Table 1.

Participant profiles

Patient no.	Sex	Age, y	Mutated gene	IgG, mg/dL	WBC, ×103/μL	% lymphocytes	% B cells	B-cell count/μL	Immune-modulating medication
1	M	19	gp91phox	1120	4.9	15.0	12.1	89	Prednisone < 0.1‡
2	M	29	gp91phox	1210	3.9	22.9	14.0	123	—
3	M	28	p47phox	1720	8.0	12.5	7.3	72	—
4	M	21	p47phox	951	24.7	9.3	3.4	77	Prednisone 0.3
5	M	21	gp91phox	638	5.0	17.4	24.0	209	Prednisone < 0.1
6	M	25	gp91phox	735	3.6	1.0	10.3	4	Prednisone < 0.1
7	M	20	gp91phox	1120	11.0	17.5	9.2	177	Prednisone < 0.1; Humira
8	M	23	gp91phox	893	5.5	11.0	4.7	28	Prednisone 0.2
9	M	23	gp91phox	1340	4.4	32.0	21.1	295	Humira
10	M	30	p47phox	1240	8.0	17.4	3.6	49	Prednisone < 0.1
11	M	28	gp91phox	1250	5.6	34.1	1.6	31	Humira
12	F	38	p47phox	836	6.5	2.8	3.8	7	Prednisone 0.5
13	M	22	p47phox	1230	9.1	12.3	6.8	76	Prednisone < 0.1; Humira
14	M	22	gp91phox	1360	6.9	31.1	20.0	429	—
15	M	40	gp91phox	1690	9.9	15.4	14.5	221	Prednisone < 0.1
16	M	22	gp91phox	1690	7.9	24.5	5.7	109	Prednisone < 0.1
17	M	23	gp91phox	1350	4.5	27.4	14.2	176	Prednisone < 0.1
18	M	42	p47phox	1280	5.9	46.0	7.0	190	Prednisone < 0.1
19	M	18	p22phox	1110	6.4	13.1	3.4	28	Prednisone 0.2
20	M	41	gp91phox	2000	6.6	17.2	12.7	145	—
21	M	20	gp91phox	774	6.0	5.4	12.7	41	Prednisone < 0.1
22	F	32	p47phox	1740	6.3	24.8	16.0	250	—
23	F	28	p47phox	1390	6.7	18.9	10.9	138	Methotrexate
24	M	21	p47phox	1660	4.8	18.4	1.8	16	Prednisone 0.3
25	M	19	gp91phox	737	6.1	20.5	4.0	50	Prednisone < 0.1
26	M	41	gp91phox	2070	9.2	9.0	24.5	202	Prednisone 1.0
27	M	25	gp91phox	1170	8.2	9.9	13.0	106	—
28	M	25	p47phox	1830	4.3	33.0	28.5	404	—
Median	25M/3F	24		1245	6.3	17.4†	10.6	107†	—
Range		18-42		638-2070	3.6-24.7	1.0-46.0	1.6-28.5	3.5-429	—
Controls (N = 21)
Median	16M/5F	27			5.8	28.6†	9.9	186†	—
Range		22-42		642-1730*	3.7-10.9	20.6-47.1	4.3-16.0	57-386	—

— indicates not applicable or no medication.

^*Normal range in clinic.

^†Significant difference between groups.

^‡Prednisone expressed in milligrams/kilogram per day.