[CIS PIDD] 8mo old boy hypereosinophilia

[Michael Albert]

I agree with Joseph that the first thing that should be done is
CD45RA/RO to rule out Omenn syndrome.
Best regards,
Michael

On Thu, Sep 20, 2012 at 5:20 PM, Hoernes Miriam
<Miriam.Hoernes at kispi.uzh.ch> wrote:

>

> Thank you very much for your input. We had already tested the T reg (CD4CD25CD127-) and they where elevated (absolute (473/ul) and relative values (38%)). In addition we have not found any autoantibodies (thyroid, pancreas). There are patients with IPEX-like Syndrome described in the literature with normal Treg numbers but not with elevated ones. Do you think we need further investigation to exclude these syndromes as well?

> Thank you very much,

>

> Miriam Hoernes

>

> Dr. med. Miriam Hoernes

> Ärztin Abteilung Immunologie/Allergologie

> Jeffrey Modell Diagnostic Center for Primary Immunodeficiencies

> Universitäts-Kinderspital Zürich

> Steinwiesstrasse 75

> CH-8032 Zürich

> Tel.: +41 (0)44-266-7311

> Fax: +41 (0)44-266-7914

> e-mail: miriam.hoernes at kispi.uzh.ch

>

>

>

>

>

>

> -----Original Message-----

> From: Dr. Carsten Speckmann [mailto:carsten.speckmann at uniklinik-freiburg.de]

> Sent: Tuesday, September 18, 2012 5:00 PM

> To: pagid at list.clinimmsoc.org; Hoernes Miriam

> Subject: Re: [CIS PIDD] 8mo old boy hypereosinophilia

>

> In addition to the mentioned differentials, you might also consider IPEX.

> We have seen a large family with very variable phenotype, some patients

> had severe eczema during their 1st year of life and later onset of

> diarrhea and other problems (so that absence of gastrointestinal

> symptoms might not be sufficient to exclude this).

>

> Carsten Speckmann

>

> --

> Dr. med. Carsten Speckmann

> Facharzt

> Zentrum fuer Kinderheilkunde und Jugendmedizin

> Centrum fuer Chronische Immundefizienz - CCI

> Universitaet Freiburg

> Mathildenstr. 1

> 79106 Freiburg

> Germany

>

> phone: +49 (0)761-270 43010

> mail: carsten.speckmann at uniklinik-freiburg.de

> web: www.cci.uniklinik-freiburg.de

>

>

> Am 18.09.12 16:24, schrieb Church, Joseph:

>> Colleagues:

>>

>> Consider Wiscott-Aldrich - ? platelet size.

>>

>> Consider Omenn's or maternal GVHD - ? CD45RO/RA, T-cell receptor oligoclonality.

>>

>> Joe Church

>> Children's Hospital Los Angeles

>>

>>

>> -----Original Message-----

>> From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Reichenbach Janine

>> Sent: Tuesday, September 18, 2012 6:57 AM

>> To: pagid at list.clinimmsoc.org; 'pagid-bounces at list.clinimmsoc.org'

>> Cc: Hoernes Miriam; Seger, Reinhard

>> Subject: [CIS PIDD] 8mo old boy hypereosinophilia

>>

>>

>>

>>

>>

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