[CIS-PAGID] Hypogam following Fontan

[Berger, Melvin]

We had a case we believed to be primary intestinal lymphangiectia who also had very dramatic "plastic bronchitis" with coughing up casts. She responded very well to MCT oil as an infant, then to ligation of the thoracic duct, although I am not sure why that worked. Interestingly, that child was borm at about 22 weeks gestational age but is now a 28 year-old College graduate who had an Op-Ed piece in the Cleveland paper advising the "supercommittee" and other members of Congress not to cut Medicaid benefits for the disabled.
 
Melvin Berger, M.D., Ph.D.
Adjunct Professor of Pediatrics and Pathology
Case Western Reserve University
Cleveland, OH 44106

________________________________

From: pagid-bounces at list.clinimmsoc.org on behalf of Matthew Greenhawt
Sent: Thu 12/1/2011 12:00 PM
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
	 
	 
	 
	 

	
	---------------------------------------------------------------------
	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. 
	---------------------------------------------------------------------






Visit us at www.UHhospitals.org.

The enclosed information is STRICTLY CONFIDENTIAL and is intended for the
use of the addressee only. University Hospitals and its affiliates disclaim
any responsibility for unauthorized disclosure of this information to anyone
other than the addressee.

Federal and Ohio law protect patient medical information, including
psychiatric_disorders, (H.I.V) test results, A.I.Ds-related conditions,
alcohol, and/or drug_dependence or abuse disclosed in this email. Federal
regulation (42 CFR Part 2) and Ohio Revised Code section 5122.31 and
3701.243 prohibit disclosure of this information without the specific
written consent of the person to whom it pertains, or as otherwise permitted
by law.
-------------- next part --------------
A non-text attachment was scrubbed...
Name: not available
Type: application/ms-tnef
Size: 11297 bytes
Desc: not available
Url : <http://seven.pairlist.net/mailman/private/pagid/attachments/20111204/20706192/attachment.bin>