Enzyme or protein defect
Aldolase B
Accumulates or becomes deficient
Fructose-1-phosphate (traps Pi)
Key features
Vomiting, hypoglycaemia, jaundice, cirrhosis after fructose/sucrose; aversion to sweets
Management
Exclude fructose, sucrose, sorbitol
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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