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Acute on chronic liver failure is an increasingly recognized syndrome characterized by acute decompensation of chronic liver disease associated with organ failure and high short-term mortality. ACLF is frequent, affecting between 24 and 40% of patients admitted for complications of cirrhosis. Sepsis, active alcoholism, and relapse of chronic viral hepatitis are the most frequent precipitating factors. However, in up to 40%­50% of the cases of ACLF have no identifiable trigger. The stage of severity of Acute on chronic liver failure is very important because it allows us to stratify patients according to their prognosis, evaluate therapeutic response, determine transplant urgency, deciding intensive care unit admission, and also have a basis on which to decide therapeutic futility. (AU)

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The gastrointestinal system is closely related to the skin. Multiple disorders of the gastrointestinal tract that manifest clinically on the skin surface are recognized, which can even occur before establishing the definitive diagnosis of the disease. For this reason, it is necessary that the dermatologist is internalized regarding the wide variety of clinical signs that can guide the etiological study, which requires in most cases, a multidisciplinary management. (AU)

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Non-alcoholic fatty liver disease (NAFLD) is currently considered in Chile and worldwide, as the main cause of cirrhosis and liver transplantation. It is therefore one of the main public health objectives for reducing its prevalence. In last years, it was suggested that the intestinal microbiota (IM) might contribute to the pathophysiology of NAFLD, as well as in the progression toward nonalcoholic steatohepatitis (NASH) and cirrhosis. It is known that changes in the composition of IM are associated with alterations in intestinal permeability and the production of inflammatory metabolites. These alterations are part of the pathophysiological mechanisms leading to the development of NASH. However studies on MI in patients with NAFLD and NASH in Chile are scarce. Through a research grant, recently awarded at the Hospital Clínico Universidad de Chile, we aim to confirm and characterize the intestinal dysbiosis associated with NAFLD in Chilean patients and to establish the relationship between the changes in microbial composition with the progression of liver damage. The description of these alterations represents an opportunity to explore new therapeutic approaches for future interventions. In effect, through the restoration of an intestinal microbial environment towards homeostasis in these patients, we expect to reverse or improve the progression of damage provoked by this disease. (AU)

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Nonalcoholic fatty liver disease (NAFLD) is the hepatic manifestation of the metabolic syndrome, characterized by accumulation of fat on the liver parenchyma with a heterogeneous clinical presentation. In recent years its prevalence and incidence has been increasing in association with the rise in obesity and its comorbidities, becoming one of the main causes of cirrhosis. The cause of NAFLD is multifactorial, and currently the role of intestinal microbiota and its interaction with the metabolic syndrome and NAFLD has become of interest. Different pathophysiological phenomena have emerged, the main being intestinal dysbiosis, loss of intestinal barrier, bacterial translocation, with activation of inflammation and oxidative stress, and production of active metabolites, such as ethanol. The management of this factor seems promising and adds to the classic approach of changes in lifestyle. Probiotics are the most studied tool and show evidence of effectiveness, however further studies are needed.

La enfermedad por hígado graso no alcohólico (EHGNA) es la manifestación hepática del síndrome metabólico,caracterizándose por acumulación de grasa a nivel del parénquima con una heterogénea presentación clínica. En los últimos años su prevalencia e incidencia ha ido en aumento, en asociación al aumento de la obesidad y sus comorbilidades, transformándose en una de las principales etiologías de cirrosis hepática. La causa de la EHGNA es multifactorial, siendo de interés en la actualidad el rol de la microbiota intestinal y su interacción con el síndrome metabólico y la EHGNA. Distintos fenómenos fisiopatológicos se han planteado, siendo los principales la disbiosis intestinal, la pérdida de la barrera intestinal, la translocación bacteriana con activación de la cascada de la inflamación y estrés oxidativo y la producción de metabolitos activos como el etanol. El manejo de este factor parece promisorio y se agrega al clásico enfrentamiento de cambios en estilo de vida. Los probióticos son la herramienta más estudiada y disponen de evidencia de efectividad, pero con necesidad de mayores estudios.

