Single-line summary items in each cell separated by semicolons for rapid bedside comparison.
| Cause | Key distinguishing features / clues |
|---|---|
| Wilson disease | Serum ceruloplasmin may be normal in ALF; very low alkaline phosphatase with high total bilirubin:alkaline phosphatase ratio; hemolytic anemia may be present; poor prognosis in fulminant presentation without liver transplant |
| Galactosemia | Infant recently fed lactose-containing formula; positive urine reducing substances; may present with Gram-negative sepsis, particularly Escherichia coli |
| Tyrosinemia | Profound coagulopathy with normal or near-normal serum aminotransferases; may be associated with Gram-negative sepsis; consider Urine succinylacetone measurement for diagnosis |
| Urea cycle defect | Markedly elevated ammonia (NH3) often without metabolic acidosis; ornithine transcarbamylase deficiency is most common and is X-linked; encephalopathy disproportionate to transaminases |
| Fatty acid oxidation defects (FAO) | Presentation like Reye syndrome with hypoketotic hypoglycemia and acute hepatic dysfunction; elevated transaminases variable; consider acylcarnitine profile and urine organic acids |
| Hereditary fructose intolerance | Symptoms triggered after introduction of fruit or sucrose-containing foods or medications; consider drugs/vitamins suspended in sucrose; hypoglycemia and liver dysfunction following fructose exposure |
| Mitochondrial disorders | Often multisystem mitochondrial dysfunction with progressive neurologic deficits, cardiomyopathy or myopathy but may present with isolated hepatic failure; elevated lactate:pyruvate ratio >20 supports mitochondrial etiology |
Use this table alongside targeted laboratory tests (serum ceruloplasmin, copper studies, urine reducing substances, succinylacetone, ammonia, acylcarnitine profile, lactate:pyruvate ratio) and clinical context to prioritize urgent management and confirm diagnosis.