FBP1 AR unreviewed
Fructose-1,6-bisphosphatase deficiency
Enzyme or protein defect
Fructose-1,6-bisphosphatase
Accumulates or becomes deficient
Lactate, ketones
Key features
Fasting hypoglycaemia with lactic acidosis and ketosis, hyperventilation
Management
Avoid fasting; glucose during illness
Not clinical guidance. Written from standard clinical biochemistry, not from the uploaded documents, and marked unreviewed. Teaching-level reference for study and database seeding only. It must not be used to manage a patient.
Recent literature
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