المؤلفون: Riveiro‐Barciela, Mar, Barreira‐Díaz, Ana, Esteban, Paula, Rota, Rosa, Álvarez‐Navascúes, Carmen, Pérez‐Medrano, Indhira, Mateos, Beatriz, Gómez, Elena, De‐la‐Cruz, Gema, Ferre‐Aracil, Carlos, Horta, Diana, Díaz‐González, Álvaro, Ampuero, Javier, Díaz‐Fontenla, Fernando, Salcedo, Magdalena, Ruiz‐Cobo, Juan‐Carlos, Londoño, María‐Carlota

المصدر: Liver International ; ISSN 1478-3223 1478-3231

الوصف: Background and Aims Small series suggest that rituximab could be effective as treatment for autoimmune hepatitis (AIH), although data are scarce. We aimed to evaluate the efficacy and safety of rituximab in different cohorts of patients with AIH. Methods Multicentre retrospective analysis of the 35 patients with AIH and its variant forms treated with rituximab and included in the ColHai registry between 2015 and 2023. Results Most patients were female (83%), 10 (29%) had cirrhosis and four (11.4%) variant forms of AIH. Indication for rituximab were as follows: 14(40%) refractory AIH, 19(54%) concomitant autoimmune or haematological disorder, 2(6%) intolerance to prior treatments. In three (9%) subjects with a concomitant disorder, rituximab was the first therapy for AIH. Overall, 31 (89%) patients achieved or maintained complete biochemical response (CBR), including the three in first‐line therapy. No difference in CBR was observed according to rituximab indication (refractory AIH 86% vs. concomitant disorders 90%, p = .824) or cirrhosis (80% vs. 92%, p = .319). Rituximab was associated with a significant reduction in corticosteroids (median dose: prior 20 vs. post 5 mg, p < .001) and the discontinuation of ≥1 immunosuppressant in 47% of patients. Flare‐free rate at 1st, 2nd and 3rd year was 86%, 73% and 62% respectively. Flares were not associated with the development of liver failure and were successfully managed with repeated doses of rituximab and/or increased corticosteroids. Three (9%) patients experienced infusion‐related adverse events (1 anaphylaxis and 2 flu‐like symptoms) and five (14%) infections. Conclusion Rituximab is safe and effective in patients with refractory AIH and those treated due to concomitant autoimmune or haematological disorders.

الإتاحة: https://doi.org/10.1111/liv.15970Test

المؤلفون: Gil, José Vicente, Miralles, Alberto, de las Heras, Sandra, Such, Esperanza, Avetisyan, Gayane, Díaz-González, Álvaro, Santiago, Marta, Fuentes, Carolina, Fernández, José María, Lloret, Pilar, Navarro, Irene, Montesinos, Pau, Llop, Marta, Barragán, Eva

المصدر: Frontiers in Molecular Biosciences ; volume 11 ; ISSN 2296-889X

مصطلحات موضوعية: Biochemistry, Genetics and Molecular Biology (miscellaneous), Molecular Biology, Biochemistry

الوصف: Introduction: Acute lymphoblastic leukemia (ALL) is a prevalent childhood cancer with high cure rate, but poses a significant medical challenge in adults and relapsed patients. Philadelphia-like acute lymphoblastic leukemia (Ph-like ALL) is a high-risk subtype, with approximately half of cases characterized by CRLF2 overexpression and frequent concomitant IKZF1 deletions. Methods: To address the need for efficient, rapid, and cost-effective detection of CRLF2 alterations, we developed a novel RT-qPCR technique combining SYBR Green and highresolution melting analysis on a single plate. Results: The method successfully identified CRLF2 expression, P2RY8::CRLF2 fusions, and CRLF2 and JAK2 variants, achieving a 100% sensitivity and specificity. Application of this method across 61 samples revealed that 24.59% exhibited CRLF2 overexpression, predominantly driven by IGH::CRLF2 (73.33%). High Resolution Melting analysis unveiled concurrent CRLF2 or JAK2 variants in 8.19% of samples, as well as a dynamic nature of CRLF2 alterations during disease progression. Discussion: Overall, this approach provides an accurate identification of CRLF2 alterations, enabling improved diagnostic and facilitating therapeutic decision-making.

