[CIS-PAGID] Cryptosporidium in CD40L

[Nelson, Robert P Jr]

Dear Jennifer,

I asked our immunocompromised host infectious disease person and got the following:

"Bob,

Have used same drugs already listed......other than this....some (not me) had  used bovine colostrum...earlier in AIDS epidemic as there is high titer antibody against cryptosporidium....but I am not sure if there is any reasonable preparation.

Mitch"

I think that MUD transplant is how we would approach this and doubt whether the infection will clear without immunological reconstitution.   The conditioning strategy would be key with respect to engraftment/incitement of further liver injury/GVHD rate-severity and tempo of immunological reconstitution.  Different centers would address this differently given that there is not a standard and the devil in this case in my opinion is in the details, other than considering an ATG-containing regimen as a poor choice.

Would be happy to talk with you if you feel like it.

Bob


Robert P. Nelson Jr., MD
Professor of Medicine and Pediatrics
Divisions of Hematology/Oncology
535 Barnhill Dr. Ste 473
Indianapolis, IN  46202
Telephone: 317-948-1186
E-mail: ronelson at iupui.edu<mailto:ronelson at iupui.edu>
pager:  317-312-1773

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Sullivan, Kathleen
Sent: Monday, May 23, 2011 7:04 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] Cryptosporidium in CD40L

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<x-msg://74/puckj@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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