[CIS PIDD] [cis-pidd] CGD carriers with mucositis

[CIS-PIDD]

I find it hard to base such an important decision on only one parameter. I think clinical course in combination with genetics, oxidative burst percentage, donor availability and patient will should lead to the decision.

Envoyé de mon iPhone

> Le 31 août 2016 à 17:17, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> a écrit :
> 
> If she ends up being steroid dependent-I’m not so sure that is different than CGD IBD for which we would do a transplant.  I too wool have a hard time moving forward with a transplant but perhaps we should be.
> 
> Just food for thought.
> 
> Kate
> Kate Sullivan, MD PhD
> Wallace Chair 
> Chief of Allergy Immunology
> ARC 1216 CHOP
> 3615 Civic Center Blvd.
> Philadelphia, PA 19104
> (p) 215-590-1697
> (f) 267-426-0363
> 
> 
>> On Aug 31, 2016, at 11:09 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> wrote:
>> 
>> She has been to the NIH and her oxburst measured at what she said was 15%
>> which is above what they would consider for transplant (per mom)
>> 
>> She doesn't get infections, an her symptoms are limited to her mouth and
>> skin so hard to push for a BMT.
>> 
>>> On 8/25/16, 12:51 AM, "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> wrote:
>>> 
>>> Dear James,
>>> 
>>> we have done allogeneic bone marrow transplantation in a X-CGD female
>>> carrier with non-random X-chromosome inactivation and therapy-refractory
>>> inflammatory bowel disease. You could check the degree of X-chromosome
>>> inactivation by DHR-123 testing  first and reevaluate histopathology
>>> (pigment-laden macrophages etc. ) and then see if transplantation might
>>> be an option (PMID: 26464403).
>>> 
>>> Best regards, Fabian
>>> 
>>> -----Ursprüngliche Nachricht-----
>>> Von: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
>>> Gesendet: Mittwoch, 24. August 2016 22:32
>>> An: CIS-PIDD
>>> Betreff: [cis-pidd] CGD carriers with mucositis
>>> 
>>> I replied to a post about scid so resending
>>> 
>>> I have a quick question about CGD carriers
>>> 
>>> I have a mother of a son with X-linked CGD who has had horrible oral
>>> mucositis as well as discoid lupus on her arms.  I know this can occur in
>>> carriers.  She dealt with this her entire life.  No other infections.
>>> 
>>> She has been tried on plaquenil, Imuran, topical steroids, and oral
>>> steroids (as a teenager, stopped since she didn't like the side effects)
>>> 
>>> Anyone care for moms of CGD patients with similar symptoms and if so has
>>> anything worked?
>>> 
>>> We are going to try a short course of steroids, and then perhaps
>>> colchicine.  Our next thought was TNF blockers (a little nervous with her
>>> SLE like rashes that things could worsen but if nothing else works we may
>>> try this.)
>>> 
>>> Thanks
>>> 
>>> James
>>> 
>>> 
>>> James W. Verbsky M.D./Ph.D.
>>> Associate Professor of Pediatrics and Microbiology Medical Director,
>>> Clinical Immunology Research Laboratory Medical Director, Clinical and
>>> Translational Research Medical College of Wisconsin Milwaukee, WI
>>> 
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