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

[luciana Cunha]

Dear Dewton,
we can't perform STAT3  phosphorylation and we'll be very thankful if you
could find someone to do this. Thank you very much,

Dear Fabrício,
She's a girl and doesn't present any other finding suggesting
Wiskott-Aldrich syndrome except of the eczema. We are thinking first in
Hyper-Ige.  Thank you very much,

Luciana.

2013/3/30 Richard Wasserman <drrichwasserman at gmail.com>


> 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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