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

[CIS-PIDD]

Hi, good night from Brazil 


Dr. Myrthes and her group has a large experience with cvid and portal hypertension. Fabiana Lima is not enrolled in the  web-list but she is studying these patients.  Lima.fms at Gmail.com myrtb at uol.com

Good night.

Leonardo Oliveira Mendonça
Médico Especialista em Imunologia Clínica e Alergia, Doenças Autoimunes e Autoinflamatórias
Médico Especialista em Clínica Médica/Medicina Interna

Leonardo Oliveira Mendonça, MD
Specialist in Clinical Immunology and Allergy, Autoimmune and Autoinflammatory disorders
Consultant Specialist in Internal Medicine

E-mail: leonardo.oliveira.mendonca at gmail.com
Telefone/Phone Number: +55-11-3864-2246
Endereço/Adress: rua Heitor penteado, 477 - Sumarezinho - São Paulo - Brasil

> On Dec 13, 2016, at 8:13 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> wrote:
> 
> Thanks!   Yes of course- ideally we get a bunch of others to respond as well -  then we really know what to say about this.  Would you offer it to a pt such as mine? 
> 
> Sent from my iPhone
> 
> On Dec 13, 2016, at 5:10 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org> wrote:
> 
>> 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
>>  
>> 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
>>  
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