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One percent of all infants are born with non-immune hydrops, a condition with a mortality rate of up to 98%. We report a female infant with normal birth weight born in good condition that died after few hours of life with acute liver failure. She had a heavy jaundice, associated with severe hemoconcentration and a dramatic elevation of plasma ammonia levels and a liver biopsy was characterized by centrilobular necrosis without fibrosis, as reported in acute liver failure cases with positive prognosis. We present the pathogenesis, diagnostic approach and therapeutic measures taken in this case.div.contentbox {
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