The purpose of this pictorial essay is to present and summarize findings of various images of chronic granulomatous disease (CGD). CGD represents a heterogeneous group of disorders caused by defective generation of respiratory bursts in human phagocytes. This defect results in abnormal phagocytic functions and defective killing of bacteria by phagocytes. CGD may involve many organs and present ...

Mice with X-linked chronic granulomatous disease (CGD) generated by targeted disruption of the gp91phox subunit of the NADPH-oxidase complex (X-CGD mice) were examined for their response to respiratory challenge with Aspergillus fumigatus. This opportunistic fungal pathogen causes infection in CGD patients due to the deficient generation of neutrophil respiratory burst oxidants important for da...

Interferon-gamma (IFN-gamma) is recommended as prophylaxis against infections in patients with chronic granulomatous disease (CGD). However, since the optimal dose, the dosing interval, and the mechanisms of action are not well-defined, we studied the effects on CGD neutrophil (PMN) functions ex vivo of interferon-gamma (IFN-gamma). Evaluations were made on oxidative capacity, measured by super...

Chronic granulomatous disease (CGD) is characterized by inherited immune defects resulting from mutations in the NADPH oxidase complex genes. The X-linked type of CGD is caused by defects in the CYBB gene that encodes gp91-phox, a fundamental component of the NADPH oxidase complex. This mutation originates the most common and severe form of CGD, which typically has absence of NADPH oxidase func...

Phagocytic cells of patients with chronic granulomatous disease (CGD) are severely impaired in the killing process, on the basis of defective oxygen metabolism. In this study we investigated the antigen-presenting function of monocytes (i.e. adherent cells) of CGD patients. Adherent cells of CGD patients were investigated for their capacity to present ovalbumin (OA) in such a way that T helper ...

CD27(+) memory B cells are reduced in the blood of patients with chronic granulomatous disease (CGD) for reasons and consequences that remain unclear. Here we confirm not only decreased CD27(+) but also IgG(+) B cells in the blood of CGD patients compared with healthy donors (HDs). However, among IgG(+) B cells, the ratio of CD27(-) to CD27(+) was significantly higher in CGD patients compared w...

Chronic granulomatous disease (CGD) is one of the congenital immunodeficiencies, which affects intracellular biocidal activity through the impairment of superoxides production. Aspergillus species are a major cause of death in CGD patients and the mortality rate of cerebral aspergillosis in immunocompromised patients is reported to be nearly 100%1). Very few reports to date have described survi...

Chronic granulomatous disease (CGD), an often fatal syndrome of recurrent infections results from the inability of patients' peripheral blood phagocytic leukocytes to generate superoxide despite otherwise normal phagocytic functions such as ingestion and degranulation. Circulating granulocytes and monocytes are the progeny of bone marrow progenitor cells, colony-forming units in culture. We com...

OBJECTIVE To develop an application that is simple and reliable using the nitroblue tetrazolium (NBT) method that clearly differentiates chronic granulomatous disease (CGD) patients with heterozygous carriers in groups suspected with CGD. METHODS This study was carried out in Shiraz University of Medical Sciences from October 2002 and March 2004. The study included 260 samples consisting of 1...

BACKGROUND Patients with chronic granulomatous disease (CGD) experience immunodeficiency because of defects in the phagocyte NADPH oxidase and the concomitant reduction in reactive oxygen intermediates. This may result in a reduction in atherosclerotic injury. METHODS AND RESULTS We prospectively assessed the prevalence of cardiovascular risk factors, biomarkers of inflammation and neutrophil...