[CIS-PAGID] Hypogam following Fontan

[Elie Haddad]

Dear all,
we had one case like this also with severe hypoIgG in this context, without infections. Since like Joe we were not comfortable with leaving a so low IgG level untreated, we gave SubcuIgG.
Interestingly, both patient and her parents asked us why we have waiten so long before giving her this "miracle" drug...She felt much better with a higher energy, ...and all these classical comments that we hear from patients who have an antibody deficiency in addition to the reduction of their infections... Overall, the "quality of life" of this patient was increased. Of course this is just a "one-case-story" and not a scientific evidence but I wanted to share this experience to feed the discussion.
Also, if you decide to treat her by Immunoglobulins, I would advise Subcu, because under IVIG, given that you induce a peak, there is more leakage of Immunoglobulins and IgG levels are more difficult to increase than under SCIg.
All the best
Elie


Elie Haddad, MD, PhD,
Professor of Pediatrics, University of Montreal,
Head, Pediatric Immunology and Rheumatology Division,
CHU Sainte-Justine, 3175 Cote Sainte-Catherine
Montreal, QC, H3T 1C5, Canada
Ph: 1 514 345 4713
fax: 1 514 345 4897
e-mail: elie.haddad at umontreal.ca





Le 2011-12-01 à 10:17, Church, Joseph a écrit :


> 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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