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1. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

Author

Baiges, A., Turon, F., Simón-Talero, M., Tasayco, S., Bueno, J., Zekrini, K., Plessier, A., Franchi-Abella, S., Guerin, F., Mukund, A., Eapen, C.E., Goel, A., Shyamkumar, N.K., Coenen, S., Gottardi, A. De, Majumdar, A., Onali, S., Shukla, A., Carrilho, F.J., Nacif, L., Primignani, M., Tosetti, G., Mura, V. La, Nevens, F., Witters, P., Tripathi, D., Tellez, L., Martínez, J., Álvarez-Navascués, C., López, M.L. Fraile, Procopet, B., Piscaglia, F., Koning, B. de, Llop, E., Romero-Cristobal, M., Tjwa, E.T., Monescillo-Francia, A., Senzolo, M., Perez-LaFuente, M., Segarra, A., Sarin, S.K., Hernández-Gea, V., Patch, D., Laleman, W., Hartog, H., Valla, D., Genescà, J., García-Pagán, J.C., Baiges, A., Turon, F., Simón-Talero, M., Tasayco, S., Bueno, J., Zekrini, K., Plessier, A., Franchi-Abella, S., Guerin, F., Mukund, A., Eapen, C.E., Goel, A., Shyamkumar, N.K., Coenen, S., Gottardi, A. De, Majumdar, A., Onali, S., Shukla, A., Carrilho, F.J., Nacif, L., Primignani, M., Tosetti, G., Mura, V. La, Nevens, F., Witters, P., Tripathi, D., Tellez, L., Martínez, J., Álvarez-Navascués, C., López, M.L. Fraile, Procopet, B., Piscaglia, F., Koning, B. de, Llop, E., Romero-Cristobal, M., Tjwa, E.T., Monescillo-Francia, A., Senzolo, M., Perez-LaFuente, M., Segarra, A., Sarin, S.K., Hernández-Gea, V., Patch, D., Laleman, W., Hartog, H., Valla, D., Genescà, J., and García-Pagán, J.C.

Abstract

Contains fulltext : 220051.pdf (Publisher’s version ) (Closed access), Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty-six patients were included; median age at the end of follow-up was 30 years. Nineteen patients (28%) presented HE. Ten-, 20-, and 30-year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty-five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.

Published
2020

4. The role of liver steatosis as measured with transient elastography and transaminases on hard clinical outcomes in patients with COVID-19

Author

Campos Varela, Isabel, Villagrasa, Ares, Simon-Talero, Macarena, Riveiro Barciela, Mar, Ventura-Cots, Meritxell, Aguilera-Castro, Lara, Álvarez-López, Patricia, Nordahl, Emilie A., Anton, Adrian, Bañares, Juan, Barber, Claudia, Barreira-Diaz, Ana, Biagetti, Betina, Camps-Relats, Laura, Ciudin, Andreea, Cocera, Raul, Dopazo, Cristina, Fernandez, Andrea, Jiménez, Cesar, Jiménez, María M., Jofra, Mariona, Gil, Clara, Gómez Gavara, Concepción, Guanozzi, Danila, Guevara, Jorge A., Lobo, Beatriz, Malagelada Prats, Carolina, Martinez-Camprecios, Joan, Mayorga, Luis, Miret Alomar, Enric, Pando, Elizabeth, Pérez-Lopez, Ana, Pigrau Pastor, Marc, Prio, Alba, Rivera-Esteban, Jesus M., Romero, Alba, Tasayco, Staphanie, Vidal-Gonzalez, Judit, Vidal, Laura, Minguez, Beatriz, Augustin Recio, Salvador, Genescà, J., Universitat Autònoma de Barcelona, Institut Català de la Salut, [Campos-Varela I, Augustin S] Unitat del Fetge, Servei de Medicina Interna, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Villagrasa A, Alvarez-Lopez P, Anton A, Bañares J, Barreira-Diaz A, Camps-Relats L, Jimenez C, Gil C, Martinez-Camprecios J, Prio A, Rivera-Esteban JM, Romero A, Vidal-Gonzalez J] Unitat del Fetge, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. [Simon-Talero M, Riveiro-Barciela M, Ventura-Cots M, Minguez B, Genesca J] Unitat del Fetge, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Instituto de Salud Carlos III, Madrid, Spain. [Aguilera-Castro L, Barber C, Guanozzi D, Lobo B, Malagelada C, Mayorga L, Tasayco S] Servei de l’Aparell Digestiu, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Nordahl EA] Servei de Farmacologia Clínica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Biagetti B, Ciudin A] Servei d’Endocrinologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Cocera R, Miret E] Servei d’Urologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Dopazo C, Fernandez A, Jofra M, Gomez-Gavara C, Pando E, Vidal L] Servei de Cirurgia Hepatobiliopancreàtica i Trasplantaments, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Jimenez MM, Pérez-Lopez A] Servei d’Hematologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Gomez-Gavara C, Pigrau M] Servei d’Endoscòpia, Vall d’Hebron Hospital Universitari, Barcelona, Spain, and Vall d'Hebron Barcelona Hospital Campus

