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Chronic Granulomatous Disease

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.

People with CGD are unable to fight off common germs and get very sick from infections that would be mild in healthy people. This is because the presence of CGD makes it difficult for cells called neutrophils to produce hydrogen peroxide. The immune system requires hydrogen peroxide to fight specific kinds of bacteria and fungi.

These severe infections can include skin or bone infections and abscesses in internal organs (such as the lungs, liver or brain).

Aside from the defective neutrophil function in CGD, the rest of the immune system is normal. People with CGD can be generally healthy until they become infected with one of these germs. The severity of this infection can lead to prolonged hospitalizations for treatment.

Children with CGD are often healthy at birth, but develop severe infections in infancy or early childhood. The most common form of CGD is genetically inherited in an X-linked manner, meaning it only affects boys. There are also autosomal recessive forms of CGD that affect both sexes.

Therapeutic options for CGD include prophylactic antibiotics and antifungal medications, interferon-gamma injections, and aggressive management of acute infections. Bone marrow transplantation can cure CGD, however this therapy is complex and transplant candidates and donors must be carefully selected, weighing the risks and benefits carefully. Researchers are investigating other approaches including gene therapy as a future option. 

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