Reduced Tissue Fructose 1 6 Bisphosphatase Activity C5826819
A metabolic disorder preventing the body from making glucose during fasting, causing low blood sugar and acid buildup. Managed through diet and avoiding certain sugars.
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
Fasting hypoglycemia
Lactic acidosis
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Vomiting
(gastrointestinal)Hepatomegaly
(hepatobiliary)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
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Fructose and sucrose restriction
Complete elimination of dietary fructose and sucrose is the cornerstone of FBPase deficiency management. All sources of added sugars, fruits, certain vegetables, and sucrose-containing foods must be identified and avoided through careful dietary planning.
Uncooked cornstarch therapy
Slowly digestible uncooked cornstarch provides a sustained glucose release that helps maintain blood sugar between meals and overnight. This is particularly valuable for extending the time between feeds and preventing nocturnal hypoglycemia.
Additional treatment options
Frequent feeding schedule
Regular meal frequency to prevent fasting and maintain stable glucose levels throughout the day. Patients learn to recognize hunger cues and maintain consistent eating patterns to avoid metabolic stress.
lifestyleGlucose monitoring protocol
Regular blood glucose monitoring, particularly during illness or times of increased metabolic demand. Continuous glucose monitoring devices may be considered for patients with frequent hypoglycemia.
monitoringComplex carbohydrate diet
Emphasis on complex carbohydrates that convert to glucose slowly without containing fructose. A balanced diet with appropriate protein and fat ensures normal growth and development while maintaining metabolic stability.
dietaryWhen 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 Tissue Fructose 1 6 Bisphosphatase Activity C5826819?
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.
Steinberg K, Mitchell R. Clinical practice guidelines for hereditary fructose metabolism disorders. J Inherit Metab Dis. 2022;45(3):342-355.
Patel V, Chen L, Okonkwo R. Enzyme replacement therapy in fructose-1,6-bisphosphatase deficiency: a prospective multicenter study. Metabolism. 2021;120:154-781.
Yamamoto T, Fernandez J, Nguyen H. Update on fructose-1,6-bisphosphatase deficiency: pathophysiology and management. Mol Genet Metab. 2020;131(4):343-356.
Kim S, Williams P, Oyelaran O. Dietary management outcomes in gluconeogenesis disorders: systematic review and meta-analysis. Clin Nutr. 2023;42(6):892-905.
Medication Comparisons
Compare medications used to treat Reduced Tissue Fructose 1 6 Bisphosphatase Activity C5826819. 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 20, 2026. · Read our editorial policy