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Immunoglobulin A Deficiency, Pediatric

Nashmia Qamar, DO, MSc and Ramsay L. Fuleihan, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

Selective IgA deficiency is the most common primary antibody deficiency; diagnosed by: 
  • Increased susceptibility to infection

  • Autoimmune manifestations

  • Serum IgA <7 mg/dL and a norma...

DIAGNOSIS

HISTORY

  • Patients with IgA deficiency

    • Can have recurrent sinopulmonary infections

    • Can have frequent GI infections

    • Tend to have atopic disease such as allergic rhinitis, allergic conjunctivitis, u...

TREATMENT

GENERAL MEASURES

  • There is no specific drug therapy.

  • Recurrent infections should be treated aggressively with broad-spectrum antibiotics.

  • Antibiotic prophylaxis to prevent recurrent sinopulmonar...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Patients should be observed for the following:

    • Sinopulmonary infections, particularly signs/symptoms of bronchiectasis

    • GI infections

    • Autoimmune diseases

    • Inflammatory ...

ADDITIONAL READING

  • Burrows PD, Cooper MD. IgA deficiency. Adv Immunol.  1997;65:245–276. [View Abstract on OvidInsights]

  • Janzi M, Kull I, Sjöberg R, et al. Selective IgA deficiency in early life: ...

CODES

ICD9

279.01 Selective IgA immunodeficiency 

ICD10

D80.2 Selective deficiency of immunoglobulin A [IgA] 

SNOMED

29260007 Immunoglobulin A deficiency (disorder) 

FAQ

  • Q: Should patients with IgA deficiency be monitored for the development of progressive immunodeficiency?

  • A: Patients with IgA deficiency should be monitored for progression to CVID, as this is asso...

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