Vascular liver diseases (VLD) are rare and their diagnosis can be difficult and late, leading to a delay in treatment. Diagnosis is most often multidisciplinary, involving primarily the clinician but also the radiologist...Vascular liver diseases (VLD) are rare and their diagnosis can be difficult and late, leading to a delay in treatment. Diagnosis is most often multidisciplinary, involving primarily the clinician but also the radiologist and pathologist. In all cases, attention should be drawn to the absence of the usual causes of liver disease; conversely, a context of pathology associated with VLD may be a suggestive factor. The two main ways in which porto-sinusoidal vascular disease is discovered are unexplained abnormal liver tests and portal hypertension (PHT) without cirrhosis. In the case of acute splanchnic thrombosis (mainly portal), the main symptom is abdominal pain. The intensity of symptoms varies greatly, and the diagnosis may be overlooked and established late, at the stage of portal cavernoma and possibly complications of PHT. Budd-Chiari syndrome can mimic any acute or chronic liver disease, with the most common presentation being ascites/hepatomegaly/abdominal pain. Many clinical situations should raise the suspicion of VLD, which is based on dialogue between the clinician, radiologist, and pathologist. Early diagnosis allows for optimal patient management, particularly through anticoagulant therapy and treatment of PHT.
Vascular diseases of the liver include damage to arterial and venous vessels, both hepatic and perihepatic. The main venous forms are Budd-Chiari syndrome (BCS), portal vein thrombosis (PVT) and porto-sinusoidal vascular...Vascular diseases of the liver include damage to arterial and venous vessels, both hepatic and perihepatic. The main venous forms are Budd-Chiari syndrome (BCS), portal vein thrombosis (PVT) and porto-sinusoidal vascular disease (PSVD), defined by the location of the thrombotic obstruction. BCS corresponds to an obstruction of the hepatic veins, PVT to an obstruction of the portal vein with a risk of cavernoma, and PSVD to damage the intrahepatic microcirculation without cirrhosis. These rare diseases mainly affect young adults and cause portal hypertension in the absence of underlying liver disease. Myeloproliferative neoplasms are a common risk factor, among other systemic causes (coagulation disorders, antiphospholipid antibodies, infections, pregnancy) or local causes (inflammation, surgery).
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...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.
Foureur N, Ferrié SM, Gross M
… +1 more, Messager D
Rev Prat
· 2025 Dec · PMID 41467831
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...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.
Chronic kidney disease (CKD) in children has an epidemiology that differs significantly from that in adults. Its main causes vary with age with a predominance of congenital anomalies of the kidney and the urinary tract....Chronic kidney disease (CKD) in children has an epidemiology that differs significantly from that in adults. Its main causes vary with age with a predominance of congenital anomalies of the kidney and the urinary tract. Early diagnosis is essential to prevent complications affecting growth, cognition, and long-term cardiovascular health, and to avoid, or at least to slow, progression to end-stage renal disease. Specific clinical contexts warrant targeted screening: family history, perinatal abnormalities, at-risk conditions, or suggestive clinical signs. Screening relies on simple tools such as growth charts, dipstick urinalysis, blood pressure measurement, glomerular filtration rate estimation, and renal ultrasound. The general practitioner plays a key role in the early detection of CKD, and in ensuring early referral to pediatric nephrology, helping to preserve nephron mass and improve long-term outcomes.
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 t...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.
The "Assessment - Acute Treatment - Reassessment - Relay" approach is still the basis of acute pain management for adults in the emergency department, and intravenous morphine titration remains the cornerstone of severe...The "Assessment - Acute Treatment - Reassessment - Relay" approach is still the basis of acute pain management for adults in the emergency department, and intravenous morphine titration remains the cornerstone of severe pain management. However, new recommendations, incorporating all the latest scientific and contextual developments, have led to challenge its place, and to modify the treatment approach. As a general rule, an initial protocol at triage should be followed by multimodal, individualized management for each patient, taking into account the possibility of induced pain, and analgesic relays after the emergency stay should be adapted to the new benefit/risk balance of opioids (from all classes) especially for ambulatory patients.
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-sinuso...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.
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, w...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.
Borderline Personality Disorder (BPD) is common, affecting 2 to 6 % of the general population, with a high prevalence in psychiatric settings. It is characterized by emotional, relational instability and impulsivity, oft...Borderline Personality Disorder (BPD) is common, affecting 2 to 6 % of the general population, with a high prevalence in psychiatric settings. It is characterized by emotional, relational instability and impulsivity, often associated with suicidal behaviors and comorbid disorders (anxiety, depression, addictions). Understanding BPD relies on the concept of relational hypersensitivity, rooted in an altered self-concept. The biopsychosocial approach explains its origins through the interaction between genetic vulnerability and emotional invalidation during childhood, exacerbated by trauma. Treatment primarily involves cognitive-behavioral therapies (CBT), such as Dialectical Behavior Therapy (DBT), which promote emotional regulation and reduce self-harming behaviors. Management should include thorough evaluation and education focused on relational hypersensitivity. Although limited, pharmacological treatments can address specific dimensions of BPD but require cautious prescription.
Stauffer E, Tankere P, Carin R
… +2 more, Connes P, Nader E
Rev Prat
· 2025 Dec · PMID 41467820
The World Tourism Organization estimates that between 195 and 375 million people traveled to high altitudes in 2019. The high prevalence of cardiorespiratory diseases in the general population, combined with the increasi...The World Tourism Organization estimates that between 195 and 375 million people traveled to high altitudes in 2019. The high prevalence of cardiorespiratory diseases in the general population, combined with the increasing number of elderly travelers, suggests that a significant proportion of these individuals may have underlying health conditions. Hypoxia, the main physiological challenge at high altitude, requires adaptive responses from the cardiorespiratory system, which are often impaired in patients with cardiac or pulmonary diseases. This population is at particular risk of decompensation and altitude intolerance symptoms. Therefore, a thorough medical assessment, including evaluation of comorbidities and consultation with a mountain medicine specialist, may be essential prior to high-altitude travel or long-haul flights.