[CIS-PAGID] Hypogam following Fontan

[Burcin Uygungil]

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:****

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

> procedure.****

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> 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.****

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> 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).****

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> Has anyone seen this combination of findings?****

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> I am leaning toward beginning Ig replacement because of the progressive

> decrease in IgG levels.  ****

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> Thanks for your help.****

>

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>

> Joe Church****

>

> Children's Hospital Los Angeles****

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> ****

>

>

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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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