الإتاحة: https://doi.org/10.3389/fmolb.2024.1362081Test

المؤلفون: Barreira-Díaz, Ana, Riveiro-Barciela, Mar, Fernández-Bonilla, Eva María, Bernal, Vanesa, Castiella, Agustín, Casado-Martín, Marta, Delgado, Carolina, Londoño, María-Carlota, Díaz-González, Álvaro, Pérez-Medrano, Indhira, Conthe, Andrés, Sala, Margarita, Mateos, Beatriz, Gómez-Camarero, Judith, Antón-Conejero, Dolores, Pozo-Calzada, Carmen Del, Cuenca, Francisca, Villagrasa-Vilella, Ares, Salcedo, Magdalena

المصدر: Annals of Hepatology ; volume 29, issue 3, page 101489 ; ISSN 1665-2681

مصطلحات موضوعية: Hepatology, General Medicine

الإتاحة: https://doi.org/10.1016/j.aohep.2024.101489Test
https://api.elsevier.com/content/article/PII:S1665268124002837?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S1665268124002837?httpAccept=text/plainTest

المؤلفون: Díaz González, Álvaro, Hernández Guerra, Manuel, Pérez Medrano, Indhira, Sapena, Víctor, Riveiro Barciela, Mar, Barreira Díaz, Ana, Gómez, Elena, Morillas, Rosa M., Barrio, María del, Escudé, Laia, Mateos, Beatriz, Horta, Diana, Gómez, Judith, Conde, Isabel, Ferre Aracil, Carlos, El Hajra, Ismael, Arencibía, Ana, Zamora, Javier, Fernández, Ainhoa, Salcedo, Magdalena, Molina, Esther, Soria, Anna, Estévez, Pamela, López, Carmen, Álvarez Navascués, Carmen, García Retortillo, Montserrat, Crespo, Javier, Londoño, María Carlota, ColHai Registry

المصدر: Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)

مصطلحات موضوعية: Hepatitis, Malalties autoimmunitàries, Corticosteroides, Autoimmune diseases, Adrenocortical hormones

الوصف: In patients with non-severe acute or chronic autoimmune hepatitis (AIH) without cirrhosis, clinical practice guidelines recommend indistinct use of prednisone or budesonide. However, budesonide is infrequently used in clinical practice. We aimed to describe its use and compare its efficacy and safety with prednisone as first-line options.This was a retrospective, multicenter study of 105 naive AIH patients treated with budesonide as the first-line drug. The control group included 276 patients treated with prednisone. Efficacy was assessed using logistic regression and validated using inverse probability of treatment weighting propensity score. The median time to biochemical response (BR) was 3.1 months in patients treated with budesonide and 4.9 months in those with prednisone. The BR rate was significantly higher in patients treated with prednisone (87% vs. 49% of patients with budesonide, p < 0.001). The probability of achieving BR, assessed using the inverse probability of treatment weighting propensity score, was significantly lower in the budesonide group (OR = 0.20; 95% CI: 0.11-0.38) at any time during follow-up, and at 6 (OR = 0.51; 95% CI: 0.29-0.89) and 12 months after starting treatment (0.41; 95% CI: 0.23-0.73). In patients with transaminases <2 × upper limit of normal, BR was similar in both treatment groups. Prednisone treatment was significantly associated with a higher risk of adverse events (24.2% vs. 15.9%, p = 0.047).In the real-life setting, the use of budesonide as first-line treatment is low, and it is generally prescribed to patients with perceived less disease activity. Budesonide was inferior to prednisone as a first-line drug but was associated with fewer side effects.Copyright © 2023 American Association for the Study of Liver Diseases.

وصف الملف: 39 p.; application/pdf

العلاقة: Reproducció del document publicat a: https://doi.org/10.1097/hep.0000000000000018Test; Hepatology, 2023, vol. 77, num. 4, p. 1095-1105; https://doi.org/10.1097/hep.0000000000000018Test; http://hdl.handle.net/2445/203640Test; 9336848

الإتاحة: https://doi.org/10.1097/hep.0000000000000018Test
http://hdl.handle.net/2445/203640Test

المؤلفون: Díaz González, Álvaro, Avetisyan, Gayane, Garcia-Ruiz, Cristian, Vicente Gil, José, Santiago, Marta, José Domínguez-García, Juan, Llop, Marta, Barragan, Eva, Leonor Senent Peris, Maria, DE LA Rubia, Javier, Cervera, José, Such, Esperanza

المصدر: HemaSphere ; volume 7, issue S3, page e8338249 ; ISSN 2572-9241

الإتاحة: https://doi.org/10.1097/01.hs9.0000976612.83382.49Test

المؤلفون: Díaz-González, Álvaro, Fontanillas, Noelia, Gil-Hernández, Eva, Guilabert, Mercedes, Londoño, Maria-Carlota, Noguerol, Mar, Pérez Escanilla, Fernando, Mira, José J., Santiñá, Manuel

المساهمون: Advanz Pharma Canada Inc

المصدر: Gastroenterología y Hepatología ; ISSN 0210-5705

مصطلحات موضوعية: Gastroenterology, Hepatology

الإتاحة: https://doi.org/10.1016/j.gastrohep.2023.12.002Test
https://api.elsevier.com/content/article/PII:S0210570523004922?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S0210570523004922?httpAccept=text/plainTest

