Global survey of stigma among physicians and patients with nonalcoholic fatty liver disease.

BACKGROUND & AIMS: Patients with fatty liver disease may experience stigma from the disease or comorbidities. In this cross-sectional study, we aimed to understand stigma among patients with nonalcoholic fatty liver disease (NAFLD)/nonalcoholic steatohepatitis (NASH) and healthcare providers. METHODS: Members of the Global NASH Council created two surveys about experiences/attitudes toward NAFLD and related diagnostic terms: a 68-item patient and a 41-item provider survey. RESULTS: Surveys were completed by 1,976 patients with NAFLD across 23 countries (51% Middle East/North Africa [MENA], 19% Europe, 17% USA, 8% Southeast Asia, 5% South Asia) and 825 healthcare providers (67% gastroenterologists/hepatologists) across 25 countries (39% MENA, 28% Southeast Asia, 22% USA, 6% South Asia, 3% Europe). Of all patients, 48% ever disclosed having NAFLD/NASH to family/friends; the most commonly used term was "fatty liver" (88% at least sometimes); "metabolic disease" or "MAFLD" were rarely used (never by >84%). Regarding various perceptions of diagnostic terms by patients, there were no substantial differences between "NAFLD", "fatty liver disease (FLD)", "NASH", or "MAFLD". The most popular response was being neither comfortable nor uncomfortable with either term (56%-71%), with slightly greater discomfort with "FLD" among the US and South Asian patients (47-52% uncomfortable). Although 26% of patients reported stigma related to overweight/obesity, only 8% reported a history of stigmatization or discrimination due to NAFLD. Among providers, 38% believed that the term "fatty" was stigmatizing, while 34% believed that "nonalcoholic" was stigmatizing, more commonly in MENA (43%); 42% providers (gastroenterologists/hepatologists 45% vs. 37% other specialties, p = 0.03) believed that the name change to metabolic dysfunction-associated steatotic liver disease (or MASLD) might reduce stigma. Regarding the new nomenclature, the percentage of providers reporting "steatotic liver disease" as stigmatizing was low (14%). CONCLUSIONS: The perception of NAFLD stigma varies among patients, providers, geographic locations and sub-specialties. IMPACT AND IMPLICATIONS: Over the past decades, efforts have been made to change the nomenclature of nonalcoholic fatty liver disease (NAFLD) to better align with its underlying pathogenetic pathways and remove any potential stigma associated with the name. Given the paucity of data related to stigma in NAFLD, we undertook this global comprehensive survey to assess stigma in NAFLD among patients and providers from around the world. We found there is a disconnect between physicians and patients related to stigma and related nomenclature. With this knowledge, educational programs can be developed to better target stigma in NAFLD among all stakeholders and to provide a better opportunity for the new nomenclature to address the issues of stigma.

Severe impairment of patient-reported outcomes in patients with chronic hepatitis C virus infection seen in real-world practices across the world: Data from the global liver registry.

Cure of chronic hepatitis C (CHC) can lead to improvement of health-related quality of life and other patient-reported outcomes (PROs). While extensive PRO data for CHC patients who were enrolled in clinical trials are available, similar data for patients seen in real-world practices are scarce. Our aim was to assess PROs of CHC patients enrolled from real-world practices from different regions and to compare them with those enrolled in clinical trials. CHC patients seen in clinical practices and not receiving treatment were enrolled in the Global Liver Registry (GLR). Clinical and PRO (FACIT-F, CLDQ-HCV, WPAI) data were collected and compared with the baseline data from CHC patients enrolled in clinical trials. N = 12,171 CHC patients were included (GLR n = 3146, clinical trial subjects n = 9025). Patients were from 30 countries from 6 out of 7 Global Burden of Disease (GBD) super-regions. Compared with clinical trial enrollees, patients from GLR were less commonly enrolled from High-Income GBD super-region, older, more commonly female, less employed, had more type 2 diabetes, anxiety and clinically overt fatigue but less cirrhosis (all p < 0.001). Out of 15 PRO domain and summary scores, 12 were lower in GLR patients than in subjects enrolled in clinical trials (p < 0.001). In multiple regression models, anxiety, depression, and fatigue were associated with significant PRO impairment in CHC patients (p < 0.05). After adjustment for the clinico-demographic confounders, the association of PRO scores of CHC patients with enrolment settings was no longer significant (all p > 0.05). In conclusion, hepatitis C patients seen in the real-world practices have PRO impairment driven by fatigue and psychiatric comorbidities.

Health-related quality of life in Cuban patients with chronic liver disease: A real-world experience.

INTRODUCTION AND OBJECTIVES: Patient-reported outcomes (PROs) are important for comprehensive assessment of chronic liver disease (CLD). Latin America and the Caribbean have a high burden of CLD, but PROs are lacking. We assessed health-related quality of life (HRQL) in Cuban patients with compensated CLD. MATERIALS AND METHODS: A cross sectional study performed of adult patients with a diagnosis of chronic viral infection B and C (HBV, HCV), non-alcoholic fatty liver diseases (NAFLD) and autoimmune liver diseases (AILD) including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and overlap syndrome (AIH+PBC). PROs were collected using: Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F), Work Productivity and Activity-Specific Health Problem (WPAI: SHP), and the Chronic Liver Disease Questionnaire (CLDQ)-disease-specific. RESULTS: 543 patients enrolled, n=91 (HBV), n=188 (HCV), n=221 (NAFLD), n=43 (AILD). Of those with AILD, 22 had AIH, 14 PBC, and 7 overlap AIH/PBC. Mean age was 53.5 years, 64.1% female, 69.2% white, and 58.0% employed. Patients with HCV and AILD had more severe liver disease. A significant impairment in PROs was observed in HCV group whereas the AILD patients had more activity impairment. CLDQ-HRQL scores were significantly lower for patients with NAFLD and AILD compared to HBV. Male gender and exercising ≥90min/week predicted better HRQL. The strongest independent predictors of HRQL impairment were fatigue, abdominal pain, anxiety, and depression (p<0.05). CONCLUSIONS: HRQL for Cuban patients with compensated CLD differs according to the CLD etiology. Patients with HCV and AILD had the worst PRO scores most likely related to severe underlying liver disease and/or extrahepatic manifestations.

