Chronic Granulomatous Disease

A recessive X-linked defect of leukocyte function in which phagocytic cells ingest but fail to digest bacteria, resulting in recurring bacterial infections with granuloma formation.

Also Known As:

Networked: 1408 relevant articles (32 outcomes, 89 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Infection
2.	Aspergillosis
3.	X-Linked Combined Immunodeficiency Diseases (X Linked Severe Combined Immunodeficiency)
4.	Wiskott-Aldrich Syndrome (Syndrome, Wiskott-Aldrich)
5.	Acquired Immunodeficiency Syndrome (AIDS)

Experts

1.	Malech, Harry L: 35 articles (04/2015 - 03/2002)
2.	Holland, Steven M: 34 articles (11/2015 - 03/2002)
3.	Roos, Dirk: 26 articles (12/2015 - 03/2002)
4.	Dinauer, Mary C: 18 articles (01/2014 - 03/2002)
5.	Gallin, John I: 17 articles (11/2015 - 03/2002)
6.	Segal, Brahm H: 16 articles (05/2014 - 03/2002)
7.	de Boer, Martin: 15 articles (12/2015 - 03/2002)
8.	Roesler, Joachim: 13 articles (12/2013 - 12/2002)
9.	Zarember, Kol A: 10 articles (11/2015 - 05/2007)
10.	Marciano, Beatriz E: 10 articles (11/2015 - 09/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Chronic Granulomatous Disease:

1.	Interferon-gamma (Interferon, gamma)IBA 12/01/1996 - "During the international placebo-controlled trial on the efficacy of interferon-gamma (IFN-gamma) in chronic granulomatous disease (CGD), 19 patients entered the study via our Institute. " 04/01/1995 - "This was an uncontrolled, open-label follow up study of a previous 12-month, randomized, double-blind, placebo-controlled trial performed to assess the long-term efficacy and safety of Recombinant Human Interferon Gamma (rIFN-gamma) in patients with chronic granulomatous disease (CGD). " 07/21/1988 - "This study demonstrates partial correction of the cellular defects in chronic granulomatous disease by interferon gamma and provides a basis for clinical trials of the agent." 11/01/2008 - "The present article is an informative review of recent patents related to the use of interferon gamma therapy in chronic granulomatous disease." 11/01/2008 - "The use of interferon-gamma therapy in chronic granulomatous disease."
2.	Adrenal Cortex Hormones (Corticosteroids)IBA 09/01/2007 - "Important role of corticosteroids in chronic granulomatous disease." 09/01/1987 - "Corticosteroids for chronic granulomatous disease." 09/01/1987 - "Two patients with chronic granulomatous disease had obstructive lesions of the gastrointestinal tract, esophagus, and genitourinary tract, which were successfully treated with corticosteroids. " 09/01/1987 - "Corticosteroids in treatment of obstructive lesions of chronic granulomatous disease." 09/01/2011 - "We present 2 chronic granulomatous disease patients with severe Nocardia pneumonia whose pulmonary status worsened despite appropriate antimicrobials, but then improved clinically and radiographically with the addition of corticosteroids."
