[CIS PIDD] [cis-pidd] Fwd: Hyper IgE syndrome

[Richard Wasserman]

I would be very cautious with the use of prednisone. Fungal pneumonia in
the setting of antibiotics and steroids is a real risk.
Richard Wasserman
Dallas


On Fri, Mar 29, 2013 at 8:00 PM, <dmvascon at usp.br> wrote:


> Dear Luciana, good evening

>

> I think that your description is very suggestive of an AD hyper IgE due to

> STAT3 mutation.

> Can you perform STAT3 phosphorylation by flow?

> It is interesting to look for IL17 production, that is probably very low

> and at least partially responsible for the susceptibility to Staph

> infection.

> I will look for someone here in our lab who should perform STAT3

> phosphorylation and, if positive I contact you.

>

> All the best,

>

> Dewton Vasconcelos

> University of São Paulo School of Medicine

>

> ----- luciana Cunha &lt;lucianaaoc at gmail.com&gt; escreveu:

> > Dear friends:

> >

> > I would like to discuss a case.

> > We admitted at ICU a 7 year-old girl who was being treated as a severe

> > atopic dermatitis, presenting with a staphylococcal and pseudomonas

> sepsis.

> > She also has eosinophilia (>2000/mL), normal immunoglobulins levels,

> normal

> > lymphocyte immunophenotyping ( CD3,CD4, CD8 CD19, CD16/56), primary teeth

> > retention, chronic eczema, characteristic face and high palate. She

> doesn't

> > present pneumonias or parenchimal lung anomalies. She is from a

> > consanguinous family with many cases of less severe atopic dermatitis.

> > At the moment, she is using vancomycin, cefepime, prednisone 1mg/kg/day

> and

> > diphenydramine. We are considering the use of gammaglobulin or

> omalyzumab.

> > What's your opinion? Any other suggestion?

> > Thank you very much,

> >

> > Luciana Cunha

> > Primary Immunodeficiency Service

> > Federal University of Minas Gerais

> > Brazil

> >

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-- 
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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