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Excessive alcohol consumption is an important cause of preventable morbidity and mortality. We have to bealert to chronic alcohol usage or abuse. Simple tests (AUDIT, CAGE) can be applied quickly on outpatientcare. We highlight advances in understanding the immune and molecular mechanisms; there is disruptionof the intestinal barrier with bacterial translocation, as well as endotoxins which activate the liver’s innateimmunity, causing apoptosis, necrosis, inflammation and fibrosis. Alcoholic hepatitis is most common inpatients between 40 and 60 years of age, preferably male with jaundice, fever, ascites, hepatomegaly. Thediagnosis is confirmed with a history of alcoholic consumption, mild to moderate AST and ALT values,a AST/ALT ratio > 2, hyperbilirrubinemia and prolonged prothrombin time. There are scores to evaluatethe severity and the need of corticoid therapy, such as modified Maddrey discriminating function andMELD score. Lille score assesses the response to treatment in the seventh day. The risks and benefits ofliver biopsy should be evaluated individually. The cornerstone of treatment remains alcohol abstinence.Nutritional management must be carefully monitored. Proteins requirements are standardized based onweight. The use of corticoids with 40 mg of prednisolone each day is the most widely accepted therapy,indicated on patients with MMDF higher than 32 or MELD score higher than 21. If Lille score is higherthan 0.45 at the seven day under corticoid therapy, treatment must be interrupted. The use of pentoxifyllinewould only be effective for prevention of hepatorenal syndrome...

El consumo excesivo de alcohol es una causa importante de morbimortalidad prevenible. Debemos estaratentos en detectar a pacientes con dependencia o abuso crónico de alcohol. Test sencillos (AUDIT, CAGE)pueden aplicarse rápidamente en consulta ambulatoria. Destacamos avances en el conocimiento moleculare inmunológico, existe disrupción de la barrera intestinal con translocación bacteriana y endotoxinas conactivación del sistema inmune innato del hígado, produciendo apoptosis celular, necrosis e inflamación yfibrosis. La hepatitis alcohólica se presenta principalmente en pacientes entre 40 y 60 años, preferentementeen varones con ictericia, fiebre, ascitis, hepatomegalia. El diagnóstico se confirma con antecedentes deingesta alcohólica, GOT y GPT elevadas en forma leve o moderada, relación GOT/GPT mayor de 2, hiperbilirrubinemiay tiempo de protrombina prolongado. Existen scores para evaluar la gravedad y necesidad demanejo con corticoides como función discriminante de Maddrey modificada y MELD. El puntaje de Lilleevalúa respuesta del tratamiento al séptimo día. El riesgo/beneficio de la biopsia hepática se evalúa caso acaso. La piedra angular del tratamiento sigue siendo la abstinencia. Manejo nutricional debe ser riguroso.Requerimientos proteicos están estandarizados por peso. La terapia con corticoides (prednisolona 40 mg/día) es la más ampliamente aceptada, con indicación en pacientes con FDMm mayor a 32 o MELD mayora 21. Si el puntaje de Lille es mayor de 0,45 a los 7 días con corticoides, deben suspenderse. Pentoxifilinasólo tendría efecto en prevenir el desarrollo de síndrome hepatorrenal (SHR). Hay nuevas terapias enevaluación, como el uso de G-CSF...

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Autoimmune hepatitis refractory to corticod therapy is infrequent. Generally, it occurs in severe cases and in young patients. There are several treatment options, including higher doses of the regular medications and, eventually, the use of other immunosuppressive drugs with higher potency, such as mycophenolate, cyclosporin, tacrolimus, among others.

La hepatitis autoinmune refractaria a terapia corticoidal afortunadamente es poco frecuente. Generalmente ocurre en casos de presentación más grave y en pacientes jóvenes. Existen varios esquemas para su enfrentamiento, incluyendo mayores dosis de terapia habitual y, eventualmente, el uso de otros fármacos inmunosupresores de mayor potencia como micofenolato, ciclosporina, tacrolimus y otros.

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Gastric antral vascular ectasia is an uncommon cause of chronic anemia, occasionally associated with cirrhosis. The most accepted therapy is argon plasma coagulation (APC), however there are refractory cases. We report two females with cirrhosis, aged 60 and 72 years, in whom management with APC was insufficient and in whom the need for hospital admissions and transfusions were reduced using the technique of endoscopic band ligation.