3.	Superoxides (Superoxide)IBA 03/01/1982 - "Differences in the ability of human peripheral blood monocytes and in vitro monocyte-derived macrophages to produce superoxide anion: studies with cells from normals and patients with chronic granulomatous disease." 06/01/2015 - "Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by failure of superoxide production in phagocytic cells. " 06/01/2011 - "This method will be valuable for prompt diagnosis of neonatal chronic granulomatous diseases in which neutrophils aberrantly produce superoxide." 06/01/2011 - "A highly sensitive chemiluminescence assay for superoxide detection and chronic granulomatous disease diagnosis." 08/15/2008 - "Superoxide release and CYBB gene expression by EDA-ID cells were significantly decreased compared with healthy cells and similar to cells from patients with X-linked chronic granulomatous disease (X91(0) CGD). "
4.	Itraconazole (Sporanox)FDA LinkGeneric 12/01/1994 - "These results require further evaluation through a comparative randomized trial to assess the possible benefit of itraconazole prophylaxis in patients with chronic granulomatous disease." 05/01/2004 - "We have investigated the effects of itraconazole (0.1-10 micro m), an antimycotic which is often used prophylactically in primary and secondary immunodeficiency disorders, including chronic granulomatous disease, on mobilization of Ca(2+) and restoration of Ca(2+) homeostasis following activation of neutrophils with FMLP or PAF. " 11/01/1998 - "We describe a case of cutaneous purulent aspergillosis in a 19-year-old man with chronic granulomatous disease (CGD) treated successfully with a 6-month regimen of itraconazole. " 06/12/2003 - "Itraconazole prophylaxis appears to be an effective and well-tolerated treatment that reduces the frequency of fungal infections in chronic granulomatous disease, but monitoring for long-term toxic effects is warranted." 06/12/2003 - "We assessed the efficacy of itraconazole as prophylaxis against serious fungal infections in chronic granulomatous disease. "
5.	Trimethoprim (Proloprim)FDA LinkGeneric 10/01/1996 - "We present a 5-year-old boy with a severe form of X-linked chronic granulomatous disease and hypersensitivity to sulphamides preventing prophylaxis with trimethoprim-sulphomethoxazole. " 07/01/1983 - "We conclude that prophylactic treatment with sulfamethoxazole-trimethoprim is beneficial in patients with chronic granulomatous disease." 10/01/1983 - "Clindamycin and trimethoprim penetrated into normal and chronic granulomatous disease (CGD) PMN equally and were at least transiently concentrated in the cells. " 07/01/1983 - "In a retrospective study, the effect of long-term treatment with sulfamethoxazole-trimethoprim was evaluated in nine male patients with chronic granulomatous disease. " 07/01/1983 - "Continuous therapy with sulfamethoxazole-trimethoprim in patients with chronic granulomatous disease."
6.	SulfamethoxazoleFDA LinkGeneric 07/01/1983 - "We conclude that prophylactic treatment with sulfamethoxazole-trimethoprim is beneficial in patients with chronic granulomatous disease." 07/01/1983 - "In a retrospective study, the effect of long-term treatment with sulfamethoxazole-trimethoprim was evaluated in nine male patients with chronic granulomatous disease. " 07/01/1983 - "Continuous therapy with sulfamethoxazole-trimethoprim in patients with chronic granulomatous disease." 12/01/1981 - "Chronic granulomatous disease: mode of action of sulfamethoxazole/trimethoprim." 01/01/1981 - "Chronic granulomatous disease: effect of sulfamethoxazole/trimethoprim on neutrophil microbicidal function."
