Evaluation and management of emergencies in the patient with cirrhosis.

The approach to and management of critically ill patients is one of the most versatile themes in emergency medicine. Patients with cirrhosis of the liver have characteristics that are inherent to their disease that can condition modification in acute emergency treatment. Pathophysiologic changes that occur in cirrhosis merit the implementation of an analysis as to whether the overall management of a critically ill patient can generally be applied to patients with cirrhosis of the liver or if they should be treated in a special manner. Through a review of the medical literature, the available information was examined, and the evidence found on the special management required by those patients was narratively synthesized, selecting the most representative decompensations within chronic disease that require emergency treatment.

Systematic review: recurrent autoimmune liver diseases after liver transplantation.

BACKGROUND: Autoimmune liver diseases (AILD) constitute the third most common indication for liver transplantation (LT) worldwide. Outcomes post LT are generally good but recurrent disease is frequently observed. AIMS: To describe the frequency and risk factors associated with recurrent AILD post-LT and provide recommendations to reduce the incidence of recurrence based on levels of evidence. METHODS: A systematic review was performed for full-text papers published in English-language journals, using the keywords 'autoimmune hepatitis (AIH)', 'primary biliary cholangitis and/or cirrhosis (PBC)', 'primary sclerosing cholangitis (PSC)', 'liver transplantation' and 'recurrent disease'. Management strategies to reduce recurrence after LT were classified according to grade and level of evidence. RESULTS: Survival rates post-LT are approximately 90% and 70% at 1 and 5 years and recurrent disease occurs in a range of 10-50% of patients with AILD. Recurrent AIH is associated with elevated liver enzymes and IgG before LT, lymphoplasmacytic infiltrates in the explants and lack of steroids after LT (Grade B). Tacrolimus use is associated with increased risk; use of ciclosporin and preventive ursodeoxycholic acid with reduced risk of PBC recurrence (all Grade B). Intact colon, active ulcerative colitis and early cholestasis are associated with recurrent PSC (Grade B). CONCLUSIONS: Recommendations based on grade A level of evidence are lacking. The need for further study and management includes active immunosuppression before liver transplantation and steroid use after liver transplantation in autoimmune hepatitis; selective immunosuppression with ciclosporin and preventive ursodeoxycholic acid treatment for primary biliary cholangitis; and improved control of inflammatory bowel disease or even colectomy in primary sclerosing cholangitis.

New concepts in liver cirrhosis: clinical significance of sarcopenia in cirrhotic patients.

The natural history of cirrhotic patients is highly variable due to several factors including hepatic synthetic function, presence and degree of portal hypertension, the cause of cirrhosis, the possibility of resolution of the underlying damaging process, and the occurrence of liver cancer. Currently, D'Amico stage classification and Child-Pugh and Model for End-Stage Liver Disease (MELD) scores constitute the best tools to predict mortality in patients with cirrhosis; however, one of their main limitations is the lack of evaluation of the nutritional and functional status. Most widely recognized complications in cirrhotic patients include ascites, hepatic encephalopathy, variceal bleeding, kidney dysfunction, and hepatocellular carcinoma; however, sarcopenia or severe muscle wasting is one of the most common and frequently hidden complications which negatively impact survival, quality of life, and response to stressor, such as infection and surgery. In this review, we discuss the current accepted and new methods to evaluate prognosis in cirrhosis, and also analyze the current knowledge regarding incidence and clinical impact of malnutrition and sarcopenia in cirrhosis and their impact after liver transplantation. We also discuss existing and potential novel therapeutic strategies for malnutrition in cirrhosis, emphasizing the recognition of sarcopenia in cirrhosis in an effort to improve survival and reduced morbidity related to cirrhosis. Finally, we propose that future studies including sarcopenia with the MELD score may allow better prediction of mortality among cirrhotic patients waiting for liver transplantation; however, due to the worldwide shortage of organs for transplants, one of the vital clinical questions is the feasibility to treat sarcopenia in cirrhosis without the need of liver transplant.

Systematic review: managing suboptimal treatment responses in autoimmune hepatitis with conventional and nonstandard drugs.

