Asociación entre polimorfismos en los genes PNPLA3 y TM6SF2 y presencia de fibrosis en pacientes con infección crónica por virus hepatitis C / The association between polymorphisms in the genes PNPLA3 and TM6SF2 and the presence of fibrosis in patients with chronic hepatitis C virus infection

Hepatitis C virus (HCV) is a globally prevalent pathogen and a leading cause of death and morbidity. The most recent estimates of disease burden show an increase in seroprevalence over the last 15 years to 2.8 percent, equating to >185 million infections worldwide. Persistent hepatitis C infection is associated with the development of liver cirrhosis, hepatocellular cancer, liver failure and death. The magnitude of disease progression in chronic infection varies significantly among individuals. Several factors have been recognized as being associated with the progression of HCV-related liver fibrosis and with clinical outcomes. As liver fibrosis progression remains variable between individuals with similar environmental or virological risks, host genetic predispositions have been suggested as another critical determinant. The single nucleotide polymorphisms in Patatin-like phospholipase domain-containing 3 (PNPLA3) and Transmembrane 6 Superfamily Member 2 (TM6SF2) genes are genetic determinants of nonalcoholic fatty liver disease, in terms of inflammation and fibrosis. The possible action of the PNPLA3 and TM6SF2 polymorphisms on fibrosis development in chronic hepatis C is being studied, with controversial results.

Hiperplasia nodular regenerativa del hígado secundaria a uso de azatioprina por trasplante renal previo, tratado con trasplante combinado hepático y renal / Combined hepatic and renal transplant after nodular regenerative hyperplasia of the liver secondary to azathioprine in renal transplant patient

Nodular regenerative hyperplasia is an uncommon condition, characterized by the presence of regenerative nodules with minimal or absence of fibrosis, which can lead to non-cirrhotic portal hypertension. There are numerous diseases, conditions and drugs that can cause it. Thiopurines, a group of immunosuppressors used in transplanted patients, has been linked to this entity. We report a case of a renal transplant woman, who has been on chronic therapy with azathioprine and that develops portal hypertension and end-stage renal disease, undergoing combined hepatic and renal transplant. Histological examination of the explanted liver was compatible with nodular regenerative hyperplasia. How azathioprine causes this entity is unknown, but endothelial vascular damage in a dose-dependent manner is postulated as the main mechanism. To our knowledge, this is the first case report of a renal transplant patient who develops nodular regenerative hyperplasia of the liver in association with azathioprine, and undergoes combined hepatic and renal transplant, with a favorable outcome 5 years post procedure.

La hiperplasia nodular regenerativa es una entidad infrecuente, que se caracteriza por la presencia de nódulos hepáticos con ausencia o mínima fibrosis y que puede llevar a hipertensión portal de origen no cirrótico. Existen diversas enfermedades, condiciones y medicamentos que la causan, destacando entre estos últimos las tiopurinas, inmunosupresores utilizados habitualmente en trasplantados. Se presenta el caso de una paciente trasplantada renal usuaria crónica de azatioprina, que desarrolla hipertensión portal además de deterioro de la función renal, requiriendo de un doble trasplante hepático y renal, destacando en la biopsia del explante hallazgos histológicos compatibles con hiperplasia nodular regenerativa. Los mecanismos de daño por azatioprina en esta entidad son desconocidos, pero se postula al daño endotelial dosis-dependiente como principal causa. La revisión de la literatura demuestra que este caso corresponde al primero de hiperplasia nodular regenerativa secundaria a azatioprina en trasplantado renal, que requiere de doble trasplante hepático y renal con evolución favorable hasta 5 años post trasplante.

Espectro clínico de la hepatotoxicidad por nitrofurantoína en serie clínica de 12 pacientes / Clinical spectrum of nitrofurantoin hepatotoxicity in a series of 12 patients

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.

