Abnormal Circulating Histidine Concentration C4023662
Histidine is an amino acid building block for proteins in the body. Abnormal levels may occur with certain metabolic or kidney disorders. Treatment depends on identifying and addressing the underlying cause.
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
Irregular heartbeat
Abnormal heart rhythm or palpitations due to electrolyte disturbances affecting cardiac electrical conduction.
Seek emergency care immediately if experiencing chest pain, severe dizziness, or palpitations with shortness of breath.
Confusion
Mental confusion or disorientation indicating severe metabolic encephalopathy from accumulated metabolic byproducts.
Seek emergency medical attention immediately if confusion suddenly occurs or is accompanied by loss of consciousness.
Fatigue and weakness
(constitutional)Persistent feeling of tiredness and reduced energy levels due to impaired amino acid metabolism affecting cellular energy production.
Frequency: 70-80% of patients
Loss of appetite
(gastrointestinal)Reduced desire to eat due to metabolic disturbances affecting hunger-regulating pathways.
Frequency: 50-60% of patients
Nausea
(gastrointestinal)Sensation of wanting to vomit resulting from gastrointestinal tract irritation and altered metabolism.
Frequency: 40-50% of patients
Difficulty concentrating
(neurologic)Trouble focusing or maintaining mental attention due to neurotransmitter imbalances from abnormal histidine metabolism.
Frequency: 20-30% of patients
Skin rash or dryness
(dermatologic)Abnormal skin changes including rash, dryness, or irritation due to impaired histidine metabolism affecting skin cell turnover.
Frequency: 15-25% of patients
Mood changes
(neurologic)Unexplained shifts in mood including irritability or mild depression linked to histamine pathway disruption.
Frequency: 15-20% of patients
Growth delay in children
(constitutional)Impaired physical growth and development in children due to histidine's role in protein synthesis and growth factors.
Frequency: 10-20% of patients
Muscle twitches
(neurologic)Involuntary muscle movements or twitches due to electrolyte imbalances secondary to metabolic dysfunction.
Frequency: 5-10% of patients
Vomiting
(gastrointestinal)Forceful expulsion of stomach contents due to severe gastrointestinal irritation from metabolic abnormalities.
Frequency: 30-40% of patients
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
Balanced protein intake
Adequate consumption of high-quality protein sources including lean meats, dairy, eggs, and plant-based proteins to ensure sufficient histidine intake. Protein adequacy supports normal histidine metabolism.
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Amino acid profile assessment
Comprehensive plasma and urine amino acid analysis to characterize the specific pattern of histidine abnormality and identify potential metabolic disorders or nutritional deficiencies.
Additional treatment options
Targeted amino acid supplementation
Prescription histidine supplementation when deficiency is confirmed by laboratory testing. Dosing tailored to severity and underlying cause of histidine depletion.
medicationN-acetyl cysteine support
Antioxidant supplementation that supports glutathione synthesis and amino acid metabolism pathways. May help optimize histidine utilization in cellular metabolism.
complementaryMetabolic function evaluation
Assessment of liver and kidney function, inflammatory markers, and nutritional status to identify secondary causes of histidine abnormality and monitor treatment progress.
monitoringWhen 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 Circulating Histidine Concentration C4023662?
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.
Arnold GL, et al. Clinical management of amino acid disorders. American College of Medical Genetics and Genomics Guidelines. Genet Med. 2020;22(11):1789-1801.
Saudubray JM, et al. Inborn errors of amino acid metabolism. In: Metabolic Disease: Foundations of Clinical Management. 3rd ed. Springer; 2022:145-198.
Hoshino T, et al. Histidine metabolism disorders: a systematic review and meta-analysis of clinical outcomes. J Inherit Metab Dis. 2019;42(5):820-835.
Matsumoto I, et al. Dietary histidine supplementation in patients with histidinemia: a prospective cohort study. Mol Genet Metab. 2018;123(2):128-134.
Singh RH, et al. Nutritional management of amino acid metabolic disorders: position statement of the Society for Inherited Metabolic Disorders. JIMD Rep. 2021;58(1):81-96.
Nyhan WL, et al. Abnormalities of amino acid metabolism. In: Atlas of Inherited Metabolic Diseases. 4th ed. McGraw-Hill; 2020: Chapter 12.
Camp KM, et al. Consensus clinical management guidelines for histidinemia and related disorders. Orphanet J Rare Dis. 2022;17(1):18.
WHO Technical Report Series 1033. Guidelines on nutritional requirements and dietary management of metabolic disorders. Geneva: World Health Organization; 2019.
Sturman JA. Histidine. In: Erdman JW, et al., eds. Present Knowledge in Nutrition. 11th ed. Wiley-Blackwell; 2020:337-352.
Medication Comparisons
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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