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  • Title: [Hereditary fructose intolerance with early onset].
    Author: Mercier JC, Bourrillon A, Beaufils F, Odievre M.
    Journal: Arch Fr Pediatr; 1976 Dec; 33(10):945-53. PubMed ID: 1015980.
    Abstract:
    Four cases of hereditary fructose intolerance with an early onset are reported. The features of acute liver failure in the neonatal period include a haemorrhagic syndrome, collapse, neurological features, hypoglycaemia, disturbed bleeding and clotting studies and abnormal liver function tests. Investigations into the aetiology include a search for bacterial or viral infection but particularly for a metabolic cause: especially for hereditary fructose intolerance which may be difficult to distinguish from tyrosinosis. Finally, methods of treatment are discussed: continuous glucose infusion, exchange transfusion, assisted ventilation, and dietary measures beginning with protein exclusion. The importance of careful observation is stressed (particularly sequential studies of bloodclotting factors).
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