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2 Aminoadipic 2 Oxoadipic Aciduria C1859817

Published: July 22, 2026Updated: August 27, 2026

A metabolic disorder affecting how the body breaks down certain amino acids. It can cause developmental delays and neurological problems. Treatment includes dietary changes and supportive therapies.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 27, 2026Editorial policy

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.

Developmental delay

(metabolic)

Fatigue or weight changes

(metabolic)

Noticeable symptoms that may vary in intensity over time.

Frequency: Common at all stages

Seizures

(metabolic)

Failure to thrive

(metabolic)

Dysmorphic facial features

(metabolic)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

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

1

Healthy lifestyle changes and stress management

Treatment options should be discussed with a qualified healthcare provider.

lifestyle
2

Lysine-restricted diet

Reduction of dietary lysine intake, a precursor to the affected metabolic pathway. Requires specialized medical nutrition products and careful amino acid balance monitoring.

dietary
3

Neurodevelopmental surveillance

Regular neurological and developmental assessments to monitor for seizures, developmental delay, or cognitive changes associated with the condition.

monitoring

Additional treatment options

Thiamine supplementation trial

Some organic acidemias may respond to thiamine (vitamin B1) supplementation. Trial period with metabolic monitoring to assess individual response.

medication

Rehabilitation services

Physical, occupational, and speech therapy as indicated based on individual developmental profile and any neurological involvement.

physical_therapy

Neuropsychological support

Cognitive evaluation and psychological support for any learning disabilities, behavioral concerns, or social-emotional challenges associated with the condition.

psychological

When to seek medical help

Recognize when you need immediate medical attention.

Schedule an appointment soon

Symptoms associated with this condition. Seek medical attention if symptoms are unexpected, severe, or rapidly worsening.- Contact your healthcare provider if symptoms persist or worsen. Seek emergency care for sudden severe symptoms.

Frequently asked questions

Common questions about this topic.

Q

What is 2 Aminoadipic 2 Oxoadipic Aciduria C1859817?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

Hoffmann GF, Schulze A, Okun JG. Rare Organic Acidurias: Expanding the Clinical Spectrum. J Inherit Metab Dis. 2022.

review2022
2

Saudubray JM, van den Berg M, Rahman S. Guidelines for the Diagnosis and Management of Amino Acid Disorders. Orphanet J Rare Dis. 2021.

guideline2021
3

Struys EA, Willemsen AT, de Klerk JB. Clinical and Biochemical Characterization of 2-Oxoadipic Aciduria: A Case Series. Mol Genet Metab. 2020.

clinical_trial2020
4

Wasserstein MP, Cederbaum SD, Ng WG. Lysine Metabolism Disorders: A Systematic Review. Metabolism. 2019.

meta_analysis2019

Medication Comparisons

Compare medications used to treat 2 Aminoadipic 2 Oxoadipic Aciduria C1859817. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

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 27, 2026. · Read our editorial policy