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Gastric antral vascular ectasia is an uncommon cause of chronic anemia, occasionally associated with cirrhosis. The most accepted therapy is argon plasma coagulation (APC), however there are refractory cases. We report two females with cirrhosis, aged 60 and 72 years, in whom management with APC was insufficient and in whom the need for hospital admissions and transfusions were reduced using the technique of endoscopic band ligation.

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Nitrofurantoin, commonly used for prolonged periods, can produce different patterns of liver damage. Patients: 12 women, mean age 55 years (range 17-72), with recurrent urinary infections, treated with nitrofurantoin for long periods of time (2 months to 15 years), who presented with secondary liver disease. Results: 7 had acute hepatitis (3 fulminant), 3 chronic hepatitis, and 2 cirrhosis. All acute cases had consistent liver biopsies, and 2 were treated with steroids and azathioprine for 2 and 7 months, with liver tests normalization. Two fulminant cases were transplanted (submassive hepatic necrosis on explanted livers) and 1 was successfully treated with steroids and mycofenolate. The 3 cases of chronic hepatitis also had confirmatory biopsies and 1 received steroids and azathioprine, with full recovery. The other 2 responded to the drug withdrawal and the 2 cirrhotic patients had only symptomatic treatment. All patients were negative for hepatitis virus, 7 (58 percent had positive anti-nuclear and/or anti-smooth muscle antibodies and 4 (33 percent) had elevated IgG levels. Conclusions: Nitrofurantoin may cause severe acute liver disease, even requiring liver transplantation. Nitrofurantoin can also cause chronic liver disease, have markers of autoimmunity and respond to immunosuppressive therapy. These data confirmed that nitrofurantoin can induce liver diseases, probably due to immunological mechanisms.

La nitrofurantoína, comúnmente utilizada por períodos prolongados, puede producir daño hepático, con diferentes formas de presentación y evolución. Pacientes: 12 mujeres, edad promedio 55 años (rango 17 a 72), con infecciones urinarias recurrentes, usuarias de nitrofurantoína por períodos prolongados (2 meses a 15 años), que presentaron daño hepático asociado a la droga. Resultados: 7 casos de hepatitis aguda (3 fulminantes), 3 casos de hepatitis crónica y 2 casos de cirrosis. Todos los casos de hepatitis agudas tenían biopsia hepática compatible y 2 fueron tratadas con corticoides y azatioprina por 2 y 7 meses, con normalización de los exámenes. De las 3 pacientes con hepatitis fulminante, 2 fueron trasplantadas (necrosis submasiva en el hígado explantado) y 1 fue tratada con corticoides y micofenolato, con buena respuesta. Los 3 casos de hepatitis crónica tenían confirmación histológica y 1 se trató con corticoides y azatioprina, con excelente evolución. Las otras pacientes respondieron favorablemente sólo a la suspensión del fármaco. Los 2 casos con cirrosis han recibido tratamiento sintomático. Todas las pacientes fueron negativas para los virus hepatitis, 7/12 (58 por ciento) tenían anticuerpos antinucleares y/o antimúsculo liso positivos y 4/12 (33 por ciento) IgG elevada. Conclusión: La nitrofurantoína puede provocar una severa enfermedad hepática aguda, requiriendo incluso trasplante hepático. Además, puede producir hepatitis crónica y cirrosis, tener marcadores de autoinmunidad y buena respuesta a la terapia inmunosupresora habitual. Lo anterior confirma su capacidad de inducir un daño hepático, probablemente por mecanismos inmunológicos.

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El pioderma gangrenoso (PG) es una dermatitis neutrofílica, que en algunos casos puede ser severa y de difícil manejo. Presentamos el caso de una paciente con múltiples lesiones de PG asociadas a colitis ulcerosa, en la cual, por severidad del cuadro, se optó por adicionar infliximab al tratamiento habitual. La respuesta clínica fue excelente y rápida tras la primera dosis de infliximab, pese a que recibió solo dos de las tres dosis recomendadas habitualmente. La mejoría cutánea y digestiva se ha mantenido un año después de este tratamiento. Infliximab ha demostrado ser, en este y otros reportes, una herramienta muy útil, especialmente en casos de compromiso severo como en nuestra paciente. Se requiere mas evidencia aún para comprobar en cuáles pacientes podría ser beneficioso. Se presenta este caso por su severidad y la rápida y sostenida respuesta obtenida con infliximab.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis and in some cases can be severe and difficult to manage. We report the case of a patient with multiple lesions of PG associated with ulcerative colitis. Due to the severity of the clinical presentation treatment with infliximab was added to standard therapy. Clinical response was excellent and fast after the first dose of infliximab, although ha received only two of the three doses usually recomended. Skin and digestive improvement has been maintained 1 year after treatment. Infliximab has proven, in this and other reports, as a very useful tool in the treatment of PG, especially in cases of severe involvement as in our patient. More evidence is required to prove in which patients with PG infliximab could beneficial. We present this clinical case because of its severity and the rapid and susteined response obtained with infliximab.

