What type of organisms cause the recurrent infections of CGD?
People with CGD are highly susceptible to infections caused by certain bacteria and fungi, such as Staphylococcus aureus, Serratia marcescens, Burkholderia cepacia, Nocardia species, and Aspergillus species.
What does CGD stand for?
Chronic granulomatous disease (CGD) is an inherited disorder in which certain immune system cells do not function properly.
What cell is affected in CGD?
Chronic granulomatous disease is a genetic disease. In CGD, mutations in any one of five different genes can cause a defect in an enzyme called phagocyte NADPH oxidase. Certain white blood cells use this enzyme to produce hydrogen peroxide, which these cells need in order to kill certain bacteria and fungi.
What bacteria causes chronic granulomatous disease?
Among the most common organisms that cause disease in CGD patients are:
- Bacteria (particularly those that are catalase-positive) Staphylococcus aureus. Serratia marcescens.
- Fungi. Aspergillus species.
What causes granulomatous inflammation?
Granulomatous inflammation is caused by a variety of conditions including infection, autoimmune, toxic, allergic, drug, and neoplastic conditions. The tissue reaction pattern narrows the pathologic and clinical differential diagnosis and subsequent clinical management.
What is the fundamental immune defect in chronic granulomatous disease?
The main defect in chronic granulomatous disease (CGD) is a failure of neutrophils, monocytes, macrophages, and eosinophils to mount a respiratory burst and, therefore, to generate superoxide anions and other reactive oxygen species derived from superoxide, such as hydrogen peroxide.
What is CGD pathology?
CGD is an inherited disorder of phagocytic cells in which those cells are unable to kill bacteria and fungi because of a gene defect which does not allow the necessary respiratory burst. For this reason, the patient is subject to recurrent chronic infections. Most patients present during the first five years of life.
How common is CGD?
CGD is not common. Doctors diagnose it in about 1 out of every 200,000 to 250,000 people worldwide. CGD occurs more often in males than in females.
What is CGD immunology?
Chronic granulomatous disease (CGD) is an inherited primary immunodeficiency disease (PIDD) which increases the body’s susceptibility to infections caused by certain bacteria and fungi. Granulomas are masses of immune cells that form at sites of infection or inflammation.
Why is CGD susceptible to catalase positive organisms?
Catalase is an enzyme that can inactivate the hydrogen peroxide that is produced by some bacteria and fungi. It is believed that patients with CGD can use hydrogen peroxide produced by catalase-negative microbes to form reactive oxidants and, consequentially, bypass the intrinsic CGD defect.