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Hereditary Angioedema: Diagnostic Algorithm and Current Treatment Concepts.

Ankur Kumar Jindal¹, Anuradha Bishnoi², Sunil Dogra²

¹Department of Pediatrics, Advanced Pediatrics Centre, Chandigarh, India.

Indian Dermatology Online Journal

|December 22, 2021

Summary

This summary is machine-generated.

Hereditary angioedema (HAE) is underrecognized in India. This review outlines diagnostic strategies and current treatment options for HAE, emphasizing resource-limited settings.

Keywords:

Androgens C1-Inhibitor fresh frozen plasma hereditary angioedema tranexamic acid

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Area of Science:

Immunology
Genetics
Rare Diseases

Background:

Hereditary angioedema (HAE) is a rare genetic disorder affecting 1 in 10,000 to 50,000 people globally.
HAE is significantly underdiagnosed in India due to limited awareness and diagnostic facilities.
Clinical symptoms include swelling, abdominal pain, and potentially life-threatening laryngeal edema.

Purpose of the Study:

To review the diagnostic strategies for HAE.
To discuss current HAE treatment concepts.
To highlight available treatment options in resource-constrained settings like India.

Main Methods:

Review of diagnostic criteria for HAE, including C1, C1-INH, and C1q levels.
Analysis of HAE management strategies: acute attack treatment and prophylaxis.
Evaluation of treatment options available in India, including fresh frozen plasma, tranexamic acid, and attenuated androgens.

Main Results:

HAE diagnosis should be considered in patients with angioedema unresponsive to standard treatments.
Current HAE management in India relies on non-first-line therapies due to medication scarcity.
Attenuated androgens are used for prophylaxis but carry significant side effect concerns, especially in children and females.

Conclusions:

Individualized HAE treatment is crucial, balancing risks and benefits of long-term prophylaxis.
Developing accessible diagnostic and treatment protocols for HAE in India is essential.
Further research into safer and more effective HAE therapies for resource-limited settings is needed.