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

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

Osteoporosis and Fragility Fractures in Patients with Liver Cirrhosis: Usefulness of FRAX® as a Screening Tool.

PURPOSE: The purpose of this study is to assess the prevalence of osteoporosis and fragility fractures in patients with liver cirrhosis (LC) and determine the associated risk factors, evaluating the usefulness of FRAX® as a screening method to identify patients at a higher risk of fracture. METHODS: This was a cross-sectional study. Demographic, clinical, and analytical data were collected in a randomized sample of LC patients attending the Hepatology Department of a university hospital. We assessed the absolute risk of fracture at 10 years (FRAX®) and based on the bone mineral density (BMD), the presence of morphometric vertebral fracture with a vertebral fracture assessment (VFA), or a thoracic and lumbar X-ray and bone microarchitecture with a trabecular bone score (TBS). RESULTS: Ninety-two patients were included (71% male); the mean age was 63 ± 11.3 years. The main etiology of LC was alcoholism (52.2%), and most patients were Child-Pugh A (80.4%), with a mean model for end-stage liver disease (MELD) score of 10.1 ± 3.6. Sixteen patients (17.4%) had osteoporosis, and fifty-four (58.7%) had osteopenia. Eight patients (8.7%) had suffered at least one fragility fracture. The absolute risk of a major fracture according to FRAX without the BMD was 5.7 ± 4.5%. Risk factors associated with osteoporosis were age and the female sex. BMI > 30 was a protective factor. A FRAX cut-off point for a major fracture > 6.6% had a sensitivity of 69% and a specificity of 85% for a diagnosis of osteoporosis. CONCLUSIONS: The prevalence of osteoporosis and fractures in patients with LC is high, particularly in older women. FRAX® may be a useful method to identify candidates for bone densitometry. A FRAX value below 6.6% without the BMD can avoid unnecessary testing.

Corrigendum to 'A multicenter analysis of the role of prophylactic transfusion of blood products in patients with cirrhosis and esophageal varices undergoing endoscopic band ligation' [JHEP Reports 6 (2021)].

[This corrects the article DOI: 10.1016/j.jhepr.2021.100363.].

A multicenter analysis of the role of prophylactic transfusion of blood products in patients with cirrhosis and esophageal varices undergoing endoscopic band ligation.

BACKGROUND & AIMS: Prophylactic administration of platelets and fresh frozen plasma (FFP) has been recommended in patients with cirrhosis with low platelets and/or prolonged international normalized ratio (INR) without scientific evidence to support this practice. In this analysis, we evaluated the use of prophylactic administration of blood products in outpatients with cirrhosis undergoing endoscopic band ligation (EBL). METHODS: This is a multicenter retrospective analysis of consecutive EBL procedures in patients with cirrhosis at 4 hospitals in Spain from 01/2010-01/2017. FFP and/or platelet transfusion were given at the discretion of the physician if INR was >1.5 and/or platelet count <50x109/L. Patient demographics, endoscopic findings, bleeding events after EBL, and the use of prophylactic FFP or platelets were recorded. RESULTS: A total of 536 patients underwent 1,472 EBL procedures: 72% male; main etiology HCV and alcohol (72%); median MELD score 11; Child-Pugh A/B/C (59/33/8%). EBL procedures were performed for primary (51%) or secondary (49%) prophylaxis. A median of 2 procedures per patient were performed.1-4 FFP and/or platelets were administered in 41 patients (7.6%). The prophylactic transfusion protocol was followed in 16% and 28% of procedures with high INR and/or low platelets, respectively. Post-EBL bleeding occurred in 26 out of 536 patients (4.8%) and in 33 out of 1,472 procedures (2.2%). Bleeding was due to post-EBL ulcers in 21 patients and due to band dislodgment in 5. In 6 patients, bleeding occurred within 24 hours and in the remaining patients it occurred within 2 weeks after EBL. In those that bled, 7 met criteria for transfusion (2 for FFP and 5 for platelets), of whom only 1 received FFP and 4 received platelets; the remaining 19 patients did not meet criteria for transfusion. There was no association between INR or platelet count and bleeding events. Univariate and multivariate analysis revealed that Child-Pugh and MELD scores were risk factors for post-EBL bleeding. CONCLUSIONS: The incidence of post-EBL bleeding is low and is associated with advanced liver disease. Post-EBL bleeding was not related to baseline INR/platelet count and most outpatients with post-EBL bleeding did not meet criteria for prophylactic transfusion. LAY SUMMARY: Patients with chronic liver disease or cirrhosis and enlarged veins (varices) of the esophagus that can potentially bleed commonly need an endoscopy to treat these varices with elastic rubber bands (endoscopic band ligation). Some patients have low platelet counts or prolonged coagulation tests. This analysis of 4 centers evaluated the use of prophylactic administration of blood products in outpatients with cirrhosis undergoing endoscopic band ligation. The results showed that bleeding after band ligation is uncommon and that if bleeding occurs it does not seem to be related with coagulation tests or the administration of blood products to prevent bleeding after band ligation of esophageal varices.

