Fructose-1,6-diphosphatase deficiency (Q86969)
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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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_2128680017 |
||
| 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. |
Statements
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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