Chronic granulimatous disease
Prepared by Mohammed Areefrubsamjani Occupational Therapist & Physiotherapist.
Chronic Granulomatous Disease (CGD), is a condition in which white blood cells are unable to protect the body from harmful microbes. This can cause serious infections in the internal organs of the body.
CGD is a genetic disorder that affects the body's immune system.
In people with this condition, phagocytes, which are white blood cell types such as neutrophils and macrophages - are unable to attack and destroy certain germs. Bacteria associated with these germs can be life threatening.
In people with CGD, infections often occur in the skin, lungs, lymph nodes and liver. CGD may increase the risk of developing abscesses in the internal organs. Some people with CGD develop inflammation in various parts of the body.
CGD is rare. Doctors diagnose it from about 1 in 200,000 to 250,000 people worldwide. CGD occurs more often in men than in women.
Who is at risk for developing CGD?
People with a family member with CGD are at greater risk of contracting the disease. In many cases, inheritance. Rarely do genetic mutations cause it.
Symptoms: -
The signs and symptoms of CGD usually appear in childhood and are rarely found in adults. Can include:
Recurrent infections caused by bacteria and fungi
Abscesses in the liver, lungs, skin or spleen
Granulomas (large numbers of cells that grow in areas of inflammation or infection)
Chronic abdominal pain associated with diarrhea, nausea and vomiting, constipation
Abnormal liver function tests
Inflammation of the lymph nodes.
CGD Problems:
X-linked CGD: This most common form of the disease involves a genetic mutation in the CYBB. It almost always affects men.
Autosomal recessive CGD: Genetic modification in CYBA, NCF1, NCF2, CYBC1, or NCF4 causes this type of CGD.
Diagnosis: -
Physical examination: During a physical examination, doctors can diagnose inflammation and granulomas.
Blood tests: Doctors use blood tests, which include a special test called DHR or nitroblue tetrazolium, which adds blood samples to measure the ability of white blood cells to make active forms of oxygen.
Genetic testing: The doctor reads a sample of blood or tissue to identify the defective gene that causes CGD.
Treatment: -
Doctors use several different drugs to treat the symptoms of CGD:
Antibiotics: Antibiotics are used for both prevention (prevention) and treatment of serious illnesses.
Antifungals: Antifungal drugs such as itraconazole can be used for both prevention and treatment of serious illnesses.
Corticosteroids: These may be used to treat the manifestations of inflammation, but they are used with caution, as broader immunosuppressants may lead to infections.
Interferon-gamma injections: A synthetic (man-made) version of an antibody product may reduce the severity and frequency of infections. (This treatment may be less effective because of your cost, side effects and efficiency compared to prophylaxis itraconazole.)