Hígado graso no alcohólico: principal causa de enfermedad hepática ambulatoria en un centro privado en Chile: estudio prospectivo marzo-julio 2010 / Non alcoholic fatty liver disease: main cause of outpatient liver disease in a private center in Chile: prospective study March-July 2010

The epidemiology of liver diseases has been changing over the time, with increasing importance of non-alcoholic fatty liver disease (NAFLD). Objective: To investigate the principal diagnosis of liver diseases in a reference center. Material and Methods: Prospective study of 405 consecutive patients attending theClinica Las Condes Gastroenterology Department, between March and July 2010. Results: 207 (51 percent) were men and 198 (49 percent) women, with a mean age of 54 years old (Range 14-89). From these, 134 (33 percent) had NAFLD, 40 (10 percent) autoimmune hepatitis, 36 (9 percent) primary biliary cirrhosis, 25 (9 percent) chronic hepatitis C, 26 (6,5 percent) Gilbert syndrome, 23 (5,5 percent) acute or chronic hepatitis B, 22 (5 percent) liver transplantation and 87 (65 percent) others diagnosis. From 134 NAFLD cases, 88 (66 percent) were men and 46 (34 percent) women, 72 (54 percent) had overweight, 39 (29 percent) obesity, 100 (75 percent) dyslipidemia and 87 (65 percent) glucose intolerance, insulin resistance and/or diabetes mellitus. Conclusion: NAFLD is currently the main cause of liver disease in a reference center, associated to well known risk factors, increasingly presented in Chile.

La epidemiología de las enfermedades hepáticas ha cambiado a través del tiempo, adquiriendo crecienteimportancia el hígado graso no alcohólico (HGNA). Objetivo: Investigar los principales diagnósticoshepatológicos en un centro de referencia. Material y Método: Estudio prospectivo de 405 pacientes consecutivos ambulatorios, consultantes entre marzo y julio de 2010, al Departamento de Gastroenterología de Clínica Las Condes. Resultados: 207 (51 por ciento) eran hombres y 198 (49 por ciento) mujeres, con edad promedio de 54 años (rango 14-89). De éstos, 134 (33 por ciento) tenían HGNA, 40 (10 por ciento) hepatitis autoinmune, 36 (9 por ciento) cirrosis biliar primaria, 25 (9 por ciento) hepatitis crónica C, 26 (6,5 por ciento) Síndrome de Gilbert, 23 (5,5 por ciento) hepatitis aguda o crónica B, 22 (5 por ciento) trasplante hepático y 89 (22 por ciento) otros diagnósticos. De los 134 casos de HGNA, 88 (66 por ciento) eran hombres y 46 (34 por ciento) mujeres, 72 (54 por ciento) tenían sobrepeso, 39 (29 por ciento) obesidad, 100 (75 por ciento) dislipidemia y 87 (65 por ciento) intolerancia a hidratos de carbono, resistencia a insulina y/o diabetes mellitus. Conclusiones: El HGNA constituye en la actualidad la principal enfermedad hepática en un centro privado en Chile, asociado a factores de riesgo conocidos, cada vez más presentes en nuestro medio.

Interferones lambdas (IFNs-lambda) en infección con virus hepatitis C / Lamba interferons in infection with hepatitis C virus

The current standard therapy for patients chronically infected with hepatitis C virus (HCV) is the administration of pegylated interferon-alpha (PEG-IFN) plus ribavirin (RBV), eliminating the virus in only about half of patients infected with the genotype most common in Chile and the world (genotype 1), being higher for genotypes 2 and 3. Genotyping of the HCV is a strong predictor of treatment response, and it defines the treatment duration (48 weeks for genotype 1 and 24weeks for genotypes 2 and 3). Genome studies revealed the association of polymorphisms (SNPs) close to IL28B gene with increased spontaneous and treatment-inducing clearance for HCV, which are now evaluated as a strong predictor of treatment response. These SNPs are close to genes coding for type III IFNs family, known as IFNs lambda (IFNs lambda), composed by IFN lambda1 (IL29), IFN lambda2 (IL28A) and IFN lambda3 (IL28B). It has been shown that these cytokines are highly involved in antiviral immune responses, including HCV, playing IFN lambda1 a central role. Today, there is an ongoing study where pegylated IFN alpha1 was administrated in chronic HCV patients as alternative to IFN lambda therapy, seeking for a more specific response to infected hepatocytes and with fewer adverse effects.