Autoimmune Hepatitis in Cuban Patients: A Retrospective Analysis of Clinical and Histological Profiles, Treatments, and Outcomes.

BACKGROUND: Population-based studies on the clinical course and prognosis of autoimmune hepatitis (AIH) from Caribbean countries are limited. OBJECTIVE: The aim of this study was to provide information regarding the clinical and laboratory findings, histological profile, treatments, and outcomes of patients with AIH with long-term follow-up in a tertiary referral center. METHODS: A retrospective study was performed at the National Institute of Gastroenterology in Havana, Cuba, by enrolling 82 patients with a well-documented, long-term clinical course of AIH. Clinical and laboratory findings, histological profiles, treatments, and outcomes were analyzed. RESULTS: At diagnosis, 73 (89%) patients had AIH type 1, 84.1% were women, and their median age was 46.5 years (range, 17-79 years). The median follow-up period was 84 months (interquartile range, 12-276 months). Clinical onset was mild or subclinical in 72% of patients and asymptomatic in 12.2%. At diagnosis, the Hennes's median score was 6 (range, 3-8). Complications were seen in 44 (53.6%) patients, 42 (51.2%) with liver-related complications and 9 (10.9%) without liver-related complications. Cirrhosis was present at diagnosis in 32 (39%) patients. Cirrhosis was subsequently diagnosed in the other 28 patients who were not cirrhotic at diagnosis, over a median follow-up of 12 (IQR, 2-84) months. During follow-up, 6 patients died (7.3%). Cumulative survival at 5 and 10 years was 98.4% and 89%, respectively. A complete biochemical response was achieved in 79% of patients in a mean (SD) of 11.7 (11.6) months. Side effects due to treatment were reported in 76 (92.7%) patients, and no pretreatment factors were found to predict treatment response. CONCLUSIONS: These Cuban patients with AIH had acceptable disease remission rate and a prompt treatment response. Although most patients had advanced-stage liver disease at diagnosis or developed during follow-up, the cumulative survival rate was high when patients were receiving and complying with treatment.

Effects of Alcohol Consumption and Metabolic Syndrome on Mortality in Patients With Nonalcoholic and Alcohol-Related Fatty Liver Disease.

BACKGROUND & AIMS: Non-alcoholic and alcohol-related fatty liver disease are overlapping diseases in which metabolic syndrome and alcohol consumption each contribute to progressive liver disease. We aimed to assess the effects of alcohol consumption and metabolic syndrome on mortality in individuals with fatty liver. METHODS: We searched the National Health and Nutrition and Examination Survey III for adults (20-74 years old) with hepatic steatosis, detected by ultrasound, for whom mortality and follow-up data were available. We collected data from the alcohol use questionnaire (self-reported number of days a participant drank alcohol; the number of drinks [10 g alcohol] per day on a drinking day; the number of days the participant had 5 or more drinks) and calculated the average amount of alcohol consumption in drinks/day for each participant during the year preceding enrollment. Excessive alcohol consumption for men was >3 drinks/day and for women was >1.5 drinks/day. We also collected clinical data, and mortality data were obtained from the National Death Index. Demographic and clinical parameters were compared among consumption groups using the χ2 test for independence or survey regression models. We used Cox proportional hazard models to identify independent predictors of all-cause and cause-specific mortality. RESULTS: The study cohort included 4264 individuals with hepatic steatosis (mean age, 45.9 years; 51% male; 76% white; 46% with metabolic syndrome; 6.2% with excessive alcohol use). There was no significant difference in mean age between individuals with vs without excessive alcohol consumption (P=.65). However, overall mortality was significantly higher among participants with excessive alcohol consumption (32.2%) vs participants with non-excessive alcohol use (22.2%) after mean 20 years of follow up (P=.003), as well as after 5 years of follow up. In multivariate analysis, the presence of metabolic syndrome (adjusted hazard ratio [aHR], 1.43; 95% CI, 1.12-1.83) and excessive alcohol consumption (aHR, 1.79; 95% CI, 1.21-2.66) were independently associated with an increased risk of death in individuals with hepatic steatosis; any lower average amount of alcohol consumption was not associated with mortality (all P>.60). In a subgroup analysis, the association of excessive alcohol use with mortality was significant in individuals with metabolic syndrome (aHR, 2.46; 95% CI, 1.40-4.32) but not without it (P=.74). CONCLUSION: In review of data from the National Health and Nutrition and Examination Survey III, we associated alcohol consumption with increased mortality in participants with fatty liver and metabolic syndrome. These findings indicate an overlap between non-alcoholic and alcohol-related fatty liver disease.

