[CIS-PAGID] Antwort: Re: Ivasive pneumococcal disease.

[Nacho Gonzalez]

Dear Joe,

as far as I know, the only complement protein deficiency with normal CH50
and AH50 is properdin deficiency.

Regards,

Luis Ignacio Gonzalez-Granado
Immunodeficiencies Unit.
Hospital 12 octubre. Madrid. Spain

2011/3/17 Church, Joseph <JChurch at chla.usc.edu>


>  Thank you all.

>

>

>

> I forgot to list that the patient has a spleen by ultrasonography and no

> H-J bodies on peripheral blood smear.

>

>

>

> Will a patient with properdin deficiency have a normal AH50?

>

>

>

> Joe Church

>

>

>  ------------------------------

>

> *From:* pagid-bounces at list.clinimmsoc.org [mailto:

> pagid-bounces at list.clinimmsoc.org] *On Behalf Of *

> stephan.ehl at uniklinik-freiburg.de

> *Sent:* Wednesday, March 16, 2011 12:07 PM

> *To:* pagid

> *Subject:* Re: [CIS-PAGID] Antwort: Re: Ivasive pneumococcal disease.

>

>

>

> Ivemark syndrome and there are probably others, ST

>  ------------------------------

>

> *  Von: *"Saxon, Andy M.D." [ASaxon at mednet.ucla.edu]

> *  Gesendet: *16.03.2011 11:43 MST

> *  An: *"'pagid at list.clinimmsoc.org'" <pagid at list.clinimmsoc.org>

> *  Betreff: *Re: [CIS-PAGID] Antwort: Re:  Ivasive pneumococcal disease.

>

>

>

> Absolutely good thought as I've seen that  but it would be strange if both

> sibs had no spleen. Is there a congenital absence of  spleen in some

> syndrome?

>

>

>

> Andy Saxon, MD

>

> UCLA

>

>

>  ------------------------------

>

> *From:* pagid-bounces at list.clinimmsoc.org [mailto:

> pagid-bounces at list.clinimmsoc.org] *On Behalf Of *

> stephan.ehl at uniklinik-freiburg.de

> *Sent:* Wednesday, March 16, 2011 11:12 AM

> *To:* pagid at list.clinimmsoc.org

> *Subject:* [CIS-PAGID] Antwort: Re: Ivasive pneumococcal disease.

>

> Spleen is present and functional?

>

>

>

> Beste Grüße

>

> Prof. Dr. Stephan Ehl

> Wissenschaftlicher Direktor

>

> CCI - Centre of Chronic Immunodeficiency

> UNIVERSITÄTSKLINIKUM FREIBURG

> Breisacher Str. 117 - 2. OG, 79106 Freiburg i. Brsg., Germany

> phone: +49(0)761.270-7730

> Sekretariat +49(0)761.270-7755 fax +49(0)761.270-7760

> e-mail: stephan.ehl at uniklinik-freiburg.de

>

>

>

> -----pagid-bounces at list.clinimmsoc.org schrieb: -----

>

> An: pagid at list.clinimmsoc.org

> Von: Nacho Gonzalez

> Gesendet von: pagid-bounces at list.clinimmsoc.org

> Datum: 16.03.2011 07:07PM

> Betreff: Re: [CIS-PAGID] Ivasive pneumococcal disease.

>

> Dear Joe,

>

>

> has this patient detectable levels of *properdin *(either by radial

> immunodiffusion or Western blot) ?

>

>

>

> Regards

>

>

>

> Luis Ignacio Gonzalez-Granado

>

> Immunodeficiencies Unit

>

> Hospital 12 octubre. Madrid. Spain.

>

> 2011/3/16 Church, Joseph <JChurch at chla.usc.edu>

>

> Colleagues:

>

>

>

> I have evaluated a 4yo boy who had pneumococcal pneumonia + empyema at

> 2.5yo.

>

>

>

> His fraternal twin died at 6mo from pneumococcal meningitis.

>

>

>

> Normal labs have included:

>

> -          CBCs

>

> -          IgG, A, M, E

>

> -          Tetanus, Hib, pneumococcal vaccine responses (including

> persistently +19/23 serotypes following Pneumovax)

>

> -          CH50

>

> -          AH50

>

> -          T, B numbers

>

> -          Mitogen and antigen LPAs

>

> -          HIV PCR

>

> -          Mannose binding lectin

>

> -          TLR function (measured at IBT Labs).

>

>

>

> What am I missing?

>

>

>

> Joe Church

>

> Clinical Immunology and Allergy

>

> Children’s Hospital Los Angeles

>

>

>

>

>

>

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