[CIS PIDD] [cis-pidd] CGD colitis

[Philipp Henneke]

Have you considered anakinra, and if it works, more lasting IL1beta suppression (PNAS. 2014 Mar 4;111(9):3526-31)?
Philipp


Dr. Philipp Henneke
Professor
Head Ped. Infectious Dis & Rheumatology
UNIVERSITY MEDICAL CENTER FREIBURG
Center for Chronic Immundefiency (CCI)
Center for Pediatrics & Adolesc. Medicine
phone assist/ office + 49(0)761270 77640
mobil +49 (0)162 285 2481
fax +49 (0) 761 270 77600
Breisacherstr. 117-2.OG, 79106 Freiburg
Germany
philipp.henneke at uniklinik-freiburg.de
http://www.cci.uniklinik-freiburg.de

Am 28.07.2014 um 16:25 schrieb Bodo Grimbacher <bodo.grimbacher at uniklinik-freiburg.de>:

> 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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