[CIS-PAGID] liver abscess and aortitis?

[dmvascon at usp.br]

Dear Yae Jean,

Your patient seems to me most probably a phagocyte defect (or maybe an  
innate immunity defect, such as NEMO, or less probably MyD88 or  
IRAK4). I agree with Jack, sometimes AR CGD diagnosis is missed if the  
flow cytometry histograms are evaluated by someone without experience  
in PID flows). It is interesting to observe the absolute neutrophil  
counts despite leukocytosis - you reported that it was predominantly  
lymphocytes, that are normal at that age), but it is important to rule  
out primary neutropenias, which are commonly associated to abscesses  
and septic states. I have also seen aortic and cerebral aneurysms with  
CMCC and I wonder about the possibility that neutropenic or innate  
immunity defects might be associated with infectious arteritis.
IgA at this age is very low or indetectable. It is not possible to  
diagnose IgA deficiency before 5 years of age, as adult levels of IgA  
are attained only in the pre-adolescence.
Low IgG is also typical of this age, as well as low hemolytic  
complement (physiological). Maybe he could benefit of IVIg?
I think that a prolonged course of antibiotics for the treatment of a  
supposed infectious arteritis and the liver abscesses might be prudent.

All the best,

Dewton de Moraes Vasconcelos, MD, PhD
University of São Paulo School of Medicine


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



Dear All,

Thanks a lot for my previous question regarding IVIG in
agammaglobulinemia pt. He is receiving high dose intravenous Ig and
still hanging in there.

I now have another question about this 4 month old boy who has been
treate with antibiotics for more than 2 months...

Sorry but the story is a little long..

----------------------------

. previously healthy, no obvious family hx (except his dad had IgA
nephropathy) or pre/perinatal hx

. at 2 mo of age, developed fever and diagnosed with UTI d/t
Enterobacter

. started antibiotics and abdominal US revealed multiple liver
abscesses and aortitis (infiltrative thickening around abdominal
aorta)

. referred to me (at that time, no fever and stable condition), I
started meropenem..no fever but persistent leukocytosis (lympho
dominant) over 20x10^3

. continued Abt for 3 more weeks, little changes for liver abscesses
and aortitis (checked multiple times by US, and liver MRI)

. suddenly liver enzyme increased, no fever still -> discontinued
antibiotics in fear of Abt side effect and I started to suspect other
possibilities such as liver mass (at that time slightly high alpha
fetoprotein) in addition to abscess..

. at this point, we did liver biopsy -> negative culture and negative
bacterial PCR, pathlogy told typical for abscess

. by the time after bx and pathology report came out, it was around 2
week off from antibiotics, he developed fever again and leukocytosis

. I started meropenem again (normla liver ez at this time), continued
mero for 4 wks until yesterday..

A f/u liver MRI showed that there is no abscess left...

I plan to discontinue meropenem...but what shall I do about this
aortitis? I am not sure whether this was incidental finding or in fact
this child had infectious, septic phlebitis of the aorta..

Anyway, he has low IgA and I am going to f/u this IgA level.

DHR: normal

IgG (Immunoglobulin G)                   ?/?     252

IgA (Immunoglobulin A)                   ?/?     5

IgM (Immunoglobulin M)                   ?/?     58

IgE (Immunoglobulin E)                   IU/?     3.3

C3 231, C4 26, CH50 53

FANA (-), ANCA (-), VDRL (-), HIV (-), Toxoplasma (-)

Echo: normal

----------------------------------------------------------------

So, my questions are

Anybody has advice or experience with Ig A def and liver abscess and
aortitis?

One time IgA less than 5 is suffieicent to diagnose IgA deficiency?

Additional tests to do? What is this aortitis? any immune deficiency
with aortitis?

Thanks a lot in advance.

YaeJean

--------------------------------------------------------------------------


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

-------------------------
????: "Church, Joseph" [JChurch at chla.usc.edu]
????: pagid at list.clinimmsoc.org
? ?: 2010? 9? 29?(?) 23:00:14
? ?: Re: [PAGID] intraventricular IVIG

The only medication that had been proposed for enteroviral infection
was pleconaril, but I don?t know if it is available anywhere.  We
have not used intraventricular IVIG.

Joe Church

Childrens Hospital Los Angeles

-------------------------

FROM: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] ON BEHALF OF ???
SENT: Tuesday, September 28, 2010 5:50 PM
TO: pagid at list.clinimmsoc.org
SUBJECT: [PAGID] intraventricular IVIG

Dear all,

I have a 20 year-old agammagloulinemia pt who came down with chronic
enterovirus meningoencephalitis.

The pt has been on multiple antibiotics, dexamethasone, and Tb
medication already several months...in other hospital.

Anybody has an advice on intraventricular IVIG?  how about antiviral
agent? Thanks in advance

YaeJean

--------------------------------------------------------------------------


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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