[CIS-PAGID] Hypogam following Fontan

[Daniel H. Conway]

I have followed a number of these individuals.  

1.  They tend to have T cell lymphopenia and low RA numbers.  I am unclear as to the ultimate significance or cause for this.  This may make sense since all have had operative thymectomies.
2.  I have "followed" them mainly.  Some individuals clearly had invasive bacterial infections on occasion.  On replacement with IgG, I found the kinetics of the IgG to be incredibly short with half-lives more than once less than 24 hours.  I have chosen to replace only with the incidence of severe bacterial infections and then only until recovery commences.
3.  Sometimes this has presented with a profound protein-losing enteropathy, sometimes not.  

Sincerely,
Daniel H. Conway, MD
Assistant Professor of Pediatrics
St. Christopher's Hospital for Children
Drexel University College of Medicine


On 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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