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

Church, Joseph JChurch at chla.usc.edu
Wed Mar 16 19:44:14 EDT 2011


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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