Chronic Granulomatous Disease (CGD)

by Brian Alverson, MD

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    00:01 In this lecture, I'm going to briefly review chronic granulomatous disease.

    00:07 So, chronic granulomatous disease is a defect in NADPH oxidase.

    00:13 What this means is patients have an inability to manufacture a superoxide free radical in their neutrophils and in their macrophages.

    00:23 And as we know, this is how they break things down.

    00:27 So they have a reduced ability to kill bacteria through encapsulation.

    00:32 As a result, these patients form granulomas.

    00:37 In many organs, that can cause problems and they have an immunodeficiency.

    00:42 So these patients have a susceptibility to catalase positive organisms which can be remembered by the mnemonic BEANS.

    00:51 These include Burkholderia species, such as Borkholderia cepacia, Enterobacteriacea, Aspergillus, Nocardia, and Staph aureus.

    01:03 These are the ones that are a problem, and in particular, it's Staph aureaus that can rear its ugly head quite a bit.

    01:10 So, these children will present typically before 2 years of age.

    01:15 And they often will develop a pneumonia, such a staph pneumonia.

    01:20 Remember, staph pneumonia looks a little bit different than classical pneumonia in that the children are much sicker and they can have a parapneumonic effusion or an abscess in their lung.

    01:31 In fact, they can develop abscesses thoughout their body and in many organs.

    01:37 These abscesses need to be drained and it can be very care-challenging.

    01:42 They frequently develop cellulitis and it's usually Staph aureus.

    01:47 Likewise, they can develop a septic athritis or an osteomyelitis.

    01:52 Anywhere where staph can get, these kids will get it.

    01:56 They often have bacteremia and they may occasionally have invasive fungal infections that can be challenging to treat.

    02:04 So, how do we test for chronic granulomatous disease in children? The best test is the dihydrorhodamine test or the DHR.

    02:14 So send some blood off for a DHR.

    02:17 If phagocytes fluoresce, they are normal.

    02:21 If they don't, we suspect chronic granulomatous disease.

    02:24 This test has replaced the older test which was the nitroblue-tetrazolium.

    02:30 That's less commonly in use now.

    02:34 How do we treat chronic granulomatous disease? We typically treat it with interferon gamma and we'll give them that during infections and as prophylaxis.

    02:45 It's not cheap, but it's reasonably effective.

    02:48 These patients generally require prophylaxis with trimethoprim/sulfamethoxazol for specifically Burkholderia.

    02:56 And we'll give them antifungals as needed for their fungal infections, either topical or systemic.

    03:03 So that's my brief summary of chronic granulomatous disease.

    03:07 Thanks.

    About the Lecture

    The lecture Chronic Granulomatous Disease (CGD) by Brian Alverson, MD is from the course Pediatric Allergy and Immunology. It contains the following chapters:

    • Chronic Granulomatous Disease
    • DHR Test and Interferon

    Included Quiz Questions

    1. It is a defect in the ability to make peroxide in the lysosome.
    2. These patients have abnormal immunoglobulins.
    3. These patients are at risk for tuberculosis, hence the risk for granulomas.
    4. These patients are at increased risk of infection by catalase negative organisms.
    5. Typically patients present with urinary tract infections.
    1. S. pneumoniae.
    2. Borkholderiacepacia.
    3. Enterobacteriacea.
    4. Aspergillus.
    5. Staph aureus.

    Author of lecture Chronic Granulomatous Disease (CGD)

     Brian Alverson, MD

    Brian Alverson, MD

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    Great lecture!
    By Rosangela S. on 28. September 2017 for Chronic Granulomatous Disease (CGD)

    this lecture was great, the Doctor has an amazing teaching skill!