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. | |||||||||||||||
| description / en | description / en | ||||||||||||||
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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| Property / Canonical URI: https://id.who.int/icd/entity/2128680017 / rank | |||||||||||||||
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CID11:ID_2128680017 | |||||||||||||||
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dki-india-ID_2128680017 | |||||||||||||||
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| Property / Knowledge Architect: https://pauloleads.com.br/cases-publicos/ / rank | |||||||||||||||
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13 August 2026
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Latest 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.
| Language | Label | Description | Also known as |
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| default for all languages | ID_2128680017 |
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| English | 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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CID11:ID_2128680017
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dki-india-ID_2128680017
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Concluído
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13 August 2026
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