Fructose-1,6-diphosphatase deficiency (Q86969): Difference between revisions

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A deficiência de frutose 1,6 bifosfatase é uma doença autossômica recessiva de neoglicogênese. É caracterizada por recorrentes e, às vezes, ameaçadores episódios de hipoglicemia de jejum com acidose lática. Os pacientes ficam assintomáticos entre os episódios ou após a ingestão de frutose. O tratamento é baseado na alimentação frequente de bebês e crianças pequenas com uma dieta enriquecida com glicose ou maltodextrina e limitada em frutose.
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Fructose 1,6 diphosphatase deficiency is an autosomal recessive disorder of neoglucogenesis. It is characterised by recurrent, and sometimes life-threatening, episodes of fasting hypoglycaemia with lactic acidosis. Patients are asymptomatic between episodes or after ingestion of fructose. Treatment is based on frequent feeding for infants and young children with a diet enriched with glucose or maltodextrin and limited in fructose.

Revision as of 12:32, 15 August 2026

Fructose 1,6 diphosphatase deficiency is an autosomal recessive disorder of neoglucogenesis. It is characterised by recurrent, and sometimes life-threatening, episodes of fasting hypoglycaemia with lactic acidosis. Patients are asymptomatic between episodes or after ingestion of fructose. Treatment is based on frequent feeding for infants and young children with a diet enriched with glucose or maltodextrin and limited in fructose.
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    Fructose-1,6-diphosphatase deficiency
    Fructose 1,6 diphosphatase deficiency is an autosomal recessive disorder of neoglucogenesis. It is characterised by recurrent, and sometimes life-threatening, episodes of fasting hypoglycaemia with lactic acidosis. Patients are asymptomatic between episodes or after ingestion of fructose. Treatment is based on frequent feeding for infants and young children with a diet enriched with glucose or maltodextrin and limited in fructose.

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