[PAGID] CF and hypogamma

[Routes, John]

Agree with prior studies but are there secondary causes of hypogamma (eg.
drugs (steroids?), intractable diarrhea leading to protein losing
enteropathy etc...)---would also check  memory, switched memory B cells
Jack

John M. Routes, MD
Chief, Section of Allergy and Clinical Immunolog
Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics
Department of Pediatrics
Children's Hospital of Wisconsin
Medical College of Wisconsin
9000 W. Wisconsin Ave.
Milwaukee, WI  53226-4874
 
Phone: 414-456-4802; 456-4803
Fax: 414-456-6487 (Clinical)
Fax: 414-456-6323 (Laboratory)
Email: jroutes at mcw.edu
 




From: "Junker, Anne" <ajunker at cw.bc.ca>
Reply-To: "pagid at list.clinimmsoc.org" <pagid at list.clinimmsoc.org>
Date: Mon, 19 Jan 2009 22:17:00 -0800
To: <pagid at list.clinimmsoc.org>
Conversation: CF and hypogamma
Subject: Re: [PAGID] CF and hypogamma

We have had several patients with this combo, including one who didn't
present until around age 20y with agammaglobulinemia! We started him on IVIg
but he was an irregular attender. We have not recommended Ig in the others
because they had variable hypogam and were able to make specific antibodies.
I agree with Mel's suggestions as to investigations to verify the need for
Ig replacement. If I recall, there is only the one case series that reports
this association, and this was in an era before there were more adults than
children living with CF. Might be time for a new systematic survey of the CF
population.  

Anne Junker, MD
Associate Professor, Division of infectious and Immunological Diseases
BC Children's
Vancouver, BC, Canada
4480 Oak St, Vancouver, BC
604-875-3591
ajunker at cw.bc.ca

----- Original Message -----
From: pagid-bounces at list.clinimmsoc.org <pagid-bounces at list.clinimmsoc.org>
To: pagid at list.clinimmsoc.org <pagid at list.clinimmsoc.org>
Sent: Mon Jan 19 19:47:05 2009
Subject: RE: [PAGID] CF and hypogamma

1. Re: assessment of her CF: What is her FEV1 as % predicted for age, and
her weight, % predicted for age ? What other organisms besides pseudomonas
are frequent in her sputum ?

2. re: assessment of her hypogam: Have you measured specific antibodies
against Haemophilus influenzae and pneumococcal polysaccharides ?  Has she
ever had bacteremia ?  Does she have sinus diisease and/or otitis
disproporpionate to her lung disease ?

Older literature suggests an inverse correlation between hypogam and CF lung
disease (ie- the lower the IgG the better) but it is difficult to know which
was cause and which, effect.



Melvin Berger, M.D., Ph.D.
Adjunct Professor of Pediatrics and Pathology
Case Western Reserve University
phone 216 844 3237

Director, Jeffrey Modell Center for Primary Immune Deficiencies
Division of Allergy-Immunology
Rainbow, Babies and Children's Hospital
University Hospitals of Cleveland
RB&C Rm 504, MS 6008B
11100 Euclid Ave.
Cleveland, OH 44106

  _____ 

From: pagid-bounces at list.clinimmsoc.org on behalf of Brynn Wainstein
Sent: Mon 1/19/2009 10:20 PM
To: pagid at list.clinimmsoc.org
Subject: [PAGID] CF and hypogamma



Dear all,

I am hoping for some advice regarding a patient of mine. This is a 9 year
old girl with DF508 homozygous cystic fibrosis who was found on routine
testing to have hypogamma with results as follows:

        28/07/08        08/10/08        18/12/08
IgG     2.89            3.27            3.41    g/L (6 - 13)
IgM     0.4             0.39            0.46    g/L (0.4 - 1.6)
IgA     0.25            0.26            0.27    g/L (0.5 - 3)

Normal B cell numbers and T cell subsets. Otherwise clinically well with no
significant lung infections despite CF (she is colonised with pseudomonas).

We are considering IVIg replacement despite her clinical wellness because of
concurrent CF and persistently low Ig levels. But some reports in the
literature suggest worse lung function in patients with hypergamma but don't
talk specifically about IVIg.

I would appreciate peoples thoughts and advice on whether this girl should
receive IVIg.

Kind Regards,

Brynn Wainstein.

Dr Brynn Wainstein
Paediatric Clinical Immunologist
Staff Specialist
Sydney Children's Hospital
Tel: +61-2-9382-1515
Fax: +61-2-9382-1580


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