[CIS PIDD] [cis-pidd] CD40L

[CIS-PIDD]

Dear all;

We are caring for a 2 y/o male with CD40L deficiency who is being worked up for BMT.  He had MRSA and PJP at diagnosis but has been doing very well for over a year, on bactrim, hizentra, and azithro prophylaxis.  As part of his workup, he underwent an abdominal CT scan, which shows mild periportal edema.  He has normal liver enzymes and normal bilirubin, with normal synthetic function, and has never had concern for liver disease.  He has been tested multiple times for crypto and been negative.

He appears quite well, but I am concerned about this finding prior to transplant (we were planning myeloablative busulfan dosing).  Has anyone seen this before, at such an early age and in the absence of crypto?  I am leaning towards further testing but was interested in the group's experience.

Thanks in advance.

-Blachy


Blachy J. Dávila Saldaña, MD
Attending Physician, Division of Blood and Marrow Transplantation
Children's National Health System
Assistant Professor of Pediatrics
George Washington University School of Medicine and Health Sciences
111 Michigan Avenue, N.W.
Washington, DC 20010
Phone: 202-476-4561
Fax: 202-476-2280

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