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Transient Hypogammaglobulinemia of Infancy Treatment & Management

  • Author: Alan P Knutsen, MD; Chief Editor: Harumi Jyonouchi, MD  more...
 
Updated: Feb 07, 2014
 

Medical Care

Transient hypogammaglobulinemia of infancy (THI) treatment is conservative and depends on the severity of infections and the patient's response to therapy. Appropriate antibiotic treatment may be sufficient. However, given emerging evidence that THI is an intrinsic B-cell immunodeficiency, with antibody deficiencies to polysaccharide and conjugated-polysaccharide immunizations (eg, S pneumoniae), treatment with prophylactic antibiotics is reasonable.

Furthermore, in patients with THI who develop severe life-threatening infections or who develop recurrent respiratory tract infections despite antibiotic therapy, a trial of antibody replacement therapy in the form of intravenous immunoglobulin (IVIG) is indicated. Recently, Memmedova et al reported that IVIG treatment in children with THI significantly decreased infections.[24] Furthermore, IVIG therapy did not prolong resolution of THI. Investigators have recommended IVIG for 6-12 months using the usual therapeutic dose of IVIG of 400-800 mg/kg intravenously every 3-4 weeks.[3, 4] Subcutaneous forms of gammaglobulin (Hizentra, Gammagard 10%, Gamunex c) have become available as an alternative to IVIG. The usual therapeutic dose is 100-200 mg/kg subcutaneously per week.

Allergic rhinitis contributes to recurrent otitis media and sinusitis. If allergic rhinitis occurs, the child should be aggressively treated with topical nasal corticosteroids and antihistamines.

Routine immunizations are continued in children with THI. Recently, a conjugated heptavalent pneumococcal vaccine has been recommended for routine immunization in children beginning at age 2 months. Whether this immunization can significantly reduce otitis media in children with THI is unclear. The conjugated heptavalent pneumococcal vaccine covers approximately 85% of the serotype responsible for invasive pneumococcal infection in children.

In studies of healthy children, the pneumococcal vaccine significantly eliminated invasive infections but reduced the frequency of otitis media by only 20%. Sorensen et al have reported that a significant percentage of children with a selective antibody deficiency to bacterial polysaccharide antigens following immunization with the unconjugated vaccine (Pneumovax) develop protective antibody levels following immunization to the conjugated vaccine (Prevnar), with a reduction in infections.[25]

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Surgical Care

Many of these children are referred to otolaryngologists for placement of tympanostomy tubes for recurrent otitis media and functional endoscopic sinus surgery (FESS) for chronic sinusitis. Tympanostomy tubes are of uncertain benefit in the prevention of recurrent otitis media, and the potential adverse anatomic and audiologic sequelae of tube placement must be considered. Likewise, some have suggested that FESS is not the cure for chronic sinusitis but that the underlying immunodeficiency disease must be appropriately treated.

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Consultations

These children need to be referred to an allergist, immunologist, or both to evaluate for THI and to ascertain that another immunodeficiency is not present. A definitive diagnosis of THI is a retrospective diagnosis when the immunodeficiency resolves. These patients need to be evaluated over time.

Atopic diseases associated with THI need to be looked for and treated.

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Diet

No special diet is required unless a food allergy is present.

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Activity

The child should not attend a daycare center to reduce his or her increased susceptibility to infections. However, physicians need to consider each family's dynamics and economic situation when giving this recommendation.

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Contributor Information and Disclosures
Author

Alan P Knutsen, MD Professor of Pediatrics, Director of Pediatric Allergy and Immunology, Director Jeffrey Modell Diagnostic & Research Center for Primary Immuodeficiences (CGCMC), Director of Pediatric Clinical Immunology Laboratory, Department of Pathology, St Louis University Health Sciences Center

Alan P Knutsen, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, Clinical Immunology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

David J Valacer, MD 

David J Valacer, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Academy of Pediatrics, American Association for the Advancement of Science, American Thoracic Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Harumi Jyonouchi, MD Faculty, Division of Allergy/Immunology and Infectious Diseases, Department of Pediatrics, Saint Peter's University Hospital

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 Pediatric Research, Society for Mucosal Immunology

Disclosure: Nothing to disclose.

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