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

[Giclas, Patsy]

Dear Dr Church,

Usually the AH50 is low if Properdin is deficient.  It is required for the efficient function of the C3 amplification loop, so without it there is less activation in the terminal pathway.  Properdin can be measured accurately by ELISA as well as the methods mentioned earlier.  I assume the fraternal twin who died was also a male?  Were there other historic male relatives who either died or had documented Neisserial infections.?  

Patsy Giclas

Patricia C. Giclas, PhD
Director, Diagnostic Complement Laboratory
Professor, Pediatric Allergy and Immunology
National Jewish Health
1400 Jackson
Denver Colorado, 80206 USA
-------------------------------
office 303-398-1217
Fax 303-270-2128
email giclasp at njhealth.org
Cell: 303-808-8958
________________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] On Behalf Of Church, Joseph [JChurch at chla.usc.edu]
Sent: Wednesday, March 16, 2011 5:44 PM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Antwort: Re:  Ivasive pneumococcal disease.

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<mailto: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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