[CIS-PAGID] liver abscess and aortitis?

[Routes, John]

Agree with prior email, this is not IgA deficiency--(at least one cannot make the diagnosis at this age when there is IgA present)-
Am curious if the abscess actually contains PMNs? In our patient with RAC2 deficiency there were few WBCs in the abscess, consistent with a defect in chemotaxis (later confirmed by in vitro assays)---to exclude this diagnosis would also check PMN oxidative burst in response to  fMLP,


John M. Routes, MD
Chief, Section of Allergy and Clinical Immunology
Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
Department of Pediatrics
Children's Hospital of Wisconsin
Medical College of Wisconsin
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874

Phone: 414-456-4802; 414-266-6997
Fax: 414-456-6487 (Clinical)
Fax: 414-456-6323 (Laboratory)
Email: jroutes at mcw.edu




________________________________
From: 김예진 <yaejeankim at skku.edu>
Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Date: Tue, 12 Oct 2010 20:22:27 -0500
To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Subject: [CIS-PAGID] liver abscess and aortitis?

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


________________________________
보낸사람: "Church, Joseph" [JChurch at chla.usc.edu]
받는사람: pagid at list.clinimmsoc.org
날 짜: 2010년 9월 29일(수) 23:00:14
제 목: 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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