Hepatic Venous Pressure Gradient: Measurement and Pitfalls.

Measurement of hepatic venous pressure gradient (HVPG) effectively mirrors the severity of portal hypertension (PH) and offers valuable insights into prognosis of liver disease, including the risk of decompensation and mortality. Additionally, HVPG offers crucial information about treatment response to nonselective beta-blockers and other medications, with its utility demonstrated in clinical trials in patients with PH. Despite the widespread dissemination and validation of noninvasive tests, HVPG still holds a significant role in hepatology. Physicians treating patients with liver diseases should comprehend the HVPG measurement procedure, its applications, and how to interpret the results and potential pitfalls.

Combination of Model for End-Stage Liver Disease (MELD) and Sarcopenia predicts mortality after transjugular intrahepatic portosystemic shunt (TIPS).

TIPS is the most effective treatment for portal hypertension. Patient selection remains important to achieving optimal post-TIPS outcomes. The study evaluates 1-year mortality factors in cirrhotic patients receiving TIPS. METHODS: 87 cirrhotic patients received a TIPS between 2015 - 2021. Predictors of 1-year and overall mortality were assessed by estimating cumulative incidence functions and Grey's test to adjust for liver transplantation as a risk competing with mortality. Variables with p < 0.05 were checked for collinearity and included in the multivariate Cox proportional hazards model. Model discrimination was evaluated by calculating the area under the ROC curve. RESULTS: 87 patients were included (68% men; 22% ≥70 years). ALD was the primary cirrhosis cause. Most patients were Child-Pugh class B, MELD-Na score was 13.6 ± 6.0 points. The most frequent indication for TIPS was bleeding (51.7%), followed by refractory ascites (42.5%). The variables positively associated with mortality in univariate analysis were ascites, clinically overt sarcopenia and MELD-Na score, while ongoing nutritional supplementation improved survival. In the multivariate analysis, only clinically overt sarcopenia and MELD-Na score remained independently associated with mortality. A MELD-Na/sarcopenia model demonstrated a good discrimination, AUROC: 0.86 (95% CI 0.77 - 0.95). CONCLUSION: MELD-Na score, and sarcopenia were significantly associated with 1-year survival in cirrhotic patients who received TIPS.

2D shear wave elastography of the rectus femoris muscle in patients with cirrhosis: Feasibility and clinical findings. A pilot study.

BACKGROUND AND AIMS: Frailty is frequent in cirrhosis and associated with skeletal muscle abnormalities and worse prognosis. 2D shear-wave elastography (2D-SWE) could mirror biomechanical properties of skeletal muscle reflecting muscle quality. However, there is no data on 2D-SWE on skeletal muscle stiffness assessment in cirrhosis and on frailty. METHODS: Outpatients with cirrhosis were prospectively included in a single center. Skeletal muscle stiffness was studied at the rectus femoris by 2D-SWE. Ileo-psoas area and index (area/height2), and antero-posterior diameter of rectus femoris (RF) was measured on ultrasound. RESULTS: We included 44 patients (24 male, age 59 [IQR 49-66]) with a median liver frailty index (LFI) of 3.7 (IQR 3.2-4.0). Measurement of RF muscle stiffness (RFMS) was feasible in all with high inter-measurement reproducibility. RFMS did not correlate with LFI, liver function and skeletal muscle diameters. Ileo-psoas index was lower in frail patients (1.7 vs 1.0 cm2/m2, p = 0.024). RF antero-posterior diameter inversely correlated with LFI (r -0.578: p<0.001). CONCLUSION: RFMS by 2D-SWE is feasible and reproducible in cirrhosis and is independent of liver function and LFI, and warrants further studies in this setting. RF antero-posterior diameter could be reported as an objective parameter mirroring sarcopenia and frailty.

Leptin receptor blockade reduces intrahepatic vascular resistance and portal pressure in an experimental model of rat liver cirrhosis.

