Hépatopathies métaboliques congénitales — Recommandations pour la prise en charge nutritionnelle

Many hereditary errors of metabolism may cause hepatopathy in children. In most cases, treatment is mainly or exclusively based on dietetics. Management of acute hepatopathy must take into account the possible metabolic origin, and urgent diet adaptations have to be begun until a metabolic disease has been excluded. If a metabolic disease is diagnosed, therapeutic strategies, whether to limit the accumulation of toxic compounds above the metabolic block (as in galactosemia, tyrosinemia type I, congenital hyperammonemias) or to compensate deficient compounds below the enzymatic block (as in glycogenosis, gluconeogenesis defects, fatty acid oxidation defects), have to be undertaken. In this paper we discuss dietary therapy principles in hereditary errors of metabolism that give rise to hepatopathies. These dietetic strategies have to cope with the nutritional needs of the child for growth and development. They have to be realistic enough to be accepted in the long term (whole life) by the child and his family. Diet therapy is a complex treatment and has to be rigorously managed by experienced doctors and dieticians.