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Hereditary fructose intolerance (HFI) is a recessively inherited condition, caused by hepatic, renal and intestinal aldolase B deficiency. The characteristic symptoms of nausea, vomiting, abdominal pain and sweating are induced by the ingestion of large quantities of fructose, sucrose or sorbitol. In severe intoxications glucagon-resistant hypoglycaemia, metabolic acidosis anduor hepatorenal failure may occur. More than 20 fatal or near-fatal insults following the administration of fructose or sorbitol containing solutions have been reported [1]. We report a case of a young male in whom suggestive clinical and biochemical manifestations in temporal relationship with infusion of fructose led to the diagnosis of HFI.

A young patient with unexplained acute hepatorenal dysfunction