Table 5. Treatment and outcome of the included case series (case number ≥10).
| Treatment or outcome | Frequency (range) | ES (95% CI) | References (min and max) |
|---|---|---|---|
| Treatment | |||
| IVIG | 67% to 100% | 78% (70%, 87%) | (4,14,16,22-26,28) |
| Anticoagulants | 50% to 91% | 70% (50%, 89%) | (14,16,24,25) |
| Inotropic agents | 20% to 95% | 61% (41%, 81%) | (4,22-26,28) |
| Steroids | 10% to 95% | 53% (29%, 77%) | (4,14,16,22-26,28) |
| Aspirin | 20% to 100% | 53% (14%, 93%) | (4,16,22,24,28) |
| Fluid resuscitation | 44% to 67% | 52% (43%, 61%) | (22-24,28) |
| Intubation | 2% to 63% | 37% (15%, 60%) | (14,22-26,28) |
| IVIG resistance | 3% to 70% | 25% (6%, 45%) | (4,22,24,25,28) |
| Immunomodulators (anti-IL-1, anti-IL-6 or anti-TNF-α agents) | 9% to 19% | 14% (9%, 19%) | (14,23-26) |
| ECMO | 29% [10/35] | NA | (25) |
| Antibiotics | 86% to 100% | NA | (22,28) |
| Outcome | |||
| ICU | 44% to 100% | 72% (54%, 90%) | (16,22,24-26,28) |
| Discharge from hospital | 98% to 100% | NA | (4,14,16,22,24,26,28) |
| Mortality | 2% [1/58] | NA | (23) |
ES, effect size; CI, confidence interval; IVIG, intravenous immunoglobulin; ECMO, extracorporeal membrane oxygenation; ICU, intensive care unit; NA, not applied.