Nonalcoholic Fatty Liver Disease in Latin America and Australia.

The epidemiologic and demographical features of nonalcoholic fatty liver disease (NAFLD) vary significantly across countries and continents. In this review, we analyze current data regarding prevalence of NAFLD in Latin America and Caribbean and Australia and review some peculiarities found in these regions. We stress the need of greater awareness of NAFLD and the development of cost-effective risk stratification strategies and clinical care pathways of the disease. Finally, we highlight the need of effective public health policies to control the main risk factors for NAFLD.

The establishment of public health policies and the burden of non-alcoholic fatty liver disease in the Americas.

Non-alcoholic fatty liver disease (NAFLD) affects 20-25% of the general population and is associated with morbidity, increased mortality, and elevated health-care costs. Most NAFLD risk factors are modifiable and, therefore, potentially amenable to being reduced by public health policies. To date, there is no information about NAFLD-related public health policies in the Americas. In this study, we analysed data from 17 American countries and found that none have established national public health policies to decrease NAFLD-related burden. There is notable heterogeneity in the existence of public health policies to prevent NAFLD-related conditions. The most common public health policies were related to diabetes (15 [88%] countries), hypertension (14 [82%] countries), cardiovascular diseases (14 [82%] countries), obesity (nine [53%] countries), and dyslipidaemia (six [35%] of countries). Only seven (41%) countries had a registry of the burden of NAFLD, and efforts to raise awareness in the Americas were scarce. The implementation of public health policies are urgently needed in the Americas to decrease the burden of NAFLD.

The Latin American Association for the Study of the Liver (ALEH) position statement on the redefinition of fatty liver disease.

The Latin American Association for the Study of the Liver (Asociación Latinoamericana para el Estudio del Hígado; ALEH) represents liver professionals in Latin America with the mission of promoting liver health and quality patient care by advancing the science and practice of hepatology and contributing to the development of a regional health policy framework. Fatty liver disease associated with metabolic dysfunction is of specific concern in the ALEH region, where its prevalence is one of the highest globally, second only to the Middle East. A recent consensus from an international panel recommended a new definition of fatty liver disease associated with metabolic dysfunction, including a shift in name from non-alcoholic fatty liver disease (NAFLD) to metabolic-associated fatty liver disease (MAFLD), and adoption of a set of positive criteria to diagnose the disease, independent of alcohol intake or other liver diseases. Given, the importance of this proposal, ALEH invited leading members of regional nations to come to a consensus on it from a local perspective. We reached a consensus to endorse the proposal that the disease should be renamed as MAFLD and that the disease should be diagnosed by the proposed simple and easily applicable criteria. We expect that this change in nosology will result in improvements in disease awareness and in advances in scientific, economic, public health, political, and regulatory aspects of the disease.

Non-Alcoholic Fatty Liver Disease in Cuba.

INTRODUCTION: With a global adult prevalence of 24%, non-alcoholic fatty liver disease is a global health problem that parallels the worldwide increase of obesity. Its frequency, clinical characteristics and related diseases in Cuba remain unknown. OBJECTIVE: Describe the clinical characteristics, comorbidities and personal habits of patients with non-alcoholic fatty liver disease who are being treated in secondary and tertiary health facilities in seven Cuban provinces. METHODS: A cross-sectional, multicenter study was carried out in 6601 adults seen at gastroenterology outpatient clinics of nine hospitals in seven Cuban provinces from September 2018 through May 2019. Non-alcoholic fatty liver disease was diagnosed by abdominal ultrasound. The study included 1070 patients who met the diagnostic and study criteria and agreed to participate. Their personal habits and anthropometric and clinical characteristics, comorbidities and other aspects of their medical histories were recorded. RESULTS: Of the 1070 participants, 60.7% (649) were women. Participants' average age was 54.5 years and average body mass index was 30.5 kg/m2. A total of 397 (37.1%) were overweight and 574 (53.6%) were obese, 945 (88.3%) led a sedentary lifestyle, 564 (52.7%) had high blood pressure, 406 (37.9%) had lipid disorders and 301 (28.1%) were diabetic. While 484 (45.2%) of patients were asymptomatic, the most frequent clinical signs and symptoms were fatigue (262; 24.5%), dyspepsia (209; 19.5%), abdominal pain (306; 28.5%) and hepatomegaly (189; 17.7%). Liver cirrhosis was present in 37 (3.5%) patients at the time of diagnosis. Family history of type 2 diabetes mellitus and obesity were identified in 391 (36.5%) and 279 (26.1%) of participants, respectively. CONCLUSIONS: Prevalence of non-alcoholic fatty liver disease in these Cuban patients coincides with that reported in the Caribbean region, which has high levels of obesity, overweight and sedentary lifestyles. Most were asymptomatic, female or had metabolism-related comorbidities such as high blood pressure, type 2 diabetes mellitus and dyslipidemia.

Liver Stiffness Reference Values for Healthy Cuban Adults.