Micronutrient Deficiencies in Patients with Decompensated Liver Cirrhosis.

Patients with cirrhosis often develop malnutrition and micronutrient deficiencies, leading to a worse prognosis and increased mortality. Our main goal was to assess the prevalence of micronutrient deficiencies in patients with decompensated cirrhosis. This was a prospective single-center study including 125 consecutive patients hospitalized for acute decompensation of cirrhosis (mostly of alcoholic etiology). A blood test including trace elements and vitamins was performed on admission. The main micronutrient deficiencies observed were vitamin D (in 94.5%), vitamin A (93.5%), vitamin B6 (60.8%) and zinc (85.6%). Patients in Child-Pugh class C had lower levels of vitamin A (p < 0.0001), vitamin E (p = 0.01) and zinc (p < 0.001), and higher levels of ferritin (p = 0.002) and vitamin B12 (p < 0.001) than those in Child-Pugh class A and B. Patients with a higher model of end-stage liver disease (MELD) score had lower levels of vitamin A (p < 0.0001), vitamin E (p < 0.001), magnesium (p = 0.01) and zinc (p = 0.001), and higher levels of ferritin (p = 0.002) and vitamin B12 (p < 0.0001). Severe hepatic insufficiency correlated with lower levels of zinc, vitamin E and vitamin A, and higher levels of vitamin B12 and ferritin.

Current trends in access to treatment for hepatitis B in immigrants vs non-immigrants.

BACKGROUND: Universal vaccination for hepatitis B virus (HBV) and migratory movements have changed the demographic characteristics of this disease in Spain and in Europe. Therefore, we evaluated the characteristics of the disease and the possible differences according to origin (immigrants vs non-immigrants) and access to treatment. METHODS: This is a multicenter cross-sectional study (June 2014 to May 2015) in which outpatients with a positive HBsAg were seen and followed in four Hepatology units. Demographic and clinical data and indication and access to treatment were collected in two different regions of Catalonia (Spain) where there are no barriers to treatment due to a comprehensive coverage under the National Health System. RESULTS: A total of 951 patients were evaluated (48.1% men). Of these, 46.6% were immigrants (58.7% of them were born in Africa) and were significantly younger compared to non-immigrants. The proportions of patients with alcohol consumption, being overweight, and other indicators of metabolic co-morbidities were significantly higher in non-immigrants. Among the 937 patients receiving HBeAg examination, 91.7% were HBeAg-negative. Chronic HBeAg-positive infection was significantly higher in immigrants (3.9% vs 0.6%, P = 0.001) and chronic HBeAg-negative hepatitis was higher non-immigrants (31.7% vs 21.4%, P < 0.001). Not only was the proportion of patients who met treatment criteria significantly higher among non-immigrants (38.4% vs 29.2%, P = 0.003), but also the proportion of those with indication of effectively receiving therapy at the time of data collection (83.2% vs 57.8 %, P < 0.001). CONCLUSIONS: The immigrant population with HBV is younger and has a lower prevalence of metabolic co-morbidities and a higher frequency of chronic HBeAg infection. Despite having access to care and an indication for treatment, some do not get adequately treated due to several factors including local adaptation that precludes access to treatment.

Bacteremia caused by Paracoccus yeei in patient with compensated cirrhosis of the liver / Bacteremia por Paracoccus yeei en un paciente con cirrosis hepática compensada

No disponible

Correction: Impact of treatment with direct-acting antivirals on anxiety and depression in chronic hepatitis C.

[This corrects the article DOI: 10.1371/journal.pone.0208112.].

Genetic and Clinical Heterogeneity in Thirteen New Cases with Aceruloplasminemia. Atypical Anemia as a Clue for an Early Diagnosis.

