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Background: Compliance with treatment is very important for patients who suffer from Wilson's disease, a rare genetic disorder. They can benefit a long-life and effective treatment. The purpose of our study is to identify the level of compliance in Wilson's disease patients and features associated with compliance as well., Methods: This is a prospective study carried out in the National Reference Center for Wilson Disease (based in Paris and Lyon) over a 8 months period. Patients were evaluated on the first (M0) and last month (M8) with a questionnaire evaluating the number of missed treatment doses, a self-questionnaire collecting the reasons for non-compliance, and analogic scales analyzing the doctor-patient relationship and their behavior towards the treatment. The severity of depression symptoms was investigated by the Beck Depression Inventory (BDI). A detailed phone call interview was conducted by a psychologist every two months to evaluate their compliance and feeling., Results: Thirty-nine patients were included. The mean age of patients was 34 years (±9.9). At M0, 84.6% had a poor compliance with treatment. They were diagnosed more recently (P=0.049) with a higher proportion involving neurological disorders (P=0.007). Age, the type of treatment and the quality of the doctor-patient relationship were not associated with the outcome; 38.5% suffered from depressive symptoms. At M8, 56.8% of patients were poor compliants and 21.6% presented depressive symptoms., Conclusion: Wilsons's disease patients have important problems with compliance, without necessary being depressed. A close follow-up may help them become compliant, particularly those with neurological symptoms., (Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Context: Smoking whose prevalence is higher in patients with Crohn's disease (CD) worsens its evolution. Ulcerative colitis mostly affect non- or ex-smokers; smoking may improve the course of the disease., Objectives: Systematic literature review of data on the relationship between smoking, smoking cessation and Crohn'disease., Documentary Sources: Medline, on the period 1980-2015 with the keywords "Crohn's disease" or "inflammatory bowel disease" and "smoking" or "smoking cessation"; limits "Title/Abstract"; the selected languages were English or French., Study Selection: Among 1315 articles, 168 abstracts have given rise to a dual reading to select 69 studies (case-control, retrospective, reviews or meta-analysis). Data were extracted using a reading gate., Results: Smoking increases the risk of complications, recurrences and resort of surgery, corticosteroids or immunosuppressants. These deleterious effects are more common in women. Stopping smoking improves the course of the disease and represents an essential component of its management., Limits: Heterogeneity of the studies collected according to the type, population characteristics, definition of smoking status and the validation of smoking cessation., Conclusion: Smokers suffering from CD must routinely be made aware of the disadvantages of smoking, benefits of abstinence and helped to quit smoking., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

The Transjugular Intrahepatic Portosystemic Shunt (TIPS) procedure is now performed in almost twenty hospitals in France, including Tours university hospital. The aim is to reduce portal hypertension (PHT) by diverting the portal system to the caval system within the liver. The main cause of PHT is cirrhosis, which may be of alcoholic, viral, dysmetabolic or autoimmune origin., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)