INTRODUCTION: Reference values for liver stiffness for healthy individuals vary worldwide. Different optimal cutoff values correspond to the stages of fibrosis in chronic liver disease. OBJECTIVES: Characterize the distribution of liver stiffness in Cuban adults without liver disease and its association with age, serum uric acid and body mass index. METHODS: A cross-sectional study was performed of 110 plasma donors recruited from the Havana Province Blood Bank January 2016 through February 2017. Measurements of liver stiffness were performed using a FibroScan elastography device on the same day of laboratory analyses and abdominal ultrasound. The Pearson coefficient was used to assess correlations, and the reference range was calculated using the mean and its 95% confidence interval. RESULTS: Liver stiffness values observed ranged from 2.2-6.3 kPa. The reference range (95% CI) for the 110 subjects without known liver disease was 4.2-4.6 kPa (mean 4.4). A positive correlation was observed between liver stiffness measurements and body mass index (r = 0.255, p ⟨0.01) and serum uric acid (r = 0.266, p ⟨0.01). There was no correlation between liver stiffness and age. Liver stiffness in women was similar to that of men, 4.3 (2.4-6.1) and 4.5 (2.2-6.3) kPa, respectively (p = 0.086). CONCLUSIONS: Liver stiffness in Cuban adults without liver disease ranges from 2.2-6.3 kPa. The reference range is 4.2-4.6 kPa. Body mass index and serum uric acid levels are positively associated with liver stiffness. CONTRIBUTION OF THIS RESEARCH: This is the first Cuban study using FibroScan to measure liver stiffness; its results will enable better assessment of liver disease in clinical practice.

Calidad de vida relacionada con la salud en pacientes con infección crónica por virus de la Hepatitis C y B

Introducción: La calidad de vida relacionada con la salud medida a través de los "resultados reportados por pacientes", del inglés: patient reported outcomes (PROs) permite la detección efectiva de problemas físicos y psicológicos en pacientes con hepatitis crónica. Objetivo: Describir las dimensiones de calidad de vida más afectadas reportados por pacientes con infección crónica por virus de la Hepatitis C y B. Material y Métodos: Se realizó un estudio descriptivo, transversal desde junio 2018 hasta diciembre 2020 en el Instituto de Gastroenterología (IGE). Entre 1 706 pacientes con diagnóstico VHB y VHC atendidos, la muestra quedó constituida por 366 adultos con infección crónica por los virus de hepatitis B (VHB) y C (VHC). Se registraron los resultados de las encuestas: Evaluación Funcional para el Tratamiento de Enfermedades Crónicas -Fatiga (FACIT-F) y Cuestionario de Impedimento de la Productividad y Actividad Laboral- Problema de salud específico (WPAI-SPH) y parámetros clínico-demográficos. Resultados: Se identificaron 271 (74,0 %) pacientes con diagnóstico de VHC y 95 (26,0 %) de VHB, con edad media 54,0 ± 12,7 años, 209 (57,1 %) mujeres. La puntuación total de la FACIT-F estuvo más afectada en VHC (FACIT-F: HVB: 129,0 ± 15,9 vs. VHC: 111,2 ± 23,5; p<0,0001), quienes a su vez tuvieron mayor deterioro de la actividad laboral (WPAI-SPH: VHB: 0,309 ± 0,312 vs. VHC: 0,386 ± 0,333; p<0,05). Conclusiones: Los pacientes con VHC vivencian una peor calidad de vida que compromete su bienestar, rendimiento laboral y cotidiano.

Introduction: Health-related quality of life measured through "patient-reported outcomes" (PROs) allows effective detection of physical and psychological problems in patients with chronic hepatitis. Objective: To identify the quality of life outcomes reported by patients with chronic hepatitis C and B virus infection. Material and Methods: A descriptive, cross-sectional study was conducted from June 2018 to December 2020 at the Institute of Gastroenterology. Of 1 706 patients with chronic hepatitis B virus (HBV) and hepatitis C virus (HCV) infection, 366 adults were included in the sample. Data was collected using validated instruments: Functional Assessment for Chronic Illness Treatment-Fatigue Scale (FACIT-F) and Work Productivity and Activity Impairment Questionnaire: Specific Health Problem (WPAI-SPH). Clinical and demographic parameters were also recorded. Results: A total of 271 (74.0%) patients with HCV and 95 (26.0%) HBV diagnosis were identified, mean (SD) age 54.0 ± 12.7, and 209 (57.1%) women. The FACIT-F total score was more affected in HCV (FACIT-F: HBV: 129.0 ± 15.9 vs. HCV: 111.2 ± 23.5; p<0.0001); these patients also had greater impairment in work activity (WPAI-SPH: HBV: 0.309 ± 0.312 vs. HCV: 0.386 ± 0.333; p<0.05). Conclusions: Patients with HCV have a worse quality of life that compromises their well-being, work and daily performance.

Prospects for US-Cuba Cooperation in Gastroenterology, Hepatology and Liver Transplantation.

Gastroenterology, hepatology and liver transplant exchanges between the USA and Cuba have mainly consisted of scientific events and short visits. This has facilitated Cuba's inclusion in recognized scientific organizations, familiarity with Cuba's biotech products for treatment of liver disease, and access by Cuban professionals to the highest level of scientific information for clinical practice. It has also given health professionals in the US a more accurate picture of Cuba's health sector. The results of the Global Alcoholic Liver Disease Survey, which included Cuba and was designed and coordinated in the USA, opened doors to joint research and scientific publications. Until now, there have been no protocols for ongoing cooperation to enable bilateral clinical trials or continuing professional development in diagnostic, therapeutic and surgical techniques for hepatology and liver transplantation. There are many mutually beneficial research prospects in these areas. What has been accomplished to date, described in this article, is encouraging and sets the stage for future collaboration. KEYWORDS Hepatology, liver transplant, health, medicine, science, Cuba, USA.

