[CIS-PAGID] Cryptosporidium in CD40L

[Sullivan, Kathleen]

Condino has some data to suggest that neutrophil function may be abnormal and g- interferon might improve the function.  It is a small thing to try in this terrible situation.

Kate
On May 22, 2011, at 12:55 PM, Puck, Jennifer wrote:


> 

> 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


Kate Sullivan, MD PhD
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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