an inherited disorder that occurs when phagocytes don't work properly. As a result, they can't protect your body from bacterial and fungal infections.

People with chronic granulomatous disease may develop infections in their lungs, skin, lymph nodes, liver, stomach and intestines, or other areas. They may also develop clusters of white blood cells in infected areas. Most people are diagnosed with CGD during childhood, but some people may not be diagnosed until adulthood.

Epidemiology

0.5 per 100,000 population

Aetiology

multiple possible defects (most frequently X-linked) → phagocytes being unable to destroy ingested microbes

Pathology

Lack of NADPH oxidase reduces ability of phagocytes to produce reactive oxygen species

Lack of oxidative burst to produce free radicals - leads to exessive granuloma formation

PC

Persistant and recurrent infections, especially: