[CIS PIDD] [cis-pidd] CGD colitis

[Haddad Elie]

Dear Kate, 
Yes, HSCT clears colitis in most CGD patients. In our hands, we experienced this I at least  6 cases, including 3 patients with AR CGD. Actually, this outcome is very satisfactory, the patient saying : " I had forgotten what it means to have normal stools"...Also, this is associated with a growth recuperation (I hope it is the way to say in English)...
It should cure also the fistula and all the inflammation.
All the best
Elie

> On Jul 28, 2014, at 7:11, "Sullivan, Kathleen" <sullivak at mail.med.upenn.edu> wrote:
> 
> I have an unfortunate 14 year old girl with AR CGD who has had essentially untreated colitis for about 10 years.  She has fistulas and tags and extensive vulvar disease.  It is extremely sad.  Her infection pattern has been minimal.
> 
> Her colitis initially responded to steroids but is now unresponsive to high dose solumedrol and anakinra.  Her disease is sufficiently inflammatory that she has chronic hypoalbuminemia and her nutrition is abysmal even on TPN.  I am looking to hear other folks experience with HSCT in this circumstance.  If it was done in the face of such extensive inflammation- would the HSCT likely clear it or just transform into gut GVHD?
> 
> Any words of wisdom will be appreciated.
> 
> Kate
> Kate Sullivan, MD PhD
> Wallace Chair of Pediatrics
> Professor of Pediatrics
> ARC 1216 Immunology CHOP
> 3615 Civic Center Blvd.
> Philadelphia, PA 19104
> (p) 215-590-1697
> (f) 267-426-0363
> 
> 
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