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Case 12345Gene of mutationIL10RAIL10RAIL10RAIL10RAIL10RAAge when tested4 y2 y4 y1 m2 yCD4+/CD8+ ratio2.4 (0.9-2.18),↑0.67 (1.33-2.77),↓NA3.53 (1.31-3.16),↑1.88 (1.33-2.77),-CD16+/CD56+?(% lymphocytes)47.43 (6.5-21.1),↑14.12 (4.4-15.6),-NA6.71 (4.0-18.2),-20.76 (4.4-15.6),↑CD16+/CD56+??(absolute counts?)×109/L0.69 (0.1-1.0),-1.63 (0.1-1.0),↑NA0.18 (0.2-1.4),↓1.36 (0.1-1.0),↑CD19+(absolute counts) ×109/L0.14 (0.2-2.1),↓1.88 (0.2-2.1),-NA0.22 (0.6-1.9),↓0.75 (0.2-2.1),-CD19+?(% lymphocytes)9.75 (14-44),↓16.3 (17-41),↓NA8.08 (4-26),-11.45 (14-44),↓CD3+(absolute counts)×109/L0.6 (0.9-4.5),↓7.9 (1.4-8.0),-NA2.32 (2.3-7.0),-4.36 (0.9-4.5),-CD3+?(% lymphocytes)40.96 (43-76),↓68.29 (29-73),-NA8.08 (60-85),↓66.3 (43-76),↑CD4+(absolute counts)×109/L0.40 (0.5-2.4),↓3.27 (0.5-2.4),↑NA1.80 (1.7-5.3),-2.83 (0.5-2.4),↑CD4+?(% lymphocytes)27.44 (27.4-44.0),-28.26 (31.7-45.7),↓NA65.21 (32.3-47.3),↑43.06 (31.7-45.7),-CD8+(absolute counts10) ×9/L0.17 (0.3-1.6),↓4.88 (0.3-1.6),↑NA0.51 (0.4-1.7),-1.50 (0.3-1.6),-CD8+?(% lymphocytes)11.41 (18.6-33.4),↓42.2 (15.5-26.6),↑NA18.47 (14.4-26.9),-22.86 (15.5-26.6),-IgA (g/L)3.3 (0.31-2.16),↑3.63 (0.24-1.56),↑NA0.36 (0.23-0.45),-2.97 (0.24-1.56),↑IgG (g/L)7.47 (5.51-12.4),-16.05 (3.8-9.08),↑NA11.62 (2.26-5.4),↑15.49 (4.86-11.4),↑IgM (g/L)1.68 (0.56-2.13),-2.1 (0.56-2.13),-NA1.95 (0.21-1.2),↑6.42 (0.56-2.13),↑AutoantiboidesNANANANANACase678910Gene of mutationIL10RAIL10RAIL10RAIL10RAIL10RAAge when tested1 y1 m2 y1 y9 mCD4+/CD8+ ratio0.69 (1.31-3.16),↓0.44 (1.06-2.73),↓0.35 (1.06-2.54),↓0.32 (1.31-3.16),↓1.18 (1.06-2.73),-CD16+/CD56+?(% lymphocytes)22.01 (4.4-15.6),↑15.14 (3.5-17.0),-23.49 (4.1-17.3),↑24.1 (4.4-15.6),↑20.71 (3.5-17.0),↑CD16+/CD56+??(absolute counts?)×109/L1.09 (0.1-1.4),-2.09 (0.2-1.4),↑0.99 (0.1-1.0),↓0.17 (0.1-1.4),-0.83 (0.2-1.2),-CD19+(absolute counts) ×109/L0.23 (0.6-3.1),↓1.20 (0.6-1.9),-0.08 (0.2-2.1),↓0.00 (0.6-3.1),↓0.34 (0.6-2.7),↓CD19+?(% lymphocytes)4.15 (17-41),↓8.74 (4-26),-1.90 (14-44),↓0.45 (17-41),↓8.60 (15-39),↓CD3+(absolute counts)×109/L4.06 (1.4-8.0),-10.3 (2.3-7.0),↑3.13 (0.9-4.5),-0.53 (1.4-8.0),↓2.79 (1.6-6.7),-CD3+?(% lymphocytes)74.71 (39-73),↑74.73 (60-85),-74.09 (43-76),-73.81 (39-73),↑69.61 (54-76),-CD4+?(absolute counts?)×10 9/L1.61 (0.9-5.5),-2.13 (1.7-5.3),-0.74 (0.5-2.4),-0.13 (0.9-5.5),↓1.49 (1.0-4.6),-CD4+?(% lymphocytes)29.64 (31.7-45.7),↓22.69 (29.4-49.1),↓17.51 (28.3-46.5),↓17.98 (31.7-45.7),↓37.22 (29.4-49.1),-CD8+?(absolute counts?)×10 9/L2.33 (0.4-2.3),↑7.14 (0.4-1.7),↑2.10 (0.3-1.6),↑0.40 (0.4-2.3),-1.26 (0.4-2.1),-CD8+?(% lymphocytes)42.99 (15.5-26.6),↑51.81 (15.6-30.0),↑49.77 (16.0-29.5),↑56.51 (15.5-26.6),↑31.49 (15.6-30.0),↑IgA (g/L)0.87 (0.24-1.56),-1.73 (0.23-0.45),↑4.18 (0.26-1.93),↑0.12 (0.24-1.56),↓1.65 (0.23-0.92),-IgG (g/L)10.79 (3.8-9.08),↑7.47 (2.26-5.4),↑20.47 (4.86-11.4),↑8.02 (3.8-9.08),-12.45 (3.38-8.07),↑IgM (g/L)1.92 (0.44-2.17),-2.02 (0.21-1.2),↑3.98 (0.56-2.13),↑0.39 (0.44-2.17),↓2.01 (0.38-2.16),-AutoantiboidesNANANANANACase11121314Gene of mutationIL10RAIL10RAIL10RAIL10RAAge when tested1 y2 m1 m1 yCD4+/CD8+ ratio0.87 (1.06-2.73),↓4.46 (1.31-3.16),↑1.24 (1.31-3.16),↑0.45 (1.33-2.77),↓CD16+/CD56+?(% lymphocytes)21.58 (4.1-17.3),↑1.48 (4.0-18.2),↓9.12 (4.0-18.2),-10.4 (4.4-15.6),-CD16+/CD56+?(absolute counts?)×109/L1.37 (0.1-1.4),-0.07 (0.1-1.3),↓1 (0.2-1.4),-0.36 (0.1-1.4),-CD19+(absolute counts) ×109/L0.96 (0.6-2.7),-1.44 (0.6-3.0),-0.82 (0.6-1.9),-0.10 (0.6-3.1),↓CD19+?(% lymphocytes)15.07 (17-41),↓31.99 (14-39),-7.5 (4-26),-2.73 (17-41),↓CD3+(absolute counts)×109/L3.86 (1.4-8.0),-2.98 (2.3-6.5),-9.02 (2.3-7.0),↑2.97 (1.4-8.0),-CD3+?(% lymphocytes)62.39 (39-73),-66.14 (48-75),-82.14 (60-85),-84.71 (39-73),↑CD4+?(absolute counts?)×10 9/L1.87 (0.9-5.5),-2.56 (1.5-5.0),-5.10 (1.7-5.3),-0.92 (0.9-5.5),-CD4+?(% lymphocytes)29.36 (28.3-46.5),-56.9 (32.3-47.3),↑46.39 (32.3-47.3),-26.15 (31.7-45.7),↓CD8+?(absolute counts?)×10 9/L2.15 (0.4-2.3),-0.57 (0.5-1.6),-4.10 (0.4-1.7),↑2.03 (0.4-2.3),-CD8+?(% lymphocytes)33.78 (16.0-29.5),↑12.75 (14.4-26.9),↓37.29 (14.4-26.9),↑57.88 (15.5-26.6),↑IgA (g/L)1.43 (0.24-1.56),-0.85 (0.23-0.45),↑0.57 (0.23-0.45),↑2.48 (0.24-1.56),↑IgG (g/L)6.92 (3.8-9.08),-8.58 (2.26-5.4),↑9.68 (2.26-5.4),↑10.79 (3.8-9.08),↑IgM (g/L)1.44 (0.44-2.17),-1.97 (0.21-1.2),↑2.15 (0.21-1.2),↑3.38 (0.44-2.17),↑AutoantiboidesNAanti-dsDNA titer 1:100; MPO-ANCA 29.3 RU/ml; PR3-ANCA 30.1 RU/ml; ANA titer 1:100, staining pattern nuclear/cytoplasmic-NACase15161718Gene of mutationIL10RAIL10RAIL10RAIL10RAAge when tested6 m7 m6 m1 mCD4+/CD8+ ratio0.71 (1.06-2.73),↓2.63 (1.31-3.16),-0.95 (1.06-2.73),↓4.13 (1.06-2.73),↑CD16+/CD56+?