Pediatric Chronic Granulomatous Disease Medication

  • Author: Lawrence C Wolfe, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Apr 16, 2010
 

Antimicrobials

Class Summary

These agents are used prophylactically to prevent infections in susceptible individuals or to treat active infections.

Trimethoprim-sulfamethoxazole (TMP-SMZ, Cotrimoxazole, Septra, Bactrim)

 

Antimicrobial drug of choice administered prophylactically to prevent infections in patients with CGD.

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Biologic response modifiers

Class Summary

These agents regulate the immune system by various mechanisms, including enhancing activity of macrophages and cytotoxic actions of T lymphocytes.

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Interferon gamma 1b (Actimmune)

 

Indicated in CGD to reduce frequency and severity of bacterial infections (50 mcg = 1 million IU).

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Antifungal agents

Class Summary

The mechanism of action may involve increasing the permeability of the cell membrane, which, in turn, causes intracellular components to leak.

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Itraconazole (Sporanox)

 

Continuous antifungal therapy effective in preventing infection due to Aspergillus species. Synthetic triazole antifungal agent that slows fungal cell growth by inhibiting CYP–dependent synthesis of ergosterol, vital component of fungal cell membranes.

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Voriconazole (VFEND)

 

Used for primary treatment of invasive aspergillosis and salvage treatment of Fusarium species or Scedosporium apiospermum infections. A triazole antifungal agent that inhibits fungal CYP450-mediated 14 alpha-lanosterol demethylation, which is essential in fungal ergosterol biosynthesis.

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Posaconazole (Noxafil)

 

Triazole antifungal agent. Blocks ergosterol synthesis by inhibiting the enzyme lanosterol 14-alpha-demethylase and sterol precursor accumulation. This action results in cell membrane disruption. Available as oral susp (200 mg/5 mL). Indicated for prophylaxis of invasive Aspergillus and Candida infections in patients at high risk because of severe immunosuppression.

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Immunomodulators

Class Summary

These agents suppress an overactive immune system that leads to formation of granulomas.

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Prednisone (Deltasone, Orasone)

 

Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear (PMN) activity.

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Contributor Information and Disclosures
Author

Lawrence C Wolfe, MD  Senior Associate in Pediatric Hematology/Oncology, Schneider Children's Hospital

Lawrence C Wolfe, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association of Blood Banks, American Society of Hematology, Children's Oncology Group, and Eastern Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Sharada A Sarnaik, MBBS  Professor of Pediatrics, Wayne State University School of Medicine; Director, Sickle Cell Center, Attending Hematologist/Oncologist, Children's Hospital of Michigan

Sharada A Sarnaik, MBBS is a member of the following medical societies: American Association of Blood Banks, American Association of University Professors, American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

James L Harper, MD  Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

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Scanning electron micrograph of Aspergillus species.
 
 
 
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