[CIS-PAGID] Cryptosporidium in CD40L

[Puck, Jennifer]

Would appreciate ideas for a young man with CD40L deficiency and a bad case
of cryptosporidium. He was previously healthy on immunoglobulin, G-CSF for
neutropenia, and TMP/SMX 3x per week for prophylaxis against pneumocystis
(his presenting infection in infancy).
He first got a headache and then diarrhea 6-8 weeks ago, and his stool was
positive for cryptosporidium as an outpatient. The diarrhea became explosive
and bloody requiring hospital admission, and he continued to have secretory
diarrhea and positive stools despite paromomycin, nitazoxanide, and
azithromycin and TPN/complete NPO bowel rest.

There is no HLA-matched sibling for BMT, and we are looking at the unrelated
donor registry. BMT may be the best long term option, and I have read that
BMT be required to clear the infection.

Do others have experience with severe cryptosporidium in CD40L deficiency?
Will symptoms eventually subside? What type of lab tests and imaging are
useful to follow these patients? LFTs? Ultrasound? MRI? Endoscopy? Will
attempts at oral feeding be helpful, neutral, or just induce further
diarrhea and delay gut healing?

Jennifer M. Puck, M.D.
Professor of Pediatrics
University of California, San Francisco, Box 0519
513 Parnassus Avenue, HSE 301A
San Francisco, CA  94143-0519

Email: puckj at peds.ucsf.edu
Phone: 415 476-3181
FAX: 415 502-5127 
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