Table 1.
Comparison of cases with PD with and without anaphylaxis to ERT
| Characteristics | Patients with anaphylaxis (n = 4) | Patients without anaphylaxis (n = 9) | Total (n = 14) |
|---|---|---|---|
| Male, n (%) | 1 (25) | 4 (44.4) | 5 (35.7) |
| Age at diagnosis, months | 3 (1–40) | 3 (1–15) | 3.2 (1–40) |
| Positive CRIM status,a n (%) | 3 (75) | 5 (71.4) | 8 (72.7) |
| Parental consanguinity | 4 (100) | 7 (77.8) | 12 (85.7) |
| Factors related with ERTb | |||
| Age at the onset of ERT, months | 4 (1–44) | 4 (2–15) | 4 (1–44) |
| High-dose ERT (40 mg/kg/dose), n (%) | 3 (75) | 2 (22.2) | 5 (38.5) |
| Weekly ERT administration, n (%) | 1 (25) | 1 (11.1) | 2 (15.4) |
ERT, enzyme replacement therapy; CRIM, cross-reactive immune material; PD, Pompe disease.
a
CRIM status was available in 11 patients.
b
Calculated in 13 patients who received ERT.