Table 2.
Comparison of clinical characteristics between sporadic and familial cases of isolated congenital asplenia
| Sporadic n = 32 | Familial n = 41 | P value | |
|---|---|---|---|
| Age at diagnosis, years | 2.1 (0.8–34.2) | 1.0 (0.6–3.0) | 0.04 |
| Male sex | 11a (44) | 25(61) | 0.18 |
| Clinical events | |||
| Sepsis and/ or meningitis | 25(78) | 24 (58) | 0.08 |
| Others | 6 (19) | 2 (5) | 0.06 |
| Asymptomatic | 1 (3) | 15 (37) | 0.001 |
| Pathogens | |||
| S. pneumoniae | 16b (62) | 14c (56) | 0.69 |
| H. influenzae | 5b (19) | 3c (12) | 0.48 |
| Others | 3b (12) | 0c (0) | 0.08 |
| Howell- Jolly bodies | 18 (56) | 17 (41) | 0.21 |
| Outcome | |||
| Death | 12 (38) | 16 (39) | 0.68 |
Values are presented as median (interquartile range) or number (percentage). P < 0.05 is considered to be significant. adata from 25 patients were available. bdata from 26 patients were available. cdata from 25 patients were available