Budd-Chiari syndrome with short-length stenosis: still room for the angioplasty and wait-and-see strategy

We read with interest the study in The Lancet Gastroenterology & Hepatology by Qiuhe Wang and colleagues. 1 This randomised controlled trial aimed to elucidate whether routine stenting plus angioplasty was superior to angioplasty alone for preventing restenosis in patients with Budd-Chiari syndrome with short-length stenosis. The authors found that patients treated with angioplasty plus routine stenting had a lower incidence of restenosis than did patients treated with angioplasty alone, with no differences in survival. Based on these findings, the authors suggested that stenting combined with angioplasty should be used as a first-line invasive treatment in patients with Budd-Chiari syndrome with short-length stenosis

Prevalence of Radiological Chronic Pancreatitis and Exocrine Pancreatic Insufficiency in Patients with Decompensated Liver Disease: Is Fecal Elastase Useful in This Setting?

Chronic pancreatitis; Decompensated cirrhosis; Micronutrient deficienciesPancreatitis crónica; Cirrosis descompensada; Deficiencias de micronutrientesPancreatitis crònica; Cirrosi descompensada; Deficiències de micronutrientsChronic alcohol consumption is a well-known etiological factor for both chronic pancreatitis (CP) and liver cirrhosis. However, there is discussion over how often these two entities are present together in the same patient. The main goal of our study is to establish the prevalence of CP and low fecal elastase (FE-1) in patients with decompensated liver disease (DLD). In addition, we aim to identify the demographic, epidemiological and clinical factors associated with EPI and CP in patients with decompensated liver cirrhosis. This was an observational single-center study including 119 consecutive patients hospitalized for acute decompensation of cirrhosis, mostly of alcoholic etiology. Patients underwent computed tomography (CT) or magnetic resonance imaging (MRI) to assess the radiological features of CP. We also performed two FE-1 tests and complete blood tests to assess the presence of exocrine pancreatic insufficiency (EPI) and nutritional status, including micronutrients. The results of our study show that 32 patients (26.9%) had low fecal elastase suggesting EPI and 11 (9.2%) had CP. Patients meeting radiological CP criteria had lower FE-1 than patients without CP. There were no statistically significant differences in micronutrient deficiencies according to the presence of CP or not. Likewise, we did not find any statistically significant differences in micronutrient deficiencies among patients with normal and low FE-1 indicative of EPI. FE-1 alone may not be suitable for assessing EPI in patients with acute DLD. Detecting co-existing pancreatic disease may be important in a subset of patients with DLD, when the FE-1 levels are significantly low, potentially suggestive of a pancreatic anomaly. Moreover, the clinical manifestations of EPI and CP are not useful in detecting CP in DLD patients. Likewise, CP cannot explain all causes of EPI in these patients

Circulating levels of butyrate are inversely related to portal hypertension, endotoxemia, and systemic inflammation in patients with cirrhosis

Short-chain fatty acids (SCFAs) are gut microbiota-derived products that participate in maintaining the gut barrier integrity and host's immune response. We hypothesize that reduced SCFA levels are associated with systemic inflammation, endotoxemia, and more severe hemodynamic alterations in cirrhosis. Patients with cirrhosis referred for a hepatic venous pressure gradient (HVPG) measurement (n = 62) or a transjugular intrahepatic portosystemic shunt placement (n = 12) were included. SCFAs were measured in portal (when available), hepatic, and peripheral blood samples by GC-MS. Serum endotoxins, proinflammatory cytokines, and NO levels were quantified. SCFA levels were significantly higher in portal vs. hepatic and peripheral blood. There were inverse relationships between SCFAs and the severity of disease. SCFAs (mainly butyric acid) inversely correlated with the model for end-stage liver disease score and were further reduced in patients with history of ascites, hepatic encephalopathy, and spontaneous bacterial peritonitis. There was an inverse relationship between butyric acid and HVPG values. SCFAs were directly related with systemic vascular resistance and inversely with cardiac index. Butyric acid inversely correlated with inflammatory markers and serum endotoxin. A global reduction in the blood levels of SCFA in patients with cirrhosis is associated with a more advanced liver disease, suggesting its contribution to disease progression.-Juanola, O., Ferrusquía-Acosta, J., García-Villalba, R., Zapater, P., Magaz, M., Marín, A., Olivas, P., Baiges, A., Bellot, P., Turon, F., Hernández-Gea, V., González-Navajas, J. M., Tomás-Barberán, F. A., García-Pagán, J. C., Francés, R. Circulating levels of butyrate are inversely related to portal hypertension, endotoxemia, and systemic inflammation in patients with cirrhosis