Respuesta terapéutica al ácido ursodesoxicólico en pacientes cubanos con colangitis biliar primaria / Therapeutic response to ursodeoxycholic acid in cuban patients with primary biliary cholangitis

Introducción: La colangitis biliar primaria es una enfermedad hepática, crónica y progresiva. El tratamiento con ácido ursodesoxicólico ha ampliado la esperanza de vida de estos pacientes. Objetivo: Describir la respuesta terapéutica al ácido ursodesoxicólico en pacientes con colangitis biliar primaria. Métodos: Estudio descriptivo, longitudinal y ambispectivo en pacientes atendidos en el Instituto de Gastroenterología entre septiembre de 2003 y enero de 2020. Se evaluaron variables clínicas, de laboratorio, histológicas y terapéuticas. El análisis de los resultados se realizó con el paquete SPSS. Resultados: Se incluyeron 45 pacientes. Hubo un predominio del sexo femenino (95,6 %) y una mediana de edad de 54 años. Los niveles bajos de aspartato amino transferasa (p=0,009 HR=0,98) y fosfatasa alcalina (p=0,005, HR=0,99), así como la presencia del síndrome de superposición (p=0,046 HR=3,08) se relacionaron con una buena respuesta al ácido ursodesoxicólico. La mayoría de los que no respondieron al tratamiento tenían cirrosis hepática (68 %). No se observaron diferencias en la supervivencia de los pacientes de acuerdo con su respuesta al tratamiento (p =0,585). Conclusiones: La respuesta terapéutica fue efectiva en menos de la mitad de los tratados con ácido ursodesoxicólico. La cirrosis hepática, el síndrome de superposición y los niveles elevados de aspartato amino transferasa y fosfatasa alcalina se asociaron a la mala respuesta terapéutica.

Introduction: Primary biliary cholangitis is a chronic and progressive liver disease. Treatment with ursodeoxycholic acid has extended the life expectancy of these patients. Objective: To describe the therapeutic response to ursodeoxycholic acid in patients with primary biliary cholangitis. Methods: Descriptive, longitudinal and ambispective study in patients treated at the Institute of Gastroenterology between September 2003 and January 2020. Clinical, laboratory, histological and therapeutic variables were evaluated. The analysis of the results was performed with the SPSS package. Results: Forty-five patients were included, with a predominance of female gender (95.6%) and a average age of 54 years. Low levels of aspartate amino transferase (p=0.009 HR=0.98) and alkaline phosphatase (p=0.005, HR=0.99), as well as the presence of overlap syndrome (p=0.046 HR=3.08) were associated with a better response to ursodeoxycholic acid. Less than half of the patients responded to conventional treatment with UDCA (47.7 %), most of the non-responders suffer from liver cirrhosis (68 %). No differences were observed in patient survival according to their response to treatment (p =0.585). Conclusions: Therapeutic response was effective in less than half of those treated with ursodeoxycholic acid. Liver cirrhosis, overlap syndrome, and elevated aspartate amino transferase and alkaline phosphatase levels were associated with poor therapeutic response.

Avances en la inmunopatogenia de la hepatitis autoinmune / Advances in the immunopathogenesis of autoimmune hepatitis

RESUMEN Introducción: en la hepatitis autoinmune los mecanismos inmunopatogénicos no están totalmente esclarecidos, múltiples son las investigaciones en este campo, con vistas a enriquecer los conocimientos y ampliar las opciones terapéuticas. Objetivo: sintetizar los conocimientos más recientes acerca de la inmunopatogenia de esta enfermedad. Material y Método: se efectúa una búsqueda exhaustiva de la bibliografía disponible en SciELO, ScienceDirect, Google Académico y PubMed, incluyendo artículos de revisión, estudios experimentales, clínicos, de cohorte y metaanálisis. Desarrollo: se explican los principales mecanismos de tolerancia central y periférica, así como el papel de las subpoblaciones linfoides, las citocinas y el microambiente en la patogenia de la enfermedad. Conclusiones: los avances en el conocimiento de la inmunopatogenia de la hepatitis autoinmune permiten una mejor comprensión de esta enfermedad y son el referente para el diseño de estrategias futuras de tratamiento.

ABSTRACT Introduction: Immunopathogenic mechanisms are not fully clarified in autoimmune hepatitis; there are many investigations in this field with a view to enriching knowledge and expanding therapeutic options. Objective: To synthesize the most recent knowledge about the immunopathogenesis of this disease. Material and Methods: An exhaustive search of the bibliography available in SciELO, ScienceDirect, Google Scholar and PubMed was carried out, including review articles, experimental, clinical, cohort and meta-analysis studies. Development: The main mechanisms of central and peripheral tolerance are explained, as well as the role of lymphoid subpopulations, cytokines and the microenvironment in the pathogenesis of the disease. Conclusions: Advances in the knowledge of the immunopathogenesis of autoimmune hepatitis allow a better understanding of this disease and are the referents in the design of future treatment strategies.

