[CIS-PAGID] Cryptosporidium in CD40L

[Prof. Dr. Antonio Condino Neto]

We have data of ongoing studies in X-LINKED HIGM patients showing an  
impairment of neutrophil and dendritic cells function that can be  
improved by IFN-gama in vitro

In this particular case I think we have nothing to loose in trying to  
do it, as all we know that this infection will not clear.

-- 
Antonio Condino-Neto
Professor of Experimental Medicine
Institute of Biomedical Sciences, University of São Paulo
1730 Lineu Prestes Avenue, São Paulo - SP. ZIP 05508-000. Brazil
Tel (55) (11) 3091-7387 / Fax (55) (11) 3091-7224



Citando "Puck, Jennifer" <puckj at peds.ucsf.edu>:


>

> 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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