Abdominal Surgery in Patients With Idiopathic Noncirrhotic Portal Hypertension: A Multicenter Retrospective Study

In patients with idiopathic noncirrhotic portal hypertension (INCPH), data on morbidity and mortality of abdominal surgery are scarce. We retrospectively analyzed the charts of patients with INCPH undergoing abdominal surgery within the Vascular Liver Disease Interest Group network. Forty‐four patients with biopsy‐proven INCPH were included. Twenty‐five (57%) patients had one or more extrahepatic conditions related to INCPH, and 16 (36%) had a history of ascites. Forty‐five procedures were performed, including 30 that were minor and 15 major. Nine (20%) patients had one or more Dindo‐Clavien grade ≥ 3 complication within 1 month after surgery. Sixteen (33%) patients had one or more portal hypertension–related complication within 3 months after surgery. Extrahepatic conditions related to INCPH (P = 0.03) and history of ascites (P = 0.02) were associated with portal hypertension–related complications within 3 months after surgery. Splenectomy was associated with development of portal vein thrombosis after surgery (P = 0.01). Four (9%) patients died within 6 months after surgery. Six‐month cumulative risk of death was higher in patients with serum creatinine ≥ 100 μmol/L at surgery (33% versus 0%, P < 0.001). An unfavorable outcome (i.e., either liver or surgical complication or death) occurred in 22 (50%) patients and was associated with the presence of extrahepatic conditions related to INCPH, history of ascites, and serum creatinine ≥ 100 μmol/L: 5% of the patients with none of these features had an unfavorable outcome versus 32% and 64% when one or two or more features were present, respectively. Portal decompression procedures prior to surgery (n = 10) were not associated with postoperative outcome. Conclusion: Patients with INCPH are at high risk of major surgical and portal hypertension–related complications when they harbor extrahepatic conditions related to INCPH, history of ascites, or increased serum creatinine

Hipertensión portal: Avances en su diagnóstico e impacto en el riesgo quirúrgico

[spa] La hipertensión portal es un síndrome clínico que consiste en el aumento patológico y sostenido de la presión en el territorio venoso portal. Su relevancia se ve determinada por las graves complicaciones que produce, las cuales condicionan una elevada morbilidad y mortalidad en pacientes con enfermedades crónicas del hígado. El gradiente de presión venosa hepática, la diferencia entre la presión suprahepática enclavada y la presión suprahepática libre, es la técnica de elección para evaluar la hipertensión portal en pacientes con cirrosis, ya que proporciona información pronóstica relevante relacionada con la supervivencia y el riesgo de descompensación. No obstante, su valor pronóstico depende de la existencia de una buena correlación entre la presión suprahepática enclavada y la presión portal. Por desgracia, los estudios que han demostrado que la presión suprahepática enclavada es capaz de estimar con precisión la presión portal no han incluido a pacientes con enfermedad hepática grasa no alcohólica, la cual se ha posicionado como la causa más frecuente de hepatopatía crónica en nuestro medio. Por otra parte, los avances continuos en las técnicas quirúrgicas y el manejo médico han dado lugar a un aumento en el número de pacientes con hipertensión portal que son derivados para una evaluación prequirúrgica. Por lo tanto, es fundamental comprender los riesgos y los beneficios de la realización de procedimientos quirúrgicos en pacientes con enfermedades crónicas del hígado. La mayoría del conocimiento actual sobre el riesgo quirúrgico en pacientes con hipertensión portal proviene de estudios realizados en pacientes con cirrosis. Sin embargo, la evidencia relacionada con el pronóstico postquirúrgico en pacientes con hipertensión portal idiopática es escasa y se limita a cirugías cada vez menos indicadas como la esplenectomía o la creación quirúrgica de una derivación portosistémica. Los trabajos incluidos en la presente tesis pretenden evaluar, por un lado, la correlación entre la presión suprahepática enclavada y la presión portal en pacientes con enfermedad hepática grasa no alcohólica, y por otro, el pronóstico postquirúrgico de los pacientes con hipertensión portal idiopática que son sometidos a una cirugía abdominal