المؤلفون: Díaz‐González, Álvaro, Forner, Alejandro, Turnes, Juan

المصدر: Liver International ; volume 44, issue 7, page 1575-1577 ; ISSN 1478-3223 1478-3231

الإتاحة: https://doi.org/10.1111/liv.15952Test

المؤلفون: Ampuero, Javier, Lucena, Ana, Berenguer, Marina, Hernández-Guerra, Manuel, Molina, Esther, Gómez-Camarero, Judith, Valdivia, Carlos, Gómez, Elena, Casado, Marta, Álvarez-Navascuez, Carmen, Jorquera, Francisco, García-Buey, Luisa, Díaz-González, Álvaro, Morillas, Rosa, García-Retortillo, Montserrat, Sousa, Jose M., Pérez-Medrano, Indhira, Simón, Miguel Á., Martínez, Javier, Arenas, Juan, Londoño, María Carlota, Olveira, Antonio, Fernández-Rodríguez, Conrado

المصدر: Hepatology ; ISSN 0270-9139

الوصف: Background and Aims: The landscape in primary biliary cholangitis (PBC) has changed with the advent of second-line treatments. However, the use of obeticholic acid (OCA) and fibrates in PBC-related cirrhosis is challenging. We assessed the impact of receiving a second-line therapy as a risk factor for decompensated cirrhosis in a real-world population with cirrhosis and PBC, and identify the predictive factors for decompensated cirrhosis in these patients. Approach and Results: Multicenter study enrolling 388 patients with PBC-cirrhosis from the Spanish ColHai registry. Biopsy (20%), ultrasound (59%), or transient elastography (21%) defined cirrhosis, and the presence of varices and splenomegaly defined clinically significant portal hypertension (CSPH). Paris-II and PBC OCA international study of efficacy criteria determined the response to ursodeoxycholic acid (UDCA), fibrates (n=93), and OCA (n=104). The incidence of decompensated cirrhosis decreased for UDCA versus OCA or fibrates in the real-world population, but they were similar considering the propensity score–matched cohort (UDCA 3.77 vs. second-line therapy 4.5 100 persons-year, respectively), as patients on second-line therapy exhibited advanced liver disease. Consequently, GGT, albumin, platelets, clinically significant portal hypertension, and UDCA response were associated with a decompensating event. OCA response (achieved in 52% of patients) was associated with bilirubin (OR 0.21 [95% CI: 0.06–0.73]) and AST (OR 0.97 [95% CI: 0.95–0.99]), while fibrate response (achieved in 55% of patients) with AST [OR 0.96 (95% CI: 0.95–0.98]). In patients treated with OCA, drug response (sHR 0.23 [95% CI: 0.08–0.64]), diabetes (sHR 5.62 [95% CI: 2.02–15.68]), albumin (sHR 0.34 [95% CI: 0.13–0.89]), and platelets (sHR 0.99 [95% CI: 0.98–1.00]) were related to decompensation. In patients treated with fibrate, drug response (sHR 0.36 (95% CI: 0.14–0.95]), albumin (sHR 0.36 (95% CI: 0.16–0.81]), and clinically significant portal hypertension (sHR 3.70 (95% CI: ...

الإتاحة: https://doi.org/10.1097/hep.0000000000000826Test

المؤلفون: Marti-Aguado, David, Pazó, Javier, Diaz-Gonzalez, Alvaro, de las Heras Páez de la Cadena, Berta, Conthe, Andres, Gallego Duran, Rocio, Rodríguez-Gandía, Miguel A., Turnes, Juan, Romero-Gomez, Manuel

المصدر: Gastroenterología y Hepatología ; ISSN 0210-5705

مصطلحات موضوعية: Gastroenterology, Hepatology

الإتاحة: https://doi.org/10.1016/j.gastrohep.2024.04.001Test
https://api.elsevier.com/content/article/PII:S021057052400075X?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S021057052400075X?httpAccept=text/plainTest

المؤلفون: Riveiro-Barciela, Mar, Carballal, Sabela, Díaz-González, Álvaro, Mañosa, Míriam, Gallego-Plazas, Javier, Cubiella, Joaquín, Jiménez-Fonseca, Paula, Varela, María, Menchén, Luis, Sangro, Bruno, Fernández-Montes, Ana, Mesonero, Francisco, Rodríguez-Gandía, Miguel Ángel, Rivera, Fernando, Londoño, María-Carlota

المصدر: Gastroenterología y Hepatología ; volume 47, issue 4, page 401-432 ; ISSN 0210-5705

مصطلحات موضوعية: Gastroenterology, Hepatology

الإتاحة: https://doi.org/10.1016/j.gastrohep.2023.10.009Test
https://api.elsevier.com/content/article/PII:S0210570523004557?httpAccept=text/xmlTest
https://api.elsevier.com/content/article/PII:S0210570523004557?httpAccept=text/plainTest