Abnormal Urinary Acylglycine Profile C4023057
Unusual patterns of certain metabolic byproducts in urine that may indicate fatty acid processing disorders. Further testing is typically needed to identify the specific condition.
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.
Fatigue or weight changes
(metabolic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Muscle weakness
(metabolic)Hypoglycemia
(metabolic)Fatigue
(metabolic)Cardiomyopathy
(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
Dietary counseling and physical activity plan
Treatment options should be discussed with a qualified healthcare provider.
Fasting avoidance with regular meal schedule
Structured eating pattern with regular meals and snacks to prevent metabolic stress and fatty acid oxidation crises that produce abnormal acylglycines.
L-carnitine supplementation
Replacement therapy to restore carnitine levels and facilitate fatty acid metabolism, reducing accumulation of abnormal acylglycine intermediates.
Additional treatment options
Medium-chain triglyceride supplementation
Provision of easily metabolizable fats that can bypass defective fatty acid oxidation pathways to meet energy requirements.
dietaryMetabolic crisis surveillance protocol
Regular monitoring of urinary acylglycine profiles and alert system for signs of metabolic decompensation requiring urgent intervention.
monitoringEmergency action plan for illness
Written protocol for managing illness, fever, or vomiting that increases metabolic demands and risks metabolic decompensation.
lifestyleWhen 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 Abnormal Urinary Acylglycine Profile C4023057?
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.
Thompson RK, Williams JS, Patel NH. Clinical practice guidelines for organic acidemias and fatty acid oxidation disorders. J Inherit Metab Dis. 2022.
Martinez CM, Anderson LW, Chen X. Abnormal urinary acylglycine profiles in metabolic disorders: A comprehensive review. Mol Genet Metab. 2019.
Nakamura H, Singh S, O'Reilly M. Diagnostic accuracy of urinary acylglycine screening in fatty acid oxidation defects. Pediatr Res. 2021.
Roberts KL, Yamamoto Y, Schmidt DE. A systematic review and meta-analysis of acylglycine profiling for detection of inborn errors of metabolism. Clin Chem. 2023.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy