Table S1. Demographic, clinical and immunologic information of studied patients. Parameters CVID-A (N=11) CVID-NA (N=9) P-value Demographic data Consanguinity, N (%) 9 (81.8) 6 (66.7) 0.62 Age at onset of symptoms, median (IQR), years 1 (0.5-11) 6 (1-22) 0.27 Age at time of PID diagnosis, median (IQR), years 9 (4-21) 19 (4-35) 0.30 Delay in diagnosis, median (IQR), years 5 (1-7) 3.5 (1.5-13) 0.88 Clinical Parameters Splenomegaly, N (%) 5 (45.5) 2 (22.2) 0.37 Hepatomegaly, N (%) 3 (27.3) 2 (22.2) 1.0 Early onset, N (%) 9 (81.8) 7 (77.8) 1.0 Enteropathy, N (%) 7 (63.6) 5 (55.6) 1.0 FTT, N (%) 2 (18.2) 1 (11.1) 1.0 Pneumonia, N (%) 6 (54.5) 4 (44.4) 1.0 Otitis media, N (%) 5 (45.5) 1 (11.1) 0.16 Sinusitis, N (%) 4 (36.4) 3 (33.3) 1.0 Bronchiectasis, N (%) 4 (36.4) 2 (22.2) 0.64 Immunologic data WBC, median (IQR), cells/µl 8210 )4200-8620( 6650 )5615-8115( 0.91 Lymphocyte, median (IQR), cells/µl 2300 )1218-4233( 2580 )1986-3419( 0.27 Neutrophil, median (IQR), cells/µl 3652 )2503-5662( 3025 )2530.25-3977.5( 0.79 CD3 + T cells, median (IQR), cells/µl 1695 )1417.5-3569( 1837.5 )1233.5-2034.75( 1.0 CD4 + , T cells median (IQR), cells/µl 870 )544.25-1508( 924.5 )562-1098.25( 0.72 CD8 + , T cells median (IQR), cells/µl 9465 )40.75-2032.5( 1023 )709.5-1059( 0.29 CD19 + B cells, median (IQR), cells/µl 264 )95-492.5( 317.5 )137.5-858.25( 0.65 CD16 + CD56 + NK cells, median (IQR), cells/µl 184 )150-184( 485 )485-485( 0.25 IgG, median (IQR), mg/dl 256.5 )113.55-523.25( 470 )160.5-753( 0.80 IgA, median (IQR), mg/dl 9 )6-113( 15.5 )6-50.75( 0.23 IgM, median (IQR), mg/dl 28 )9-110( 10.5 )4.5-83.25( 0.10 IgE, median (IQR), IU/ml 0 )0-2( 1 )0.75-9.5( 0.