Enfermedad linfoproliferativa difusa postrasplante en adultos: comunicación de un caso y revisión de la literatura / Post-transplant lymphoproliferative disorder in adults: case report and review of the literature

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.

Síndrome hepatorenal: patogénesis y tratamiento / Hepatorenal syndrome: pathogenesis and treatment

El Síndrome Hepatorenal (SHR) es una insuficiencia renal aguda funcional y reversible, que se desarrolla en pacientes con cirrosis hepática descompensada o en insuficiencia hepática aguda severa. La característica principal del SHR es la intensa vasoconstricción renal causada por la interacción entre alteraciones hemodinámicas a nivel sistémico y portal. El aumento del volumen intravascular y un prolongado tratamiento con fármacos vasoconstrictores pueden revertir la falla renal en un porcentaje significativo de pacientes. El SHR Tipo 2, el más frecuente, generalmente presenta una evolución lenta y un mejor pronóstico que el SHR Tipo 1. La terapia vasoconstrictora con terlipresina asociado a albúmina es el tratamiento de elección en pacientes con SHR. Apesar del avance en las diferentes estrategias terapéuticas, el pronóstico a largo plazo es aún pobre y depende generalmente del grado de reversibilidad de la enfermedad hepática asociada o del acceso al trasplante hepático. En la presente revisión se discutirán los avances más recientes en el diagnóstico, patogénesis y tratamiento del SHR.

The Hepatorenal Syndrome (HRS) is a functional and reversible form of acute renal failure, which develops in decompensated cirrhosis or acute liver failure. The distinctive hallmark feature of HRS is the intense renal vasoconstriction caused by interactions between systemic and portal hemodynamics. Increasing intravascular volume and prolonged treatment with vasoconstrictors drugs reverses renal failure in a significant proportion of patients. Type 2 HRS, the most frequent, generally follows a slower course and has a better prognosis than Type 1 HRS. Vasopressor therapy with terlipressin plus intravenous albumin is the medical treatment of choice for patients with HRS. Despite improvements in the therapeutic strategies, long term prognosis is still poor and generally depends on the degree of reversibility of the underlying liver disease or access to liver transplantation. In the present review, the most recent advances in diagnosis, pathophysiology, and treatment of HRS are discussed.

Pancreatitis autoinmune: experiencia clínica y revisión de la literatura / Autoimmune pancreatitis: report of 10 cases

Background: Autoimmune pancreatitis is a special form of chronic pancreatitis, more common in men and usually presenting as obstructive jaundice or abdominal pain. It may be associated with other immunological disorders and sometimes it is possible to find positive serological markers. Typical images show pancreatic enlargement with focal or diffuse stenosis of the pancreatic duct but sometimes it presents as a focal pancreatic mass that is difficult to differentiate from pancreatic carcinoma. Aim: To report ten cases of autoimmune pancreatitis. Material and Methods: Retrospective review of clinical records of 10 patients aged 26 to 56 years (six males) with autoimmune pancreatitis. Results: The clinical presentation was obstructive jaundice in six cases, acute pancreatitis in two, persistent increase in serum amylase and Upase in one, and permanent abdominal pain and weight loss in one. On imaging studies, a circumscribed mass was founded in six patients. An endoscopic retrograde colangiopancreatography was performed in four patients showing an abnormal pancreatic duct in all. Six patients were operated and tissue for pathological study was obtained in five, showing inflammatory infiltration. Vive patients were treated with steroids with a good clinical response. Conclusions: Autoimmune pancreatitis must be borne in mind in the differential diagnosis of pancreatic lesions.

