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| printer friendly | CHRONIC GRANULOMATOUS DISORDER | ||||||||||||||||||||
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Chronic Granulomatous Disorder (CGD) is a rare, inherited disorder of the immune system that affects 1 in 250,000 people. The basic defect lies in phagocytic cells which are made in the bone marrow (neutrophils and monocytes). These fail to effectively destroy certain invading bacteria and fungi. Affected individuals are therefore susceptible to serious bacterial and fungal infection. They also experience symptoms associated with chronic inflammation, often granulomatous in nature. Infections may occur as abscesses in lymph glands. Other sites of infection include bones and joints, the liver, the bowels, the lungs and skin. However, individuals with the condition are able to combat viral infection normally. Most CGD patients should be on daily antibacterial and antifungal prophylaxis (preventive drug therapy). CGD can be diagnosed by a simple blood test. If CGD is suspected it is important that referral is made to a specialist centre and diagnostic tests carried out in a laboratory that is familiar with doing these tests on a regular basis. Bone marrow transplantation is a curative treatment option for some patients, but requires a fully matched donor. New treatments based on gene therapy are also being developed. Early diagnosis and treatment of the symptoms with appropriate antibiotics as well as lifelong antibiotic and antifungal prophylaxis, has greatly mitigated the effects of this condition. In conjunction with Great Ormond Street Hospital, the CGD Research Trust has established a CGD Registry, the first comprehensive collection of information on CGD, which includes information about patients from the UK and Europe. Inheritance patterns Prenatal diagnosis Medical text written February 2006 by the CGD Research Trust. Approved February 2006 by Dr W Qasim, Specialist Registrar and Clinical Lecturer in Immunology, Institute of Child Health, London, UK.
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