DISEASE SCANNER

Global Incurable Diseases Tracker

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Genetic Disorder

Hereditary Angioedema (HAE)

HIGH SEVERITY

An autosomal dominant disorder causing recurrent episodes of severe swelling (angioedema) affecting skin, gastrointestinal tract, and airway. Caused by C1 inhibitor deficiency (HAE types I/II) or factor XII mutation (HAE type III).

Global Affected

150.0K

Countries

15

Symptoms

Recurrent angioedema (not urticaria)
Abdominal pain/cramping
Vomiting
Diarrhea
Airway swelling (life-threatening)
Skin swelling
Laryngeal edema

Treatment Options

Genetic counseling
Supportive care
Symptom management
Enzyme replacement (if applicable)
Physical therapy
Regular monitoring
Multidisciplinary care

Risk Factors

1Family history
2Genetic mutations
3Consanguinity
4Advanced paternal age
5Ethnic predisposition

Diagnostic Methods

  • 1Genetic testing
  • 2Clinical evaluation
  • 3Family history assessment
  • 4Specialized laboratory tests
  • 5Imaging studies
  • 6Biopsy (if applicable)

Prognosis

Good with prophylaxis. Laryngeal attacks can be fatal without treatment. Attacks preventable with C1 inhibitor replacement. Normal life expectancy with modern therapy.

Prevention

  • Genetic counseling
  • Carrier screening
  • Prenatal diagnosis
  • Preimplantation genetic diagnosis
  • Family planning

Research Status

C1 inhibitor concentrate, ecallantide, icatibant for acute attacks. Prophylactic C1 inhibitor, lanadelumab, or berotralstat. Self-administration training. Excellent prognosis with modern treatment. Avoid ACE inhibitors.

Sources

  • https://www.ncbi.nlm.nih.gov/books/NBK1116
  • https://medlineplus.gov/genetics
  • https://rarediseases.org

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.