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. 2023 Apr 21;43(6):1289–1301. doi: 10.1007/s10875-023-01472-0

Table 4.

Characteristics of patients in the GAIN registry

Characteristics Total (n = 419) No. %
Gender Male 201 48.0%
Female 217 51.8%
Missing 1 0.2%
Agea Mean age (SD), 40 (±19)
0 – 9 25 6.0%
10 – 19 49 11.7%
20 – 29 67 16.0%
30 – 39 69 16.5%
40 – 49 61 14.6%
50 –59 84 20.0%
60 – 69 41 9.8%
70 – 79 17 4.1%
80+ 6 1.4%
Status Alive 399 95.2%
Deceased 14 3.3%
Discharged 1 0.2%
Lost to follow-up 5 1.2%
Mutated geneb NFKB1 48 11.5%
CTLA4 40 9.5%
TNFRSF13B 39 9.3%
NFKB2 18 4.3%
LRBA 13 3.1%
STAT3 GOF 12 2.9%
ADA2 10 2.4%
STAT1 9 2.1%
STAT3 9 2.1%
IKZF1 7 1.7%
PIK3CD 7 1.7%
ICOS 6 1.4%
FAS 5 1.2%
UNC13D 3 0.7%
BACH2 2 0.5%
CARMIL2 2 0.5%
FLG 2 0.5%
MST1 2 0.5%
SH2D1A 2 0.5%
Other 22 5.3%
Not tested 43 10.3%
No mutation found 87 20.8%
No information availablec 41 9.8%

aIn case of death, lost to follow-up, and discharge, age at death or last information was used

bSeveral mutated genes per patient are possible. Therefore, percentages do not sum up to 100%

cPending result, or no information