Variabilidad genética del virus hepatitis B y su significado clínico / Genetic variability hepatitis b virus, the clinical meaning

The Hepatitis B virus (HBV) is the prototype member of the Hepadnaviridae family, which can cause acute or chronic hepatic illness. The virus has a partially double-stranded DNA genome of3.2 kb. Molecular variations and change in the genome over time have resulted in the emergence of at least eight genotypes and multiple subgenotypes. The distribution of HBV genotypes varies widely across geographic regions, been the genotype F the most prevalent in Chile. In recent years, substantial progress has been made toward understanding the epidemiology and virologic significance of HBV variants. Actually, accumulating evidence suggests that hepatitis B genotypes and subgenotypes can influence the severity, course and likelihood of complications, and response to treatment of HBV infection and possibly vaccination against the virus.

Hepatitis crónica viral / Chronic viral hepatitis

Los Virus Hepatitis B (VHB) y Hepatitis C (VHC) en la actualidad son infecciones frecuentes en el hombre, afectando a una proporción significativa de la población mundial. Estos son causa frecuente de hepatitis aguda y crónica, cirrosis hepática, hepatocarcinoma y trasplante hepático. En 105 últimos años ha habido un gran progreso en el conocimiento de la epidemiología e historia natural de la infección con el VHB y VHC. Además, se han logrado grandes avances en su tratamiento y con numerosas drogas actualmente en evaluación.

Hepatitis B (HBV) and Hepatitis C (HCV) virus are common viral infections, affecting at a large worldwide population. These viruses are frequent cause of acute and chronic hepatitis, cirrhosis, hepatocellular carcinoma and liver transplantation. In the last few years, the knowledge of the epidemiology and natural history of HBV and HCV infections have markedly improved. Furthermore, considerable progress have been achieved in the efficacy of treatment and several new drugs are currently under evaluation.

Timoma, síndrome de Good y carcinoide intestinal: caso clínico y revision de la literatura / Case report: thymoma Good's syndrome and intestinal carcinoid

The association of thymoma, with myasthenia gravis, pure red cell aplasia and hypogammaglobulinemia (Good's syndrome), has been described. We report a case of a 51 years old woman who after a surgieal resection of a thymoma had several Symptoms and study showed hypogammaglobulinemia. During the study of a persitent diarrhea distal ileum carcinoid tumor was diagnosed. The forgotten association of thymoma and malignancies is of a great clinical significance. Evidence linking thymoma with other neoplasms is provided by the clinical literature.

La asociación de timoma con miastenia gravis, aplasia eritrocítica pura e hipogamaglobulinemia (Síndrome de Good) ha sido descrita. Presentamos el caso de una mujer de 51 años que después de la operación de un timoma presentó varios síntomas confirmándose una hipogammaglobulinemia. En el estudio de una diarrea persistente se encontró un tumor carcinoide del íleon distal. La asociación olvidada entre timoma y cáncer es de gran importancia clínica.

Infección por virus de la hepatitis B: características epidemiológicas / Epidemiology of hepatitis B virus infection

The Hepatitis B (HBV) has a low frequency in Chile, there are only few studies on the epidemiological characteristics, for either the acute or chronic infection. Aim: to investigate the epidemiology of HBV infection in our country. Patients and methods: a retrospective analysis of cases of HBV infection between 2001 an 2007 from a private medical centre of east area in Santiago was informed. Results: 81 cases, range 15-61 years were studied: 67 patients (83 percent) were male, mainly between 35 - 44 years. Sexual route was the main mechanism of transmission (62 cases, 77 percent). In 8 cases (10 percent) co infection with HIV was diagnosed. Fifty one patients (63 percent of patients who completed follow-up developed immunity against HBV, and 2 cases evolved to chronic infection (5 percent). Thirty patients (27 percent) had chronic HBV infection. The predominant characteristics of this group were: male sex 86 per cent , ages range 45-54 years (30 percent), being the main mechanism of infection homosexual contact (33 percent). Conclusions: the infection by HBV in Chile seems to be more frequent, that previously reported, associated to sexual behaviour and with a significant level of co infection with HIV.

