[CIS PIDD] Fwd: [cis-pidd] NEMO pt with enterocolitis/SBO

[Dr. Carsten Speckmann]

  Dear Dr.Hernandez,

We observed good longterm control of very severe IBD (occurring after 
SCT) in a NEMO patient using anti-TNFa (Infliximab).
However, to achieve full remission we needed high doses (6mg/kg) and 
short intervalls (4-weekly), which obviously increases the risk for 
infections...which we luckily did not experienced too often in our 
patient (post SCT).

Best wishes,

Carsten Speckmann

Funktionsoberarzt/Consultant Immunologist
Zentrum fuer Kinderheilkunde und Jugendmedizin
Centrum fuer Chronische Immundefizienz - CCI
Universitaet Freiburg
Mathildenstr. 1
79106 Freiburg
Germany

phone: +49 (0)761-270 43010
mail: carsten.speckmann at uniklinik-freiburg.de
web: www.cci.uniklinik-freiburg.de


>
>
>
> -------- Original-Nachricht --------
> Betreff: 	[cis-pidd] NEMO pt with enterocolitis/SBO
> Datum: 	Wed, 8 Oct 2014 09:08:47 -0700
> Von: 	Joseph Hernandez <joseph.demetrius.hernandez at gmail.com>
> Antwort an: 	CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> An: 	CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
>
>
>
> Hi all,
>
> I'm posting this question on behalf of one of my colleagues. We 
> recently inherited a challenging 22yo patient at Stanford with NEMO 
> deficiency. He has had a history complicated by multiple infections, 
> but his bigger problem has ben his gut disease. He has a history of 
> enterocolitis complicated by multiple enter-entero, and I think at 
> least one enterocutaneous fistula. He has had multiple bowel 
> resections and may now have some degree of short gut syndrome. Some of 
> the bowel resections have been because of obstruction/SBO and have 
> found inflammatory masses with no isolated infectious agent. We have 
> not been able to get all of his medical records but I am fairly 
> certain that he has received steroids in the past for his gut disease, 
> I'm not certain about non steroidal agents.
>
> He is currently been hospitalized with an organizing PNA (no organism 
> identified) and a RLQ fluid collection in the abdomen. The fluid grew 
> VRE and Candida glabgrata. He has another SBO identified on enema and 
> is being taken to the OR. Our GI service is reluctant to give much 
> immunosuppression given his infections.
>
> 1) Does anyone have any specific preoperative recommendations for this 
> patient in terms of improving his gut healing/function?
>
> 2) Any other ideas in terms of long term management of his gut 
> disease? I should add that he he a LONG history of non adherence to 
> therapy.
>
> Thanks,
>
> Joseph Hernandez
> Stanford University
>
> ---
>
> The CIS-PIDD listserv is supported by:
>
>
> /The science & practice of human immunology/
>
> P: +1.414.224.8095
> E: info at clinimmsoc.org
>
> Not a member of CIS? Please visit www.clinimmsoc.org 
> <https://cis.execinc.com/edibo/Signup> to join!
>
> You are currently subscribed to cis-pidd as: 
> jan.rohr at uniklinik-freiburg.de <mailto:jan.rohr at uniklinik-freiburg.de>.
> To unsubscribe click here: 
> http://lm.clinimmsoc.org/u?id=183824737.5b5212fc937bf3eee6f2e19506d4417c&n=T&l=cis-pidd&o=45593837 
> <http://lm.clinimmsoc.org/u?id=183824737.5b5212fc937bf3eee6f2e19506d4417c&n=T&l=cis-pidd&o=45593837>
>
>
>

---
The CIS-PIDD listserv is supported by the Clinical Immunology Society
The science & practice of human immunology

P: +1.414.224.8095
E: info at clinimmsoc.org

Not a member of CIS? Please visit www.clinimmsoc.org to join!

You are currently subscribed to cis-pidd as: pagid at list.clinimmsoc.org.
To unsubscribe click here: http://lm.clinimmsoc.org/u?id=183939985.3ea13d40a15475ac00ebbd9cd8a37d6d&n=T&l=cis-pidd&o=45602330
or send a blank email to leave-45602330-183939985.3ea13d40a15475ac00ebbd9cd8a37d6d at lists.clinimmsoc.org
-------------- next part --------------
An HTML attachment was scrubbed...
URL: <https://pairlist7.pair.net/pipermail/pagid/attachments/20141010/018fe4f5/attachment-0001.html>