Hipertensión portal: Avances en su diagnóstico e impacto en el riesgo quirúrgico

Programa de Doctorat en Medicina i Recerca Translacional / Tesi realitzada a l'Hospital Clínic de Barcelona[spa] La hipertensión portal es un síndrome clínico que consiste en el aumento patológico y sostenido de la presión en el territorio venoso portal. Su relevancia se ve determinada por las graves complicaciones que produce, las cuales condicionan una elevada morbilidad y mortalidad en pacientes con enfermedades crónicas del hígado. El gradiente de presión venosa hepática, la diferencia entre la presión suprahepática enclavada y la presión suprahepática libre, es la técnica de elección para evaluar la hipertensión portal en pacientes con cirrosis, ya que proporciona información pronóstica relevante relacionada con la supervivencia y el riesgo de descompensación. No obstante, su valor pronóstico depende de la existencia de una buena correlación entre la presión suprahepática enclavada y la presión portal. Por desgracia, los estudios que han demostrado que la presión suprahepática enclavada es capaz de estimar con precisión la presión portal no han incluido a pacientes con enfermedad hepática grasa no alcohólica, la cual se ha posicionado como la causa más frecuente de hepatopatía crónica en nuestro medio. Por otra parte, los avances continuos en las técnicas quirúrgicas y el manejo médico han dado lugar a un aumento en el número de pacientes con hipertensión portal que son derivados para una evaluación prequirúrgica. Por lo tanto, es fundamental comprender los riesgos y los beneficios de la realización de procedimientos quirúrgicos en pacientes con enfermedades crónicas del hígado. La mayoría del conocimiento actual sobre el riesgo quirúrgico en pacientes con hipertensión portal proviene de estudios realizados en pacientes con cirrosis. Sin embargo, la evidencia relacionada con el pronóstico postquirúrgico en pacientes con hipertensión portal idiopática es escasa y se limita a cirugías cada vez menos indicadas como la esplenectomía o la creación quirúrgica de una derivación portosistémica. Los trabajos incluidos en la presente tesis pretenden evaluar, por un lado, la correlación entre la presión suprahepática enclavada y la presión portal en pacientes con enfermedad hepática grasa no alcohólica, y por otro, el pronóstico postquirúrgico de los pacientes con hipertensión portal idiopática que son sometidos a una cirugía abdominal

Prevalence of Radiological Chronic Pancreatitis and Exocrine Pancreatic Insufficiency in Patients with Decompensated Liver Disease: Is Fecal Elastase Useful in This Setting?