Increased hepatic vascular resistance mainly due to elevated vascular tone and to fibrosis is the primary factor in the development of portal hypertension in cirrhosis. Leptin, a hormone associated with reduction in nitric oxide bioavailability, vascular dysfunction, and liver fibrosis, is increased in patients with cirrhosis. We aimed at evaluating whether leptin influences the increased hepatic resistance in portal hypertension. CCl4-cirrhotic rats received the leptin receptor-blocker ObR antibody, or its vehicle, every other day for 1 wk. Hepatic and systemic hemodynamics were measured in both groups. Hepatic nitric oxide production and bioavailability, together with oxidative stress, nitrotyrosinated proteins, and liver fibrosis, were evaluated. In cirrhotic rats, leptin-receptor blockade significantly reduced portal pressure without modifying portal blood flow, suggesting a reduction in the intrahepatic resistance. Portal pressure reduction was associated with increased nitric oxide bioavailability and with decreased O2(-) levels and nitrotyrosinated proteins. No changes in systemic hemodynamics and liver fibrosis were observed. In conclusion, the present study shows that blockade of the leptin signaling pathway in cirrhosis significantly reduces portal pressure. This effect is probably due to a nitric oxide-mediated reduction in the hepatic vascular tone.

Efficacy and safety of anticoagulation on patients with cirrhosis and portal vein thrombosis.

BACKGROUND & AIMS: Portal vein thrombosis (PVT) is a frequent event in patients with cirrhosis; it can be treated with anticoagulants, but there are limited data regarding safety and efficacy of this approach. We evaluated this therapy in a large series of patients with cirrhosis and non-neoplastic PVT. METHODS: We analyzed data from 55 patients with cirrhosis and PVT, diagnosed from June 2003 to September 2010, who received anticoagulant therapy for acute or subacute thrombosis (n = 31) or progression of previously known PVT (n = 24). Patients with cavernomatous transformation were excluded. Thrombosis was diagnosed, and recanalization was evaluated by using Doppler ultrasound, angio-computed tomography, and/or angio-magnetic resonance imaging analyses. RESULTS: Partial or complete recanalization was achieved in 33 patients (60%; complete in 25). Early initiation of anticoagulation was the only factor significantly associated with recanalization. Rethrombosis after complete recanalization occurred in 38.5% of patients after anticoagulation therapy was stopped. Despite similar baseline characteristics, patients who achieved recanalization developed less frequent liver-related events (portal hypertension-related bleeding, ascites, or hepatic encephalopathy) during the follow-up period, but this difference was not statistically significant (P = .1). Five patients developed bleeding complications that were probably related to anticoagulation. A platelet count <50 × 109/L was the only factor significantly associated with higher risk for experiencing a bleeding complication. There were no deaths related to anticoagulation therapy. CONCLUSIONS: Anticoagulation is a relatively safe treatment that leads to partial or complete recanalization of the portal venous axis in 60% of patients with cirrhosis and PVT; it should be maintained indefinitely to prevent rethrombosis.

Tumor estromal gastrointestinal del intestino delgado con expresión negativa del marcador oncogénico KIT (CD117): A propósito de un caso

Los tumores estromales del tracto gastrointestinal (GIST), aunque infrecuentes, son tumores mesenquimatosos importantes de origen mesodérmico. Macroscópicamente se presentan como una masa intramural submucosal, siendo los mas comunes dentro de los tumores mesenquimatosos. La forma maligna, típicamente agresiva y resistente al tratamiento, es afortunadamente rara. Un importante avance ha sido la caracterización del marcador oncogénico KIT y su relación a la patogénesis del Tumor Estromal Gastrointestinal. Sin embargo, recientes investigaciones han demostrado la génesis de los GIST que son KIT negativos. Por lo tanto, la positividad del marcador oncogénico KIT, si bien es altamente sensible, no es requerimiento indispensable para el diagnóstico de GIST. Presentamos nuestra paciente con un GIST del intestino delgado y negatividad al c-KIT(CD 117).