Aceruloplasminemia is a rare autosomal recessive genetic disease characterized by mild microcytic anemia, diabetes, retinopathy, liver disease, and progressive neurological symptoms due to iron accumulation in pancreas, retina, liver, and brain. The disease is caused by mutations in the Ceruloplasmin (CP) gene that produce a strong reduction or absence of ceruloplasmin ferroxidase activity, leading to an impairment of iron metabolism. Most patients described so far are from Japan. Prompt diagnosis and therapy are crucial to prevent neurological complications since, once established, they are usually irreversible. Here, we describe the largest series of non-Japanese patients with aceruloplasminemia published so far, including 13 individuals from 11 families carrying 13 mutations in the CP gene (7 missense, 3 frameshifts, and 3 splicing mutations), 10 of which are novel. All missense mutations were studied by computational modeling. Clinical manifestations were heterogeneous, but anemia, often but not necessarily microcytic, was frequently the earliest one. This study confirms the clinical and genetic heterogeneity of aceruloplasminemia, a disease expected to be increasingly diagnosed in the Next-Generation Sequencing (NGS) era. Unexplained anemia with low transferrin saturation and high ferritin levels without inflammation should prompt the suspicion of aceruloplasminemia, which can be easily confirmed by low serum ceruloplasmin levels. Collaborative joint efforts are needed to better understand the pathophysiology of this potentially disabling disease.

Salivary Cortisol Determination in ACTH Stimulation Test to Diagnose Adrenal Insufficiency in Patients with Liver Cirrhosis.

PURPOSE: The prevalence of adrenal insufficiency (AI) in patients with decompensated liver cirrhosis is unknown. Because these patients have lower levels of cortisol-binding carrier proteins, their total serum cortisol (TSC) correlates poorly with free serum cortisol (FC). Salivary cortisol (SaC) correlates better with FC. We aimed to establish SaC thresholds for AI for the 250 µg intravenous ACTH test and to estimate the prevalence of AI in noncritically ill cirrhotic patients. METHODS: We included 39 patients with decompensated cirrhosis, 39 patients with known AI, and 45 healthy volunteers. After subjects fasted ≥8 hours, serum and saliva samples were collected for determinations of TSC and SaC at baseline 0'(T0) and at 30-minute intervals after intravenous administration of 250 µg ACTH [30'(T30), 60'(T60), and 90'(T90)]. RESULTS: Based on the findings in healthy subjects and patients with known AI, we defined AI in cirrhotic patients as SaC-T0< 0.08 µg/dL (2.2 nmol/L), SaC-T60 < 1.43 µg/dl (39.5 nmol/L), or ΔSaC<1 µg/dl (27.6 nmol/L). We compared AI determination in cirrhotic patients with the ACTH test using these SaC thresholds versus established TSC thresholds (TSC-T0< 9 µg/dl [248 nmol/L], TSC-T60 < 18 µg/dl [497 nmol/L], or ΔTSC<9 µg/dl [248 nmol/L]). SaC correlated well with TSC. The prevalence of AI in cirrhotic patients was higher when determined by TSC (48.7%) than by SaC (30.8%); however, this difference did not reach statistical significance. AI was associated with sex, cirrhosis etiology, and Child-Pugh classification. CONCLUSIONS: Measuring SaC was more accurate than TSC in the ACTH stimulation test. Measuring TSC overestimated the prevalence of AI in noncritically ill cirrhotic patients.

Análisis de los índices predictores de respuesta al tratamiento con ácido ursodeoxicólico en pacientes con colangitis biliar primaria / Analysis of predictive response scores to treatment with ursodeoxycholic acid in patients with primary biliary cholangitis

