[CIS-PAGID] question for CGD

[Richard Wasserman]

I strongly suggest granulocyte transfusions. You may need to give doses
every 12 hours. Be aware of the development of polycythemia. You should be
prepared to bleed the patient a ml for ml immediately prior to infusing the
granulocytes. You will certainly need to drain/excise the liver abscess. If
you do surgery now, the surgeon will find a phlegmon without clearly
identifiable margins. After several days of granulocytes, there is a good
chance that a capsule will begin to form that will guide the surgeon. With
luck the granulocytes and antibiotics will treat the pulmonary nodules. The
pulmonary infiltrate may require surgical intervention but I would try to
hold off. I would also carefully consider adding a second antibiotic. Good
luck.
Richard Wasserman
University of Texas Southwestern Medical School
Dallas

On Tue, Feb 15, 2011 at 7:29 PM, 김예진 <yaejeankim at skku.edu> wrote:


> Dear all,

>

>

>

> I have a question about a 4 month old boy who is in our PICU now.

>

>

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> This baby was transferred to our center for an evaluation of tumor in the

> liver (r/o hepatoblastoma) on Friday (2/12).

>

> Actually he was on the H-O ward when I saw hims Monday morning. But as soon

> as I heard that his liver abscess grew Sery erratia at previous hospital

> (they did open liver biopsy and culture) I sent out respiratory burst test

> and diagnosed CGD on Monday evening (2/14).

>

>

>

> He has huge mas like abscess in the liver, pleural effusion, multiple lung

> nodules with massive irregular pulmonary infiltration... He was stable until

> Tuesday morning (while we were discussing options for surgical intervention

> such as abscess drainage and diagnositc biopsy for additional pathogen), he

> suddenly developed tachypnea on Tuesday morning (2/15).

>

> He is now in PICU and got intubated with chest tube for pleural fluid

> drainage since last night.

>

>

>

> He has been on meropenem for Serratia from previous hospital but I started

> antifungal agent (Amphotericin, I prefer Vori but insurance will not cover

> at this point) right after I saw the chest CT.

>

> And in addition, because in Korea we give BCG and birth, I also started

> anti-Tb medication (INH+RFP+EMB) in fear that he might have disseminated BCG

> infection as well, esp his lung condition deteriorates and I saw massive

> mediastinal LNs enlargements with necrotic portions in them...

>

>

>

> Tons of tests were sent from blood, CSF, pleural fluid, tracheal aspiration

> (for bacteria, fungus, TB...).  our pulmonologist couldn't do BAL on this

> child since he is too small for BAL.

>

>

>

> At this point he is critical but somewhat stable in PICU. Is there anything

> that I can do for this baby? Should I add  cytokine?

>

> If the condition continues to be stable, should I persuade my surgeons and

> do the biopsy and drainage from the lung, mediastinum and liver?

>

>

>

> Obviously, the diagnosis was delayed from another hospital and since I saw

> this child his condition just deteriorated so fast within 24 hours.

>

> I will appreciate any advice and suggestion. Thanks a lot!!!  and wish him

> a good luck!

>

>

>

> YaeJean

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> --------------------------------------------------------------------------

>

> Yae-Jean Kim, MD

>

> Assistant Professor

>

> Division of Infectious Diseases

>

> Department of Pediatrics

>

> Sungkyunkwan University School of Medicine

>

> Samsung Medical Center

>

> 50 Irwon-dong Gangnam-gu

>

> Seoul, Korea

>

> Tel)  +82-2-3410-0987

>

> Fax) +82-2-3410-0043

>




-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211
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