Table 3.
Association between residual ADAMTS13 activity and phenotypic outcomes
| Phenotype | ADAMTS13 activity unadjusted analysis, % | ADAMTS13 activity adjusted for family clustering, % |
|---|---|---|
| Age at first TTP episode requiring FFP, y* | ||
| beta (95% CI) | 3.242 (1.432 to 5.052) | 3.260 (1.535 to 4.986) |
| R2 | 0.334 | 0.419 |
| P | 0.001 | 0.001 |
| Total no. of lifetime TTP episodes* | ||
| beta (95% CI) | −2.081 (−4.442 to 0.281) | −2.082 (−4.443 to 0.279) |
| R2 | 0.112 | 0.150 |
| P | 0.082 | 0.081 |
| Annual rate of TTP episodes* | ||
| beta (95% CI) | −0.306 (−0.500 to −0.111) | −0.306 (−0.502 to −0.110) |
| R2 | 0.285 | 0.309 |
| P | 0.003 | 0.004 |
| Multiple TTP episodes† | ||
| OR (95% CI) | 0.798 (0.519 to 1.225)‡ | 0.795 (0.517 to 1.225) |
| P | 0.302 | 0.299 |
| Neonatal jaundice and/or thrombocytopenia† | ||
| OR (95% CI) | 0.879 (0.616 to 1.255)‡ | 0.878 (0.615 to 1.255) |
| P | 0.478 | 0.475 |
| Persistence of renal/neurological damage† | ||
| OR (95% CI) | 2.014 (0.863 to 4.699)‡ | 2.285 (0.805 to 6.485) |
| P | 0.105 | 0.120 |
| Regular FFP prophylaxis† | ||
| OR (95% CI) | 0.625 (0.410 to 0.954)‡ | 0.625 (0.409 to 0.955) |
| P | 0.030 | 0.030 |
*
On linear regression analysis.
†
On logistical regression analysis.
‡
Per 1% increase in ADAMTS13 activity.
OR indicates odds ratio.