(% lymphocytes)15.69 (3.5-17.0),-6.15 (4.0-18.2),-13.26 (3.5-17.0),-4.99 (3.5-17.0),-CD16+/CD56+??(absolute counts?)×109/L0.52 (0.1-1.0),-0.09 (0.1-1.0),↓0.85 (0.1-1.0),-0.26 (0.2-1.4),-CD19+(absolute counts) ×109/L0.50 (0.7-2.5),↓0.60 (0.7-2.5),↓0.49 (0.7-2.5),↓0.76 (0.6-1.9),-CD19+?(% lymphocytes)14.85 (13-35),-41.13 (13-35),↑7.67 (13-35),↓14.43 (4-26),-CD3+(absolute counts)×109/L2.28 (2.4-6.9),↓0.75 (2.4-6.9),↓5.02 (2.4-6.9),-4.23 (2.3-7.0),-CD3+?(% lymphocytes)67.19 (50-77),-51.41 (50-77),-78.4 (50-77),↑79.75 (60-85),-CD4+(absolute counts?)×10 9/L0.89 (1.4-5.1),↓0.54 (1.4-5.1),↓2.36 (1.4-5.1),-33.39 (1.7-5.3),↑CD4+?(% lymphocytes)26.34 (29.4-49.1),↓37.25 (32.3-47.3),-36.81 (29.4-49.1),-63.97 (29.4-49.1),↑CD8+?(absolute counts?)×10 9/L1.26 (0.6-2.2),-0.21 (0.6-2.2),↓2.48 (0.6-2.2),↑0.82 (0.4-1.7),-CD8+?(% lymphocytes)27.13 (15.6-30.0),-14.15 (14.4-26.9),↓38.65 (15.6-30.0),↑18.48 (15.6-30.0),↓IgA (g/L)1.98 (0.23-0.79),↑0.65 (0.23-0.79),-0.89 (0.23-0.79),↑0.68 (0.23-0.45),↑IgG (g/L)12.17 (2.72-6.62),↑6.09 (2.87-7.74),-12.45 (2.72-6.62),↑13 (2.26-5.4),↑IgM (g/L)2.75 (0.3-1.62),↑0.47 (0.3-1.62),-0.59 (0.3-1.62),-1.18 (0.16-0.7),↑Autoantiboides-NA-anti-GBM 41 RU/mlCase19202122Gene of mutationIL10RAIL10RAEPCAMTNFAIP3Age when tested3 y1 m1 y3 yCD4+/CD8+ ratio0.46 (0.90-2.18),↓5.08 (1.06-2.73),↑7.41 (1.06-2.54),↑1.8 (1.03-2.09),-CD16+/CD56+?(% lymphocytes)16.84 (6.5-21.1),-11.32 (3.5-17.0),-11.08 (4.1-17.3),-20.3 (6.9-19.3),↑CD16+/CD56+??(absolute counts?)×109/L0.26 (0.1-1.0),-0.16 (0.2-1.4),↓0.25 (0.1-1.4),-0.45 (0.1-1.0),-CD19+(absolute counts) ×109/L0.23 (0.2-2.1),-0.27 (0.6-1.9),↓0.66 (0.6-3.1),-0.51 (0.2-2.1),-CD19+?(% lymphocytes)14.81 (14-44),-18.87 (4-26),-28.93 (17-41),-22.80 (14-44),-CD3+(absolute counts)×109/L1.02 (0.9-4.5),-0.97 (2.3-7.0),↓1.35 (1.4-8.0),↓1.21 (0.9-4.5),-CD3+?(% lymphocytes)66.99 (43-76),-68.69 (60-85),-58.86 (39-73),-54.36 (43-76),-CD4+?(absolute counts?)