Key points throughout the day:
- Listen carefully to CGD patients
- Avoid making assumptions
- Identify the underlying cause behind symptoms
- Give immediate attention and swift treatment to patients with CGD
Inflammation in CGD
Professor David Denning, Professor of Medicine and Medical Mycology at the University of Manchester
- When diagnosis is difficult, remember SPUR is indicative of an immunedeficiency:-
- Always find the underlying reason for a problem; granulomas in CGD can be mistaken for other things; granuloma histology holds the key
- Signs of CGD can be picked up from family history
- Common symptoms include:- lymphangitis, usually in the neck, diarrhea; peri-anal abscess/fissure; liver abscess (these are usually rare in 'normal' children and so CGD should be considered); aspergillosis, which if diagnosed late is often fatal; recurrent respiratory problems; granular colitis; sepsis; staph. infection; necrotic skin lesion; failure to thrive.
- In CGD there is need to work closely with surgeons and microbiologists.
- It is imperative to diagnose aspergillus as fast as possible in the first 11 days in patients affected by CGD,
- The incidence of Asp. nidulans infection in CGD is proportionately higher in frequency than Asp. fumigatus
- Asp. nidulans in CGD is amphoteracin B resistant, another very important reason for recognising both Aspergillus and which species it is. Correct treatment is vital.
- High levels of aspergillus are found in pillows, whatever the filling and pillows should be changed frequently. No one knows how frequently, but perhaps six or twelve monthly.
- Voriconazole has better efficacy than amphotericin for invasive aspergillosis. In Manchester, voriconazole is used with caspofungin in children until the voriconazole blood level is checked and found to be adequate. Other measures are itraconazole (as a preventive) and posaconazole for salvage treatment. Fluconazole is ineffective against Aspergillus.
- Small children metabolise voriconazole fast but adults can suffer from toxicity. The correct level in the blood can be checked.
- Never assume what a problem is. Always test. Swift, correct treatment is important.
- Gut problems are more common in X-linked CGD. The symptoms: abdominal pain, nausea, vomiting, diarrhea, blood and constipation can be mistaken for Crohns. Management is difficult.
S - evere
P - ersistent
U - nusual
R - ecurrent
IMPORTANT NOTE :
The information contained on this website is intended only as a guideline, not as a substitute for medical advice. Always consult your doctor if you or your child has any CGD symptoms or concerns.
© 2001-2007 The Chronic Granulomatous Disorder (CGD) Research Trust
Registered Charity No. 1003425 email:cgd@cgdrt.co.uk
The CGD Research Trust is a member of the Association of Medical Research Charities (AMRC), the Genetic Interest Group (GiG) and an associate member of the International Patient Organisation of Primary Immunodeficiencies (IPOPI)
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