[PAGID] please post

[Kate Sullivan]

Very very tough.  The adult folks probably see more but I am a big fan  
of Rapamycin for T cell infiltrates in the gut.  We also recently had  
a theoretical discussion on a similar patient at CHOP where we talked  
about BMT.

Kate
On Jan 20, 2010, at 9:08 AM, Routes, John wrote:


> I am posting this unusual patient for a colleague at the University of

> Virginia ( Larry Borish <LB4M at virginia.edu>) who asked me to comment  

> on this

> unusual patient----thanks

>

> Jack Routes, MD

> Chief, Section of Allergy and Clinical Immunology

> Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics

> Department of Pediatrics

> Children's Hospital of Wisconsin

> Medical College of Wisconsin

> 9000 W. Wisconsin Ave.

> Milwaukee, WI  53226-4874

>

>

> "What do you think of a 17 year old kid who 3 years ago developed in  

> no

> particular order: AIHA, autoimmune neutropenia, had his eosinophils

> disappear (AEC of 200-300 all his life to 0 for the past 3 years),  

> and had

> his B cell disappear along with all immunoglobulins.  He is in the  

> hospital

> dying of his enteropathy and secondary cachexia.  Other features are

> esophageal candidiasis, low testosterone (but nothing else hormonally

> including PTH), and maybe Menière's disease.  He has diffuse large  

> nodular

> lung disease and impressive bronchiectasis but nothing I'd call ILD  

> (also no

> thymoma).

>

>  My real question is he is about to be the 2nd antibody deficient  

> patient

> who has died on me in the past 2 years of refractory protein-losing

> enteropathy.  Gut shows excessive CD8s but no B cells.  I'm going to  

> send

> you gut DNA for HHV8, what the heck.  Anyway have you ever  

> successfully

> treated refractory enteropathy in an antibody-deficient patient?  I'm

> considering infliximab or cyclosporin but thought you might have a  

> better

> idea"

>

>

>

>

>

>

>


Kathleen Sullivan MD PhD
Professor of Pediatrics
Chief, Division of Allergy Immunology
The Children's Hospital of Philadelphia
(p) 215-590-1697
(f) 267-426-0363