BACKGROUND: Corticosteroid treatment for autoimmune hepatitis has been shown by randomised controlled clinical trials to ameliorate symptoms, normalise liver tests, improve histological findings and extend survival. Nevertheless, suboptimal responses to corticosteroid treatment still occur. AIM: To describe the current definitions, frequencies, clinical relevance and treatment options for suboptimal responses, and to discuss alternative medications that have been used off-label for these occurrences. METHODS: Literature search was made for full-text papers published in English using the keyword 'autoimmune hepatitis'. Authors' personal experience and investigational studies also helped to identify important contributions to the literature. RESULTS: Suboptimal responses to standard therapy include treatment failure (7%), incomplete response (14%), drug toxicity (13%) and relapse after drug withdrawal (50-86%). The probability of a suboptimal response prior to treatment is higher in young patients and in patients with a severe presentation, jaundice, high MELD score at diagnosis, multilobular necrosis or cirrhosis, antibodies to soluble liver antigen, or inability to improve by clinical indices within two weeks or by MELD score within 7 days of conventional corticosteroid treatment. Management strategies have been developed for the adverse responses and nonstandard drugs, including mycophenolate mofetil, budesonide, ciclosporin, tacrolimus, sirolimus and rituximab, are emerging as rescue therapies or alternative frontline agents. CONCLUSIONS: Once diagnosed, the suboptimal response should be treated by a highly individualised and well-monitored regimen, preferentially using first-line therapy. Nonstandard drugs warrant consideration as salvage or second-line therapies.

Cyclosporine A protects against primary biliary cirrhosis recurrence after liver transplantation.

Primary biliary cirrhosis (PBC) reoccurs in a proportion of patients following liver transplantation (LT). The aims of our study were to evaluate the risk factors associated with PBC recurrence and determine whether recurrent disease constitutes a negative predictor for survival. One hundred and eight patients receiving LT for end-stage PBC were studied. Recurrent disease was diagnosed in 28 patients (26%). Probability of recurrent PBC at 5 years was 13% and 29% at 10 years with an overall incidence of 3.97 cases per 100 patient years. By univariate Cox analysis use of tacrolimus (HR 6.28, 95% CI, 2.44-16.11, p < 0.001) and mycophenolate mofetil (HR 5.21, 95% CI, 1.89-14.33, p = 0.001) were associated with higher risk of recurrence; whereas use of cyclosporine A (CsA) and azathioprine were associated with reduced risk of recurrence (HR 0.13, 95% CI 0.05-0.35, p < 0.001 and HR 0.27, 95% CI 0.11-0.64, p = 0.003, respectively). In the multivariate Cox analysis, only CsA was independently associated with protection against recurrence (HR 0.17, 95% CI 0.06-0.71, p = 0.02). Five-year probability of survival was 83% and 96%, in patients without and with recurrence (log-rank test, p = 0.3). Although PBC transplant recipients receiving CsA have a lower risk of disease recurrence, the development of recurrent PBC did not impact on long-term patient survival.

Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival.

BACKGROUND: Budd-Chiari syndrome carries significant mortality, but factors predicting this outcome are uncertain. AIM: To determine factors associated with 3-month mortality and compare outcomes after surgical shunting or liver transplantation. METHODS: From 1985 to 2008, 51 patients with Budd-Chiari syndrome were identified. RESULTS: By logistic regression analysis, features associated with higher risk of 3-month mortality were Rotterdam class III, Clichy >6.6, model for end-stage liver disease (MELD) >20 and Child-Pugh C. Rotterdam class III had the best performance to discriminate 3-month mortality with sensitivity of 0.89 and specificity of 0.63, whereas Clichy >6.60 had sensitivity of 0.78 and specificity of 0.69; MELD >20 had sensitivity of 0.78 and specificity of 0.75 and Child-Pugh C had sensitivity of 0.67 and specificity of 0.72. Eighteen patients underwent surgical shunts and 14 received liver transplantation with no significant differences in survival (median survival 10 +/- 3 vs. 8 +/- 2 years; log-rank, P = 0.9). CONCLUSIONS: Rotterdam score is the best discrimination index for 3-month mortality in Budd-Chiari syndrome and should be used preferentially to determine treatment urgency. Surgical shunts constitute an important therapeutic modality that may help save liver grafts and prolong transplantation-free survival in a selected group of patients with Budd-Chiari syndrome.

Gastric capacity is related to body mass index in obese patients. A study using the water load test.

