[CIS PIDD] [cis-pidd] ADA lung disease

[Dr. Carsten Speckmann]

  We have seen quick response (within 1 week) in a SCID baby aftet start 
of PEG-ADA, and within 3 weeks after PEG-ADA in a 6 year old with 
delayed-onset ADA and  interstitial lung disease.
Enzyme levels after start of PEG-ADA were certainly higher than 
"pysiological" levels after SCT, so I do not know how this is 
comparable. Murine data suggest that adenosine mediated lung pathology 
is also treatable with anti-IL6. Kind regards, Carsten Speckmann

Dr. med. Carsten Speckmann
Funktionsoberarzt/Consultant Immunologist
Zentrum fuer Kinderheilkunde und Jugendmedizin
Centrum fuer Chronische Immundefizienz - CCI
Universitaet Freiburg
Mathildenstr. 1
79106 Freiburg
Germany

phone: +49 (0)761-270 43010
mail: carsten.speckmann at uniklinik-freiburg.de
web: www.cci.uniklinik-freiburg.de



Am 18.04.14 17:29, schrieb Sullivan, Kathleen:

> Rob Sokolic had a nice presentation on the intrinsic lung disease in ADA SCID at CIS.  We have a patent who is clearly pathogen-free but for whom the lung disease has been a major factor in her care.  In thinking about a tracheostomy post-transplant, it would be helpful to know how long this takes to resolve post-transplant.

>

> Does anyone have experience with the quite severe ADA SCIDs and the anticipated time of resolution of their lung disease post-engraftment?  I would be eager to hear it.

>

> Thanks.

>

> 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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