Evidence of advanced or progressive intestinal failure-associated liver disease: - Hyperbilirubinemia >75 micromol/L¶ (4.5 mg/dL) despite intravenous lipid modification strategies that persists for >2 months
- Any combination of elevated serum bilirubin, reduced synthetic function (subnormal albumin or elevated international normalized ratio), and laboratory indications of portal hypertension and hypersplenism, especially low platelet count, persisting for >1 month in the absence of a confounding infectious event(s)
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Thrombosis of 3 out of 4 discrete upper body central veins (left subclavian and internal jugular, right subclavian and internal jugular) or occlusion of a brachiocephalic vein in children (in adults, this criterion should be evaluated in a case-by-case basis) |
Life-threatening morbidity in the setting of indefinite parenteral nutrition dependence of either anatomical or functional cause, as suggested by: - In children, 2 admissions to an intensive care unit (after initial recovery from the event resulting in intestinal failure) because of cardiorespiratory failure (mechanical ventilation or inotrope infusion) due to sepsis or other complications of intestinal failure
- In adults, on a case-by-case basis
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Invasive intra-abdominal desmoids in adolescents and adults |
Acute diffuse intestinal infarction with hepatic failure |
Failure of first intestinal transplant |