[PAGID] oral Ig
Daniel Conway
dhconway at hotmail.com
Sat Jun 27 07:03:08 EDT 2009
If one is a "believer" in enteric Ig (and I am not), consider placement of nasojejunal tube and pass this as far as one would need to bypass gastric contents. Not that protein digestion fails to occur in the small intestine, however.
Have you considered a trial of Ivig (considering higher doses)?
Sincerely,
Daniel H. Conway, MD
Asst. Professor of Pediatrics
St. Christopher's Hospital for Children
Drexel University College of Medicine
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From: yaejeankim at skku.edu
To: pagid at list.clinimmsoc.org
Date: Sat, 27 Jun 2009 19:45:56 +0900
Subject: [PAGID] oral Ig
Hi, all
I have another
question about a patients with WAS who got transplanted and has had chronic
diarrhea due to virus called “calicivirus”.
This is a pt with WAS who was
transplanted about a year ago. He developed severe severe GVH including skin
and gut. He got better…then he developed severe diarrhea which was
suspected for GVH back then and
H-O guy put him on a
significant immunosuppresion…later calicivirus was reported. He has had
calicivirus dected for the past 7 months…still has diarrhea upto 8-10
times a day. Multiple endoscopies…the
most recent one showed no evidence of GVH but more likely viral enteritis…no
CMV or EBV were seen on staining.
I requested for EM…because I was expecting to
see virus particle if there is any invasion… this is pending and takes
long time…I am not sure whether we can identify calici by EM…anyway
I though that was worth of try.
For tx options…there is nothing to give but the
child is still suffering a lot. And finally, I have thought of oral IVIG
….giving Ig by mouth to control the viral infection in the gut
mucosa….there is no guarantee that Ig will be destroyed by gastric juice
…but I can still think and if that is the concern, I can persuade GI guy,
do the endoscopy and let him spary Ig on the surface of the gut….
Or Ig enema even!!
Do you also try things like this with IVIG? If I
give Ig, how much (the dose, and frequencies) should I give per mouth?
Thanks for any comments.
YaeJean Kim, MD
Assistant Professor
50 Ilwon-dong Gangnam-gu
Division of Infectious Diseases
Department of Pediatrics
Samsung Medical Center
Sungkyunkwan University
Seoul, 135-710
South
Korea
tel) 82-2-3410-3539, 0987
fax) 82-2-3410-0043
yaejeankim at skku.edu
From:
pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Torgerson, Troy
Sent: Friday, June 26, 2009 8:21
AM
To: pagid at list.clinimmsoc.org
Subject: Re: [PAGID] two questions
on enteritis
YaeJean,
IPEX is certainly a good
initial thought given his symptoms but I would include a couple of other
disorders:
First, for IPEX:
Has he had eczema or other rash? Has he had other autoimmunity as
well (hemolytic anemia, thrombocytopenia, liver disease, etc.)? Most of
the pts with FOXP3 mutations have other autoimmune manifestations in addition
to the enteropathy, eczema, and endocrinopathy. Have you been able to get
flow cytometry done to see if he has FOXP3+ cells in his CD4+ population, would
also look at CD25 expression by flow to make sure it is there (see James’
comment)? When they sequenced FOXP3, do you know what they sequenced and
how much of the gene they looked at? About 5% of the mutations we have
identified are in non-coding regions of the gene (polyadenylation site or
upstream non-coding region). If they sequenced genomic DNA did they look
at all of the exon/intron junctions to rule out a splicing mutation or did they
sequence cDNA?
Second, Leaky SCID:
Some Leaky SCID/Omenn or maternally engrafted SCID patients can look a lot like
IPEX. Pts with IPEX can get infections for sure but pneumonias are not
all that common and “frequent” infections are not all that
common. What are the T/B/NK numbers? Are they his T/B/NK cells or
his mom’s? Do his T cells proliferate to mitogens/antigens?
Do you know whether his T cells are mostly CD45RA+ (naïve) or CD45RO+ (mature)?
– leaky SCID’s & maternally engrafted SCID’s are usually
very skewed to CD45RO+. Are his parents consanguineous?
Third, CGD:
Incidence of IBD/IBD-like symptoms is very high in CGD and we recently
saw an infant here in Seattle with initial presentation of early onset
“Crohn’s” disease with granulomatous lesions on biopsy that
has X-CGD. Pneumonia also common in CGD. Would check neutrophil
oxidative burst as this would dramatically alter the therapeutic approach.
Best,
TT
Troy
R. Torgerson, MD PhD
Assistant Professor,
Pediatric Immunology/Rheumatology
University of Washington,
Department of Pediatrics
Co-Director, Immunology
Diagnostic Laboratory
Center for Immunity and
Immunotherapies
Seattle
Children’s Research Institute
1900 9th
Ave., C9S-7
Seattle, WA 98101-1305
Tel: (206)
987-7450
Fax: (206)
987-7310
Email: Troy.Torgerson at seattlechildrens.org
From:
pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of YaeJean Kim
Sent: Thursday, June 25, 2009 2:46
PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] two questions on
enteritis
Dear all,
I am YaeJean
Kim who attended the 2007 PID summer school while I was a fellow in Seattle and
now am relocated to Seoul, Korea.
I see a
patient in my institute and have questions as below.
This is a now
18 month boy who is suspected for IPEX or IPEX like syndrome. He has had
intractable diarrhea, recurrent infections including pneumonia and anal
infection, fistula, hypothyroidism (only endocrinopathy manifestiation he has)
since age of 1 month. His brother died too with similar features.
He was
initially presented with failure to thrive. Intestinal biopsy (at the age of 7
mo) was suspected for IPEX but FOXP3 mutation was not observed. Because of
severe anal fistula, he received cecostomy. He is now on azathioprine by our GI
guy who is the main doctor for him. He still seems to have frequent infection
with fevers and get hospitalized for antibiotics. Do you have any suggestions?
Or should we repeat the test? All the tests were done about 10 months ago.
I would
appreciate any feedback. Thanks a lot.
Jean
--------------------------------------------
YaeJean Kim, MD
Assistant Professor
50 Ilwon-dong Gangnam-gu
Division of Infectious Diseases
Department of Pediatrics
Samsung Medical Center
Sunggyungwan University
Seoul, 135-710
South Korea
tel) 82-2-3410-3539, 0987
fax) 82-2-3410-0043
yaejeankim at skku.edu
Children's Hospital and Regional Medical Center is now
Seattle Children's.
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