La Revue de médecine interne
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Splanchnic vein thrombosis includes Budd-Chiari syndrome and portal vein thrombosis. These diseases share common features: (i) they are rare diseases and (ii) they can lead to portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, the most common being myeloproliferative neoplasms. ⋯ Because of the rarity of these diseases, high-level evidence studies are rare. However, collaborative studies have provided a better understanding of their natural history and allowed to improve the management of these patients. This review focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, patients with portal vein thrombosis without underlying liver disease, and patients with cirrhosis and portal vein thrombosis.
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Review
[Diagnosis and evaluation of metabolic dysfunction associated steatotic liver disease (MASLD)].
Non-alcoholic fatty liver disease (NAFLD) or recently called Metabolic Dysfunction Associated Steatotic Liver Disease (MASLD), is the leading cause of liver disease, with an estimated worldwide prevalence of 25%. MASLD is suspected, in a metabolic condition, in the presence of hepatic steatosis, moderate hepatic cytolysis or hyperferritinemia. ⋯ Only a small minority of patients with MASLD will develop advanced liver disease and require liver specialist. Given the high prevalence of MASLD, the identification of these patients cannot be envisaged without the taking part in the screening of all physicians (general practitioners and specialists).
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Hemophagocytic lymphohistiocytosis is a rare syndrome with a poor prognosis, characterized by an uncontrolled dysregulation of the immune system. The rarity of this disease makes it difficult to obtain large cohorts. In this study, we analyzed the data of 66 patients: the objective was to describe the epidemiological, clinical, biological and therapeutic characteristics and to compare our results with those already published. ⋯ The study of the clinico-biological, epidemiological and survival data of the patients in our cohort allowed us to confirm previously published data but also to discuss some of them.