[CIS PIDD] [cis-pidd] CGD colitis

[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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