Curso clínico y supervivencia de pacientes cubanos con colangitis biliar primaria. Instituto de Gastroenterología, 2003-2020 / Clinical course and response to treatment of Cuban patients with primary biliary cholangitis. Institute of Gastroenterology, 2003-2020

Introducción: La colangitis biliar primaria es una enfermedad hepática colestásica. La historia natural y el pronóstico de esta enfermedad ha sido difícil de caracterizar. En el área de Latinoamérica y el Caribe son muy escasos los trabajos que investigan el tema. Objetivo: Describir las características clínicas, evolutivas, y supervivencia en pacientes con colangitis biliar primaria. Material y Métodos: Estudio descriptivo, longitudinal y ambispectivo de pacientes mayores de 18 años, con diagnóstico de CBP, atendidos en el Instituto de Gastroenterología entre septiembre de 2003 y enero de 2020; se evaluaron variables demográficas, clínicas, bioquímicas e histológicas a partir de los registros de historias clínicas. Para el procesamiento de los datos se utilizó el paquete estadístico SPSS versión 21.0. Resultados: Se incluyeron 45 pacientes, la mayoría del sexo femenino (95 %) y con forma de presentación sintomática (55,6 %). El 71,1 % de los pacientes presentó alguna complicación, siendo la cirrosis la más frecuente, en 28,8 % de los cirróticos hubo complicaciones secundarias a la lesión hepática predominando la ascitis, seguido del sangrado digestivo variceal, la encefalopatía, el carcinoma hepatocelular (CHC) y las infecciones bacterianas asociadas a la cirrosis. La supervivencia acumulativa superó 95 % a los cinco años. Conclusiones: La supervivencia de los pacientes con CBP fue elevada en el tiempo a pesar del elevado índice de complicaciones.

Introduction: Primary biliary cholangitis (PBC) is a cholestatic liver disease. The natural history and prognosis of PBC has been difficult to characterize. In ​​Latin America and the Caribbean, there are very few research works on the subject. Objective: To describe the clinical and developmental characteristics and survival of patients with PBC. Material and Methods: Descriptive, longitudinal and ambispective study of patients older than 18 years with a diagnosis of PBC, treated at the Institute of Gastroenterology between September 2003 and January 2020. Demographic, clinical, biochemical and histological variables were evaluated from the data of medical records. For data processing, the statistical package SPSS version 21,0 was used. Results: A total of 45 patients were included, most of them female (95 %) and with symptomatic presentation (55,6 %). In addition, 71,1 % of the patients presented some complications, cirrhosis being the most frequent; in 28,8 % of the cirrhotic patients, there were complications secondary to liver injury, predominantly ascites, followed by variceal digestive bleeding, encephalopathy, hepatocellular carcinoma (HCC), and bacterial infections associated with cirrhosis. Cumulative survival exceeded 95 % at five years. Conclusions: The survival of patients with PBC was high over time despite the high rate of complications.

Infección crónica por virus de hepatitis B. Instituto de Gastroenterología de Cuba, 2016-2018 / Chronic hepatitis B virus infection. Institute of Gastroenterology, Havana, Cuba; 2016-2018

Introducción: La efectividad del programa nacional de inmunización ha impactado en la reducción de la incidencia del virus de la hepatitis B en Cuba; sin embargo, no es despreciable la cantidad de pacientes infectados crónicos, que por esta causa, se detectan en la práctica asistencial, aunque insuficientes los estudios epidemiológicos que los caracterizan. Objetivo: Describir las principales características clínicas, biomoleculares e inmunológicas de los pacientes con VHB crónica atendidos en el Instituto de Gastroenterología de Cuba. Materiales y métodos: 97 pacientes que tenían al menos un historial de 6 meses de infección crónica con VHB fueron reclutados en la propia institución desde enero 2016 hasta enero 2018. Se realizaron análisis estadísticos descriptivos para las características clínicas, estudios bioquímicos, virológicos, grado de dureza hepática (medido por elastografía transitoria) y terapia antiviral. Resultados: Todos los pacientes completaron el seguimiento; 61,9 por ciento eran varones y la mediana (rango) de edad fue de 46 (18-84) años. La media de tiempo desde el diagnóstico de la infección fue de 11,7 ± 8,9 años. El 61,9 por ciento tenían enfermedad inactiva sin fibrosis hepática o fibrosis ligera. Solamente el 2 por ciento eran negativos para el antígeno de superficie de la hepatitis B con el DNA cuantificable del VHB, el 81,4 por ciento tenían carga viral detectable y el 85,5 por ciento recibieron uno o más tratamientos antivirales, principalmente los análogos del nucleótido/sido. Conclusiones: Los pacientes con la infección crónica del VHB estudiados, en su mayoría se encuentran en fase inactiva de su enfermedad, sin evidencia significativa de daño hepático, con niveles detectables de viremia y han recibido algún tratamiento antiviral(AU)

Introduction: The effectiveness of the national immunization program has impacted on the reduction of the incidence of hepatitis B virus (HBV) infection in Cuba; however, the number of chronically infected patients is not negligible. These patients are diagnosed in the clinical practice, although the epidemiological studies that indicate the presence of the disease are insufficient. Objective: To describe the main clinical, biomolecular and immunological characteristics of patients with chronic hepatitis B virus infection treated at the National Institute of Gastroenterology, Havana, Cuba. Materials and methods: A total of 97 patients who had at least a 6-month history of chronic HBV infection were recruited at the above mentioned institution from January 2016 to January 2018. Descriptive statistical analyzes were performed to identify the clinical characteristics. Biochemical and virological studies, analysis of both liver stiffness values measured by transient elastography and use of antiviral therapy were also carried out. Results: All patients completed the follow-up. It was observed that 61,9 percent of them were male and the median (range) age was 46 (18-84) years. The mean time since the diagnosis of the infection was 11.7 ± 8,9 years. Inactive disease without liver fibrosis or light fibrosis was present in 61,9 percent. Only 2 percent were negative for hepatitis B surface antigen with quantitative analysis of HBV DNA; also, 81,4 percent of patients had detectable viral load and 85,5 percent received one or more antiviral treatments, mainly nucleotide analogues. Conclusions: The patients with chronic HBV infection studied are mostly in the inactive phase of their disease, without significant evidence of liver damage and detectable levels of viremia. All of them have received some antiviral treatment(AU)

Disfunción cardiaca en la cirrosis hepática / Cardiac dysfunction in liver cirrhosis

Objetivo: determinar las alteraciones de la función cardiaca en las personas que padecen cirrosis hepática. Métodos: se desarrolló un estudio descriptivo transversal en el Instituto de Gastroenterología durante el período 2011-2012, en 33 cirróticos de causa viral y alcohólica, 57,6 por ciento del sexo masculino, con una edad promedio de 50 años, la mayoría (84,8 por ciento) tenía la enfermedad compensada. Resultados: la disfunción diastólica fue la alteración cardiaca más frecuente (39,3 por ciento) seguida de la prolongación del intervalo QT (12,1 por ciento), disfunción sistólica (6,1 por ciento) y miocardiopatía cirrótica (3 por ciento). No se identificaron rasgos distintivos epidemiológicos y/o clínicos que caracterizara a estos pacientes. La circulación hiperdinámica fue más evidente en los que presentaron disfunción diastólica y en la cirrosis de origen alcohólico; las dimensiones cardiacas fueron normales en todos los casos. Conclusiones: las personas que padecen cirrosis son susceptibles de presentar alteraciones de la función cardiaca, incluso, desde la etapa compensada de la enfermedad, lo que debe considerarse por las implicaciones terapéuticas que demanda este tipo de paciente...

Objective: to determine abnormalities of cardiac function in subjects with liver cirrhosis. Methods: a descriptive cross-sectional study was conducted at the Institute of Gastroenterology from 2011 to 2012, in 33 cirrhotic patients due to alcoholic and viral causes, 57.6 percent male, with an average age of 50 years, most (84,8 percent) had compensated disease. Results: diastolic dysfunction was the most common cardiac disorders (39.3 percent) followed by QT prolongation (12.1 percent), systolic dysfunction (6.1 percent) and cirrhotic (3 percent) cardiomyopathy interval. No distinctive epidemiological and/or clinical studies were identified to characterize these patients. The hyperdynamic circulation was more evident in those presenting diastolic dysfunction and alcohol-related cirrhosis. Cardiac dimensions were normal in all cases. Conclusions: people with cirrhosis are susceptible to alterations in cardiac function, even from the compensated stage of the disease, which should be considered by the therapeutic implications of this type of patient demand...

Disfunción cardiaca en la cirrosis hepática / Cardiac dysfunction in liver cirrhosis

Objetivo: determinar las alteraciones de la función cardiaca en las personas que padecen cirrosis hepática. Métodos: se desarrolló un estudio descriptivo transversal en el Instituto de Gastroenterología durante el período 2011-2012, en 33 cirróticos de causa viral y alcohólica, 57,6 por ciento del sexo masculino, con una edad promedio de 50 años, la mayoría (84,8 por ciento) tenía la enfermedad compensada. Resultados: la disfunción diastólica fue la alteración cardiaca más frecuente (39,3 por ciento) seguida de la prolongación del intervalo QT (12,1 por ciento), disfunción sistólica (6,1 por ciento) y miocardiopatía cirrótica (3 por ciento). No se identificaron rasgos distintivos epidemiológicos y/o clínicos que caracterizara a estos pacientes. La circulación hiperdinámica fue más evidente en los que presentaron disfunción diastólica y en la cirrosis de origen alcohólico; las dimensiones cardiacas fueron normales en todos los casos. Conclusiones: las personas que padecen cirrosis son susceptibles de presentar alteraciones de la función cardiaca, incluso, desde la etapa compensada de la enfermedad, lo que debe considerarse por las implicaciones terapéuticas que demanda este tipo de paciente(AU)

Objective: to determine abnormalities of cardiac function in subjects with liver cirrhosis. Methods: a descriptive cross-sectional study was conducted at the Institute of Gastroenterology from 2011 to 2012, in 33 cirrhotic patients due to alcoholic and viral causes, 57.6 percent male, with an average age of 50 years, most (84,8 percent) had compensated disease. Results: diastolic dysfunction was the most common cardiac disorders (39.3 percent) followed by QT prolongation (12.1 percent), systolic dysfunction (6.1 percent) and cirrhotic (3 percent) cardiomyopathy interval. No distinctive epidemiological and/or clinical studies were identified to characterize these patients. The hyperdynamic circulation was more evident in those presenting diastolic dysfunction and alcohol-related cirrhosis. Cardiac dimensions were normal in all cases. Conclusions: people with cirrhosis are susceptible to alterations in cardiac function, even from the compensated stage of the disease, which should be considered by the therapeutic implications of this type of patient demand(AU)

Particularidades del carcinoma hepatocelular en los pacientes con cirrosis hepática / Special features of hepatocellular carcinoma in patients presenting with liver cirrhosis

