A recent review (SA Taylor, PF Whittington. Liver Transplantation 2016; 22: 677-85) provides several important concepts for practitioners who may need to manage neonatal acute liver failure.
The most common etiologies (in parenthesis the approximate percentage of cases in their experience):
- Gestational alloimmune liver disease (GALD) (60-90%)
- Viral hepatitis (20-30%)-particularly HSV, followed by HHV-6, and rarely CMV
- Hemophagocytic lymphohistiocytosis (HLH) (<10%)
- Mitochondrial hepatopathy (<5%)
- Rare causes include galactosemia, hereditary tyrosinemia type 1, and hereditary fructose intolerance. (<1%) In addition, bile acid synthetic defect 5-beta-reductase deficiency can cause neonatal liver failure.
While INR ≥2.0 was used in the PALF studies as a primary defining feature of liver failure, since an INR of 2.0 can occur in the normal newborn, the authors recommend using an INR≥ 3.0 for neonatal liver failure.
Their Table 1 helps provide some important differences, Distinguishing features:
- With GALD, ALT values are typically <100 due to underdeveloped hepatic parenchyma and ferritin is typically >800 and <7000. IUGR is frequent (70-90%) as is hypoglycemia. Hepatosplenomegaly is uncommon.
- With viral infections and HLH, ALT values are typically high, ferritin often very high, hepatosplenomegaly is common. IUGR is rare.
- With mitochondrial disorders, ALT typically is between 100-500, ferritin levels are variable, and IUGR occurs in 20-30%. A distinguishing feature is lactate: pyruvate ratio and ketone body ratios.
- By thinking carefully about the reasons for liver failure in the neonatal period and not trying to examine for every possible liver disease, the use of these variables can expedite the evaluation and decrease the cost. Genetic testing is not recommended due to the slow turnaround time, “and many diseases that are prominent causes of cholestatic disease …just do not cause NALF.”
With regard to treatment, the authors advocate use of IVIG if suspicion for GALD. If workup (lip biopsy and/or MRI) confirms GALD then exchange transfusion and repeat IVIG is recommended.
My take: This reference should be helpful when managing a neonate with severe liver disease.
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