Pediatric Angioedema Workup

  • Author: Shih-Wen Huang, MD; Chief Editor: Harumi Jyonouchi, MD   more...
 
Updated: Jan 6, 2012
 

Other Tests

Acquired angioedema (AAE) is characterized by low functional C1INH levels, low C4 levels, and C3 levels within the reference range. Concentration of C1q is often very low.

Plasma levels for the diagnosis of hereditary angioedema (HAE) include the following:

  • C4 level less than 14 mg/L (diagnostic)
  • C1q level greater than 77 mg/L
  • C1INH (antigenic) level less than 199 mg/L (diagnostic)
  • C1INH (functional) level less than 72% of the reference range (diagnostic)

The summary of complement profiles in different forms of angioedema is as follows:

  • HAE1: C1INH levels are low; C4 levels are almost always low and can be used as the first step of screening; C1, C3, and C1q levels are all within the reference range. These changes are seen in both hereditary and spontaneous mutation conditions.
  • HAE2: C1INH levels may be normal or elevated but are dysfunctional; C1, C3, and C1q levels are within the reference range, but C4 levels are almost always low.
  • HAE3: The complement profile is normal.
  • AAE1: C1INH and C4 levels are low. C1q levels are usually, but not always, reduced.
  • AAE2: The findings are the same as in AAE1. Autoantibody testing may be appropriate.
  • Idiopathic angioedema: The complement profile is normal.
  • Nonhistaminergic angioedema (INAE): The complement profile is normal.
  • Allergic angioedema: The complement profile is normal.
  • ACE inhibitor–induced angioedema: The complement profile is normal.

Prothrombin fragment (F1 + 2) and D-dimer levels may have an important diagnostic value. A study in 28 patients with C1INH deficiency during acute attacks and remission, in 35 patients without C1INH deficiencies during abdominal colic, and in 20 healthy subjects found high levels of these biomarkers during acute angioedema attacks in patients with C1INH deficiency.[12]

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Approach Considerations

The diagnostic workup for urticaria-related angioedema is the same for urticaria. Perform an immunoglobulin E (IgE) antibody skin test or radioallergosorbent test (RAST) if the history is suggestive of a rash caused by foods, drugs, insect venom, or latex.

Obtain a bacterial culture with sensitivity if the patient has a history of fever and sore throat.

Obtain a thyroid profile, antithyroid microsomal antibodies, and antithyroglobulin antibody if the patient has a strong family history of thyroid disorder or symptoms of hypothyroidism; this is more frequent in females. However, patients are often euthyroid.

Measure C1 inhibitor (C1INH) and C3 and C4 levels if the patient has a family history of angioedema.

Obtain stool for ova and parasites if the patient reports ingestion of poorly cooked meats or travel in unsanitary areas.

Perform antinuclear antibody testing and urinalysis with microscopic examination if the patient may have arthritis, photosensitivity, or other signs or symptoms of collagen vascular disease.

Obtain a complete blood cell count (CBC) with differential, C-reactive protein level, and erythrocyte sedimentation rate if the patient's history indicates underlying vasculitis or inflammatory diseases.

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Ultrasonography

Ultrasonography of the GI tract may help differentiate between HAE and conditions of surgical abdomen.

In hereditary angioedema, ascites and edema of the intestinal walls are present in more than 80% of patients during acute attacks.

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Contributor Information and Disclosures
Author

Shih-Wen Huang, MD  Professor Emeritus of Pulmonology and Allergy, Department of Pediatrics, University of Florida College of Medicine

Shih-Wen Huang, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD  Associate Professor, Division of Pulmonary, Allergy/Immunology, and Infectious Diseases, Department of Pediatrics, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Harumi Jyonouchi, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association of Immunologists, American Medical Association, Clinical Immunology Society, New York Academy of Sciences, Society for Experimental Biology and Medicine, Society for Mucosal Immunology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

C Lucy Park MD, Head, Division of Allergy, Immunology, and Pulmonology, Associate Professor, Department of Pediatrics, University of Illinois at Chicago College of Medicine

C Lucy Park is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Medical Association, Chicago Medical Society, Clinical Immunology Society, and Illinois State Medical Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

References

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The mechanism of angioedema resulting from C1-esterase inhibitor deficiency.
Angioedema secondary to ACE inhibitors
 
 
 
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