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 · fetched just now · sorted by publication date
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1
Hereditary Fructose Intolerance Presenting with Hematochezia.
Ks A, Bana SK, Mohan N. · 2026-07-27
unreviewed -
2
Spectrum of inherited metabolic disorders diagnosed through newborn screening and symptomatic referrals in Eastern Türkiye.
Arslan S, Ekinci Uğan F, Yaralı O, Kahveci H. · 2026-09-04
open access unreviewed -
3
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 -
4
Genetic testing in liver diseases: Clinical applications.
Keitel V, Mentzinger A, Dröge C, Vilarinho S. · 2026-05-28
open access unreviewed -
5
From Traditional Remedy to Evidence-Based Phytotherapeutic Agent: <i>Hedera helix</i> L. in Respiratory Medicine.
Pawłowska AM. · 2026-08-27
open access unreviewed -
6
Vaccination of children with inborn errors of metabolism: safety, immunogenicity, and practical implications.
Lipiński P, Jaczewska M, Jackowska T. · 2026-08-27
open access unreviewed -
7
Current Dietary Practices in Hereditary Fructose Intolerance: Results from a National Survey Across 15 Italian Metabolic Centres.
Bruni G, Zuvadelli J, Bernabei SM, Bonfanti C, Borsani B, Dianin A, Finizii A, Favaro A, Giorda S, Gugelmo G,… · 2026-08-10
open access unreviewed -
8
Sugar Shockwaves: How the Fructose-Glucose-ChREBP Pathway Hijacks Liver Metabolism.
Gugliucci A. · 2026-08-11
open access unreviewed -
9
Declared Inactive Ingredients in US Oral Drug Listings: A Reproducible Census of Prevalence, Labeling Coverage, and Within-Name Variation.
Staiger B. · 2026-08-21
cited 1× open access unreviewed -
10
Ketohexokinase: A central mediator of fructose-associated pathogenesis and promising therapeutic target.
Zhang H, Zhu Y, Xu C. · 2026-06-30
unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.