BACKGROUND: The role of gastrointestinal function in obesity is unknown. Recent studies have shown that satiety in obese patients is influenced by an abnormal gastric capacity. AIM: An easy and non-invasive tool, the water load test (WLT) was used to evaluate gastric capacity and how it relates to body mass index (BMI) in obese patients. METHODS: The WLT was performed in 32 patients with high BMI and 12 healthy volunteers. Water was ingested at a 15 mL/min rate. The maximal tolerable volume (MTV) was defined as the total ingested volume when patients stopped the test. RESULTS: A BMI > 30 was significantly associated with higher water consumption (2339 ± 306 mL) compared to controls (1830 ± 240 mL, p = 0.001). The MTV had a positive correlation with BMI (r = 0.68, p = 0.001). CONCLUSIONS: Obese subjects have an increased gastric capacity, as measured with the WLT. This greater drinking capacity has a positive correlation with the subjects' BMI.

[Effect of the administration of rectal indomethacin on amylase serum levels after endoscopic retrograde cholangiopancreatography, and its impact on the development of secondary pancreatitis episodes]. / Efecto de la administración de indometacina rectal sobre los niveles séricos de amilasa posteriores a colangiopancreatografía retrógrada endoscópica y su impacto en la aparición de episodios de pancreatitis secundaria.

BACKGROUND: Hyperamylasemia and acute pancreatitis represent the most frequent major complication after endoscopic retrograde cholangiopancreatography (ERCP), developing in 1-30% of cases. OBJECTIVE: To determine the incidence of hyperamylasemia and acute pancreatitis after ERCP, and to assess the utility of rectal indomethacin to prevent these events. MATERIAL AND METHODS: A randomized clinical trial. During a 12-month period 150 patients were included. They were divided up into a study group (n = 75), where 100 mg of rectal indomethacin were administered 2 hours prior to the procedure, and a control group (n = 75), which received rectal glycerin. Two hours after ERCP serum amylase levels were measured and classified as follows: 0or=600 IU/L. Clinical pancreatitis episodes were quantified and classified according to Ranson's criteria. RESULTS: Gender distribution: 100 women and 50 men. Mean age: 55.37 +/- 18.0 for the study group, and 51.1 +/- 17.0 for the control group. A diagnosis of benign pathology was present in 56 (74.7%) cases in the study group, and 59 (78.7%) controls. After ERCP 13 (17.3%) patients in the study group and 28 (37.3%) in the control group developed hyperamylasemia (p (2) 0.05). Hyperamylasemia > 600 IU/L was found in 3 patients in the study group, and in 10 in the control group (p = 0.001). Mild pancreatitis was detected in 4 (5.3%) patients in the study group, and in 12 (16%) patients in the control group (p = 0.034). There were no deaths or adverse drug reactions. CONCLUSIONS: Rectal indomethacin before ERCP decreases the risk of hyperamylasemia and pancreatitis. Indomethacine is a feasible, low-cost drug with minimal or nil side effects.

Efecto de la administración de indometacina rectal sobre los niveles séricos de amilasa posteriores a colangiopancreatografía retrógrada endoscópica y su impacto en la aparición de episodios de pancreatitis secundaria / Effect of the rectal administration of indomethacin on amylase serum levels after endoscopic retrograde cholangiopancreatography, and its impact on the development of secondary pancreatitis episodes

Introducción: hiperamilasemia y pancreatitis aguda representan las complicaciones mayores más frecuentes posteriores a colangiopancreatografía retrógrada endoscópica (CPRE), apareciendo en 1-30% de los casos. Objetivo: determinar la incidencia de hiperamilasemia y pancreatitis posterior a CPRE y evaluar la utilidad de indometacina rectal para la prevención de estos. Material y métodos: ensayo clínico controlado. Durante un periodo de 12 meses se incluyeron 150 pacientes. Estos fueron divididos en grupo de estudio (n = 75), a quienes se administró indometacina rectal 100 mg 2 horas previas al procedimiento, y control (n = 75) que recibió glicerina. Dos horas posteriores a la CPRE se determinó el nivel de amilasa sérica y se clasificaron en: 0 = 600 UI/l. Los episodios de pancreatitis clínica se cuantificaron y clasificaron de acuerdo a los criterios de Ranson. Resultados: distribución por género: 100 mujeres y 50 hombres. Edad media: 55,37 ± 18,0 para el grupo de estudio y 51,1 ± 17,0 para el control. El diagnóstico de patología benigna se presentó en 56 (74,7%) casos del grupo de estudio y 59 (78,7%) del control. Posterior al procedimiento, 13 (17,3%) pacientes del grupo experimental y 28 (37,3%) del control desarrollaron hiperamilasemia (p 600 UI/l en 3 pacientes del grupo de estudio y 10 del control (p = 0,001). Se detectó pancreatitis leve en 5,3% de los pacientes del grupo de estudio y 16% del control (p < 0,05). No hubo mortalidad ni eventos adversos. Conclusiones: indometacina rectal previo a CPRE disminuye el riesgo de hiperamilasemia y pancreatitis. La indometacina es accesible, de bajo costo con mínimos o nulos efectos secundarios