×10 9/L0.31 (0.5-2.4),↓0.80 (1.7-5.3),↓1.15 (0.9-5.5),-0.77 (0.3-2.0),-CD4+?(% lymphocytes)20.5 (27.4-44.0),↓56.5 (29.4-49.1),↑50.31 (28.3-46.5),↑34.4 (27.8-44.1),-CD8+?(absolute counts?)×10 9/L0.68 (0.3-1.6),-0.16 (0.4-1.7),↓0.16 (0.4-2.3),↓0.42 (0.3-1.6),-CD8+?(% lymphocytes)44.66 (18.6-33.4),↑11.12 (15.6-30.0),↓6.79 (16.0-29.5),↓19.08 (18.2-30.6),-IgA (g/L)3.85 (0.26-1.93),↑0.67 (0.23-0.45),↑1.87 (0.24-1.03),↑4.18 (0.26-1.93),↑IgG (g/L)14.39 (4.97-11.7),↑12.17 (2.26-5.4),↑11.34 (3.8-9.08),↑15.77 (4.97-11.7),↑IgM (g/L)1.32 (0.21-1.2),↑0.59 (0.21-1.2),-2.34 (0.44-2.17),↑2.72 (0.56-2.13),↑AutoantiboidesMPO-ANCA 31.9 RU/mlanti-GBM 91.4 RU/ml; MPO-ANCA 28.2 RU/ml--Case23242526Gene of mutationLRBANegativeNegativeNegativeAge when tested2 y1 m3 m2 mCD4+/CD8+ ratio1.19 (1.33-2.77),-0.9 (1.31-3.16),↓1.8 (1.06-2.73),-2.16 (1.06-2.73),-CD16+/CD56+?(% lymphocytes)10.34 (4.4-15.6),-13.89 (4.0-18.2),-7.13 (3.5-17.0),-22.21 (3.5-17.0),↑CD16+/CD56+??(absolute counts?)×109/L0.3 (0.1-1.0),-1.1 (0.2-1.4),-0.38 (0.1-1.3),-0.52 (0.1-1.3),-CD19+(absolute counts) ×109/L0.54 (0.2-2.1),-0.63 (0.6-1.9),-0.97 (0.6-3.0),-0.84 (0.6-3.0),-CD19+?(% lymphocytes)18.63 (14-44),-7.97 (4-26),-18.32 (14-39),-35.72 (14-39),-CD3+(absolute counts)×109/L2 (0.9-4.5),-6.11 (2.3-7.0),-3.87 (2.3-6.5),-0.9 (2.3-6.5),↓CD3+?(% lymphocytes)69.66 (43-76),-76.98 (60-85),-73.32 (48-75),-38.35 (48-75), ↓CD4?+(absolute counts?)×10 9/L1.07 (0.5-2.4),-2.82 (1.7-5.3),-2.41 (1.5-5.0),-0.61 (1.5-5.0),↓CD4+?(% lymphocytes)37.37 (31.7-45.7),-35.56 (32.3-47.3),-45.71 (29.4-49.1),-26.01 (29.4-49.1),↓CD8+?(absolute counts?)×10 9/L0.90 (0.3-1.6),-3.13 (0.4-1.7),↑1.34 (0.5-1.6),-0.28 (0.5-1.6),↓CD8+?(% lymphocytes)31.4 (15.5-26.6),↑39.4 (14.4-26.9),↑25.34 (15.6-30.0),-12.07 (15.6-30.0),↓IgA (g/L)0.13 (0.24-1.56),↓0.13 (0.23-0.45),↓0.34 (0.26-1.93),-0.81 (0.23-0.45),↑IgG (g/L)8.02 (4.86-11.4),-11.07 (2.26-5.4),↑5.81 (2.26-5.4),↑6.36 (2.26-5.4),↑IgM (g/L)0.79 (0.56-2.13),-0.98 (0.21-1.2),-1.89 (0.21-1.2),↑0.93 (0.21-1.2),-Autoantiboides--NA-Case27282930Gene of mutationNegativeNegativeNegativeNegativeAge when tested1 m2 m1 m1 mCD4+/CD8+ ratio0.68 (1.31-3.16),↓1.86 (1.06-2.73),-2.9 (1.06-2.73),↑3.83 (1.06-2.73),↑CD16+/CD56+?