7.	NADPH Oxidase (NAD(P)H oxidase)IBA 05/01/2002 - "We hypothesize that clearance of stimulated neutrophils would be delayed in chronic granulomatous disease (CGD) neutrophils, which lack a functional NADPH oxidase. " 05/01/2007 - "[The X+ chronic granulomatous disease as a fabulous model to study the NADPH oxidase complex activation]." 03/01/2002 - "Using an established murine model of X-linked chronic granulomatous disease (X-CGD) created by homologous recombinant disruption of the gene encoding the gp91phox component of the NADPH oxidase, in this study we examined cutaneous reactivity to sterile Aspergillus fumigatus hyphae. " 02/16/1997 - "The aim of this study was to give a short description of the structure and function of the NADPH oxidase enzyme and to summarize the results obtained during the diagnostic of 10 patients with chronic granulomatous disease. " 03/01/1996 - "The study of patients with chronic granulomatous disease (CGD), an inherited disorder characterized by deficient of absent respiratory burst activity, has contributed greatly to our understanding of the NADPH-oxidase. "
8.	Adenosine DeaminaseIBA 07/01/2008 - "Both clinical successes and severe adverse events continue to be reported in trials of gammaretroviral gene therapy for severe combined immune deficiency-X1, adenosine deaminase-deficient forms of severe combined immune deficiency and chronic granulomatous disease. " 01/01/2009 - "In the past decade, substantial progress has been achieved in the treatment of severe combined immundeficiencies (SCID)-X1, adenosine deaminase (ADA)-deficient SCID, and chronic granulomatous disease (CGD). " 04/01/2008 - "Results from clinical trials for X-linked severe combined immunodeficiency (SCID-X1), adenosine deaminase deficient SCID (ADA-SCID), and X-linked chronic granulomatous disease (X-CGD) are discussed. " 06/01/2009 - "Previous clinical trials based on the genetic correction of purified CD34(+) cells with gamma-retroviral vectors have demonstrated clinical efficacy in different monogenic diseases, including X-linked severe combined immunodeficiency, adenosine deaminase deficient severe combined immunodeficiency and chronic granulomatous disease. " 06/01/2014 - "This review details gene therapy trials for X-linked and adenosine deaminase (ADA)-deficient severe combined immunodeficiency (SCID), Wiskott-Aldrich syndrome (WAS) and chronic granulomatous disease (CGD). "
9.	Nitroblue TetrazoliumIBA 06/01/1986 - "Heterogeneity in chronic granulomatous disease detected with an improved nitroblue tetrazolium slide test." 11/30/1993 - "Study of chronic granulomatous disease by a nitroblue tetrazolium densitometric kinetic test: a new research method." 05/30/1992 - "Immunologic studies initiated after this time showed negative tetrazolium nitroblue test, the results of which led to the diagnosis of chronic granulomatous disease. " 01/01/1976 - "Nitroblue tetrazolium reduction by human macrophages: studies in chronic granulomatous disease." 06/01/2009 - "Subsequently, he was diagnosed with X-linked chronic granulomatous disease (CGD) by the nitroblue tetrazolium assay when he was 4 years old. "
10.	Peroxidase (Myeloperoxidase)IBA 10/01/1997 - "In this study, normal, myeloperoxidase-deficient, and respiratory burst-deficient (chronic granulomatous disease [CGD]) neutrophils killed Escherichia coli with equivalent, high efficiencies. " 01/01/2014 - "These include the leukocyte adhesion deficiencies, actin defects, and other disorders of chemotaxis, hyperimmunoglobulin E syndrome, Chédiak-Higashi syndrome, neutrophil-specific granule deficiency, chronic granulomatous disease, and myeloperoxidase deficiency. " 01/01/2012 - "Neutrophils with reduced ROS production, such as those from patients with chronic granulomatous disease or myeloperoxidase (MPO) deficiency, produce fewer NETs in response to inflammatory stimuli. " 01/01/2007 - "These include leukocyte adhesion deficiencies, actin defects, and other disorders of chemotaxis; hyperimmunoglobulin E syndrome; Chédiak-Higashi syndrome; neutrophil specific granule deficiency; chronic granulomatous disease; and myeloperoxidase deficiency. " 11/15/2002 - "Therefore, healthy, chronic granulomatous disease (CGD) or myeloperoxidase (MPO)-deficient human neutrophils were examined for their ability to consume NO and attenuate its signaling. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 09/01/2012 - "Disseminated disease mainly occurs in immunocompromised patients with underlying illnesses such as chronic granulomatous disease, human immunodeficiency virus (HIV) infection, in patients undergoing cytotoxic chemotherapy, organ transplantation or prolonged glucocorticoid treatment. " 07/01/1991 - "Disseminated infection with Pseudallescheria boydii in a patient with chronic granulomatous disease: response to gamma-interferon plus antifungal chemotherapy." 11/01/2000 - "Cytokines are increasingly used for the therapy of infections in patient populations with special defects in immunity (chemotherapy, bone marrow transplantation, chronic granulomatous disease). " 06/01/2009 - "Granulocyte transfusion is a useful adjunct to antimicrobials and growth factors in post chemotherapy neutropenic sepsis and is highly effective in children with chronic granulomatous disease and life threatening infections." 07/01/1986 - "Because of their central role in host defense (in inflammatory responses, in antigen presentation, and in immunoregulatory networks), monocyte/macrophage dysfunction may result in one or more pathophysiologic consequences: defects in monocyte maturation, deficiencies in the clearance of physiologic substrates in lysosomal diseases (e.g., Gaucher's disease, mucopolysaccharidoses, osteopetrosis, metachromatic leukodystrophy), decreased synthesis and secretion of mediators (complement component deficiencies), defects in microbicidal activity (chronic granulomatous disease) and defects which are acquired following infection and during chemotherapy (e.g., acquired immune deficiency syndrome)."
