Clinical characteristics of the 35 patients
Kindred . | Patient . | Sex . | Origin . | Age at diagnosis . | Status . | Age at last follow-up, y . | Protracted diarrhea . | ENT infection . | Pneumonia . | Failure to thrive . | Severe viral Inf. . | P liver disease . | Cholangitis . | AI cytopenia . | Reference(s) . |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | P1 | F | Algeria | 4 y | Dead | 10 | + | + | + | + | - | + | + | + | 2,31 |
2 | P2 | M | Algeria | 2 mo | HSCT/dead | 4 | + | + | + | + | + | + | + | - | 2,7,31 |
3 | P3 | F | Algeria | 3 mo | HSCT/dead | 6 | + | + | + | + | + | + | + | - | 2,7,31 |
3 | P4 | M | Algeria | 3 mo | Dead | 1 | - | + | + | + | + | - | - | - | 2,31 |
4 | P5 | M | Algeria | 3 y | HSCT/alive | 16 | + | + | + | + | - | - | - | - | 10,31 |
5 | P6 | M | Algeria | 5 y | Alive | 32 | + | + | + | + | + | + | - | + | 2,31 |
6 | P7 | F | Tunisia | 5 y | Dead | 5 | + | + | + | + | + | - | - | - | 2,31 |
7 | P8 | M | Algeria | 8 y | Dead | 17 | + | + | + | + | - | + | + | + | 2,31 |
8 | P9 | M | Algeria | 18 mo | HSCT/dead | 8 | + | + | + | + | - | - | - | - | 31 |
9 | P10 | M | Algeria | 2 y | HSCT/dead | 9 | + | + | + | + | + | - | - | + | 2,7,31 |
9 | P11 | M | Algeria | 1 mo | HSCT/dead | 1 | - | - | - | - | + | - | - | - | 2,7,31 |
10 | P12 | F | Morocco | 2 y | Dead | 2 | + | + | - | + | + | - | - | + | This report |
10 | P13 | F | Morocco | 1 y | HSCT/dead | 2 | + | + | + | + | - | - | - | - | 31 |
12 | P14 | M | Algeria | 2 y | Alive | 23 | + | + | + | + | - | + | - | - | 2,31 |
12 | P15 | F | Algeria | 1 mo | HSCT/dead | 1 | + | + | + | - | + | - | - | - | This report |
13 | P16 | F | Algeria | 1 y | HSCT/alive | 22 | + | + | + | + | - | + | - | - | 2,7,31 |
14 | P17 | F | Algeria | 5 mo | HSCT/alive | 18 | + | + | + | - | - | - | - | - | 7,31 |
15 | P18 | F | Algeria | 6 mo | HSCT/alive | 23 | + | + | + | + | - | + | - | - | 2,7,10,31 |
16 | P19 | F | Tunisia | 2 y | HSCT/alive | 26 | + | + | + | + | - | + | - | + | 2,7,31 |
17 | P20 | F | Tunisia | 1 mo | HSCT/dead | 5 | - | - | - | - | + | - | - | ND | 2,7,31 |
19 | P21 | M | Algeria | 2 y | HSCT/dead | 4 | + | + | + | + | + | + | - | - | 2,31 |
20 | P22 | F | Algeria | 1 y | HSCT/alive | 19 | + | + | + | + | - | + | - | - | 2,31 |
27 | P23 | M | Algeria | 4 y | HSCT/alive | 16 | + | + | + | + | - | - | - | - | 10,31 |
27 | P24 | M | Algeria | At birth | HSCT/alive | 4 | - | - | - | - | - | - | - | - | This report |
30 | P25 | F | Morocco | 6 mo | HSCT/dead | 4 | + | + | + | + | + | - | - | - | This report |
31 | P26 | M | Algeria | 9 mo | Alive | 6 | - | + | + | + | + | - | - | + | This report |
32 | P27 | F | Tunisia | 21 mo | HSCT/dead | 2.5 | + | - | + | + | + | - | - | + | 10 |
33 | P28 | F | Tunisia | 2.5 y | HSCT/dead | 4 | + | + | + | + | + | - | - | - | 10 |
34 | P29 | M | Morocco | 4.5 y | Alive | 9 | + | + | + | + | - | - | - | - | This report |
35 | P30 | F | Algeria | 7.5 y | HSCT/alive | 11 | + | + | - | + | + | + | - | - | This report |
36 | P31 | M | Algeria | 21 mo | HSCT/alive | 4 | + | + | - | + | + | + | - | - | This report |
37 | P32 | F | Algeria | 10 y | Alive | 12 | + | - | + | + | - | + | + | - | This report |
38 | P33 | F | Algeria | 7 mo | HSCT/alive | 2 | - | - | + | + | + | - | - | - | This report |
39 | P34 | F | Morocco | 5.5 y | Alive | 7 | + | + | + | - | + | + | - | - | This report |
40 | P35 | M | Algeria | 15 y | Alive | 16 | + | + | + | - | - | - | - | - | This report |