La infección por el Virus de la Hepatitis B (VHB) es de baja frecuencia en Chile, existiendo pocos estudios sobre las características epidemiológicas, tanto de su infección aguda como crónica. Objetivo: investigar las características epidemiológicas de infección por VHB en nuestro país. Pacientes y método: revisión retrospectiva y prospectiva de casos de infección por VHB entre los años 2001-2007 en un centro médico privado de área oriente de Santiago. Resultados: 81 casos entre los 15 y 61 años de edad: 67 pacientes (83 por ciento) eran de sexo masculino, siendo el grupo etario con mayor frecuencia (40 por ciento) el comprendido entre los 35-44 años. El principal mecanismo de contagio fue el contacto sexual en 62 casos (77 por ciento) y en 8 casos (10 por ciento)se diagnosticó coinfección con Virus de Inmunodeficiencia Humana (VIH). La infección aguda por VHB correspondió a 51 casos (63 por ciento), con una evolución a la inmunidad en el 92 por ciento de los pacientes que completaron el seguimiento y 2 casos evolucionaron a la cronicidad (5 por ciento). La infección crónica correspondió a 30 pacientes (27 por ciento), predominantemente hombres (86 por ciento), entre 45 - 54 años (30 por ciento), siendo el principal mecanismo de contagio el contacto homosexual (33 por ciento). Conclusiones: La infección por VHB en Chile pareciera ser más frecuente que lo comunicado, con un patrón epidemiológico asociado a conductas sexuales y con un nivel de coinfección con VIH relativamente importante.

Hepatitis aguda alcohólica asociada a pancreatitis aguda grave / Acute alcoholic hepatitis and severe acute pancreatitis

Alcohol is a common cause of hepatic and pancreatic damage. Despite the widespread alcohol consumption simultaneous presentation of acute alcoholic hepatitis and pancreatitis is uncomnon. We describe a case of a patient with alcoholic hepatitis in association acute pancreatitis, who developed multiorgan failure and died. The association of liver and pancreatic disease in clinical practice is discussed.

El alcohol es un factor etiológico común de daño hepático y pancreático. A pesar de su extenso consumo la presentación simultánea de hepatitis y pancreatitis aguda alcohólica es excepcional. Se presenta un caso de esta situación, que evolucionó hacia la falla orgánica múltiple con desenlace fatal. Se discute la asociación de daño hepático y pancreático agudo en la práctica Clínica.

Enfermedad inflamatoria intestinal y enfermedad celíaca: una asociación infrecuente / Inflammatory bowel disease and celiac disease: an uncommon association

The prevalence of inflammatory bowel disease (IBD) and celiac disease (CD) have increased during the last decades; however the occurrence of both conditions together is uncommon. In spite of this fact, the literature has suggested an association between IBD and CD because an aberrant innate immune reaction is a contributory risk factor in both diseases. The combined presentation of IBD and CD in five patients is presented and we analyzed the clinical and laboratory characteristics of this condition. The occurrence of both diseases should be kept in mind at the time of diagnosis and at relapse, or when the remission is difficult to maintain.

La prevalencia de las enfermedades inflamatorias intestinales (EII) y de la enfermedad celiaca (EC) ha aumentado durante las ultimas décadas; sin embargo la asociación de ambas condiciones es infrecuente. A pesar de ello, la literatura ha sugerido una asociación entre ambas entidades, ya que una respuesta inmune innata aberrante es un factor de riesgo de ambas enfermedades. Presentamos cinco pacientes con la presentación combinada de EII y EC y se analizan las características clínicas y de laboratorio de esta condición. La presencia de ambos cuadros debe ser tenida en mente al momento del diagnostico, recaída de la enfermedad sin causa clara o si existe alguna dificultad en mantener la etapa de remisión de la enfermedad.

Cirrosis biliar primaria: experiencia de trece años en dos centros de referencia / Primary biliary cirrhosis: a thirteen years experience

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.

