[CIS-PAGID] Hypogam following Fontan

[Notarangelo, Luigi]

Dear Joe:

I have seen several cases like this after Fontan procedure. Typically, they remain infection-free and are capable to mount antibody responses. I have not used IVIG in the vast majority of them and reserved substitution therapy only to the very few ones who develop infections.


Luigi D. Notarangelo
Professor of Pediatrics and Pathology, Harvard Medical School
Jeffrey Modell Chair of Pediatric Immunology Research
Division of Immunology, Children’s Hospital Boston
Karp Research Building, Room 9210
1 Blackfan Circle
Boston, MA 02115

teL: +1-(617)-919-2276
FAX: +1-(617)-730-0709




On 12/1/11 10:23 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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