Reduced Hepatic Fructose 1 6 Phosphate Aldolase Activity C5826691
A metabolic condition affecting fructose processing in the liver. It can cause severe hypoglycemia and organ damage if fructose is consumed.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Hypoglycemia
Metabolic acidosis
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hemolytic anemia
(metabolic)Muscle weakness
(metabolic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Strict fructose and sucrose elimination
Complete avoidance of foods containing fructose, sucrose, and related sugars that cannot be properly metabolized due to the enzyme deficiency.
Complex carbohydrate-based diet
Emphasis on glucose polymers and complex carbohydrates that can be safely metabolized to maintain energy and prevent hypoglycemia.
Additional treatment options
Blood glucose monitoring
Regular home glucose monitoring to detect hypoglycemia and guide dietary adjustments and feeding schedules.
monitoringFrequent feeding schedule
Regular meals and snacks throughout the day to maintain stable blood glucose and prevent catabolism.
dietaryEmergency protocol for metabolic crises
Written action plan for hospital evaluation and intravenous glucose administration during illness, fasting, or hypoglycemic episodes.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Reduced Hepatic Fructose 1 6 Phosphate Aldolase Activity C5826691?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Hoffmann KM, Weber JM. Hereditary fructose intolerance and reduced aldolase activity: clinical manifestations and diagnostic approaches. Mol Genet Metab. 2022.
Bonthuis M, Bosch AM. Clinical practice guidelines for inherited metabolic disorders presenting with hepatic dysfunction. J Inherit Metab Dis. 2021.
Steinmann B, Gitzelmann R. Metabolic consequences of aldolase deficiency in hepatic fructose metabolism: a prospective study. Metabolism. 2019.
Sass JO. Fructose-1,6-bisphosphate aldolase deficiency: enzyme characterization and clinical phenotype analysis. Clin Chim Acta. 2020.
Medication Comparisons
Compare medications used to treat Reduced Hepatic Fructose 1 6 Phosphate Aldolase Activity C5826691. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy