[CIS PIDD] [cis-pidd] Problems with TIPS in CVID with liver disease?

[CIS-PIDD]

Hi Charlotte,
as you know we finally have put together our questionnaire on experience with TIPS in CVID like patients. Sorry for taking so long, but we wanted to test it in our cohort first to make sure our questions are not too complicated.
We have a total of 3 patients with TIPS I am currently aware off. Our gastroenterologists require previous bleeding episode in order to perform TIPS.

One died later on of gram negative sepsis, with known enteropathy and the septic infection possibly favored by TIPS
One patient did well so far
One other patient died also of systemic infection (I don't have the detailed information at this time).
We did not see clinically relevant encephalopathy in our three patients, but this may have to do also with the remaining liver function which was not too bad in our patients despite severe portal hypertension. I wonder whether we have to worry about gram negative systemic infections in our patients due to underlying enteropathy and disturbed gastrointestinal barriers.

We are very interested in collecting the available clinical data on patients with CVID like disorders and TIPS.
If you are willing to contribute to this survey please contact me or Dr. Brass so we are sending the questionnaire out to you

Thank you

klaus
Prof. Dr. med. Klaus Warnatz

MEDICAL CENTER - UNIVERSITY OF FREIBURG
Center for Chronic Immunodeficiency - CCI
at the Center for Translational CellResearch
Department of Rheumatology and Clinical Immunology

Breisacher Str. 115, 79106 Freiburg, Germany
Tel. +49 761 270 77640 / FAX -77600 / Pager 12-7100
klaus.warnatz at uniklinik-freiburg.de<mailto:klaus.warnatz at uniklinik-freiburg.de>

www.uniklinik-freiburg.de/cci

Von: CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
Gesendet: Dienstag, 13. Dezember 2016 22:48
An: CIS-PIDD
Betreff: [cis-pidd] Problems with TIPS in CVID with liver disease?

Hi,

We saw a  CVID  patent yesterday with nodular regenerative  hyperplasia  of the liver with portal hypertension and ascites-  no bleed but had varices banded.
Had 2 L fluid removed a few days before.  (TIPS) procedure  (transjugular intrahepatic portosystemic shunt) is being considered.

We have only one CVID patient with a TIPS procedure  and she has been doing well overall but with one episode of encephalopathy in about 6 or more years.

Anyone have other experiences, positive or negative?

Charlotte

Charlotte Cunningham-Rundles, MD, PhD

Departments of Medicine and Pediatrics

The David S Gottesman Professor

The Immunology Institute

Mount Sinai School of Medicine

1425 Madison Avenue

New York, NY 10029

Phone: 212 659 9268

Fax: 212 987 5593

Email: Charlotte.Cunningham-Rundles at mssm.edu<mailto:Charlotte.Cunningham-Rundles at mssm.edu>


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