Patients can develop rapid swelling of the hands, feet, limbs, face, intestinal tract, or airway (larynx or trachea). HANE is characterized by recurrent episodes of angioedema involving any part of the body. Laryngeal
edema is common, and it is the major cause of death. Angioedema of the gastrointestinal tract may frequently mimic an acute abdomen.
Diagnosis
Diagnostic recommendations
Additional Information
- C1 inhibitor deficiency: consensus document
- Angioedema, Atlas of Dermatology, Health Portal
- Angioedema, Hereditary, National Organization for Rare Disorders
- Angioedema, Hereditary, eMedicine
- Angioedema, Acquiered, eMedicine
- C1 esterase deficiency, eMedicine
- Angioedema ereditario,Telethon
- Angioneurotic edema, ORPHANET
- C1 inhibitor deficiency: consensus document
- Hereditary angioedema, MedlinePlus
Diagnostic laboratories
Clinical:
Genetic:
Therapeutic options
- C1 inhibitor concentrate is preferred for acute treatment. Androgens such as winstrol, danazol, and oxandrolone for possible prevention of episodes. Hypotension accompanies abdominal attacks in some patients and fluid replacement therapy is required. A combination of Demerol and Compazine suppositories, and possibly Dicyclomine is useful to relieve abdominal pain and vomiting.
- Angioedema, Hereditary, eMedicine