Kindred . | Patient . | Sex . | Origin . | Age at diagnosis . | Status . | Age at last follow-up, y . | Protracted diarrhea . | ENT infection . | Pneumonia . | Failure to thrive . | Severe viral Inf. . | P liver disease . | Cholangitis . | AI cytopenia . | Reference(s) . |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | P1 | F | Algeria | 4 y | Dead | 10 | + | + | + | + | - | + | + | + | 2,31 |
2 | P2 | M | Algeria | 2 mo | HSCT/dead | 4 | + | + | + | + | + | + | + | - | 2,7,31 |
3 | P3 | F | Algeria | 3 mo | HSCT/dead | 6 | + | + | + | + | + | + | + | - | 2,7,31 |
3 | P4 | M | Algeria | 3 mo | Dead | 1 | - | + | + | + | + | - | - | - | 2,31 |
4 | P5 | M | Algeria | 3 y | HSCT/alive | 16 | + | + | + | + | - | - | - | - | 10,31 |
5 | P6 | M | Algeria | 5 y | Alive | 32 | + | + | + | + | + | + | - | + | 2,31 |
6 | P7 | F | Tunisia | 5 y | Dead | 5 | + | + | + | + | + | - | - | - | 2,31 |
7 | P8 | M | Algeria | 8 y | Dead | 17 | + | + | + | + | - | + | + | + | 2,31 |
8 | P9 | M | Algeria | 18 mo | HSCT/dead | 8 | + | + | + | + | - | - | - | - | 31 |
9 | P10 | M | Algeria | 2 y | HSCT/dead | 9 | + | + | + | + | + | - | - | + | 2,7,31 |
9 | P11 | M | Algeria | 1 mo | HSCT/dead | 1 | - | - | - | - | + | - | - | - | 2,7,31 |
10 | P12 | F | Morocco | 2 y | Dead | 2 | + | + | - | + | + | - | - | + | This report |
10 | P13 | F | Morocco | 1 y | HSCT/dead | 2 | + | + | + | + | - | - | - | - | 31 |
12 | P14 | M | Algeria | 2 y | Alive | 23 | + | + | + | + | - | + | - | - | 2,31 |
12 | P15 | F | Algeria | 1 mo | HSCT/dead | 1 | + | + | + | - | + | - | - | - | This report |
13 | P16 | F | Algeria | 1 y | HSCT/alive | 22 | + | + | + | + | - | + | - | - | 2,7,31 |
14 | P17 | F | Algeria | 5 mo | HSCT/alive | 18 | + | + | + | - | - | - | - | - | 7,31 |
15 | P18 | F | Algeria | 6 mo | HSCT/alive | 23 | + | + | + | + | - | + | - | - | 2,7,10,31 |
16 | P19 | F | Tunisia | 2 y | HSCT/alive | 26 | + | + | + | + | - | + | - | + | 2,7,31 |
17 | P20 | F | Tunisia | 1 mo | HSCT/dead | 5 | - | - | - | - | + | - | - | ND | 2,7,31 |
19 | P21 | M | Algeria | 2 y | HSCT/dead | 4 | + | + | + | + | + | + | - | - | 2,31 |
20 | P22 | F | Algeria | 1 y | HSCT/alive | 19 | + | + | + | + | - | + | - | - | 2,31 |
27 | P23 | M | Algeria | 4 y | HSCT/alive | 16 | + | + | + | + | - | - | - | - | 10,31 |
27 | P24 | M | Algeria | At birth | HSCT/alive | 4 | - | - | - | - | - | - | - | - | This report |
30 | P25 | F | Morocco | 6 mo | HSCT/dead | 4 | + | + | + | + | + | - | - | - | This report |
31 | P26 | M | Algeria | 9 mo | Alive | 6 | - | + | + | + | + | - | - | + | This report |
32 | P27 | F | Tunisia | 21 mo | HSCT/dead | 2.5 | + | - | + | + | + | - | - | + | 10 |
33 | P28 | F | Tunisia | 2.5 y | HSCT/dead | 4 | + | + | + | + | + | - | - | - | 10 |
34 | P29 | M | Morocco | 4.5 y | Alive | 9 | + | + | + | + | - | - | - | - | This report |
35 | P30 | F | Algeria | 7.5 y | HSCT/alive | 11 | + | + | - | + | + | + | - | - | This report |
36 | P31 | M | Algeria | 21 mo | HSCT/alive | 4 | + | + | - | + | + | + | - | - | This report |
37 | P32 | F | Algeria | 10 y | Alive | 12 | + | - | + | + | - | + | + | - | This report |
38 | P33 | F | Algeria | 7 mo | HSCT/alive | 2 | - | - | + | + | + | - | - | - | This report |
39 | P34 | F | Morocco | 5.5 y | Alive | 7 | + | + | + | - | + | + | - | - | This report |
40 | P35 | M | Algeria | 15 y | Alive | 16 | + | + | + | - | - | - | - | - | This report |
ENT infection indicates ear nose and throat infections; P liver disease, progressive liver disease; AI cytopenia, autoimmune cytopenia; y, year(s); and mo, month(s).