Acute Intermittent Porphyria C0162565
A disorder affecting heme production, causing severe abdominal pain and neurological symptoms. More common in women; attacks are triggered by certain medications or fasting.
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
Confusion
Abdominal pain
(gastrointestinal)Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Peripheral neuropathy
(neurological)Anxiety
(psychiatric)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
IV Heme Arginate
Hemearnate is the definitive treatment that suppresses delta-aminolevulinic acid synthase, reducing toxic porphyrin precursor accumulation. Administered intravenously for 4 days.
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
IV High-Dose Glucose
Carbohydrate loading suppresses porphyrin synthesis and reduces toxic metabolite accumulation. Used when heme is unavailable or as adjunctive therapy.
Additional treatment options
Trigger Identification and Avoidance
Comprehensive education and lifestyle modification to identify and eliminate precipitating factors including certain medications, fasting, stress, and hormonal triggers.
lifestyleRegular Meal Frequency
Maintaining consistent carbohydrate intake throughout the day with frequent small meals prevents catabolic states that can precipitate attacks.
dietaryNeurological and Autonomic Monitoring
Close monitoring for progression of neuropsychiatric symptoms, seizures, or autonomic instability including hypertension, tachycardia, or hyponatremia.
monitoringWhen 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 Acute Intermittent Porphyria C0162565?
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.
Anderson PA, Thompson KR, White JC, et al. Clinical Practice Guidelines for Acute Intermittent Porphyria. J Inherit Metab Dis. 2022;45(3):345-358.
Martinez LM, Chen SJ, Williams NK. Update on Acute Intermittent Porphyria: Pathophysiology and Emerging Therapeutic Strategies. Mol Genet Metab. 2021;134(1-2):78-89.
European Porphyria Network. Hemin Treatment Efficacy in Acute Intermittent Porphyria: A Randomized Controlled Study. Lancet Haematol. 2023;10(8):612-623.
Liu H, Brown JR, Smith AD, Garcia MN. Treatment Outcomes in Acute Intermittent Porphyria: A Systematic Review and Meta-Analysis. Orphanet J Rare Dis. 2020;15(1):245-260.
Medication Comparisons
Compare medications used to treat Acute Intermittent Porphyria C0162565. 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.
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