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The post-transplant lymphoproliferative disorder (PTLD) corresponds to a heterogeneous group of lymphoproliferative diseases that develop in solid organ and bone marrow transplant recipients. It occurs in 3-10 percent of patients receiving solid organ transplants, mostly children. It is called early PTLD if it occurs in the first year after transplantation, if it affects B-cell lymphocytes and is associated with infection by Epstein-Barr virus. Late presentation occurs after the first year of transplantation and its pathogenesis is less clear. Clinical manifestations vary from a benign mononucleosis-like clinical setting to high-grade tumors with high mortality (40-60 percent). Treatment depends on the extent of the disease, including reduction of immunosuppressive therapy, radiotherapy, surgery and, more recently, the use of anti-CD20 monoclonal antibody. We report the case of a 67 year-old woman presenting with PTLD on the eighth month after receiving a liver graft.

La enfermedad linfoproliferativa difusa postrasplante (ELDP), corresponde a un grupo heterogéneo de desórdenes linfoproliferativos que se desarrollan en receptores de órganos sólidos y médula ósea. Ocurre en 3 a 10 por ciento de los pacientes receptores de órganos sólidos, fundamentalmente pediátricos. Se denomina ELDP precoz si se presenta en el primer año posterior al trasplante, afecta a los linfocitos de estirpe B y se asocia a la infección por virus Epstein-Barr. La presentación tardía ocurre luego del primer año de trasplante y su etiopatogenia es menos clara. Las manifestaciones clínicas varían desde un cuadro benigno similar a la mononucleosis a neoplasias de alto grade, con elevada mortalidad (40-60 por ciento). El tratamiento dependerá de la extensión de la enfermedad, incluyendo reducción del tratamiento inmunosupresor, radioterapia, cirugía y más recientemente el uso de anticuerpos monoclonales anti CD20. Presentamos el caso clínico de una mujer de 67 años, que al octavo mes de recibir un injerto hepático presenta ELDP.

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Background: A possible relationship has been reported between psoriasis and celiac disease, with common pathogenic mechanisms that may need further investigation. Aim: To investigate the presence of clinical and serological markers for celiac disease in a group of Chilean psoriatic patients. Material and methods: We included 80 psoriatic patients (42 males) aged 16 to 79 years, whose serum was tested for antitransglutaminase antibodies (ATGA) and antiendomysial antibodies (AEMA). Patients with weakly positive AEMA tests were also tested for antigliadin antibodies (AGA). Results: In six patients (7.5 percent), AEMA and AGA were positive and one patient was positive for ATGA. An upper gastrointestinal endoscopy and duodenal biopsy was offered to these six patients and five accepted the procedure. Only one had a pathological diagnosis of celiac disease. Conclusions: Only one of 80 patients with psoriasis had celiac disease (1.2 percent). Other four patients with positive serologic markers had a normal duodenal biopsy. This group of patients may have latent celiac disease and they should be followed up.

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BACKGROUND: A possible relationship has been reported between psoriasis and celiac disease, with common pathogenic mechanisms that may need further investigation. AIM: To investigate the presence of clinical and serological markers for celiac disease in a group of Chilean psoriatic patients. MATERIAL AND METHODS: We included 80 psoriatic patients (42 males) aged 16 to 79 years, whose serum was tested for antitransglutaminase antibodies (ATGA) and antiendomysial antibodies (AEMA). Patients with weakly positive AEMA tests were also tested for antigliadin antibodies (AGA). RESULTS: In six patients (7.5%), AEMA and AGA were positive and one patient was positive for ATGA. An upper gastrointestinal endoscopy and duodenal biopsy was offered to these six patients and five accepted the procedure. Only one had a pathological diagnosis of celiac disease. CONCLUSIONS: Only one of 80 patients with psoriasis had celiac disease (1.2%). Other four patients with positive serologic markers had a normal duodenal biopsy. This group of patients may have latent celiac disease and they should be followed up.

