[CIS-PAGID] Hypogam following Fontan

[Church, Joseph]

Thank you, Matt.  My current patient has no symptoms of airway disease
at all.  JC

 

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Matthew
Greenhawt
Sent: Thursday, December 01, 2011 9:01 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Hypogam following Fontan

 

Joe,

We are aware of a number of post-fontan children that also have this
profile.  When we evaluate them, we see almost exactly the same pattern
with respect to the labs, but have not recommended any replacement
because they have protective titers despite low total IgG and they don't
have infections.    The most interesting thing that we've seen, that no
one else has mentioned yet this morning,  is that these all tend to be
older kids (10 years or older), and many of them have plastic bronchitis
associated with the immune dysregulation (for lack of a better term).
These kids routinely will cough up thick casts (some up to several
inches long).  Our pathologists are looking at the composition of these
and have run proteomic analysis on a few of the kids, with more to come.


 

If anyone else is noting this in the presence of plastic bronchitis, I'd
love to compare notes--I am most curious about this particular aspect.  

 

Best,

 

Matt

 

Matthew Greenhawt, MD, MBA

Assistant Professor

University of Michigan Health System

University of Michigan Medical School

Division of Allergy and Clinical Immunology

mgreenha at med.umich.edu <mailto:mgreenha at med.umich.edu>   

On Dec 1, 2011, at 11:36 AM, Church, Joseph wrote:





Burcin:  Thank you for your response.  I certainly will keep that
approach in mind.  JC

 

From: pagid-bounces at list.clinimmsoc.org
[mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Burcin Uygungil
Sent: Thursday, December 01, 2011 8:32 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Hypogam following Fontan

 

Hi Joe, 

 

I am a mere fellow but I just wrote a review on primary intestinal
lymphangiectasia and I think that the same issues apply to this is case
of secondary protein losing enteropathy.  In addition to IgG replacement
(subq might be better) - you could also try a dietary approach with a
high MCT (medium chain triglyceride) diet.  This usually involves a
formula so you might need to speak with a dietician to make it more
palatable for a 7 year old.  I have seen that it really works in a
handful of patients.

 

Burcin Uygungil

Johns Hopkins Hospital

On Thu, Dec 1, 2011 at 10:17 AM, Church, Joseph <JChurch at chla.usc.edu>
wrote:

Colleagues:

 

I am seeing a 7yo girl with hypoplastic left heart syndrome s/p Fontan
procedure.

 

As many of these patients she has developed progressively decreasing
IgG.  Her tetanus, Hib and pneumococcal antibodies remain protective,
and she has had no serious infections.  She also has T-lymphopenia.

 

Of particular interest is that her low IgG (currently 155 mg/dL) seems
out of proportion to her hypoalbuminemia (2.9-3.2 mg/dL).

 

Has anyone seen this combination of findings?

 

I am leaning toward beginning Ig replacement because of the progressive
decrease in IgG levels. 

 

Thanks for your help.

 

Joe Church

Children's Hospital Los Angeles

 

 

 

 


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-- 
Burcin

 

"If you want to progress in your life and grow, act not to react"

 
-Yogi Bhajan

 


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