[CIS-PAGID] question for CGD

Prof. Dr. Antonio Condino Neto condino at icb.usp.br
Wed Feb 16 05:53:42 EST 2011


This situation requires antibiotics (ok), steroids (ok), and surgery,
specially to clean the liver.

--
Antonio Condino-Neto
Professor of Experimental Medicine
Institute of Biomedical Sciences, University of São Paulo
1730 Lineu Prestes Avenue, São Paulo - SP. ZIP 05508-000. Brazil
Tel (55) (11) 3091-7387 / Fax (55) (11) 3091-7224



Citando ??? <yaejeankim at skku.edu>:



Dear all,

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

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 Serratia 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


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