[CIS PIDD] [cis-pidd] CGD colitis

[Sullivan, Kathleen]

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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