[CIS-PAGID] Help with consult

[Chong, Hey]

Hello All:

 I have a 13 yo female with "combined immunodeficiency " (lymphopenia, with low CD4 count, low IgG and a protein losing enteropathy).  She had gastroschisis and atresias at birth now s/p numerous small and large bowel resections currently being evaluated for small bowel/colon/stomach transplant.   The transplant team would like to know what to expect with the immunosuppression used for transplantation in the setting of her immunodeficiency.  Could anyone comment specifically on the role of immunodeficiency in GVHD and infections post transplant?

Does anyone have any experience with managing transplants in CVID patients and what to expect?  Should they be monitored any differently?  Should the immunosuppression used be any different than any other patient?
.
*       More history:
   CID diagnosed in 2006
   *    Poor/absent response to vaccines
   *    Low IgG but normal IgA and IgM in the past currently on SQ IGx 2 years
   *    Decreased T cell proliferation with mitogens and antigens
   *    Lymphopenia with CD4 counts as low as 70 and history of PCP prophylaxis without hx of OI's
   *    Last 2 months with splenomegaly
   *    adrenal insufficiency



I would appreciate any help and advice and willing to answer any questions.

Thanks very much

Hey Chong



Hey Jin Chong MD PhD
Assistant Professor of Pediatrics
Division of Pulmonary Medicine, Allergy & Immunology
Children's Hospital of Pittsburgh of UPMC
One Children's Hospital Drive
4401 Penn Avenue
Pittsburgh, PA 15224
tel 412-692-7885
fax 412-692-8499





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