Chronic alcohol consumption is a well-known etiological factor for both chronic pancreatitis (CP) and liver cirrhosis. However, there is discussion over how often these two entities are present together in the same patient. The main goal of our study is to establish the prevalence of CP and low fecal elastase (FE-1) in patients with decompensated liver disease (DLD). In addition, we aim to identify the demographic, epidemiological and clinical factors associated with EPI and CP in patients with decompensated liver cirrhosis. This was an observational single-center study including 119 consecutive patients hospitalized for acute decompensation of cirrhosis, mostly of alcoholic etiology. Patients underwent computed tomography (CT) or magnetic resonance imaging (MRI) to assess the radiological features of CP. We also performed two FE-1 tests and complete blood tests to assess the presence of exocrine pancreatic insufficiency (EPI) and nutritional status, including micronutrients. The results of our study show that 32 patients (26.9%) had low fecal elastase suggesting EPI and 11 (9.2%) had CP. Patients meeting radiological CP criteria had lower FE-1 than patients without CP. There were no statistically significant differences in micronutrient deficiencies according to the presence of CP or not. Likewise, we did not find any statistically significant differences in micronutrient deficiencies among patients with normal and low FE-1 indicative of EPI. FE-1 alone may not be suitable for assessing EPI in patients with acute DLD. Detecting co-existing pancreatic disease may be important in a subset of patients with DLD, when the FE-1 levels are significantly low, potentially suggestive of a pancreatic anomaly. Moreover, the clinical manifestations of EPI and CP are not useful in detecting CP in DLD patients. Likewise, CP cannot explain all causes of EPI in these patients

The ABO blood group locus and a chromosome 3 gene cluster associate with SARS-CoV-2 respiratory failure in an Italian-Spanish genome-wide association analysis

[Background] Respiratory failure is a key feature of severe Covid-19 and a critical driver of mortality, but for reasons poorly defined affects less than 10% of SARS-CoV-2 infected patients.[Methods] We included 1,980 patients with Covid-19 respiratory failure at seven centers in the Italian and Spanish epicenters of the SARS-CoV-2 pandemic in Europe (Milan, Monza, Madrid, San Sebastian and Barcelona) for a genome-wide association analysis. After quality control and exclusion of population outliers, 835 patients and 1,255 population-derived controls from Italy, and 775 patients and 950 controls from Spain were included in the final analysis. In total we analyzed 8,582,968 single-nucleotide polymorphisms (SNPs) and conducted a metaanalysis of both case-control panels.[Results] We detected cross-replicating associations with rs11385942 at chromosome 3p21.31 and rs657152 at 9q34, which were genome-wide significant (P<5×10-8) in the meta-analysis of both study panels, odds ratio [OR], 1.77; 95% confidence interval [CI], 1.48 to 2.11; P=1.14×10-10 and OR 1.32 (95% CI, 1.20 to 1.47; P=4.95×10-8), respectively. Among six genes at 3p21.31, SLC6A20 encodes a known interaction partner with angiotensin converting enzyme 2 (ACE2). The association signal at 9q34 was located at the ABO blood group locus and a blood-group-specific analysis showed higher risk for A-positive individuals (OR=1.45, 95% CI, 1.20 to 1.75, P=1.48×10-4) and a protective effect for blood group O (OR=0.65, 95% CI, 0.53 to 0.79, P=1.06×10-5).[Conclusions] We herein report the first robust genetic susceptibility loci for the development of respiratory failure in Covid-19. Identified variants may help guide targeted exploration of severe Covid19 pathophysiology.The IKMB's core facilities received infrastructure support by the Deutsche Forschungsgemeinschaft (DFG) Cluster of Excellence "Precision Medicine in Chronic Inflammation" (PMI, EXC2167). The project also received support through a philanthropic donation by Stein Erik Hagen and Canica AS. L.V. was funded by the Fondazione IRCCS Ca’ Granda «COVID-19 Biobank» research grant. This work was also supported by the Ministero dell’Istruzione, dell’Università e della Ricerca – MIUR project "Dipartimenti di Eccellenza 2018 – 2022" (n° D15D18000410001) to the Department of Medical Sciences, University of Torino. The IKMB authors received financial support from the UKSH Foundation "Gutes Tun!" (special thanks to Alexander Eck, Jenspeter Horst and Jens Scholz) and the German Federal Ministry of Education and Research (BMBF; grant ID 01KI20197). HLA-Typing was performed and supported by the Stefan-MorschStiftung. M.A.H was supported by the Spanish Ministry of Science and Innovation ‘JdC fellowship IJC2018-035131-I.N