Hereditary C1 Esterase Inhibitor Deficiency Dysfunctional Factor C0398776
Hereditary angioedema caused by a C1 inhibitor protein that is present but does not work properly. Leads to recurrent episodes of swelling in various body parts. Managed with specific therapies and preventive measures.
Key facts at a glance
- ICD-10
- R69
- ICD-11
- MA00.0
- Body system
- immunologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Laryngeal edema
Generalized symptoms
(immunologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Abdominal pain
(gastrointestinal)Swelling of extremities
(integumentary)Headache
(neurologic)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
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
C1 esterase inhibitor replacement therapy
Intravenous or subcutaneous administration of C1 esterase inhibitor concentrate to replace deficient or dysfunctional protein, reducing frequency and severity of angioedema attacks.
Bradykinin B2 receptor antagonist
Subcutaneous injection that blocks bradykinin receptors, effectively terminating acute angioedema episodes by inhibiting the pathway causing swelling.
Additional treatment options
Plasma kallikrein inhibitor
Subcutaneous injection that inhibits plasma kallikrein, preventing bradykinin formation and reducing angioedema attack severity and duration.
medicationTrigger avoidance and attack preparedness
Identification and avoidance of individual triggers such as stress, infection, or certain medications; maintaining emergency medication access and carrying medical alert identification.
lifestyleRegular specialist follow-up and attack diary
Routine assessment by immunology or hematology specialists to monitor attack frequency, adjust prophylaxis, and evaluate treatment response using patient-maintained attack diaries.
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 Hereditary C1 Esterase Inhibitor Deficiency Dysfunctional Factor C0398776?
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.
Maurer M, Magerl M, Ansotegui I, et al. The international WAO/EAACI guideline for the management of hereditary angioedema - the 2021 revision and update. World Allergy Organ J. 2021.
Busse PJ, Christiansen SC. Hereditary Angioedema. N Engl J Med. 2020.
Banerji A, Riedl MA, Bernstein JA, et al. Efficacy of lanadelumab in patients with hereditary angioedema: Results from the HELP study. Lancet Respir Med. 2018.
Liu S, Wang X, Xu Y, et al. Efficacy and safety of C1 esterase inhibitor for hereditary angioedema: A systematic review and meta-analysis. J Allergy Clin Immunol Pract. 2022.
Medication Comparisons
Compare medications used to treat Hereditary C1 Esterase Inhibitor Deficiency Dysfunctional Factor C0398776. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy