mycobacterium avium-intracellulare Infection / Q000473:pathology*; D011169:Portal Vein / Q000473:pathology*; D017471:Receptors, Interferon / Q000172:deficiency*
chemical_list: C086185:IFNGR2 protein, human; D017471:Receptors, Interferon; D007371:Interferon-gamma