Abstract
Chronic granulomatous disease (CGD) is a genetic immunodeficiency that commonly presents in infancy and early childhood. Currently, life expectancy is beyond the fourth decade of life if the disease is promptly diagnosed and treated. CGD is characterized by increased susceptibility to recurrent and often life-threatening infections caused by bacteria and fungi. The hallmark of the disease is the formation of inflammatory granulomas, which can cause obstruction of the hollow viscera. Therapeutic management of CGD patients relies on the early and aggressive treatment of acute infections, as well as lifelong antibiotic and antifungal prophylaxis. Interferon gamma has a role in the prevention and management of intractable infections. Current efforts for the development of curative therapies focus on allogeneic bone marrow transplantation for CGD patients with HLA-identical sibling donors, and transplantation of autologous genecorrected hematopoietic stem cells for patients who lack suitable marrow donors. Significant recent achievements in these fields provide a realistic hope for a lifelong cure of CGD.
Keywords: Chronic granulomatous disease, CGD, inflammation, primary immunodeficiency, phagocyte defect, recurrent infections
Current Pediatric Reviews
Title: Chronic Granulomatous Disease in Childhood
Volume: 2 Issue: 1
Author(s): Cecilia Barese and W. S. Goebel
Affiliation:
Keywords: Chronic granulomatous disease, CGD, inflammation, primary immunodeficiency, phagocyte defect, recurrent infections
Abstract: Chronic granulomatous disease (CGD) is a genetic immunodeficiency that commonly presents in infancy and early childhood. Currently, life expectancy is beyond the fourth decade of life if the disease is promptly diagnosed and treated. CGD is characterized by increased susceptibility to recurrent and often life-threatening infections caused by bacteria and fungi. The hallmark of the disease is the formation of inflammatory granulomas, which can cause obstruction of the hollow viscera. Therapeutic management of CGD patients relies on the early and aggressive treatment of acute infections, as well as lifelong antibiotic and antifungal prophylaxis. Interferon gamma has a role in the prevention and management of intractable infections. Current efforts for the development of curative therapies focus on allogeneic bone marrow transplantation for CGD patients with HLA-identical sibling donors, and transplantation of autologous genecorrected hematopoietic stem cells for patients who lack suitable marrow donors. Significant recent achievements in these fields provide a realistic hope for a lifelong cure of CGD.
Export Options
About this article
Cite this article as:
Barese Cecilia and Goebel S. W., Chronic Granulomatous Disease in Childhood, Current Pediatric Reviews 2006; 2 (1) . https://dx.doi.org/10.2174/157339606775518368
DOI https://dx.doi.org/10.2174/157339606775518368 |
Print ISSN 1573-3963 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-6336 |
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
Related Articles
-
Cutaneous Lupus Erythematosus in Children
Current Pediatric Reviews Acute Myocardial Infarction in Systemic Mastocytosis: Case Report With Literature Review on the Role of Inflammatory Process in Acute Coronary Syndrome
Current Cardiology Reviews Medical Complications in Anorexia and Bulimia Nervosa
Endocrine, Metabolic & Immune Disorders - Drug Targets Aortic Dissection: A Review of the Pathophysiology, Management and Prospective Advances
Current Cardiology Reviews Targeted Therapies for Advanced Non-Small Cell Lung Cancer
Combinatorial Chemistry & High Throughput Screening Post Cardiovascular Surgery Atrial Fibrillation. Biomarkers Determining Prognosis
Current Medicinal Chemistry Cardiovascular Magnetic Resonance for Evaluation of Heart Involvement in ANCA-Associated Vasculitis. A Luxury or a Valuable Diagnostic Tool?
Inflammation & Allergy - Drug Targets (Discontinued) Extrapulmonary Tuberculosis- Its Management and Control
Current Drug Therapy Cyclopentenyl Cytosine (CPEC): An Overview of its in vitro and in vivo Activity
Current Cancer Drug Targets Targeted Inhibition of Angiogenic Factors in AIDS-related Disorders
Current Drug Targets - Infectious Disorders Evaluation of Cardiac Scan in Diagnosing Coronary-artery Disease
Current Medical Imaging Can Chest Computed Tomography Findings of Symptomatic COVID-19 Patients Upon Admission Indicate Disease Prognosis and Clinical Outcome?
Current Medical Imaging Thyroid Hormones and Cardiovascular System: From Bench to Bedside
Current Drug Therapy Malignant Pleural Mesothelioma in 2011. Is there a Gold Standard Therapy?
Current Respiratory Medicine Reviews Interstitial Lung Disease in Systemic Lupus Erythematosus, Sjogren’s Syndrome, and Mixed Connective Tissue Disease
Current Respiratory Medicine Reviews Refractory Cough as a Remote Manifestation of Retroperitoneal Liposarcoma
Current Cancer Therapy Reviews Pulmonary Arterial Hypertension in HIV-infected Patients
Current Hypertension Reviews Pulmonary Arterial Hypertension in Connective Tissue Diseases
Current Respiratory Medicine Reviews Impact of Immunosuppressive Drugs on the Development of Cardiac Allograft Vasculopathy
Current Vascular Pharmacology Myopericarditis, as the First Sign of Rheumatoid Arthritis Relapse, Evaluated by Cardiac Magnetic Resonance
Inflammation & Allergy - Drug Targets (Discontinued)