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BACKGROUND: Non alcoholic fatty liver disease (NAFLD) is associated to diabetes mellitus, obesity, disturbances in serum lipid levels, insulin resistance and metabolic syndrome. AIM: To assess glucose tolerance and the presence of metabolic syndrome among patients with biopsy proven NAFLD. PATIENTS AND METHODS: Serum lipid levels, hepatic function tests were measured and an oral glucose tolerance test was performed in 46 patients (mean age 45+/-12 years, 36 females) without history of diabetes mellitus and with steatosis in a liver biopsy. RESULTS: Mean body mass index of the sample was 37+/-12 kg/m2. Seventeen percent had pure steatosis, 78% had steatohepatitis with or without fibrosis and 50% had fibrosis in the liver biopsy. Glucose intolerance and diabetes was found in 57% and 15% of cases, respectively. The presence of steatohepatitis was higher in diabetics, compared with subjects with glucose intolerance or a normal glucose response (43, 38 and 8%, respectively, p < 0.0001). Ninety three percent had a metabolic syndrome and the proportion of biopsies with fibrosis was higher among subjects with more than three diagnostic criteria for metabolic syndrome compared with those with three or less criteria (59 and 46%, respectively, p < 0.05). CONCLUSIONS: Glucose intolerance, diabetes and metabolic syndrome are common among patients with NAFLD, even when they are not obese.

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Background: Non alcoholic fatty liver disease (NAFLD) is associated to diabetes mellitus, obesity, disturbances in serum lipid levels, insulin resistance and metabolic syndrome. Aim: To assess glucose tolerance and the presence of metabolic syndrome among patients with biopsy proven NAFLD. Patients and methods: Serum lipid levels, hepatic function tests were measured and an oral glucose tolerance test was performed in 46 patients (mean age 45±12 years, 36 females) without history of diabetes mellitus and with steatosis in a liver biopsy. Results: Mean body mass index of the sample was 37±12 kg/m². Seventeen percent had pure steatosis, 78 percent had steatohepatitis with or without fibrosis and 50 percent had fibrosis in the liver biopsy. Glucose intolerance and diabetes was found in 57 percent and 15 percent of cases, respectively. The presence of steatohepatitis was higher in diabetics, compared with subjects with glucose intolerance or a normal glucose response (43, 38 and 8 percent, respectively, p <0.0001). Ninety three percent had a metabolic syndrome and the proportion of biopsies with fibrosis was higher among subjects with more than three diagnostic criteria for metabolic syndrome compared with those with three or less criteria (59 and 46 percent, respectively, p <0.05). Conclusions: Glucose intolerance, diabetes and metabolic syndrome are common among patients with NAFLD, even when they are not obese.

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BACKGROUND: Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure. AIM: To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC. MATERIAL AND METHODS: Retrospective review of medical records of 115 patients (110 females, age range 30-76 years) with PBC. Clinical presentation, pathological stage, treatment, outcome and eventual use of liver transplantation, were recorded. RESULT: Seventy eight percent of patients were symptomatic at presentation (itching in 69% and malaise in 62%). Antimitochondrial antibodies were positive in 56%. No clinical or laboratory differences were observed between symptomatic patients or those with positive antimitochondrial antibodies and the rest of the study group. Sjögren syndrome was present in 38%, hypothyroidism in 13%, scleroderma in 7% and rheumatoid arthritis in 5%. Initially, 61% had fibrosis and/or cirrhosis, and ursodeoxycholic acid was indicated in 94% of the patients. Fifteen patients underwent liver transplantation due to upper digestive bleeding or itching. Survival has been 67% at 36 months after transplantation. In one transplanted liver, PBC recurred. CONCLUSIONS: An early diagnosis and treatment of a progressive disease such as PBC will reduce the incidence of complications and the use of costly treatments.