Background: hyperamylasemia and acute pancreatitis represent the most frequent major complication after endoscopic retrograde cholangiopancreatography (ERCP), developing in 1-30% of cases. Objective: to determine the incidence of hyperamylasemia and acute pancreatitis after ERCP, and to assess the utility of rectal indomethacin to prevent these events. Material and methods: a randomized clinical trial. During a 12-month period 150 patients were included. They were divided up into a study group (n = 75), where 100 mg of rectal indomethacin were administered 2 hours prior to the procedure, and a control group (n = 75), which received rectal glycerin. Two hours after ERCP serum amylase levels were measured and classified as follows: 0 = 600 IU/L. Clinical pancreatitis episodes were quantified and classified according to Ranson’s criteria. Results: gender distribution: 100 women and 50 men. Mean age: 55.37 ± 18.0 for the study group, and 51.1 ± 17.0 for the control group. A diagnosis of benign pathology was present in 56 (74.7%) cases in the study group, and 59 (78.7%) controls. After ERCP 13 (17.3%) patients in the study group and 28 (37.3%) in the control group developed hyperamylasemia (p 600 IU/L was found in 3 patients in the study group, and in 10 in the control group (p = 0.001). Mild pancreatitis was detected in 4 (5.3%) patients in the study group, and in 12 (16%) patients in the control group (p = 0.034). There were no deaths or adverse drug reactions. Conclusions: rectal indomethacin before ERCP decreases the risk of hyperamylasemia and pancreatitis. Indomethacine is a feasible, low-cost drug with minimal or nil side effects

[Seropositivity for Chlamydia pneumoniae in patients with primary biliary cirrhosis]. / Seropositividad para Chlamydia pneumoniae en pacientes con cirrosis biliar primaria.

INTRODUCTION: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by inflammatory injury and bile duct destruction. Recent studies suggest that Chlamydia pneumoniae could be associated with the development of PBC. The aim of this study was to determine the seroprevalence of C. pneumoniae in a cohort of patients with PBC. PATIENTS AND METHODS: The presence of IgG antibodies against C. pneumoniae was investigated in 46 patients with PBC and in 105 subjects without cirrhosis. RESULTS: Twenty-one patients (46%) with PBC had antibodies against C. pneumoniae compared with 74 subjects (71%) in the control group (OR = 0.6; 95% CI, 0.3-1.2; p = NS). Subanalysis of the PBC group showed that patients with C. pneumoniae antibodies had a higher frequency of advanced Child-Pugh stages (24% A, 52% B and 24% C vs 64% A, 32% B and 4% C; p = 0.01), a higher score on the Mayo Clinic Prognostic Index (7.8 +/- 2.1 vs 5.6 +/- 1.2; p = 0.004), a higher frequency of ascites (29% vs 4%; OR = 9.6; 95% CI, 1-87; p = 0.02), higher total bilirubin levels (4.5 +/- 2.5 mg/dl vs 2.4 +/- 4.3 mg/dl, p = 0.001) and lower serum albumin levels (2.6 +/- 0.9 g/dl vs 3.3 +/- 0.6 g/dl, p = 0.02). CONCLUSION: No association was found between C. pneumoniae infection and PBC in this study. An association was found between the severity of PBC and C. pneumoniae, which may suggest a deleterious effect of C. pneumoniae infection or a predisposition in advanced stages of PBC to acquire infection with this microorganism.

