[CIS-PAGID] Hypogam following Fontan

[Matthew Greenhawt]

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  

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

>  

>  

>  

>  

> 

> ---------------------------------------------------------------------

> CONFIDENTIALITY NOTICE: This e-mail message, including any attachments, 

> is for the sole use of the intended recipient(s) and may contain confidential

> or legally privileged information. Any unauthorized review, use, disclosure

> or distribution is prohibited. If you are not the intended recipient, please

> contact the sender by reply e-mail and destroy all copies of this original message. 

> ---------------------------------------------------------------------

> 

> 

> 

> 

> -- 

> Burcin

>  

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

>                                                                        -Yogi Bhajan

>  

> 

> ---------------------------------------------------------------------

> CONFIDENTIALITY NOTICE: This e-mail message, including any attachments, 

> is for the sole use of the intended recipient(s) and may contain confidential

> or legally privileged information. Any unauthorized review, use, disclosure

> or distribution is prohibited. If you are not the intended recipient, please

> contact the sender by reply e-mail and destroy all copies of this original message. 

> ---------------------------------------------------------------------

> 


-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/mailman/private/pagid/attachments/20111201/3a37915f/attachment.htm>