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

[Joseph Hernandez]

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

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