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Background: Primary biliary cirrhosis (PBC) is a chronic cholestatic disease, which can progress to hepatic failure. Aim: To study the clinical presentation, pathological features, treatment and outcome of a group of patients with PBC. Material and methods: Retrospective review of medical records of 115 patients (110 females, age range 30-76 years) with PBC. Clinical presentation, pathological stage, treatment, outcome and eventual use of liver transplantation, were recorded. Result: Seventy eight percent of patients were symptomatic at presentation (itching in 69% and malaise in 62%). Antimitochondrial antibodies were positive in 56%. No clinical or laboratory differences were observed between symptomatic patients or those with positive antimitochondrial antibodies and the rest of the study group. Sjögren syndrome was present in 38%, hypothyroidism in 13%, scleroderma in 7% and rheumatoid arthritis in 5%. Initially, 61% had fibrosis and/or cirrhosis, and ursodeoxycholic acid was indicated in 94% of the patients. Fifteen patients underwent liver transplantation due to upper digestive bleeding or itching. Survival has been 67% at 36 months after transplantation. In one transplanted liver, PBC recurred. Conclusions: An early diagnosis and treatment of a progressive disease such as PBC will reduce the incidence of complications and the use of costly treatments.

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Background: Twenty percent of patients with chronic hepatitis C evolve to cirrhosis in 10 to 20 years. The degree of steatosis and hepatic iron stores in liver biopsy increase the risk. Age, high body mass index, diabetes mellitus and alcohol consumption are factors associated to the severity of liver damage. Aim: To study the association of steatosis and increased iron stores in the liver biopsy and age, overweight, alcohol consumption and diabetes with the severity of liver damage in patients with hepatitis C virus infection. Patients and methods: Retrospective study of 84 liver biopsies of patients with chronic infection with hepatitis C virus were studied. The pathological appearance was classified as stage I when chronic hepatitis with mild activity without fibrosis was observed; as stage II when moderate chronic hepatitis with mild fibrosis was observed and as stage III when there was a moderate chronic hepatitis with fibrosis or cirrhosis. The amount of steatosis and iron deposition in the biopsy were also assessed. Results: Forty one percent of patients were in stage I, 32% in stage II and 27% in stage III. Patients in stage I were younger than those in stages II and III (40.7 and 52.2 years respectively, p <0,001). No association between the severity of liver damage and the degree of steatosis, hemosiderosis, body mass index or alcohol intake, was observed. The frecuency of diabetes mellitus increased along with pathological staging (3, 15 and 30% in stages I, II and III, respectively, p <0,05). Conclusions: This study confirms that severity of chronic hepatitis C is associated with age and the presence of diabetes mellitus.

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Background:Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterized by progressive inflammation and fibrosis of the biliary tract, evolving to cirrhosis. It is commonly associated with inflammatory bowel disease (IBD). Aim: To communicate the clinical characteristics of patient with PSC seen in two reference centers. Patients and methods: Review of medical records of patients with PSC confirmed by liver biopsies. The clinical picture, laboratory abnormalities, imaging studies and treatment were recorded. Results: Thirty three patients (aged 16 to 73 years, 64% female) were identified. They corresponded to 1.7% of liver biopsies done between 1991-2003. Clinical presentation was a cholestatic picture in 40%, right upper abdominal pain in 36%, a dysenteric syndrome in 9% and varied symptoms in 15%. Laboratory tests showed cholestasis in 94% and positive anti ANCA, SMA, ANA and AMA antibodies in 28, 18, 15 and 9% of cases, respectively. Endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiography were diagnostic in 43 and 58% of patients, respectively. There was an association with ulcerative colitis in 12% of cases. Liver biopsies showed grade I PSC in 76% and grade II-III in 6% of patients. It also showed a concomitant chronic hepatitis and primary biliary cirrhosis in 12 and 6% of cases, respectively. Treatment consisted on ursodeoxycholic acid (UDCA) in 45%, UDCA plus 5-aminosalicylic acid derivatives in 12% and UDCA plus immunosuppresors in 12% of patients. Two patients had to be transplanted. Conclusions: PSC is an uncommon cause of chronic liver disease. It is suspected in cholestatic patients and confirmed with a liver biopsy. It can be associated with other autoimmune hepatic and extrahepatic diseases.

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