La Revue du praticien最新文献

Small liver vessels disorders are a heterogeneous group of rare diseases that can affect the portal venules, the hepatic sinusoids, the centri-lobular veins, or the hepatic arteries. The most frequent is the porto-sinusoidal vascular disorder (PSVD), which is characterised by damage of the portal venules or sinusoids and may be associated with portal hypertension, in the absence of cirrhosis. Diagnosis is therefore based on a liver biopsy. PSVD is often associated with an extrahepatic condition, most commonly immune-mediated, haematological, or a toxic. Its two main complications are variceal haemorrhage and portal vein thrombosis. The latter must be screened for by imaging every six months. Liver failure, on the other hand, is very rare in this context. It is therefore important to consider the diagnosis of PSVD when there is marked portal hypertension alongside preserved liver function or low liver stiffness, particularly in the absence of an obvious cause of cirrhosis or in the presence of an extrahepatic condition known to be associated with PSVD, as well as in cases of unexplained abnormalities of liver blood tests. The management of PSVD is like that of cirrhosis.

Budd-Chiari syndrome is a rare condition characterized by obstruction of hepatic venous drainage, ranging from hepatic venules to the terminal part of the inferior vena cava. A thorough etiological workup to search for a pro-thrombotic disorder should be systematically performed. The most common cause of Budd-Chiari syndrome is myeloproliferative syndrome, present in more than 40% of cases. The clinical presentation of BCS is highly variable, ranging from asymptomatic patients (3% of cases) to those presenting with fulminant hepatitis. Diagnosis relies on imaging, notably abdominal ultrasound coupled with Doppler.A progressive therapeutic approach, combining medical measures (curative anticoagulation, treatment of the underlying cause, management of portal hypertension complications) and a strategy to restore hepatic venous flow, is recommended, preferably in a specialized center for vascular liver diseases. This strategy has significantly improved patient prognosis, with an overall 5-year survival rate exceeding 80%. The follow-up frequency for BCS patients is biannual, with hepatic imaging recommended, as more than 60% of patients may develop hepatic nodules and are at risk of hepatocellular carcinoma.

The medical treatment of children sometimes referred to as "intersex" has evolved over the last few decades. The bioethics law in 2021 in France sought to "depathologize" these situations by now referring to "variations in genital development" (disorders of sex development) and limiting treatment to "medical necessity" linked to unavoidable medical indications. Faced with this evolution, the clinical ethics center (AP-HP) conducted a qualitative study in three specialized departments (14 situations included, 38 semi-structured interviews with 17 parents and 13 professionals) and by observing 14 national meetings (new mechanism where medical decisions are made by "consensus"). The results show that people's ethical positions can be opposed, whether we think from a logic linked to the medical proposal (as is usual in pediatrics), or from a logic linked to the request of the person concerned (waiting for the "consent" of the individual concerned). More broadly, we are witnessing a re-examination of the role of medicine in relation to what it describes - or has long described - as functional disability, but also of the place of parents in pediatrics and the voice given to children or future adults in medical decisions that affect them. This paradigm shift requires a rethinking of the care pathway.

Portal vein thrombosis (PVT) in the absence of underlying chronic liver disease is a rare disease and is frequently associated with prothrombotic factors. In patients with cirrhosis, the frequency of PVT increases with the severity of cirrhosis. The main symptoms of recent PVT include abdominal pain and an increase in C-reactive protein (CRP). The main symptoms of chronic PVT are manifestations of portal hypertension (esophageal varices, thrombocytopenia, splenomegaly). The diagnosis of PVT is fortuitous in 30% of cases. Computed tomography or magnetic resonance imaging (MRI) with acquisitions without injection, at the arterial, portal, and tardive phase are mandatory to confirm the diagnosis and assess complications. Mesenteric ischemia is a medical and surgical emergency and represents the most severe complication of PVT. It must be systematically investigated using imaging. Surgery should be considered when intestinal necrosis is suspected, namely in cases of hyperlactatemia and associated organ failure. Anticoagulant therapy is the first-line treatment of recent and chronic PVT. Its modalities are based on a case-by-case assessment, considering features of thrombosis, comorbidities, and the therapeutic plan. In case of failure of anticoagulant therapy and/or severe manifestations, radiological portal vein recanalization may be considered. Treatment in a center with expertise in vascular liver diseases is recommended.

Anticoagulants play a major role in the management of vascular liver diseases (VLD). However, their use is challenging due to portal hypertension and thrombocytopenia, frequently observed in these conditions. Moreover, when the disease is associated with hepatic insufficiency, hemostatic abnormalities further complicate the use of anticoagulants. Finally, in cases of digestive resection following mesenteric ischemia, the absorption and pharmacokinetics of oral anticoagulant therapies raise numerous concerns. The benefit-risk balance regarding bleeding and thrombosis must therefore be carefully assessed. Nevertheless, anticoagulant therapy in VLD is crucial to prevent thrombus extension, improve recanalization, and reduce the risk of severe complications such as portal hypertension and mesenteric ischemia. It must be initiated as early as possible. Acute-phase treatment generally relies on low-molecular-weight heparin (LMWH), switch to Direct oral anticoagulants (DOACs) are increasingly used, however, vitamin K antagonists (VKAs) remain indicated in certain situations. Screening and management of esophageal varices should be systematic when initiating and managing anticoagulant therapy. Thrombocytopenia is most often moderate and should not lead to modification or discontinuation of anticoagulation. Severe thrombocytopenia < 50 G/L requires close monitoring and management in a specialized center. In Budd-Chiari syndrome, hepatic insufficiency may lead to reduced synthesis of coagulation factors as well as major coagulation inhibitors, allowing a degree of rebalancing of the hemostatic system. Prolonged coagulation times (aPTT) and decreased PT do not accurately reflect these changes and should not contraindicate or delay anticoagulant therapy. Women of childbearing age must be informed of the risks associated with anticoagulant therapy during pregnancy and breastfeeding. Menorrhagia is common and may require appropriate management. All patients should participate in a treatment education program, enabling them to understand the characteristics and risks of their treatment.