[CIS PIDD] [cis-pidd] CGD colitis

[Klaus Warnatz]

Dear Kate, 

we have transplanted two adults with active colitis one with X CGD  (no treatment, colitis not so active) and one with AR CGD (despite AZA + 5ASA active pan colitis in the FDG PET, long sigma stenosis, status post fistula but not during transplantation) both cleared their colitis without side effects. For me it was amazing, both are very thankful for the procedure. 
this is my small experience 

klaus
Prof. Dr. med. Klaus Warnatz

UNIVERSITÄTSKLINIKUM FREIBURG
University Medical Center Freiburg
Center for Chronic Immunodeficiency
Division of Rheumatology and Clinical Immunology

Tel: +49-761-270-77640 / FAX -71000 / Pager: 12-7100

Breisacher Str. 117, 79106 Freiburg, Germany
klaus.warnatz at uniklinik-freiburg.de
http://www.uniklinik-freiburg.de/cci

Am 28.07.2014 um 16:25 schrieb Bodo Grimbacher:

> Š in the setting of IL-10 (receptor) deficiency mediated inflammation, the
> HSCT clears the inflammation...
> 
> ****************************************
> Univ.-Prof. Dr. med. B. Grimbacher
> Scientific Director
> CCI-Center for Chronic Immunodeficiency
> UNIVERSITÄTSKLINIKUM FREIBURG
> Tel.: 0761 270-77731  Fax: -77744
> Engesserstraße 4, 79108 Freiburg
> bodo.grimbacher at uniklinik-freiburg.de
> http://www.uniklinik-freiburg.de/cci
> 
> 
> 
> 
> 
> 
> 
> 
> 
> Am 28.07.14 16:02 schrieb "Sullivan, Kathleen" unter
> <sullivak at mail.med.upenn.edu>:
> 
>> 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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