[CIS PIDD] [cis-pidd] Lung transplantation in CGD patients

[Verbsky, James]

Pere



We have had a similar AR CGD...granulomatous lung disease without infections.  We were able to improve her lung function considerably with prednisone..and she then tolerated a stem cell transplant.  Was wondering if you have tried immunosupppression to improve lung function.





James Verbsky MD/PhD
Associate Professor of Pediatrics and Microbiology and Molecular Genetics
Medical College of Wisconsin
Milwaukee, WI  53226

________________________________
From: Pere Soler Palacin [psoler at vhebron.net]
Sent: Tuesday, April 08, 2014 11:18 AM
To: CIS-PIDD
Subject: [cis-pidd] Lung transplantation in CGD patients

Dear all, a 24 year-old female with AR-CGD was sent to our centre few years ago with severe respiratory impairment due to several lung infections (including IFI) and some restrictive pattern. After intensive treatment the patient improved enough to be consdered for URD-SCT. While waiting, the patient has suffered a couple of viral infections and her lung function has worsened again and our haematologists has refused the option of transplantation.
Her RFT are FVC 1.2 (35.2%), FEV1 0.6 (20.67%) FEV1/FVC 49.4%. MMEF 0.11L/s (2.7%), she's on O2-therapy at home and our pulmologists have proposed sequential lung and SC transplantation. Any experience in lung transplantation in CGD patients?
Any other proposal?



Thanks in advance.



P.


Pere Soler Palacín, MD, PhD.
Pediatric Infectious Diseases and Immunodeficiencies Unit. Hospital Universitari Vall d'Hebron.
Assistant Professor. Universitat Autònoma de Barcelona.
Passeig de la Vall d'Hebron 119-129.
08035 Barcelona. Spain.
Tel: 0034934893140. Fax: 0034934893039.
E-mail: psoler at vhebron.net<mailto:psoler at vhebron.net>; 34660psp at comb.cat<mailto:34660psp at comb.cat>. Web: www.upiip.com<http://www.upiip.com/>.



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