[CIS-PAGID] liver abscess and aortitis?

[dmvascon at usp.br]

Dear Yae Jean, good evening

The possibility of CMCC seems distant to me too, as usually CMCC  
patients present persistent and relapsing mucocutaneous Candida  
infections after 12 months of age (I only reminded about some cases  
with arteritis).

OK, the repetition of immunological tests is sometimes fundamental for  
a correct diagnosis, such as low levels of hemolytic complement in an  
acute infection that normalizes after remission of the infectious  
episode, for example.

The possibility of a normal child affected by a severe infection in  
the context of the physiological immunodeficiency of infancy cannot be  
ruled out, certainly.

About the possibility of BCGosis, it does not occur in all cases of  
CGD and even in MSMD due to IL-12/23/IFN-gamma axis defects. Only time  
will answer to this question. The clinical features do not resemble  
those presented by SCID patients. Moreover only occasional NEMO  
patients present BCGosis. MyD88 and IRAK-4 patients are not prone to  
BCGitis.

We use PMA (phorbol myristil acetate) to stimulate blood cells in the  
DHR test, which is a very aggressive stimulation (on our hands at  
least 80% of the neutrophils are stimulated and with a MFI of at least  
200). fMLP is very interesting to stimulate for chemotaxis assays.

All the best,

Dewton Vasconcelos
University of São Paulo Medical School

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



Dear All,

Thanks a lot for the responses so far, everybody.

The pt does not have any episodes of candida infection so I guess the
possibility for CMCC seems low.

I talked to my lab medcine colleague and we plan to repeat the DHR
test again. The baby looks perfectly well and looks like a normal
healthy child unless you hear about the US/MRI report...(good weight
gain, no abnormal face or other physical morphology...).

Echo was done twice (normal). Our cardiology guys are nation-number
ones so I trust their echo expertise..We talked to pediatric radiology
people, vascular surgery people...but no surgery was ever
recommended...

By the way, I have another question. The baby already received BCG at
his first month of life since that is our part of routine childhood
immunization in Korea... However, the baby did not develop any
complications after the BCG vaccination, yet.. if in fact, he had any
of those CGD, NEMO, MYD88, IRAK 4 problems...I would bet he already
developed some mycobacterial abscess, osteo, or more serious forms or
diseeminated type infection etc... what do you think?  if AR CGD,
maybe not that severe infection?  Liver abscess biopsy was negative
for AFB, TB PCR and no caseation necrosis...

Congenital neutropenia..hmmm...I will bear the possibility in my
mind.

Anyway, I will repeat the test and this time I will check the raw
data myself.

They use PHA to stimulate the cells for DHR test in our hospital lab.
Does it matter a lot whether to use PHA or fMLP when you do DHR test?


Thanks a lot. I will give you more updates.

YaeJean

-------------------------
????: Pere Soler Palacin [psoler at vhebron.net]
????: pagid at list.clinimmsoc.org
? ?: 2010? 10? 17?(?) 01:41:20
? ?: Re: [CIS-PAGID] liver abscess and aortitis?

Dear all, I wonder if the real problem is repeated embolisms from
septic arteritis and suspicion of PID should be a second step. Has
surgical treatment been considered in this case? Has congenital heart
defects been ruled out?

Yours,

Pere.

Pere Soler Palacín MD

Pediatric Infectious Diseases and Immunodeficiencies Unit.

Vall d'Hebron Hospital, Barcelona, Spain.

----- Mensaje original -----
De: dmvascon at usp.br
Para: pagid at list.clinimmsoc.org, "???" <yaejeankim at skku.edu>
Enviados: Sábado, 16 de Octubre 2010 17:59:15
Asunto: Re: [CIS-PAGID] liver abscess and aortitis?

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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