Subjects
medicine.medical_specialty, 2019-20 coronavirus outbreak, Coronavirus disease 2019 (COVID-19), ALT, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), RC799-869, Liver injury, Gastroenterology, Otros calificadores::Otros calificadores::/complicaciones [Otros calificadores], Liver disease, Liver steatosis, Internal medicine, virosis::infecciones por virus ARN::infecciones por Nidovirales::infecciones por Coronaviridae::infecciones por Coronavirus [ENFERMEDADES], Medicine, In patient, AST, Other subheadings::Other subheadings::/diagnostic imaging [Other subheadings], COVID-19 (Malaltia) - Complicacions, Original Research, business.industry, Virus Diseases::RNA Virus Infections::Nidovirales Infections::Coronaviridae Infections::Coronavirus Infections [DISEASES], Otros calificadores::Otros calificadores::/diagnóstico por imagen [Otros calificadores], Diseases of the digestive system. Gastroenterology, medicine.disease, CAP, controlled attenuation parameter, enfermedades del sistema digestivo::enfermedades hepáticas [ENFERMEDADES], Fetge - Malalties - Imatgeria, Digestive System Diseases::Liver Diseases [DISEASES], Controlled attenuation parameter, business, Transient elastography, Other subheadings::Other subheadings::/complications [Other subheadings], liver injury
Abstract

Coronavirus SARS-CoV-2; COVID-19; 2019-nCoV; Paràmetre d'atenuació controlat; Lesió hepàtica Coronavirus SARS-CoV-2; COVID-19; 2019-nCoV; Parámetro de atenuación controlado; Daño hepático Coronavirus SARS-CoV-2; COVID-19; 2019-nCoV; Controlled attenuation parameter; Liver injury Liver injury has been widely described in patients with Coronavirus disease 2019 (COVID-19). We aimed to study the effect of liver biochemistry alterations, previous liver disease, and the value of liver elastography on hard clinical outcomes in COVID-19 patients. We conducted a single-center prospective observational study in 370 consecutive patients admitted for polymerase chain reaction (PCR)-confirmed COVID-19 pneumonia. Clinical and laboratory data were collected at baseline and liver parameters and clinical events recorded during follow-up. Transient elastography [with Controlled Attenuation Parameter (CAP) measurements] was performed at admission in 98 patients. All patients were followed up until day 28 or death. The two main outcomes of the study were 28-day mortality and the occurrence of the composite endpoint intensive care unit (ICU) admission and/or death. Alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels were elevated at admission in 130 patients (35%) and 167 (45%) patients, respectively. Overall, 14.6% of patients presented the composite endpoint ICU and/or death. Neither ALT elevations, prior liver disease, liver stiffness nor liver steatosis (assessed with CAP) had any effect on outcomes. However, patients with abnormal baseline AST had a higher occurrence of the composite ICU/death (21% versus 9.5%, p = 0.002). Patients ⩾65 years and with an AST level > 50 U/ml at admission had a significantly higher risk of ICU and/or death than those with AST ⩽ 50 U/ml (50% versus 13.3%, p