Antecedentes y objetivos: La colangitis biliar primaria (CBP) es una enfermedad autoinmunitaria que afecta a los conductos biliares de pequeño y mediano tamaño. Los únicos tratamientos aprobados actualmente en nuestro país son el ácido ursodeoxicólico (AUDC) y el ácido obeticólico. Diversos índices evalúan la respuesta al año de tratamiento. El objetivo de nuestro estudio fue evaluar los diferentes índices predictivos de respuesta al tratamiento con AUDC. Material y métodos: Estudio unicéntrico retrospectivo en el que se recogieron los datos clínicos y analíticos de los pacientes diagnosticados de CBP desde enero de 1987 hasta diciembre de 2015. Se calculó la respuesta al año de inicio del tratamiento utilizando los diferentes índices de respuesta y se evaluó su grado de concordancia mediante el índice Kappa. Se calculó el area under the receiver operating characteristic curve (AUROC, «área bajo la curva ROC») para determinar la capacidad predictiva de los índices. Asimismo, se analizaron los factores pronósticos basales. Resultados: Se incluyeron 153 pacientes. El análisis bivariante demostró una relación estadísticamente significativa entre los niveles altos iniciales de fosfatasa alcalina y colesterol y la mala respuesta al tratamiento. La mejor AUROC fue del índice París-I (0,81). La concordancia entre los diferentes índices fue baja. El índice pronóstico GLOBE fue válido para evaluar el pronóstico. Conclusión: La fosfatasa alcalina y el colesterol basales fueron factores predictores de mala evolución. El índice cualitativo que mejor predijo la supervivencia fue el París-I. Se obtuvo una mala concordancia entre los diferentes índices predictivos. El índice GLOBE es válido para evaluar el pronóstico de la CBP

Background and objectives: Primary biliary cholangitis (PBC) is an autoimmune disease that affects the small bile ducts. The only treatments currently approved in our country are ursodeoxycholic acid (UDCA) and obeticholic acid. Different indices evaluate the response after one year of treatment. The aim of our study was to evaluate the different predictive scores and prognostic factors of response to UDCA. Material and methods: Retrospective single-centre study in which clinical and analytical data of patients diagnosed with PBC were collected from January 1987 to December 2015. The response after one year of treatment was evaluated using the different response scores and their concordance degree using the Kappa index. The area under the receiver operating characteristic curve (AUROC) was calculated to determine the predictive capacity of the scores. Likewise, the prognostic factors of response to treatment were analysed. Results: We included 153 patients. The bivariate analysis showed a statistically significant relationship between the initial high levels of alkaline phosphatase and cholesterol and the poor response to treatment. The best AUROC was in Paris-I score (0.81). The concordance between the different scores was low. The GLOBE score was valid to evaluate the prognosis. Conclusion: Basal alkaline phosphatase and cholesterol were predictors of poor outcome. The best predictive qualitative score in our cohort patients was Paris-I. There was a poor concordance between the different predictive scores. GLOBE score is valid to evaluate prognosis

Systematic review: Would susceptibility-guided treatment achieve acceptable cure rates for second-line Helicobacter pylori therapy as currently practiced?

BACKGROUND: Susceptibility-guided treatment has been proposed as a way to improve Helicobacter pylori eradication rates. Evidence on its efficacy for rescue therapy is very scarce. The aim of this study was to indirectly assess the applicability and effectiveness of susceptibility-guided treatment by evaluating (a) the rate of acceptance of endoscopy, (b) its success in detecting resistances, and (c) infection cure rates in patients harboring strains found to be susceptible to the antibiotics administered in clinical trials in which the efficacy of second-line treatments was reported. METHODS: A systematic review of studies evaluating second-line H pylori treatment was carried out in multiple databases. Studies reporting antibiotic susceptibility evaluation and/or cure rates in patients harboring sensitive and resistant strains were selected. Data were extracted in duplicate. RESULTS: The systematic review identified 36 eligible studies. Acceptance was evaluated in only one study of 60 patients, of whom only 38 agreed to endoscopy. Among the 2890 patients who received endoscopy and culture, resistances were finally determined in 86.5%. Cure rate was 72.5% in the 113 patients harboring a clarithromycin-susceptible strain after previous clarithromycin treatment, 93.5% in the 765 patients harboring a metronidazole-susceptible strain, and 83.8% in the 192 patients harboring a levofloxacin-susceptible strain. No studies with repeated administration of levofloxacin or metronidazole were found. CONCLUSION: Even if the culture shows a clarithromycin-sensitive strain, repeating clarithromycin after a first failure should be discouraged. Susceptibility-guided treatment alone did not achieve adequate cure rates for rescue therapies. Additional measures are needed to design rescue treatments that consistently achieve excellent cure rates.

