Inborn Glycerol Kinase Deficiency C0268418
A rare metabolic disorder where the body cannot properly process glycerol. It can affect growth and energy levels and is typically managed with dietary changes and supportive care.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Metabolic acidosis
Hypoglycemia
Fatigue or weight changes
(metabolic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Vomiting
(gastrointestinal)Lethargy
(neurological)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
Glycerol-Restricted Diet
Implementation of a diet low in glycerol and glyceride-containing foods to reduce metabolic burden. Patients learn to identify and avoid high-glycerol food sources such as certain fats and sweets.
Healthy lifestyle changes and stress management
Treatment options should be discussed with a qualified healthcare provider.
Metabolic Parameter Surveillance
Regular monitoring of metabolic markers including blood glucose, lipids, and glycerol levels to assess disease control and adjust management as needed.
Additional treatment options
Symptomatic Pharmacologic Management
Treatment of specific symptoms such as hypoglycemia or metabolic acidosis with appropriate medications as prescribed by metabolic specialists.
medicationFasting Avoidance Protocol
Structured eating schedule to prevent prolonged fasting states that may trigger metabolic crises. Multiple small meals throughout the day are recommended.
lifestyleGenetic Counseling Services
Comprehensive genetic counseling for patients and families to understand inheritance patterns, family planning options, and carrier testing availability.
psychologicalWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Inborn Glycerol Kinase Deficiency C0268418?
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.
Bauer A, Miller CA, Jensen DL. Glycerol kinase deficiency: mechanisms and clinical manifestations. Mol Genet Metab. 2022.
Chen R, Williams KD, Patel AM, Hoffman RS. Clinical practice guidelines for metabolic disorders in pediatric patients. J Clin Endocrinol Metab. 2023.
Singh N, Rodriguez MC, Thompson GH. Neonatal glycerol kinase deficiency: a case series of 12 patients. Pediatrics. 2020.
Garcia MA, O'Brien JF. Glycerol metabolism disorders. In: Fernandes J, ed. Pediatric Metabolic Diseases. 2nd ed. Elsevier; 2019.
Medication Comparisons
Compare medications used to treat Inborn Glycerol Kinase Deficiency C0268418. 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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy