Abstract Background Autoimmune liver diseases (AILD) comprise a set of entities characterized by tissue damage as a result of the loss of self-tolerance. There are few reports of AILD from Caribbean ...countries. The aim of the study was to investigate the clinical patterns, laboratory findings, and immunological features, treatment responses, and prognoses of AILD in adult patients at a Cuban tertiary referral center. Methods A prospective study was conducted at the National Institute of Gastroenterology in Havana, Cuba from May 2012 to April 2016. Clinical, immunologic, and histologic features of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), AIH/PBC overlap syndrome, autoimmune cholangiopathy (AIC), and primary sclerosing cholangitis (PSC) were recorded. Response to therapy was assessed by serum ALT and bilirubin levels at 3, 6, 12, and 24 months after treatment-initiation. Results Of the 106 patients included in the study, 85.5% were female. The median age at presentation was 47 years. AIH was the most common AILD and was diagnosed in 60 patients (56.6%), 55 of whom had type 1 AIH. PBC was diagnosed in 22 (20.7%) patients, overlap syndrome in 16 (15%) patients, AIC in 5 (4.71%) patients, and PSC in 3 patients (2.8%). Most patients were symptomatic; 48 (45.2%) patients presented with liver cirrhosis and 14.5% with decompensated cirrhosis. Follow up of treatment was between 6 and 24 months. Prednisone monotherapy was used in 22 AIH patients (36.6%) and a combination of prednisone and azathioprine (AZA) was used in 28 (46.6%) AIH patients. Response to treatment was seen in 41 AIH patients (68.3%), 33 (55%) of whom had a complete response and 8 of whom (24.2%) relapsed after 12 months of maintenance therapy. No or incomplete response to treatment was seen in 18 (30%) patients. In 46 patients with autoimmune cholestasis, ursodeoxycholic acid was used as monotherapy in 25 (54.3%) patients. Conclusion The clinical profile of AILD in a sample of the Cuban population is similar to that reported in South areas. AIH was more frequent than PBC, and usually presented with advanced liver disease that responded poorly to treatment.
Disfunción cardiaca en la cirrosis hepática Castellanos Fernández, Marlen Ivón; Rogel Marroquín, Belkin Ismael; Rodríguez Martorell, Francisco ...
Revista Cubana de medicina,
04/2014, Letnik:
53, Številka:
2
Journal Article
Recenzirano
Odprti dostop
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 % del sexo masculino, con una edad promedio de 50 años, la mayoría (84,8 %) tenía la enfermedad compensada. Resultados: la disfunción diastólica fue la alteración cardiaca más frecuente (39,3 %) seguida de la prolongación del intervalo QT (12,1 %), disfunción sistólica (6,1 %) y miocardiopatía cirrótica (3 %). 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.
Patients with nonalcoholic fatty liver disease (NAFLD)/metabolic dysfunction-associated steatotic liver disease (MASLD) face a multifaceted disease burden which includes impaired health-related ...quality of life (HRQL) and potential stigmatization. We aimed to assess the burden of liver disease in patients with NAFLD and the relationship between experience of stigma and HRQL.
Members of the Global NASH Council created a survey about disease burden in NAFLD. Participants completed a 35-item questionnaire to assess liver disease burden (LDB) (seven domains), the 36-item CLDQ-NASH (six domains) survey to assess HRQL and reported their experience with stigmatization and discrimination.
A total of 2,117 patients with NAFLD from 24 countries completed the LDB survey (48% Middle East and North Africa, 18% Europe, 16% USA, 18% Asia) and 778 competed CLDQ-NASH. Of the study group, 9% reported stigma due to NAFLD and 26% due to obesity. Participants who reported stigmatization due to NAFLD had substantially lower CLDQ-NASH scores (all p <0.0001). In multivariate analyses, experience with stigmatization or discrimination due to NAFLD was the strongest independent predictor of lower HRQL scores (beta from -5% to -8% of score range size, p <0.02). Experience with stigmatization due to obesity was associated with lower Activity, Emotional Health, Fatigue, and Worry domain scores, and being uncomfortable with the term “fatty liver disease” with lower Emotional Health scores (all p <0.05). In addition to stigma, the greatest disease burden as assessed by LDB was related to patients’ self-blame for their liver disease.
Stigmatization of patients with NAFLD, whether it is caused by obesity or NAFLD, is strongly and independently associated with a substantial impairment of their HRQL. Self-blame is an important part of disease burden among patients with NAFLD.
Patients with nonalcoholic fatty liver disease (NAFLD), recently renamed metabolic dysfunction-associated steatotic liver disease (MASLD), may experience impaired health-related quality of life and stigmatization. Using a specifically designed survey, we found that stigmatization of patients with NAFLD, whether it is caused by obesity or the liver disease per se, is strongly and independently associated with a substantial impairment of their quality of life. Physicians treating patients with NAFLD should be aware of the profound implications of stigma, the high prevalence of self-blame in the context of this disease burden, and that providers’ perception may not adequately reflect patients’ perspective and experience with the disease.
Display omitted
•A survey of stigma and disease burden was completed by patients with NAFLD/MASLD.•Stigmatization due to liver disease was strongly associated with impaired quality of life.•Stigmatization due to obesity was also associated with lower quality of life scores.•The greatest disease burden was related to patients’ self-blame for their liver disease.•Addressing disease stigma may improve patients’ well-being and self-efficacy.
INTRODUCTIONReference values for liver stiffness for healthy individuals vary worldwide. Different optimal cutoff values correspond to the stages of fibrosis in chronic liver disease. ...OBJECTIVESCharacterize the distribution of liver stiffness in Cuban adults without liver disease and its association with age, serum uric acid and body mass index. METHODSA 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. RESULTSLiver 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). CONCLUSIONSLiver 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 RESEARCHThis is the first Cuban study using FibroScan to measure liver stiffness; its results will enable better assessment of liver disease in clinical practice.
Chronic hepatitis B (CHB) infection is one of the most common causes of cirrhosis and liver cancer worldwide. Our aim was to assess clinical and patient‐reported outcome (PRO) profile of CHB patients ...from different regions of the world using the Global Liver Registry. The CHB patients seen in real‐world practices are being enrolled in the Global Liver Registry. Clinical and PRO (FACIT‐F, CLDQ, WPAI) data were collected and compared to baseline data from CHB controls from clinical trials. The study included 1818 HBV subjects (48 ± 13 years, 58% male, 14% advanced fibrosis, 7% cirrhosis) from 15 countries in 6/7 Global Burden of Disease super‐regions. The rates of advanced fibrosis varied (3–24%). The lowest PRO scores across multiple domains were in HBV subjects from the Middle East/North Africa (MENA), the highest – Southeast/East and South Asia. Subjects with advanced fibrosis had PRO impairment in 3 CLDQ domains, Activity of WPAI (p < 0.05). HBV subjects with superimposed fatty liver had more PRO impairments. In multivariate analysis adjusted for location, predictors of PRO impairment in CHB included female sex, advanced fibrosis, and non‐hepatic comorbidities (p < 0.05). In comparison to Global Liver Registry patients, 242 controls from clinical trials had better PRO scores (Abdominal, Emotional, and Systemic scores of CLDQ, all domains of WPAI) (p < 0.05). In multivariate analysis with adjustment for location and clinicodemographic parameters, the associations of PROs with the enrollment setting (real‐life Global Liver Registry vs. clinical trials) were no longer significant (all p > 0.10). The clinico‐demographic portrait of CHB patients varies across regions of the world and enrollment settings. Advanced fibrosis and non‐hepatic comorbidities are independently associated with PRO impairment in CHB patients.
PDF