Utilidad de la histología para el diagnóstico de la gastroenteropatía por hipertensión portal. Concordancia entre la imagen endoscópica y las biopsias gastrointestinales. Papel del marcador CD34 / Utility of histology for the diagnosis of portal hypertensive gastroenteropathy. Concordance between the endoscopic image and gastrointestinal biopsies. Role of the CD34 marker

Introducción: En la endoscopia digestiva alta de pacientes con cirrosis hepática a menudo se observan lesiones inespecíficas, que se suelen orientar como gastropatía por hipertensión portal (GHP). Sin embargo, el diagnóstico de GHP puede ser difícil, tanto endoscópica como histológicamente. El estudio de expresión de CD34, que realza las células endoteliales de la microvasculatura podría ayudar al diagnóstico diferencial. Los objetivos del estudio fueron evaluar la correlación entre la endoscopia y la histología en el diagnóstico de la GHP y valorar la utilidad del CD34 en el diagnóstico de la misma. Material y métodos: Se analizaron biopsias fúndicas de 100 pacientes cirróticos y 20 controles, y se realizó inmunotinción para CD34. Se compararon con las imágenes endoscópicas. Resultados: Se observó una correlación muy baja entre la histología con el diagnóstico endoscópico de GHP (kappa=0,15). Además, la medición del diámetro de los vasos gástricos realzados mediante el uso de la tinción inmunohistoquímica (CD34) no mostró buena correlación con el diagnóstico endoscópico (p=0,26) y tampoco parece aportar información relevante para el diagnóstico histológico de GHP. Discusión: Existe una baja correlación entre la histología y la endoscopia para el diagnóstico de GHP. El uso de la inmunotinción para CD34 no mejora la rentabilidad diagnóstica del estudio histológico

Introduction: Upper gastroscopy in patients with cirrhosis often reveals non-specific lesions, which are usually oriented as portal hypertensive gastropathy (PHG). However, the diagnosis of PHG can be difficult, both from an endoscopic and histological point of view. The study of CD34 expression, which enhances the endothelial cells of the microvasculature, could help the differential diagnosis. The objectives of this study were to evaluate the correlation between endoscopy and histology in the diagnosis of PHG and to assess the utility of CD34 in the diagnosis of PHG. Material and methods: The results of immunostaining with CD34 gastric fundus biopsies from 100 cirrhotic patients and 20 controls were compared with the endoscopic images. Results: The correlation between the histology and the endoscopic diagnosis of PHG was very low (kappa=0.15). In addition, the measurement of the diameter of the gastric vessels enhanced by the use of immunohistochemical staining (CD34) did not show good correlation with the endoscopic diagnosis (p=.26) and did not provide relevant information for the histological diagnosis of PHG either. Discussion: The correlation between histology and endoscopy is low for the diagnosis of PHG. The use of immunostaining for CD34 does not seem to improve the diagnostic yield of the histological study

Reactivación de tuberculosis peritoneal y pleural durante el tratamiento de la hepatitis C con antivirales de acción directa / Reactivation of peritoneal and pleural tuberculosis during hepatitis C treatment with direct-acting antivirals

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Manejo de la insuficiencia hepática aguda grave. Documento de posicionamiento de la Societat Catalana de Digestologia / Management of acute liver failure. Clinical guideline from the Catalan Society of Digestology

La insuficiencia hepática aguda grave es una enfermedad infrecuente de etiología diversa caracterizada por la rápida aparición de insuficiencia hepatocelular grave en individuos sin enfermedad hepática previa. El manejo inicial de esta enfermedad condiciona su evolución posterior. El primer contacto con el paciente afecto de insuficiencia hepática aguda grave suele realizarse en unidades de urgencias, consultas de aparato digestivo o, en casos más graves, unidades de cuidados intensivos. En todos estos casos resulta fundamental un abordaje multidisciplinario que incluya cirujanos y hepatólogos expertos en esta enfermedad, es decir, de centros hospitalarios que dispongan de programa de trasplante hepático. El presente artículo revisa la evidencia actual en el manejo médico de la insuficiencia hepática aguda grave, desde la sospecha diagnóstica y el manejo inicial, hasta el tratamiento médico intensivo, incluyendo la necesidad de un trasplante hepático urgente. También se revisan las evidencias sobre el uso de sistemas de soporte hepático artificial en esta enfermedad

