[PAGID] any indication of IVIG in mixed ID

[Nacho Gonzalez]

Dear Sabiha,

you need to undergo complement funtional tests i.e. CH50 or CH100 if you
suspect C3 or C4 deficiency and AP50 if you suspect alternative pathway
deficiency.

Nacho Gonzalez. Immunodeficiencies Division. Hospital 12 octubre. Madrid

2009/9/9 Sabiha Anis <sabiha_anis at hotmail.com>


> Dear Dr Zuhair and Dr Dewton

>

> Thank you very much

> Specific anti body responses to diptheria and tetanus is slightly low. we

> have given a booster for tetanus and will repeat test after completion of 4

> weeks.  Though he had mounted a very good antibody response to CMV. we'll

> check the same for polysaccharide antigens. We have done lymphocyte subset

> analysis twice. during infection and then at the time of discharge. It was

> 0.3% and 0.2% with an absoloute count of 14/ul.  T (both helper and

> cytotoxic) and B cells were on the higher side (Ab counts: 1342u/l, 2153/ul

> and 673 /ul). IgG: 20 g/l (ref: 7.5 -15.6 g/l), IgM : 1.68 g/l (ref:

> 0.46-3.04g/l), IgA :0.5 g/l (ref: 0.82-4.5 g/l), C3:0.6 g/l (

> ref:0.79-1.52g/l).  yes I agree that this C3 deficiency could be due to fH

> or fI defiency, nevertheless it could lead to recurrent infections with

> encapsulated organism. This pt had meningitis as well in the past.

>

> as the patient hasbeen readmitted, I'll have his subset analysis redone. at

> the moment I dont have CD21 nor do I have the facility for NK functions.

>

> Regards

>

> Sabiha

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

>

> Date: Tue, 8 Sep 2009 10:34:44 -0300

> From: dmvascon at usp.br

> To: pagid at list.clinimmsoc.org

> Subject: Re: [PAGID] any indication of IVIG in mixed ID

>

>

> Dear Sabiha

>

> I also agree that Ab responses to proteins and polysaccharides are

> fundamental.

> Nevertheless, NK cell and C3 deficiencies are not common

> immunodeficiencies. It is important to follow-up NK cell counts and

> cytotoxic function if possible, as they could be temporarily decreased due

> to CMV infection.

> Moreover, C3 deficiency states could be dependent on C3 deficiency (very

> rare) as well as to factor I and H of complement pathway. C3 receptor on B

> cells (CD21) is important as it decreases the threshold for activation of B

> cells by antigens, being very important for the responses to polysaccharide

> antigens.

> Another point to think is about the possibility of a protein losing

> enteropathy, that could be associated to hypogammaglobulinemia (mainly IgG)

> and lymphopenia.

>

> Kind regards,

>

> Dewton

>

> Ballas, Zuhair escreveu:

>

>  Did you check specific antibody responses?

>

> Zuhair

>

>

>

> Zuhair K Ballas, M.D.

>

> University of Iowa

>

> Iowa City, Iowa 52242

>

> Phone: 319-356-3697

>

> FAX: 319-356-8280

>

> e-mail: ballasz at uiowa.edu

>

>

>

>

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

>

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

> mailto:pagid-bounces at list.clinimmsoc.org<pagid-bounces at list.clinimmsoc.org>]

> *On Behalf Of *Sabiha Anis

> *Sent:* Monday, September 07, 2009 10:48 PM

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

> *Subject:* [PAGID] any indication of IVIG in mixed ID

>

>

>

> Good Morning everyone

> We have a 22 year old patient with NK cell, C3 and IgA deficiency presented

> to us only 2 months ago with recurrent diarrhea and other infections

> including CMV and candidal esophagitis.  Accordingly he's been treated with

> antibiotics, anti viral and anti fungal and discharged on improvement.  He

> again end up with peumococcal pneumonia this time.  I want to ask if there

> is any role of giving him IVIG.  IgG subset levels report is awaited. Thanks

>

> Sabiha Anis

> Clinical Immunologist

> Sindh Institute of Urology and Transplantation

> Karachi, Pakistan

>

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

>

> Date: Mon, 7 Sep 2009 10:55:56 +0200

> From: nachgonzalez at gmail.com

> To: pagid at list.clinimmsoc.org

> Subject: [PAGID] CGD new mutation?

>

> Good morning, we have found a mutation in CYBB g.IVS6(+4) and it doesn´t

> appear in the databases

>

> Does anyone know if it has been already described?

>

>

>

> Many thanks

>

>

>

> Luis Ignacio Gonzalez Granado. Inmunoodeficiencies. Hospital 12 octubre.

>

>

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

>

> With Windows Live, you can organize, edit, and share your photos.<http://www.microsoft.com/middleeast/windows/windowslive/products/photo-gallery-edit.aspx>

>

>

>

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

> Share your memories online with anyone you want anyone you want.<http://www.microsoft.com/middleeast/windows/windowslive/products/photos-share.aspx?tab=1>

>

-------------- next part --------------
An HTML attachment was scrubbed...
URL: <http://seven.pairlist.net/mailman/private/pagid/attachments/20090909/16cdcf85/attachment-0001.html>