Gastrointestinal stromal tumors (GIST) are uncommon but important mesenchymal tumors of the Gastrointestinal Tract, defined as being of mesodermal origin. Macroscopically they present as intramural submucosal masses, being the most common mesenchymal tumors to arise from the gastrointestinal tract. The malignant form, typically aggressive and resistant to treatment, in fortunately rare. An important development has been the characterization of the oncogene marker KIT, and its relation to the pathogenesis of GIST. However recent research has shed light on the genesis of GIST that is KIT negative. KIT is highly sensitive for GIST (though not 100%) but is not a marker of malignancy or essential requirement to the diagnosis of GIST. We show a patient with a GIST of the small intestine, KIT- negative.

Valor del diagnóstico y tratamiento precoz en el pronostico de pacientes con sindrome de Budd-Chiari / The diagnose value and precocious treatment in the patient prognosis with Budd-Chari syndrome

El síndrome de Budd-Chiari es una obstrucción completa, completa o parcial del drenaje venoso hepático. En los últimos 10 años la sobrevida de los pacientes con Budd Chiari ha aumentado, debido a los avances en los métodos diagnósticos y de radiología endovascular (Angioplastia y derivaciones intra-hepáticas transyugulares portosistemicas), las nuevas técnicas radiológicas intervensionistas pueden tener un papel relevante frente al tratamiento quirúrgico. Revisamos las historias de 8 pacientes con diagnóstico de Síndrome de Budd-Chiari, 4 pacientes fueron diagnosticados y tratados precozmente con anticoagulación y terapia endovascular, los restantes 4 pacientes acudieron a la institución con hipertensión portal clínicamente significativa, en estadios avanzados de su enfermedad y no fueron susceptibles de tratamiento medico o de terapia endovascular. De los 4 pacientes diagnosticados tempranamente, 3 (2 Child B y 1 Child C) recibieron anticoagulación oral con angioplastia con balon por vía transyugular, con mejoría clínica y resolución de la ascitis, con una reducción significativa del Índice de Child-Pugh (Child –Pugh A). Durante el seguimiento solo dos de los tres pacientes requirieron una sesión adicional de angioplastia transyugular. 1 paciente con Budd Chiari y trombosis portal simultanea, recibió solamente anticoagulación oral con evolución satisfactoria. Dos pacientes fueron referidas tardíamente (con 5 y 11 años de evolución) a este centro para evaluación y diagnóstico murieron durante el seguimiento, la causa en los dos caso fue síndrome hepatorrenal. El tratamiento del SBC requiere una valoración multidisciplinaria y debe ser individualizado. En los pacientes en que el Budd Chiari no pudo ser controlado médicamente, la angioplastia con balón vía transyugular se podría establecer como medida de descompresión de elección , frente a las derivaciones quirúrgicas, reservando el trasplante hepático cuando estas no son eficaces. Los resultado de este estudi...

Hepatitis autoinmune tipo 2 sindrome de superposición: hepatitis autoinmune/cirrosis biliar primaria: a proposito de un caso / Autoimmune hepatitis type 2: overlap syndrome: premary biliar autoimmune/eirrhosis hepatitis: case report

La Hepatitis Autoinmune constituye una inflamación crónica del hígado caracterizada por la presencia de autoanticuerpos y de hepatitis de interfase en el examen histológico e hipergammaglobulinemia. Constituye el 11-23 por ciento de todos los casos de hepatitis crónica en USA, afecta principalmente a las mujeres, siendo el tipo I el más frecuente. Se presenta el caso de una paciente de 21 años, con perfil inmunológico compatible con una Hepatitis Autoinmune tipo 2, con autoanticuerpos antimicrosomales de Hígado-Riñón (LKM1) positivo, lo cual es de prevalencia muy baja .El hallazgo asociado de anticuerpos antimitocondriales (AMA) positivo, característico de Cirrosis Biliar Primaria, nos lleva a considerar: Síndrome de Superposición