[CIS-PAGID] Hypogam following Fontan

[Saxon, Andy M.D.]

Thanks for the education Joe.  My all your wines mature beautifully.

Andy Saxon, UCLA


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From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Church, Joseph
Sent: Thursday, December 01, 2011 7:26 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Hypogam following Fontan

No, Andy.

The hypogam is thought to be mechanical secibdary to increased pressure in the GI lymphatics due to the specific surgical procedure.  Patients with this process (post-Fontan hypogam+hypoalbuminemia) who go on to require heart transplant normalize Ig levels and even with post-transplant immunosuppression often have normal antibody responses.

Joe Church

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Saxon, Andy M.D.
Sent: Thursday, December 01, 2011 7:21 AM
To: 'pagid at list.clinimmsoc.org'
Subject: Re: [CIS-PAGID] Hypogam following Fontan

Hi Joe: Let me show my lack of pediatric knowledge. Is there recognized thymic developmental issues associated the hypoplastic left heart syndrome as there are in the combined cardiac/thymic issues seen in deGeorge?

Andy Saxon, MD
UCLA

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From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Church, Joseph
Sent: Thursday, December 01, 2011 7:17 AM
To: pagid at list.clinimmsoc.org
Subject: [CIS-PAGID] Hypogam following Fontan
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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