Published
2021

5. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study

Author

Baiges, Anna, Turon, Fanny, Simon-Talero, Macarena, Tasayco, Stephanie, Bueno, Javier, Zekrini, Kamal, Plessier, Aurelie, Franchi-Abella, Stephanie, Guerin, Florent, Mukund, Amar, Eapen, CE, Goel, Ashish, Shyamkumar, Nidugala Keshava, Coenen, Sandra, De Gottardi, Andrea, Majumdar, Avik, Onali, Simona, Shukla, Akash, Jose Carrilho, Flair, Nacif, Lucas, Primignani, Massimo, Tosetti, Giulia, La Mura, Vicenzo, Nevens, Frederik, Witters, Peter, Tripathi, Dhiraj, Tellez, Luis, Martinez, Javier, Alvarez-Navascues, Carmen, Fraile Lopez, Miguel Lopez, Procopet, Bogdan, Piscaglia, Fabio, de Koning, Barbara, Llop, Elba, Romero-Cristobal, Mario, Tjwa, Eric, Monescillo-Francia, Alberto, Senzolo, Marco, Perez-LaFuente, Mercedes, Segarra, Antonio, Sarin, Shiv Kumar, Hernandez-Gea, Virginia, Patch, David, Laleman, Wim, Hartog, Hermien, Valla, Dominique, Genesca, Joan, Carlos Garcia-Pagan, Juan, Garcia-Criado, Angeles, Darnell, Anna, Belmonte, Ernest, Ferrusquia-Acosta, Jose, Magaz, Marta, Vidal-Gonzalez, Judit, Horia, Stefanescu, Nicoara-Farcau, Oana, Joseph, Philip, Zachariah, Uday, Moses, Vinu, Mammen, Suraj, Ahmed, Munawwar, Koshy, George, Eapen, Anu, Sajith, KG, Vyas, FL, Raju, RS, Rymbai, Manbha L, Arulkumar, S, Ramachandran, Jeyamani, Elias, Elwyn, Baiges A., Turon F., Simon-Talero M., Tasayco S., Bueno J., Zekrini K., Plessier A., Franchi-Abella S., Guerin F., Mukund A., Eapen C.E., Goel A., Shyamkumar N.K., Coenen S., De Gottardi A., Majumdar A., Onali S., Shukla A., Carrilho F.J., Nacif L., Primignani M., Tosetti G., La Mura V., Nevens F., Witters P., Tripathi D., Tellez L., Martinez J., Alvarez-Navascues C., Fraile Lopez M.L., Procopet B., Piscaglia F., de Koning B., Llop E., Romero-Cristobal M., Tjwa E., Monescillo-Francia A., Senzolo M., Perez-LaFuente M., Segarra A., Sarin S.K., Hernandez-Gea V., Patch D., Laleman W., Hartog H., Valla D., Genesca J., Garcia-Pagan J.C., Gastroenterology & Hepatology, Pediatrics, and Surgery

Subjects
0301 basic medicine, Male, Vascular Malformations, medicine.medical_treatment, International Cooperation, Liver transplantation, 0302 clinical medicine, Malalties hereditàries, Hepatopulmonary syndrome, 610 Medicine & health, Child, Hepatic encephalopathy, Portal Vein, Malalties del fetge, Liver Neoplasms, vascular diseases of the liver, Middle Aged, Abernethy, Child, Preschool, 030211 gastroenterology & hepatology, Female, medicine.symptom, Genetic diseases, Adult, medicine.medical_specialty, Adenoma, Adolescent, Hypertension, Pulmonary, Asymptomatic, 03 medical and health sciences, Young Adult, extrahepatic portosytemic shunt, medicine, Humans, Liver diseases, Aged, Retrospective Studies, Abernethy Malformation, Hepatology, business.industry, Infant, Retrospective cohort study, medicine.disease, Pulmonary hypertension, Surgery, Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11], 030104 developmental biology, Hepatic Encephalopathy, Congenital malformation, Portosystemic shunt, business, Hepatopulmonary Syndrome
Abstract

Contains fulltext : 220051.pdf (Publisher’s version ) (Closed access) Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty-six patients were included; median age at the end of follow-up was 30 years. Nineteen patients (28%) presented HE. Ten-, 20-, and 30-year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty-five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach.

