[CIS-PAGID] Hypogam following Fontan

[Church, Joseph]

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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