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
Europe PMC · from cache · sorted by publication date
-
1
Ketohexokinase: A central mediator of fructose-associated pathogenesis and promising therapeutic target.
Zhang H, Zhu Y, Xu C. · 2026-06-30
unreviewed -
2
Hereditary Fructose Intolerance Presenting with Hematochezia.
Ks A, Bana SK, Mohan N. · 2026-07-27
unreviewed -
3
Delayed diagnosis of hereditary fructose intolerance presenting as chronic lean steatosis in an adolescent.
Hurlock A, Lah M, Hyaduck HS, Molleston JP, Jarasvaraparn C. · 2026-07-13
unreviewed -
4
Endogenous fructose production in patients and mice with aldolase B deficiency.
Buziau AM, Simons N, Tolan DR, Caiment F, Scheijen JLJM, van de Waarenburg MP, Schalkwijk CG, Brouwers MCGJ. · 2026-07-17
unreviewed -
5
A National Overview of Nutritional Care in Diet-Treated Inborn Errors of Metabolism in Brazil
Poloni S, Pesce L, Kanufre V, Farret L, Pugliese C, Silva J, Poubel M, Leite M, de Oliveira R. · 2026-06-01
open access unreviewed - 6
-
7
Fructose malabsorption associated with functional abdominal bloating: Case-control study.
Kulandaivelu A, Vaithiyam V, Mehta K, Reddy RT, Ahuja A, Sirohi N, Sonika U, Dalal A, Kumar A, Puri AS, Sachd… · 2026-06-01
open access unreviewed -
8
Consistency and clarity of pharmacogenomic guidance in UK medicine patient information leaflets: A cross-sectional analysis.
Narlawar P, Ratcliffe C, Sharma V, Newman WG, Pirmohamed M, McDermott J. · 2026-03-15
open access unreviewed -
9
Fructose 1-phosphate inhibits mannose phosphate isomerase to suppress hepatocellular carcinogenesis.
Wang Y, Zhang X, Wang N, Jiang H, Liang N, Du C, Yin C, Li R, Zhang L, Tu Q, Lv J, Ma H, Xu X, Kong X, Chen X… · 2026-05-25
open access unreviewed -
10
Fructose: metabolic signal and modern hazard.
Johnson RJ, Lanaspa MA, Tolan DR, Goncalves MD, Softic S, Stanhope KL, Sánchez-Lozada LG, Herman MA, Rabinowi… · 2026-04-17
cited 1× unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.