Introducción: La cirrosis hepática (CH) se ubica entre las principales causas de muerte en Cuba, una de sus complicaciones, el carcinoma hepatocelular (CHC), es responsable de la muerte en estos pacientes lo que ha motivado un incremento del interés clínico por los aspectos diagnósticos y de tratamiento. Métodos: Se desarrolló un estudio descriptivo, prospectivo con componente analítico, en 203 pacientes con CH, en el Instituto de Gastroenterología, en el período de 2005 a 2007. La muestra estuvo constituida por 105 hombres y 98 mujeres, de 56,4 años de edad promedio, la mayoría de causa viral y en diferentes estadios de progresión de la enfermedad. Se evaluaron las características clínicas y criterios de tratamiento en el momento del diagnóstico mediante métodos estadísticos descriptivos y el análisis de supervivencia se realizó mediante las curvas de Kaplan-Meier. Resultados: La frecuencia de presentación de CHC fue del 15,5 por ciento, la causa más frecuente asociada fue la infección crónica por el virus de la hepatitis C, prevaleció en ambos sexos y en edades avanzadas. Predominaron las lesiones nodulares únicas mayores de 2 cm en el lóbulo hepático derecho, en el 77,7 por ciento de los casos, asociadas a hipertensión portal. La supervivencia al año fue menor de 45 por ciento. Conclusiones: El diagnóstico de la enfermedad se estableció en estadios donde las alternativas de tratamiento y curación fueron escasas. La vigilancia del CHC en los pacientes con CH es insuficiente. La supervivencia pudiera mejorar con el diagnóstico precoz y la aplicación de las opciones terapéuticas pertinentes

Introduction: The liver cirrhosis (LC) is located among the leading causes of death in Cuba, one of its complications, the hepatocellular carcinoma (HCC) account for the death of these patients leading to an increase of the clinical interest by the diagnostic features and of treatment. Methods: An analytical, prospective and descriptive study was conducted in 203 patients presenting with LC in the Institute of Gastroenterology from 2005 to 2007. Sample included 105 men and 98 women aged 56,4 as average most of viral cause and in different stages of disease progression. The clinical characteristics and treatment criteria were assessed at diagnosis using descriptive statistical methods and the survival analysis was made using the Kaplan-Meier curves. Results: The presentation frequency of HCC was of 15.5 percent, the more frequent cause associated with the chronic infection by hepatitis C prevailing in both sexes and in advanced ages. The was predominance of nodular lesions unique greater than 2 cm in the right liver lobule in the 77.7 percent of the cases, associated with a portal hypertension. The 1 year survival was less than 45 percent. Conclusions: The diagnosis of disease was made in stages where the alternatives of treatment and cure were scarces. The surveillance of HCC in patients presenting with LC is insufficient. The survival could to improve with an early diagnosis and the application of appropriate therapeutic options

Particularidades del carcinoma hepatocelular en los pacientes con cirrosis hepática / Special features of hepatocellular carcinoma in patients presenting with liver cirrhosis

Introducción: La cirrosis hepática (CH) se ubica entre las principales causas de muerte en Cuba, una de sus complicaciones, el carcinoma hepatocelular (CHC), es responsable de la muerte en estos pacientes lo que ha motivado un incremento del interés clínico por los aspectos diagnósticos y de tratamiento. Métodos: Se desarrolló un estudio descriptivo, prospectivo con componente analítico, en 203 pacientes con CH, en el Instituto de Gastroenterología, en el período de 2005 a 2007. La muestra estuvo constituida por 105 hombres y 98 mujeres, de 56,4 años de edad promedio, la mayoría de causa viral y en diferentes estadios de progresión de la enfermedad. Se evaluaron las características clínicas y criterios de tratamiento en el momento del diagnóstico mediante métodos estadísticos descriptivos y el análisis de supervivencia se realizó mediante las curvas de Kaplan-Meier. Resultados: La frecuencia de presentación de CHC fue del 15,5 por ciento, la causa más frecuente asociada fue la infección crónica por el virus de la hepatitis C, prevaleció en ambos sexos y en edades avanzadas. Predominaron las lesiones nodulares únicas mayores de 2 cm en el lóbulo hepático derecho, en el 77,7 por ciento de los casos, asociadas a hipertensión portal. La supervivencia al año fue menor de 45 por ciento. Conclusiones: El diagnóstico de la enfermedad se estableció en estadios donde las alternativas de tratamiento y curación fueron escasas. La vigilancia del CHC en los pacientes con CH es insuficiente. La supervivencia pudiera mejorar con el diagnóstico precoz y la aplicación de las opciones terapéuticas pertinentes(AU)

Introduction: The liver cirrhosis (LC) is located among the leading causes of death in Cuba, one of its complications, the hepatocellular carcinoma (HCC) account for the death of these patients leading to an increase of the clinical interest by the diagnostic features and of treatment. Methods: An analytical, prospective and descriptive study was conducted in 203 patients presenting with LC in the Institute of Gastroenterology from 2005 to 2007. Sample included 105 men and 98 women aged 56,4 as average most of viral cause and in different stages of disease progression. The clinical characteristics and treatment criteria were assessed at diagnosis using descriptive statistical methods and the survival analysis was made using the Kaplan-Meier curves. Results: The presentation frequency of HCC was of 15.5 percent, the more frequent cause associated with the chronic infection by hepatitis C prevailing in both sexes and in advanced ages. The was predominance of nodular lesions unique greater than 2 cm in the right liver lobule in the 77.7 percent of the cases, associated with a portal hypertension. The 1 year survival was less than 45 percent. Conclusions: The diagnosis of disease was made in stages where the alternatives of treatment and cure were scarces. The surveillance of HCC in patients presenting with LC is insufficient. The survival could to improve with an early diagnosis and the application of appropriate therapeutic options(AU)