Seropositividad para Chlamydia pneumoniae en pacientes con cirrosis biliar primaria / Seropositivity for Chlamydia pneumoniae in patients with primary biliary cirrhosis

Introducción: La cirrosis biliar primaria (CBP) es una enfermedad hepática colestásica crónica, que se caracteriza por lesión inflamatoria y destrucción de conductos biliares. Estudios recientes indican que Chlamydia pneumoniae podría estar asociada al desarrollo de CBP. El objetivo de este estudio ha sido determinar la seroprevalencia de C. pneumoniae en una cohorte de pacientes con CBP. Pacientes y métodos: Se buscaron anticuerpos antiinmunoglobulina G contra C. pneumoniae en 46 pacientes con CBP y 105 sujetos sin cirrosis hepática. Resultados: Entre los pacientes con CBP, 21 (46%) tuvieron anticuerpos, en comparación con 74 (71%) del grupo control, con una odds ratio de 0,6 (intervalo de confizanza del 95%, 0,3-1,2; p no significativa). El subanálisis del grupo con CBP mostró que los pacientes seropositivos presentaron mayor frecuencia de estadios avanzados de Child-Pugh (el 24% A, el 52% B y el 24% C, frente a un 64% A, un 32% B y un 4% C; p = 0,01), mayor puntuación en la escala pronóstica de la Clínica Mayo (7,8 ± 2,1 frente a 5,6 ± 1,2; p = 0,004), mayor frecuencia de ascitis (un 29 frente a un 4%; odds ratio de 9,6; intervalo de confianza del 95%, 1,1-87; p = 0,02), concentraciones mayores de bilirrubina total (4,5 ± 2,5 frente a 2,4 ± 4,3 mg/dl; p = 0,001) y valores menores de albúmina (2,6 ± 0,9 frente a 3,3 ± 0,6 g/dl; p = 0,02). Conclusión: No fue posible establecer una asociación entre C. pneumoniae y CBP. Encontramos una relación entre la gravedad de la CBP y la seropositividad para C. pneumoniae, lo que podría apuntar a un efecto perjudicial de esta infección o bien a una predisposición en etapas avanzadas para adquirir infecciones por C. pneumoniae

Introduction: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by inflammatory injury and bile duct destruction. Recent studies suggest that Chlamydia pneumoniae could be associated with the development of PBC. The aim of this study was to determine the seroprevalence of C. pneumoniae in a cohort of patients with PBC. Patients and Methods: The presence of IgG antibodies against C. pneumoniae was investigated in 46 patients with PBC and in 105 subjects without cirrhosis. Results: Twenty-one patients (46%) with PBC had antibodies against C. pneumoniae compared with 74 subjects (71%) in the control group (OR = 0.6; 95% CI, 0.3-1.2; p = NS). Subanalysis of the PBC group showed that patients with C. pneumoniae antibodies had a higher frequency of advanced Child-Pugh stages (24% A, 52% B and 24% C vs 64% A, 32% B and 4% C; p = 0.01), a higher score on the Mayo Clinic Prognostic Index (7.8 ± 2.1 vs 5.6 ± 1.2; p = 0.004), a higher frequency of ascites (29% vs 4%; OR = 9.6; 95% CI, 1-87; p = 0.02), higher total bilirubin levels (4.5 ± 2.5 mg/dl vs 2.4 ± 4.3 mg/dl, p = 0.001) and lower serum albumin levels (2.6 ± 0.9 g/dl vs 3.3 ± 0.6 g/dl, p = 0.02). Conclusion: No association was found between C. pneumoniae infection and PBC in this study. An association was found between the severity of PBC and C. pneumoniae, which may suggest a deleterious effect of C. pneumoniae infection or a predisposition in advanced stages of PBC to acquire infection with this microorganism

[Predictive factors for portal hypertension in patients with primary sclerosing cholangitis]. / Factores predictores de hipertensión portal en los enfermos con colangitis esclerosante primaria.

BACKGROUND: Primary sclerosing cholangitis (PSC) is characterized by progressive destruction of bile ducts, which may lead to cirrhosis and portal hypertension. The factors associated with the presence of esophageal varices (EV) and the risk of bleeding have not been well defined. AIM: To determine the factors associated with the presence of EV and risk of bleeding in a cohort of patients with PSC. MATERIAL AND METHODS: We analyzed the demographic, biochemical and endoscopic characteristics, and follow-up of 32 patients with a diagnosis of PSC. All patients underwent endoscopic evaluation to determine the presence of EV at diagnosis and annually during follow-up. RESULTS: There were 24 men (75%) and 8 women (25%). The mean age was 40.2 years (range, 19-66). At diagnosis, none of the patients had a previous history of variceal bleeding and 4 (13%) had EV on endoscopic examination. In bivariate analysis, the factors associated with the presence of EV were: splenomegaly (4/6 vs 0/26; p < 0.001), ascites (2/4 vs 0/24; p < 0.001), thrombocytopenia (96 +/- 27 vs 299 +/- 135 x 10(3), p < 0.001), and hypoalbuminemia (2.4 +/- 0.6 vs 3.5 +/- 0.6 g/dl; p = 0.005). During a mean follow-up period of 7 years (range, 2-15 years), 6 patients developed EV and 7 patients had at least one episode of variceal bleeding. In logistic regression analysis, the factors independently associated with the presence of EV at diagnosis were thrombocytopenia (p = 0.001) and splenomegaly (p = 0.01). The factors associated with variceal bleeding were worsening of liver function (p = 0.01) and splenomegaly (p = 0.02). CONCLUSIONS: There are noninvasive indicators of portal hypertension that could predict the presence of EV and risk of bleeding in patients with PSC. The presence of thrombocytopenia, splenomegaly or worsening of liver function should be detected in these patients, as they could benefit from endoscopic surveillance.