Tromboembolismo, otra manifestación extraintestinal de las enfermedades inflamatorias intestinales / Thromboembolism, another extraintestinal manifestation of inflammatory bowel disease

El tromboembolismo (TE) arterial y venoso, posee una elevada morbimortalidad, siendo en pacientes con enfermedades inflamatorias intestinales (EII) 3-4 veces mayor que la población general, con una prevalencia de un 2-10 por ciento. El mecanismo por el cual la actividad procoagulante está aumentada en las EII es aún desconocido, sin embargo, factores de riesgo como el grado de inflamación de la mucosa han sido descritos. Objetivos: Determinar las características clínicas de TE en un grupo de pacientes con EII. Pacientes y Métodos: Se analizaron de manera retrospectiva los antecedentes clínicos de 16 pacientes con EII que presentaron TE durante su evolución. Se evaluó edad, extensión y grado de actividad de la EII al momento del TE. Resultados: Los pacientes estudiados fueron 13 con colitis ulcerosa (CU) y tres con enfermedad de Crohn (EC), edad promedio 43 años (rango 21-72), ocho mujeres. Once pacientes tenían < 50 años al momento del TE. En CU, nueve pacientes tenían pancolitis, todos con enfermedad activa moderada-severa. En EC, dos pacientes presentaban actividad de la enfermedad al momento del TE. Tres pacientes tenían otra manifestación extraintestinal (MEI), dos de ellos artralgias y uno colangitis esclerosante primaria. Los eventos de TE son: trombosis venosa profunda de extremidades inferiores en 9 casos; TE pulmonar en 3 pacientes, trombosis mesentérica/portal (dos casos), trombosis del seno venoso longitudinal (un paciente), trombosis arteria cerebral (uno) y trombosis de arteria braquial un caso). Quince casos de TE ocurrieron entre una semana y 6 años luego del diagnóstico de EII. Cinco pacientes estaban hospitalizados al momento del TE, dos de ellos después de cirugía por su EII. Ningún paciente estaba recibiendo nutrición parenteral total o tenía catéter venoso central al momento del TE. El estudio de trombofilia realizado en cinco pacientes, no demostró alteraciones características. Todos los pacientes fueron tratados con heparina sin complicaciones, y un paciente falleció por embolia pulmonar. Ningún paciente tenía antecedentes personales o familiares de TE. Conclusiones: El tromboembolismo es otra MEI de las EII que debe ser sospechado. La remisión de la EII pudiera ser el factor más importante en la prevención de estos episodios. El uso de heparina como tratamiento del TE no está contraindicado en estos pacientes, incluso en aquellos con crisis severa con sangramiento intestinal.

Hepatitis crónica por virus C: factores asociados a la severidad del daño histológico / Factors associated with the severity of liver damage in chronic hepatitis C

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.

Enfermedad Celíaca del adulto: experiencia clínica / Adult celiac disease: clinical experience

Background: The prevalence of celiac disease (CD) is unknown in Chile. We have recently noted a rise in the number of cases diagnosed among adults. Aim: To describe the clinical characteristics of a group of adult celiac patients. Patients and methods: Clinical data of patients older than 15 years with positive antitransglutaminase or antiendomysial autoantibodies and a duodenal biopsy characteristic of CD were retrospectively reviewed. Age at diagnosis, symptoms and signs and laboratory, endoscopic and histological findings, were analyzed. Results: Thirty seven patients (28 women), were studied. Median age at diagnosis was 41 years (range 15-69). Main symptoms and signs were diarrhea (78%), weight loss (38%) and abdominal pain (38%). Anemia was found in 49%, elevation of ESR in 57%, elevation of alkaline phosphatases in 54%, elevation of aspartate aminotransferase in 38% and a rise in alanine aminotransferase in 27%. Antiendomysial antibodies were positive in 17/22 (77%) and antitransglutaminase in 19/22 (86%) patients. Endoscopic findings were suggestive of CD in 47% of cases and duodenal biopsy showed intestinal villi atrophy in 34 (92%) patients. The three patients with normal histology had positive serology and a good response to gluten free diet. Conclusions: CD should be considered in the differential diagnosis of patients with unespecific digestive symptons, even when they present late in adult life. Serologic markers are a good diagnostic tool. A normal duodenal pathology does not exclude the diagnosis, if other diagnostic features are present.