Published
2018

6. Elevated Serum Triglyceride Levels in Acute Pancreatitis: A Parameter to be Measured and Considered Early.

Author

Hidalgo NJ, Pando E, Alberti P, Vidal L, Mata R, Fernandez N, Gomez-Jurado MJ, Dopazo C, Blanco L, Tasayco S, Molero X, Balsells J, and Charco R

Subjects
Acute Disease, Humans, Multiple Organ Failure, Retrospective Studies, Severity of Illness Index, Triglycerides, Pancreatitis, Acute Necrotizing
Abstract

Background: The value of serum triglycerides (TGs) related to complications and the severity of acute pancreatitis (AP) has not been clearly defined. Our study aimed to analyze the association of elevated levels of TG with complications and the severity of AP., Methods: The demographic and clinical data of patients with AP were prospectively analyzed. TG levels were measured in the first 24 h of admission. Patients were divided into two groups: one with TG values of<200 mg/dL and another with TG≥200 mg/dL. Data on the outcomes of AP were collected., Results: From January 2016 to December 2019, 247 cases were included: 200 with TG<200 mg/dL and 47 with TG≥200 mg/dL. Triglyceride levels≥200 mg/dL were associated with respiratory failure (21.3 vs. 10%, p=0.033), renal failure (23.4 vs. 12%, p=0.044), cardiovascular failure (19.1 vs. 7.5%, p=0.025), organ failure (34 vs. 18.5%, p=0.02), persistent organ failure (27.7 vs. 9.5%, p=0.001), multiple organ failure (19.1 vs. 8%, p=0.031), moderately severe and severe AP (68.1 vs. 40.5%, p=0.001), pancreatic necrosis (63.8 vs. 34%, p<0.001), and admission to the intensive care unit (27.7 vs. 9.5%, p=0.003). In the multivariable analysis, a TG level of≥200 mg/dL was independently associated with respiratory, renal, and cardiovascular failure, organ failure, persistent organ failure, multiple organ failure, pancreatic necrosis, severe pancreatitis, and admission to the intensive care unit (p<0.05)., Conclusions: In our cohort, TG≥200 mg/dL was related to local and systemic complications. Early determinations of TG levels in AP could help identify patients at risk of complications., (© 2022. The Author(s).)

Published
2022
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8. Prevalence, features and predictive factors of liver nodules in Fontan surgery patients: The VALDIG Fonliver prospective cohort.

Author

Téllez L, Rodríguez de Santiago E, Minguez B, Payance A, Clemente A, Baiges A, Morales-Arraez D, La Mura V, Llop E, Garrido E, Garrido-Lestache E, Tasayco S, Bruno O, Prieto R, Montserrat S, Pons M, Olavarría A, Dos L, Legendre A, Jesús Del Cerro M, Bañares R, García-Pagán JC, Rautou PE, and Albillos A

Subjects
Adult, Carcinoma, Hepatocellular diagnostic imaging, Cross-Sectional Studies, Europe epidemiology, Female, Humans, Liver Neoplasms diagnostic imaging, Magnetic Resonance Imaging methods, Male, Prevalence, Prognosis, Prospective Studies, Risk Factors, Tomography, X-Ray Computed methods, Ultrasonography methods, Young Adult, Carcinoma, Hepatocellular epidemiology, Carcinoma, Hepatocellular etiology, Fontan Procedure adverse effects, Heart Defects, Congenital surgery, Liver Neoplasms epidemiology, Liver Neoplasms etiology
Abstract

Background & Aims: Fontan surgery is used to treat a variety of congenital heart malformations, and may lead to advanced chronic liver disease in the long-term. This study examines the prevalence, characteristics and predictors of liver nodules in patients following Fontan surgery., Methods: This was a prospective, cross-sectional, observational study conducted at 8 European centres. Consecutive patients who had undergone Fontan surgery underwent blood tests, abdominal ultrasonography (US), transient elastography (Fibroscan®), echocardiography, haemodynamic assessments, and abdominal MRI/CT scan. The primary outcome measure was liver nodules detected in the MRI/CT scan. Predictors of liver nodules were identified by multivariate logistic regression., Results: One hundred and fifty-two patients were enrolled (mean age 27.3 years). The mean time elapsed from surgery to inclusion was 18.3 years. Liver nodule prevalences were 29.6% (95% CI 23-37%) on US and 47.7% (95% CI 39-56%) on MRI/CT. Nodules were usually hyperechoic (76.5%), round-shaped (>80%), hyperenhancing in the arterial phase (92%) and located in the liver periphery (75%). The sensitivity and specificity of US were 50% (95% CI 38-62%) and 85.3% (95% CI 75-92%), respectively. Inter-imaging test agreement was low (adjusted kappa: 0.34). In the multivariate analysis, time since surgery >10 years was the single independent predictor of liver nodules (odds ratio 4.18; p = 0.040). Hepatocellular carcinoma was histologically diagnosed in 2 of the 8 patients with hypervascular liver nodules displaying washout., Conclusion: While liver nodules are frequent in Fontan patients, they may go unnoticed in US. Liver nodules are usually hyperechoic, hypervascular and predominantly peripheral. This population is at risk of hepatocellular carcinoma, the diagnosis of which requires confirmatory biopsy., Lay Summary: Fontan surgery is the standard of care for many patients with univentricular congenital cardiopathies. Recent advances have improved the survival of Fontan patients, and nowadays most of them reach adulthood. In this setting, Fontan-associated liver disease (FALD) is increasingly recognised, and has become a significant prognostic factor. Liver nodules are considered a component of FALD yet their prevalence, imaging features and predictors have hardly been evaluated. In this study, we observed that liver nodules are frequent, typically hyperechoic, hypervascular and predominantly peripheral in patients with FALD. This population is at risk of hepatocellular carcinoma, the diagnosis of which must be confirmed by biopsy., (Copyright © 2019 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.)