Acute liver failure is an uncommon and severe disease characterised by a rapid onset of severe hepatocellular failure in individuals without previous liver disease. Initial management of this entity determines the outcome of the patient. Initial contact with the acute liver failure patients usually occurs in the emergency department, digestology clinic or, in more severe cases, intensive care units. The management of acute liver failure patients in all these cases must be multidisciplinary, involving surgeons and hepatologists who are experts in this condition, meaning those from hospitals with active liver transplant programmes. This article reviews the current body of evidence concerning the medical management of acute liver failure patients, from the suspected diagnosis and initial management to intensive medical treatment, including the need for an emergency liver transplantation. Moreover, we also review the use of artificial liver support systems in this setting

Utility of histology for the diagnosis of portal hypertensive gastroenteropathy. Concordance between the endoscopic image and gastrointestinal biopsies. Role of the CD34 marker. / Utilidad de la histología para el diagnóstico de la gastroenteropatía por hipertensión portal. Concordancia entre la imagen endoscópica y las biopsias gastrointestinales. Papel del marcador CD34.

INTRODUCTION: Upper gastroscopy in patients with cirrhosis often reveals non-specific lesions, which are usually oriented as portal hypertensive gastropathy (PHG). However, the diagnosis of PHG can be difficult, both from an endoscopic and histological point of view. The study of CD34 expression, which enhances the endothelial cells of the microvasculature, could help the differential diagnosis. The objectives of this study were to evaluate the correlation between endoscopy and histology in the diagnosis of PHG and to assess the utility of CD34 in the diagnosis of PHG. MATERIAL AND METHODS: The results of immunostaining with CD34 gastric fundus biopsies from 100 cirrhotic patients and 20 controls were compared with the endoscopic images. RESULTS: The correlation between the histology and the endoscopic diagnosis of PHG was very low (kappa=0.15). In addition, the measurement of the diameter of the gastric vessels enhanced by the use of immunohistochemical staining (CD34) did not show good correlation with the endoscopic diagnosis (p=.26) and did not provide relevant information for the histological diagnosis of PHG either. DISCUSSION: The correlation between histology and endoscopy is low for the diagnosis of PHG. The use of immunostaining for CD34 does not seem to improve the diagnostic yield of the histological study.

Analysis of predictive response scores to treatment with ursodeoxycholic acid in patients with primary biliary cholangitis. / Análisis de los índices predictores de respuesta al tratamiento con ácido ursodeoxicólico en pacientes con colangitis biliar primaria.

BACKGROUND AND OBJECTIVES: Primary biliary cholangitis (PBC) is an autoimmune disease that affects the small bile ducts. The only treatments currently approved in our country are ursodeoxycholic acid (UDCA) and obeticholic acid. Different indices evaluate the response after one year of treatment. The aim of our study was to evaluate the different predictive scores and prognostic factors of response to UDCA. MATERIAL AND METHODS: Retrospective single-centre study in which clinical and analytical data of patients diagnosed with PBC were collected from January 1987 to December 2015. The response after one year of treatment was evaluated using the different response scores and their concordance degree using the Kappa index. The area under the receiver operating characteristic curve (AUROC) was calculated to determine the predictive capacity of the scores. Likewise, the prognostic factors of response to treatment were analysed. RESULTS: We included 153 patients. The bivariate analysis showed a statistically significant relationship between the initial high levels of alkaline phosphatase and cholesterol and the poor response to treatment. The best AUROC was in Paris-I score (0.81). The concordance between the different scores was low. The GLOBE score was valid to evaluate the prognosis. CONCLUSION: Basal alkaline phosphatase and cholesterol were predictors of poor outcome. The best predictive qualitative score in our cohort patients was Paris-I. There was a poor concordance between the different predictive scores. GLOBE score is valid to evaluate prognosis.

Management of acute liver failure. Clinical guideline from the Catalan Society of Digestology. / Manejo de la insuficiencia hepática aguda grave. Documento de posicionamiento de la Societat Catalana de Digestologia.

Acute liver failure is an uncommon and severe disease characterised by a rapid onset of severe hepatocellular failure in individuals without previous liver disease. Initial management of this entity determines the outcome of the patient. Initial contact with the acute liver failure patients usually occurs in the emergency department, digestology clinic or, in more severe cases, intensive care units. The management of acute liver failure patients in all these cases must be multidisciplinary, involving surgeons and hepatologists who are experts in this condition, meaning those from hospitals with active liver transplant programmes. This article reviews the current body of evidence concerning the medical management of acute liver failure patients, from the suspected diagnosis and initial management to intensive medical treatment, including the need for an emergency liver transplantation. Moreover, we also review the use of artificial liver support systems in this setting.