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Bruton Agammaglobulinemia Medication

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 08, 2016
 

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and prevent complications.

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Immunoglobulins

Class Summary

Immunoglobulins are the mainstay of therapy. Passively supply a broad spectrum of IgG antibodies against bacterial, viral, parasitic, and mycoplasmic antigens. Check IgG levels every 3 months and then every 6 months when stable. The goal is to maintain IgG trough levels greater than 500 mg/dL in serum. Check liver function and kidney function 3-4 times a year.

Immune globulin intravenous (Gamimune, Gammar-P, Sandoglobulin, Gammagard)

 

Immune globulin intravenous neutralizes circulating myelin antibodies through anti-idiotypic antibodies; down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells and augments suppressor T cells; blocks complement cascade; promotes remyelination; and may increase CSF IgG (10%).

Adjust the dose and interval according to individual needs.

Symptomatic adverse effects may be alleviated by premedicating with acetaminophen, diphenhydramine, or methylprednisolone (Solu-Medrol).

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Antibiotics

Class Summary

These agents treat common sinopulmonary infections (eg, pneumonia, otitis media). Drugs, such as amoxicillin and amoxicillin/clavulanate, are typical agents used. Fluoroquinolone therapy is useful for respiratory staphylococcal infections and for patients with allergies to other medications. If the infection is caused by Mycoplasma organisms, the drug of choice is clarithromycin. Severe infections may require hospitalization and IV therapy with ceftriaxone or vancomycin.

Amoxicillin (Amoxil, Trimox, Biomox)

 

Amoxicillin interferes with the synthesis of cell wall mucopeptides during active multiplication, resulting in bactericidal activity against susceptible bacteria.

Ceftriaxone (Rocephin)

 

Ceftriaxone is a third-generation cephalosporin with broad-spectrum gram-negative activity; it has lower efficacy against gram-positive organisms and higher efficacy against resistant organisms. Ceftriaxone arrests bacterial growth by binding to one or more penicillin-binding proteins.

Vancomycin (Vancocin, Lyphocin, Vancoled)

 

Vancomycin is a potent antibiotic directed against gram-positive organisms and active against Enterococcus species. It is useful in the treatment of septicemia and skin structure infections. Ii indicated for patients who cannot take or in whom no response has occurred with penicillins and cephalosporins or for those who have infections with resistant staphylococci. For abdominal penetrating injuries, it is combined with an agent active against enteric flora and/or anaerobes.

To avoid toxicity, the current recommendation is to assay trough levels after the third dose, drawn 0.5 hours prior to next dosing. Use creatinine clearance to adjust the dose in patients with renal impairment.

Vancomycin is used in conjunction with gentamicin for prophylaxis in patients allergic to penicillin undergoing GI or GU tract procedures.

Clarithromycin (Biaxin)

 

Clarithromycin inhibits bacterial growth, possibly by blocking the dissociation of peptidyl tRNA from ribosomes, causing RNA-dependent protein synthesis to arrest.

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Bronchodilators

Class Summary

Bronchodilators are administered via an inhaler to reduce bronchoconstriction and inflammatory response in the lungs. Inhaled beta2-agonists, with or without steroid inhalation therapy, are the standard of care for pulmonary maintenance in XLA.

Albuterol (Proventil, Ventolin)

 

Albuterol is a beta-agonist for bronchospasm refractory to epinephrine. It relaxes bronchial smooth muscle by its action on beta2-receptors, with little effect on cardiac muscle contractility.

Salmeterol (Serevent)

 

By relaxing the smooth muscles of the bronchioles in conditions associated with bronchitis, emphysema, asthma, or bronchiectasis, salmeterol can relieve bronchospasms. The effect may also facilitate expectoration. Adverse effects are more likely to occur when administered at higher or more frequent doses than recommended.

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Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Beclomethasone, inhaled (Qvar)

 

Beclomethasone inhibits bronchoconstriction mechanisms, produces direct smooth muscle relaxation, and may decrease the number and activity of inflammatory cells, in turn, decreasing airway hyperresponsiveness.

Fluticasone inhaled (Flovent)

 

Fluticasone inhibits bronchoconstriction mechanisms, produces direct smooth muscle relaxation, and may decrease the number and activity of inflammatory cells, in turn, decreasing airway hyperresponsiveness.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Franklin Desposito, MD Professor of Pediatrics and Clinical Director, Center for Human and Molecular Genetics, Rutgers New Jersey Medical School; Consulting Staff, Department of Pediatrics, UMDNJ-University Hospital

Franklin Desposito, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Medical Genetics and Genomics, American Medical Association, American Society of Human Genetics, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Rosalie Elenitsas, MD Herman Beerman Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System

Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology

Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Julie R Kenner, MD, PhD Private Practice, Kenner Dermatology Center

Julie R Kenner, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Isabel N. Granja Jander, MD, to the development and writing of this article.

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Early stages of B-cell differentiation can be identified by the status of the immunoglobulin genes and by the cell surface markers CD34, CD19, and surface immunoglobulin (sIg). From: Conley ME. Genes required for B cell development. J Clin Invest. 2003;112: 1636-8. Reproduced with permission of American Society for Clinical Investigation via Copyright Clearance Center.
 
 
 
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