Published
2020
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9. Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation): An International Observational Study.

Author

Baiges A, Turon F, Simón-Talero M, Tasayco S, Bueno J, Zekrini K, Plessier A, Franchi-Abella S, Guerin F, Mukund A, Eapen CE, Goel A, Shyamkumar NK, Coenen S, De Gottardi A, Majumdar A, Onali S, Shukla A, Carrilho FJ, Nacif L, Primignani M, Tosetti G, La Mura V, Nevens F, Witters P, Tripathi D, Tellez L, Martínez J, Álvarez-Navascués C, Fraile López ML, Procopet B, Piscaglia F, de Koning B, Llop E, Romero-Cristobal M, Tjwa E, Monescillo-Francia A, Senzolo M, Perez-LaFuente M, Segarra A, Sarin SK, Hernández-Gea V, Patch D, Laleman W, Hartog H, Valla D, Genescà J, and García-Pagán JC

Subjects
Adolescent, Adult, Aged, Child, Child, Preschool, Female, Hepatic Encephalopathy epidemiology, Hepatopulmonary Syndrome epidemiology, Humans, Hypertension, Pulmonary epidemiology, Infant, International Cooperation, Liver Neoplasms epidemiology, Male, Middle Aged, Retrospective Studies, Vascular Malformations diagnosis, Young Adult, Hepatic Encephalopathy etiology, Hepatopulmonary Syndrome etiology, Hypertension, Pulmonary etiology, Liver Neoplasms etiology, Portal Vein abnormalities, Vascular Malformations complications
Abstract

Congenital extrahepatic portosystemic shunt (CEPS) or Abernethy malformation is a rare condition in which splanchnic venous blood bypasses the liver draining directly into systemic circulation through a congenital shunt. Patients may develop hepatic encephalopathy (HE), pulmonary hypertension (PaHT), or liver tumors, among other complications. However, the actual incidence of such complications is unknown, mainly because of the lack of a protocolized approach to these patients. This study characterizes the clinical manifestations and outcome of a large cohort of CEPS patients with the aim of proposing a guide for their management. This is an observational, multicenter, international study. Sixty-six patients were included; median age at the end of follow-up was 30 years. Nineteen patients (28%) presented HE. Ten-, 20-, and 30-year HE incidence rates were 13%, 24%, and 28%, respectively. No clinical factors predicted HE. Twenty-five patients had benign nodular lesions. Ten patients developed adenomas (median age, 18 years), and another 8 developed HCC (median age, 39 years). Of 10 patients with dyspnea, PaHT was diagnosed in 8 and hepatopulmonary syndrome in 2. Pulmonary complications were only screened for in 19 asymptomatic patients, and PaHT was identified in 2. Six patients underwent liver transplantation for hepatocellular carcinoma or adenoma. Shunt closure was performed in 15 patients with improvement/stability/cure of CEPS manifestations. Conclusion: CEPS patients may develop severe complications. Screening for asymptomatic complications and close surveillance is needed. Shunt closure should be considered both as a therapeutic and prophylactic approach., (© 2019 by the American Association for the Study of Liver Diseases.)

Published
2020
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