(% lymphocytes)25.12 (4.0-18.2),↑26.67 (3.5-17.0),↑9.06 (3.5-17.0),-3.55 (3.5-17.0),↓CD16+/CD56+??(absolute counts?)×109/L1.42 (0.2-1.4),↑1.02 (0.1-1.3),-0.28 (0.2-1.4),-0.16 (0.2-1.4),↓CD19+(absolute counts) ×109/L0.14 (0.6-1.9),↓0.57 (0.6-3.0),↓0.53 (0.6-1.9),↓0.75 (0.6-1.9),-CD19+?(% lymphocytes)2.55 (4-26),↓14.79 (14-39),-17.13 (4-26),-16.18 (4-26),-CD3+(absolute counts)×109/L4 (2.3-7.0),-2.17 (2.3-6.5),↓2.26 (2.3-7.0),↓3.62 (2.3-7.0),-CD3+?(% lymphocytes)70.58 (60-85),-56.7 (48-75),-72.36 (60-85),-78.59 (60-85),-CD4+?(absolute counts?)×10 9/L1.57 (1.7-5.3),↓1.38 (1.5-5.0),↓1.68 (1.7-5.3),↓2.83 (1.7-5.3),-CD4+?(% lymphocytes)27.76 (32.3-47.3),↓36.05 (29.4-49.1),-53.67 (29.4-49.1),↑61.4 (29.4-49.1),↑CD8+?(absolute counts?)×10 9/L2.33 (0.4-1.7),↑0.74 (0.5-1.6),-0.58 (0.4-1.7),-0.74 (0.4-1.7),-CD8+?(% lymphocytes)41.12 (14.4-26.9),↑19.35 (15.6-30.0),-18.48 (15.6-30.0),-16.02 (15.6-30.0),-IgA (g/L)0.77 (0.23-0.45),↑0.3 (0.23-0.45),-1.06 (0.23-0.45),↑0.22 (0.23-0.45),↓IgG (g/L)8.85 (2.26-5.4),↑13 (2.26-5.4),↑15.22 (2.26-5.4),↑3.2 (2.26-5.4),-IgM (g/L)0.93 (0.21-1.2),-0.54 (0.21-1.2),-1.46 (0.21-1.2),↑0.24 (0.21-1.2),-Autoantiboidesanti-GBM 43 RU/ml; MPO-ANCA 41 RU/ml; PR3-ANCA 63.2 RU/mlNAanti-GBM 39.3 RU/ml-Case31323334Gene of mutationNegativeNegativeIL10RAIL10RAAge when tested2 m2 m9 m4 mCD4+/CD8+ ratio2.11 (1.31-3.16),-2.11 (1.31-3.17),-1.07 (1.31-3.16),↓0.99 (1.31-3.16),↓CD16+/CD56+?(% lymphocytes)23.86 (3.5-17.0),↑30.93 (3.5-17.0),↑13.26 (4.0-18.2),-15.21 (4.0-18.2),-CD16+/CD56+??(absolute counts?)×109/L1.67 (0.1-1.3),↑2.64 (0.2-1.3),↑0.84 (0.2-1.3),-1.12 (0.1-1.3),-CD19+(absolute counts) ×109/L1.50 (0.6-3.0),-0.96 (0.6-3.0),-1.18 (0.6-5.7),-0.65 (0.6-3.0),-CD19+?(% lymphocytes)21.36 (14-39),-11.24 (14-39),↓18.66 (13-35),-8.85 (14-39),↓CD3+(absolute counts)×109/L3.75 (2.3-6.5),-0.47 (2.3-6.5),↓4.26 (2.4-6.9),-5.55 (2.3-6.5),-CD3+?(% lymphocytes)53.58 (48-75),-54.56 (48-75),-67.2 (50-77),-75.19 (48-75),↑CD4?(absolute counts?)×10 9/L2.52 (1.5-5.0),-4.31 (1.5-5.0),-2.17 (1.4-5.1),-2.78 (1.5-5.0),-CD4+?(% lymphocytes)35.95 (32.3-47.3),-50.57 (29.4-49.1),↑34.25 (32.3-47.3),-37.73 (32.3-47.3),-CD8+?(absolute counts?)×10 9/L1.19 (0.5-1.6),-0.32 (0.5-1.6),↓2.03 (0.6-2.2),-2.82 (0.5-1.6),↑CD8+?