[CIS PIDD] CGD and cutaneous ulcers

[M.H.Haverkamp at lumc.nl]

Dear Dr. Nelson,

My professor, Jaap van Dissel (J.T.van Dissel MD PhD FRCPedin) at the infectious diseases department of the Leiden University Medical Center (LUMC) in the Netherlands (which is about 20 kilometers from The Hague) would be very happy to see her as a patient. At our department we are working among others on primary immunodeficiencies.

I give you his email address and the phone number of the department directly:

J.T.van_Dissel at lumc.nl
+31-70-5262613


Yours sincerely,

Margje H. Haverkamp



-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Nelson, Robert P Jr
Sent: zondag 12 augustus 2012 16:11
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] CGD and cutaneous ulcers

Dear all,

I am looking for an immunologist for young female from the US that is visiting the Hague for the next year working/studying.  I think she might have IgA def and celiac disease.  I have included my recent response to her..as below.  Any help would be appreciated.

Sincerely,

Bob Nelson, M.D.



"IgA deficiency is the most common immunodeficiency in humans and affects about 1 in 400-500 people.  IgA is an antibody that helps keep us stay well and is special because it is secreted onto mucus membranes which includes  the inside of the nose, sinuses, bronchi and throughout the GI tract..  The incidence of IgA deficiency is higher in folks with allergies and other autoimmune problems.  So there is an increase in the incidence of IgA def in those with celiac disease and an increase in celiac disease in those with IgA deficiency.  Occasionally the gluten diet may improve the IGA deficiency by making the GI tract  more intact.  Most of the time I think they are 2 problems and I think that you have IGA deficiency and Celiac. This is not too awful bad because the textbooks would say that 2/3 of people with IgA deficiency don't have symptoms related to it.  And the 1/3 with symptoms have mostly recurrent sinusitis and sometime bronchitis.   Evolution, the good lord or both bestowed us with a redundant immune system that generally keeps us well even when one part of it doesn't work well.  And there may be an advantage that we don't yet understand, like a decrease in the tendency for atherosclerosis.

There isn't a replacement of IgA.  I manage folks with early and somewhat prolonged courses of antibiotic for bacterial sinusitis, which is usually heralded by colored sputum.  Your current symptoms sounds more like a virus syndrome.  The differential usually comes down to virus, bacterial sinusitis or allergic rhinitis (hay fever) related to allergies (also more common in IgA-deficient folks).  Oftentimes there seems to be a running together of problems--like a virus that causes some inflammation, then secondary bacterial sinusitis.  Gastroesophageal reflux may be present, which feels like heartburn. 

I could help you with this when you come visit but think that it would be a good idea to have a doc in your camp  while you are at the Hague.  So I'm sharing your information with others who may know specifically who would be the best person to see there.  Will share the results lif they come."

Sincerely,

Bob Nelson
________________________________________
________________________________________
From: pagid-bounces at list.clinimmsoc.org [pagid-bounces at list.clinimmsoc.org] on behalf of Richard Wasserman [drrichwasserman at gmail.com]
Sent: Saturday, August 11, 2012 11:41 PM
To: Michael H. Land, MD; pagid at list.clinimmsoc.org
Subject: Re: [CIS PIDD] CGD and cutaneous ulcers

You might consider granulocyte transfusions. If you do, I'd apply G-CSF, GM-CSF, or Regranex (PDGF) http://www.regranex.com/PI_Full_Version.pdf about 30 minutes prior to the transfusion.
Richard Wasserman
Dallas

On Sat, Aug 11, 2012 at 2:37 PM, Michael H. Land, MD <mikelandmd at yahoo.com<mailto:mikelandmd at yahoo.com>> wrote:
Dear Sara,

At the UCLA Medical Center where I trained, my mentors Dr. Richard Stiehm and Dr. Robert Roberts have been treating patients with topical GM-CSF for over a decade and have published this.  I am sure they would be approachable and interested in sharing their more recent experience.  One of their papers from 2002 is the following, if you have not already seen it:

De Ugarte DA, Roberts RL, Lerdluedeeporn P, Stiehm ER, Atkinson JB.  Treatment of chronic wounds by local delivery of granulocyte-macrophage colony-stimulating factor in patients with neutrophil dysfunction. Pediatr Surg Int. 2002 Sept; 18(5-6): 517-20.

At Duke University Medical Center we have also been treating some PIDD patients with topical GM-CSF as well, which has helped greatly (particularly in one patient with a peri-rectal ulcer that was very difficult to treat).  It is an expensive therapy, but has been an important tool.

Best regards,
Michael

Michael H. Land, MD FAAAAI
Assistant Professor of Pediatrics
Department of Pediatrics, Division of Allergy & Immunology
Duke University Medical Center, Box 2644
Durham, NC 27710
Phone: 919-862-5354<tel:919-862-5354> or for Patient Issues: 919-684-9914<tel:919-684-9914>
Fax: 919-862-5355<tel:919-862-5355>
Email: m.land at duke.edu<mailto:m.land at duke.edu>

________________________________
From: "Leo, Sara" <sleo at cw.bc.ca<mailto:sleo at cw.bc.ca>>
To: "pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>" <pagid at list.clinimmsoc.org<mailto:pagid at list.clinimmsoc.org>>
Sent: Friday, August 10, 2012 5:18 PM
Subject: [CIS PIDD] CGD and cutaneous ulcers

Dear Colleagues,

My name is Sara Leo and I'm a 2nd year Pediatric Immunology/Allergy fellow at BC Children's Hospital in Vancouver, BC. About a year ago, Dr. Stuart Turvey wrote to PAGID to see if anyone had experience with using topical GM-CSF to treat CGD patients with cutaneous ulcers. Since then, we have successfully treated our patient with topical GM-CSF. We would like to put together a case series of CGD patients with cutaneous ulcers that have been treated with topical GM-CSF and would be interested in hearing about both failed and successful regimens. If anyone has experience with the above and would like to collaborate with us, we would love to hear from you.

Yours Sincerely,
Sara

Sara Leo BSc, MD, FRCPC
Pediatric Clinical Immunology and Allergy, PGY-5
University of British Columbia
BC Children's Hospital
Email: sleo at cw.bc.ca<mailto:sleo at cw.bc.ca>
Pager: 410-1447





--
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
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Fax (972) 566-8837
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