Factores predictores de hipertensión portal en los enfermos con colangitis esclerosante primaria / Predictive factors for portal hypertension in patients with primary sclerosing cholangitis

Objetivo: Determinar en una cohorte de enfermos con CEP la frecuencia de VE y los factores asociados para predecir su presencia y riesgo de hemorragia. Material y métodos: Se evaluaron las características demográficas, bioquímicas, endoscópicas y la evolución clínica de 32 enfermos con CEP. En el momento del diagnóstico y con un seguimiento promedio anual a todos se les realizó estudio endoscópico para determinar la presencia de VE. Resultados: Veinticuatro pacientes eran varones (75%) y 8, mujeres (25%). El promedio de edad fue de 40,2 años (intervalo mínimo-máximo, 19-66). En su primera endoscopia ningún paciente tenía historia de hemorragia varicosa y 4 (13%) presentaron VE. En el análisis bivariado, los factores que se asociaron a la presencia de VE fueron: esplenomegalia (4/6 frente a 0/26; p < 0,001), ascitis (2/4 frente a 0/24; p < 0,001), trombocitopenia (96 ± 27 frente a 299 ± 135/103, p < 0,001) e hipoalbuminemia (2,4 ± 0,6 frente a 3,5 ± 0,6 g/dl; p = 0,005). Durante un seguimiento promedio de 7 años (intervalo mínimo-máximo, 2-15) 6 pacientes desarrollaron VE y 7 tuvieron, al menos, un episodio de hemorragia. En el análisis de regresión logística los factores que se asociaron de manera independiente a la presencia de varices fueron trombocitopenia (p = 0,001) y esplenomegalia (p = 0,01). Los factores asociados a hemorragia varicosa fueron el deterioro de la función hepática (p = 0,01) y la esplenomegalia (p = 0,02). Conclusiones: En pacientes con CEP existen marcadores no invasivos de hipertensión portal que pueden ser útiles para predecir la presencia de VE y un mayor riesgo para hemorragia varicosa. Es importante identificar la presencia de esplenomegalia, trombocitopenia y deterioro de la función hepática en estos pacientes, ya que podrían beneficiarse con la vigilancia endoscópica

Background: Primary sclerosing cholangitis (PSC) is characterized by progressive destruction of bile ducts, which may lead to cirrhosis and portal hypertension. The factors associated with the presence of esophageal varices (EV) and the risk of bleeding have not been well defined. Aim: To determine the factors associated with the presence of EV and risk of bleeding in a cohort of patients with PSC. Material and methods: We analyzed the demographic, biochemical and endoscopic characteristics, and follow-up of 32 patients with a diagnosis of PSC. All patients underwent endoscopic evaluation to determine the presence of EV at diagnosis and annually during follow-up. Results: There were 24 men (75%) and 8 women (25%). The mean age was 40.2 years (range, 19-66). At diagnosis, none of the patients had a previous history of variceal bleeding and 4 (13%) had EV on endoscopic examination. In bivariate analysis, the factors associated with the presence of EV were: splenomegaly (4/6 vs 0/26; p < 0.001), ascites (2/4 vs 0/24; p < 0.001), thrombocytopenia (96 ± 27 vs 299 ± 135 x 103, p < 0.001), and hypoalbuminemia (2.4 ± 0.6 vs 3.5 ± 0.6 g/dl; p = 0.005). During a mean follow-up period of 7 years (range, 2-15 years), 6 patients developed EV and 7 patients had at least one episode of variceal bleeding. In logistic regression analysis, the factors independently associated with the presence of EV at diagnosis were thrombocytopenia (p = 0.001) and splenomegaly (p = 0.01). The factors associated with variceal bleeding were worsening of liver function (p = 0.01) and splenomegaly (p = 0.02). Conclusions: There are noninvasive indicators of portal hypertension that could predict the presence of EV and risk of bleeding in patients with PSC. The presence of thrombocytopenia, splenomegaly or worsening of liver function should be detected in these patients, as they could benefit from endoscopic surveillance

