FIG 1.

Severe viral infections and inflammatory disease in patients with ZNFX1 deficiency. A, Kaplan-Meier survival curve for patients; dashes indicate ages of patients who are alive. B, Overall inflammatory organ involvement with or without a proven link to infections; number of patients affected. C, May-Gruenwald-Giemsa staining (light microscopy; magnification, ×1000) of a bone marrow aspirate from P5.2. A macrophage with engulfed leukocytes is shown: its nucleus is indicated by an ar, and the engulfed leukocytes are indicated by an arrow. D, Computed tomography image of the brain of P1.2 at the age of 15 years showing calcification of the basal ganglia and white matter abnormalities (white ars). E, A high-resolution computed tomography image of the lungs P1.2 at the age of 9 years and 11 months, showing bilateral diffuse ground glass attenuation, subpleural thickening, and septal thickening. F, Jones staining of a kidney biopsy specimen, highlighting TMA lesions in P5.2. The arrow indicates a small arteriole with endothelial cell swelling and a fibrin/red blood microthrombus obliterating the lumen. Two glomeruli with capillary lumen dilatation and red blood cell stasis are indicated by asterisks. Acute tubular lesions with epithelial cell necrosis, lumen debris, and interstitial hemorrhage are observed (scale bar = 50 μm). ARDS, Acute respiratory distress syndrome; MOF, multiorgan failure; MPGN, membranoproliferative glomerulonephritis.