Criteria by Jodele et al for MODS diagnosis and resolution
| Organ . | MODS diagnosis . | MODS resolution . |
|---|---|---|
| Kidney | ≥50% reduction of cystatin-C GFR from pre-HCT value or the lowest value during or before diagnosis of TA-TMA, whichever is lower, or requiring RRT | Cystatin-C GFR of ≥70 mL/min per 1.73 m2 or ≥50% increase in cystatin-C GFR from the lowest value during TA-TMA |
| Pulmonary | Any need for positive pressure ventilation (noninvasive or invasive) for ≥24 h | Resolution of positive pressure ventilation (noninvasive or invasive), and resolution of oxygen requirements |
| Cardiovascular | PH diagnosed by cardiologist using cardiac catheterization, or PH criteria on echocardiogram (RV pressure of ≥50% of systemic pressure, ventricular septal flattening, or RV dysfunction) | Resolution of pulmonary hypertension (may receive maintenance therapy) |
| Serositis | Clinically significant pericardial effusion requiring medical therapy (such as diuretics) or drainage (pericardiocentesis) | No evidence of clinically significant serositis requiring medical therapy or drainage |
| Severe hypertension | Hypertension requiring >2 antihypertensive medications for >24 h or continuous antihypertensive infusion for ≥12 h or resulting in complications such as CNS bleeding or posterior reversible encephalopathy syndrome | Normotensive for age on ≤2 antihypertensive medications or return to pretransplant baseline antihypertensive therapy |
| CNS | Altered mental status attributable to TA-TMA or CNS bleeding, or seizures clinically attributable to PRES | No uncontrolled seizures (may be on therapy), no active PRES (residual radiologic signs are acceptable without clinical symptomatology) |
| GI | GI bleeding and/or intestinal strictures in subject with systemic TA-TMA or intestinal TA-TMA∗ requiring medical or surgical interventions | No active GI bleeding, no evidence of unresolved intestinal strictures (history of surgical stricture correction is acceptable) |
| Organ . | MODS diagnosis . | MODS resolution . |
|---|---|---|
| Kidney | ≥50% reduction of cystatin-C GFR from pre-HCT value or the lowest value during or before diagnosis of TA-TMA, whichever is lower, or requiring RRT | Cystatin-C GFR of ≥70 mL/min per 1.73 m2 or ≥50% increase in cystatin-C GFR from the lowest value during TA-TMA |
| Pulmonary | Any need for positive pressure ventilation (noninvasive or invasive) for ≥24 h | Resolution of positive pressure ventilation (noninvasive or invasive), and resolution of oxygen requirements |
| Cardiovascular | PH diagnosed by cardiologist using cardiac catheterization, or PH criteria on echocardiogram (RV pressure of ≥50% of systemic pressure, ventricular septal flattening, or RV dysfunction) | Resolution of pulmonary hypertension (may receive maintenance therapy) |
| Serositis | Clinically significant pericardial effusion requiring medical therapy (such as diuretics) or drainage (pericardiocentesis) | No evidence of clinically significant serositis requiring medical therapy or drainage |
| Severe hypertension | Hypertension requiring >2 antihypertensive medications for >24 h or continuous antihypertensive infusion for ≥12 h or resulting in complications such as CNS bleeding or posterior reversible encephalopathy syndrome | Normotensive for age on ≤2 antihypertensive medications or return to pretransplant baseline antihypertensive therapy |
| CNS | Altered mental status attributable to TA-TMA or CNS bleeding, or seizures clinically attributable to PRES | No uncontrolled seizures (may be on therapy), no active PRES (residual radiologic signs are acceptable without clinical symptomatology) |
| GI | GI bleeding and/or intestinal strictures in subject with systemic TA-TMA or intestinal TA-TMA∗ requiring medical or surgical interventions | No active GI bleeding, no evidence of unresolved intestinal strictures (history of surgical stricture correction is acceptable) |
MODS updated from Jodele et al.3
RRT includes continues RRT and/or dialysis.
CNS, central nervous system; PH, pulmonary hypertension; PRES, posterior reversible encephalopathy syndrome; RV, right ventricular.
Intestinal TA-TMA diagnostic criteria by El-Bietar et al.17-19 are as follows. Clinical signs: severe abdominal pain, GI bleeding, clinical ileus; radiologic signs: signs of ileus, thick mucosal wall; GI endoscopy: mucosal erosions, mucosal hemorrhages; histologic signs on tissue biopsy: endothelial cell swelling and separation with mucoid subintimal thickening, schistocytes and fibrinoid debris in the vessel lumen, intravascular thrombus formation (absent does not exclude diagnosis), mucosal hemorrhages/red cell extravasation into the tissues, crypt loss/mucosal infarcts, total denudation of mucosa.