Factores predictores de hipertensión portal en los enfermos con colangitis esclerosante primaria / Predictive factors for portal hypertension in patients with primary sclerosing cholangitis

Objetivo: Determinar en una cohorte de enfermos con CEP la frecuencia de VE y los factores asociados para predecir su presencia y riesgo de hemorragia. Material y métodos: Se evaluaron las características demográficas, bioquímicas, endoscópicas y la evolución clínica de 32 enfermos con CEP. En el momento del diagnóstico y con un seguimiento promedio anual a todos se les realizó estudio endoscópico para determinar la presencia de VE. Resultados: Veinticuatro pacientes eran varones (75%) y 8, mujeres (25%). El promedio de edad fue de 40,2 años (intervalo mínimo-máximo, 19-66). En su primera endoscopia ningún paciente tenía historia de hemorragia varicosa y 4 (13%) presentaron VE. En el análisis bivariado, los factores que se asociaron a la presencia de VE fueron: esplenomegalia (4/6 frente a 0/26; p < 0,001), ascitis (2/4 frente a 0/24; p < 0,001), trombocitopenia (96 ± 27 frente a 299 ± 135/103, p < 0,001) e hipoalbuminemia (2,4 ± 0,6 frente a 3,5 ± 0,6 g/dl; p = 0,005). Durante un seguimiento promedio de 7 años (intervalo mínimo-máximo, 2-15) 6 pacientes desarrollaron VE y 7 tuvieron, al menos, un episodio de hemorragia. En el análisis de regresión logística los factores que se asociaron de manera independiente a la presencia de varices fueron trombocitopenia (p = 0,001) y esplenomegalia (p = 0,01). Los factores asociados a hemorragia varicosa fueron el deterioro de la función hepática (p = 0,01) y la esplenomegalia (p = 0,02). Conclusiones: En pacientes con CEP existen marcadores no invasivos de hipertensión portal que pueden ser útiles para predecir la presencia de VE y un mayor riesgo para hemorragia varicosa. Es importante identificar la presencia de esplenomegalia, trombocitopenia y deterioro de la función hepática en estos pacientes, ya que podrían beneficiarse con la vigilancia endoscópica

Background: Primary sclerosing cholangitis (PSC) is characterized by progressive destruction of bile ducts, which may lead to cirrhosis and portal hypertension. The factors associated with the presence of esophageal varices (EV) and the risk of bleeding have not been well defined. Aim: To determine the factors associated with the presence of EV and risk of bleeding in a cohort of patients with PSC. Material and methods: We analyzed the demographic, biochemical and endoscopic characteristics, and follow-up of 32 patients with a diagnosis of PSC. All patients underwent endoscopic evaluation to determine the presence of EV at diagnosis and annually during follow-up. Results: There were 24 men (75%) and 8 women (25%). The mean age was 40.2 years (range, 19-66). At diagnosis, none of the patients had a previous history of variceal bleeding and 4 (13%) had EV on endoscopic examination. In bivariate analysis, the factors associated with the presence of EV were: splenomegaly (4/6 vs 0/26; p < 0.001), ascites (2/4 vs 0/24; p < 0.001), thrombocytopenia (96 ± 27 vs 299 ± 135 x 103, p < 0.001), and hypoalbuminemia (2.4 ± 0.6 vs 3.5 ± 0.6 g/dl; p = 0.005). During a mean follow-up period of 7 years (range, 2-15 years), 6 patients developed EV and 7 patients had at least one episode of variceal bleeding. In logistic regression analysis, the factors independently associated with the presence of EV at diagnosis were thrombocytopenia (p = 0.001) and splenomegaly (p = 0.01). The factors associated with variceal bleeding were worsening of liver function (p = 0.01) and splenomegaly (p = 0.02). Conclusions: There are noninvasive indicators of portal hypertension that could predict the presence of EV and risk of bleeding in patients with PSC. The presence of thrombocytopenia, splenomegaly or worsening of liver function should be detected in these patients, as they could benefit from endoscopic surveillance