2.	Immunotherapy 01/01/2015 - "In this study, three kinds of failure models are considered, and our methods illustrated with an analysis of imperfect information from a trial of immunotherapy in the treatment of chronic granulomatous disease. " 04/01/2008 - "We demonstrate the method using data from a trial of immunotherapy for chronic granulomatous disease." 01/01/2004 - "We demonstrate the methods using data from a trial of immunotherapy of chronic granulomatous disease." 07/30/2001 - "We illustrate our method with an analysis of data from a trial of immunotherapy in the treatment of chronic granulomatous disease." 10/01/2013 - "Robust T cell responses to aspergillosis in chronic granulomatous disease: implications for immunotherapy."
3.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 12/01/2009 - "Successful bone marrow transplantation in chronic granulomatous disease." 09/01/2008 - "First successful bone marrow transplantation for X-linked chronic granulomatous disease by using preimplantation female gender typing and HLA matching." 07/01/2006 - "Successful bone marrow transplantation in an 8-month-old patient with chronic granulomatous disease." 01/01/2003 - "Long-term follow-up and prognosis of chronic granulomatous disease in Yugoslavia: is there a role for early bone marrow transplantation?" 12/15/2002 - "Treatment of chronic granulomatous disease (CGD) with myeloablative bone marrow transplantation is considered risky. "
4.	Transplants (Transplant) 10/01/2014 - "Our case was a relatively old male patient compared with other patients having the diagnosis of chronic granulomatous disease who underwent hematopoietic stem cell transplant. " 10/01/2014 - "We describe the successful hematopoietic stem cell transplant of a 22-year-old patient with chronic granulomatous disease at our center. " 10/01/2014 - "Allogeneic hematopoietic stem cell transplant for high-risk adult patients with chronic granulomatous disease: first case report from Iran." 08/01/2013 - "Chronic granulomatous disease (CGD) is a primary immunodeficiency managed conservatively or with hematopoietic stem cell transplant. " 08/01/2013 - "Cognitive ability in children with chronic granulomatous disease: a comparison of those managed conservatively with those who have undergone hematopoietic stem cell transplant."
5.	Hematopoietic Stem Cell Transplantation 01/01/2012 - "Matched related donor (MRD) hematopoietic stem cell transplantation (HSCT) is a successful treatment for chronic granulomatous disease (CGD), but the safety and efficacy of HSCT from unrelated donors is less certain. " 01/01/2015 - "We update and summarize the recent findings in conventional treatment and hematopoietic stem cell transplantation in chronic granulomatous disease (CGD). " 01/01/2015 - "Chronic granulomatous disease - conventional treatment vs. hematopoietic stem cell transplantation: an update." 11/01/2013 - "Clinical outcome in children with chronic granulomatous disease managed conservatively or with hematopoietic stem cell transplantation." 07/01/2013 - "Rapid determination of chimerism status using dihydrorhodamine assay in a patient with X-linked chronic granulomatous disease following hematopoietic stem cell transplantation."