[PAGID] any indication of IVIG in mixed ID

[Sabiha Anis]

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] 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.
 



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