(% lymphocytes)17.02 (14.4-26.9),-3.75 (15.6-30.0),↓32.044 (14.4-26.9),↑38.27 (14.4-26.9),↑IgA (g/L)0.2 (0.23-0.45),↓0.23 (0.23-0.45),-1.79 (0.32-0.92),↑0.46 (0.24-1.02),-IgG (g/L)6.36 (2.26-5.4),↑8.85 (2.26-5.4),↑9.13 (3.38-8.07),↑8.85 (2.72-6.62),↑IgM (g/L)1.13 (0.21-1.2),-0.36 (0.21-1.2),-2.08 (0.38-2.16),-0.84 (0.3-1.62),-AutoantiboidesMPO-ANCA 20.4 RU/mlNAanti-GBM 36.7 RU/ml, MPO-ANCA 37.2 RU/ml, PR3-ANCA 50.1 RU/mlNACase35363738Gene of mutationIL10RAIL10RANegativeNegativeAge when tested28 d1 m2 m2 mCD4+/CD8+ ratio3.2 (1.06-2.73),↑1.78 (1.06-2.73),-3.27 (1.06-2.73),↑0.41 (1.06-2.73),↓CD16+/CD56+?(% lymphocytes)3.96 (3.5-17.0),-10.18 (3.5-17.0),-15.81 (3.5-17.0),-22.58 (3.5-17.0),↑CD16+/CD56+?(absolute counts?)×109/L0.15 (0.2-1.4),↓0.37 (0.2-1.4),-0.42 (0.2-1.4),-1.69 (0.2-1.4),↑CD19+(absolute counts) ×109/L0.09 (0.6-1.9),↓0.36 (0.6-1.9),↓0.72 (0.6-1.9),-0.93 (0.6-1.9),-CD19+?(% lymphocytes)22.33 (4-26),-9.84 (4-26),-27.54 (4-26),↑12.41 (4-26),-CD3+(absolute counts)×109/L2.82 (2.3-7.0),-2.88 (2.3-7.0),-1.43 (2.3-7.0),↓4.8 (2.3-7.0),-CD3+?(% lymphocytes)72.36 (60-85),-78.66 (60-85),-54.34 (60-85),↓64.13 (60-85),-CD4?(absolute counts?)×10 9/L2.12 (1.7-5.3),-1.81 (1.7-5.3),-1.06 (1.7-5.3),↓1.36 (1.7-5.3),↓CD4+?(% lymphocytes)54.37 (29.4-49.1),↑49.47 (29.4-49.1),↑40.34 (29.4-49.1),-18.16 (29.4-49.1),↓CD8+?(absolute counts?)×10 9/L0.66 (0.4-1.7),-1.02 (0.4-1.7),-0.32 (0.4-1.7),↓3.31 (0.4-1.7),↑CD8+?(% lymphocytes)16.98 (15.6-30.0),-27.86 (15.6-30.0),-12.33 (15.6-30.0),↓44.13 (15.6-30.0),↑IgA (g/L)0.25 (0.23-0.45),-0.19 (0.23-0.45),↓0.56 (0.23-0.45),↑0.28 (0.23-0.45),-IgG (g/L)14.94 (3.59-7.9),↑14.94 (3.59-7.9),↑16.32 (2.26-5.4),↑4.7 (2.26-5.4),-IgM (g/L)0.58 (0.21-1.2),-0.97 (0.21-1.2),-0.62 (0.21-1.2),-1.06 (0.21-1.2),-Autoantiboidesanti-GBM 79 RU/ml, PR3-ANCA 31 RU/mlanti-GBM 103.7 RU/mlanti-GBM 56.1 RU/ml-Test values were compared with age-specific reference range14 15, which were shown in brackets. ANCA, anti-neutrophil cytoplasmic antibody; GBM, glomerular basement membrane; MPO, myeloperoxidase; NA, not available; PR3, proteinase 3; -, normal; ↓, decreased; ↑, increased. ................
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