Elevated Urinary Succinylacetone Level C5937353
An important marker for hereditary tyrosinemia type I, a metabolic disorder. Requires prompt evaluation by metabolic specialists and treatment.
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
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Liver dysfunction
(hepatobiliary)Renal tubular dysfunction
(renal)Rickets
(musculoskeletal)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
Nitisinone (NTBC) Therapy
First-line pharmacotherapy that inhibits the enzyme responsible for converting tyrosine to toxic metabolites, reducing succinylacetone production.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Low Tyrosine and Phenylalanine Diet
Strict dietary restriction of tyrosine and phenylalanine through specialized low-protein foods and medical formulas to minimize toxic metabolite accumulation.
Additional treatment options
Liver Transplantation
Definitive surgical treatment for patients with advanced liver disease or those unresponsive to medical therapy, replacing the deficient enzyme activity.
surgicalSuccinylacetone and Liver Function Monitoring
Serial measurement of urinary succinylacetone and liver function tests to assess treatment response and detect liver dysfunction early.
monitoringMetabolic Crisis Prevention Protocol
Development of personalized emergency protocol including sick day rules, identification of metabolic decompensation signs, and access to immediate medical care.
lifestyleWhen 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 Elevated Urinary Succinylacetone Level C5937353?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
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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