Utilidad del sistema de recirculación de adsorbentes moleculares en la falla hepática: "diálisis hepática" / Usefulness of the molecular adsorbent recirculating system in liver failure: "liver dialysis"

INTRODUCCIÓN. La falla hepática ya sea aguda o crónica reagudizada representa un reto para el clínico ya que sus complicaciones conllevan una gran mortalidad, esto se ve aún más complicado ya que las opciones terapéuticas son limitadas, incluso muchas veces no se puede acceder a un programa de trasplante hepático oportuno que mejore la sobrevida de estos pacientes, es así que se ha desarrollado un sistema de "diálisis" hepática conocido como sistema de recirculación de adsorbentes moleculares el cual hace un efecto de detoxificación para eliminar sustancias que generan una noxa en el cuerpo humano. OBJETIVO. Entender la utilidad del sistema recirculante molecular adsorbente en la falla hepática, conocer sus indicaciones y complicaciones. METODOLOGÍA. Se realizó una revisión de la literatura con un enfoque descriptivo, retrospectivo cualitativo no experimental, de documentos que tratan sobre la utilización del sistema MARS para tratar la falla hepática, con evidencia desde el año 2004 hasta el 2021. La revisión bibliográfica se llevó a cabo en bases de datos como Pubmed, Embase, BVS, Google Scholar y Elsevier. RESULTADOS. Se identificaron 30 artículos que cumplieron criterios de inclusión de un grupo original de 343 artículos revisados. Se ha determinado que la evidencia sobre este sistema está compuesta sobre todo por reportes de caso y son pocos los ensayos controlados aleatorizados que empleen su uso, sin embargo, se ha podido determinar que este sistema es un puente al trasplante renal mientras se estabiliza al paciente en la Unidad de Cuidados Intensivos, disminuye los marcadores de falla hepática. CONCLUSIÓN. En Latinoamérica su uso es casi nulo de ahí la necesidad de entender el mecanismo de este novedoso sistema.

INTRODUCTION. Hepatic failure, whether acute or chronic, represents a challenge for the clinician since its complications entail a great mortality, this is even more complicated since the therapeutic options are limited, even many times it is not possible to access a timely liver transplant program to improve the survival of these patients, Thus, a hepatic "dialysis" system known as molecular adsorbent recirculation system has been developed, which has a detoxification effect to eliminate substances that generate a noxa in the human body. OBJECTIVE. To understand the usefulness of the molecular adsorbent recirculating system in liver failure, to know its indications and complications. METHODOLOGY. A literature review was performed with a descriptive, retrospective qualitative non-experimental qualitative approach, of papers dealing with the use of the MARS system to treat liver failure, with evidence from 2004 to 2021. The literature review was conducted in databases such as Pubmed, Embase, BVS, Google Scholar and Elsevier. RESULTS. Thirty articles were identified that met inclusion criteria from an original group of 343 articles reviewed. It has been determined that the evidence on this system is mainly composed of case reports and there are few randomized controlled trials that employ its use, however, it has been determined that this system is a bridge to renal transplantation while the patient is stabilized in the Intensive Care Unit, decreasing the markers of liver failure. CONCLUSIONS. In Latin America its use is almost null, hence the need to understand the mechanism of this novel system.

Síndrome DRESS secundária ao uso de alopurinol: um relato de caso / DRESS syndrome secondary to the use of allopurinol: a case report

A Síndrome de DRESS (do inglês, Drug Rash with Eosinophilia and Systemic Symptoms) é uma patologia rara que consiste em uma severa reação medicamentosa mediada por células T. O presente relato de caso retrata uma paciente do sexo feminino, 59 anos, que apresentou icterícia, febre não termometrada, acolia, colúria, mialgia, placas hipercrômicas e lesões pruriginosas. Referiu uso recente de alopurinol, paracetamol e nimesulida, apresentando melhora importante e espontânea após a suspensão das medicações. A extensão do tempo de exposição ao medicamento agressor ocasiona um maior período de internação e risco de mortalidade. Além disso, os dados restritos sobre a Síndrome de DRESS impõe desafios ao seu diagnóstico. Sendo assim, este estudo busca destacar a importância do diagnóstico clínico precoce, a suspensão do medicamento agressor e a instituição da terapêutica adequada para um prognóstico favorável

The Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a rare pathology that consists of a severe drug reaction mediated by T cells. The present case report depicts a female patient, 59 years old, who presented jaundice, non thermometered fever, acholia, choluria, myalgia, hyperchromic plaques and pruritic lesions. She mentioned recent use of allopurinol, paracetamol and nimesulide, showing significant and spontaneous improvement after discontinuation of medications. The extension of time of exposure to the offending drug causes a longer period of hospitalization and risk of mortality. In addition, the restricted data on DRESS Syndrome poses challenges to its diagnosis. Therefore, this study seeks to highlight the importance of early clinical diagnosis, suspension of the offending drug and the institution of appropriate therapy for a favorable prognosis

Acute liver failure due to radiographically occult infiltration of urothelial cancer

Introduction Acute liver failure (ALF) due to diffuse infiltrating solid malignancy without any focal lesions on radiographic imaging is rare. Case report A 70-year-old man was admitted due to mental confusion, abdominal pain, and ALF. Three years before, he had undergone a left nephrectomy for urothelial carcinoma followed by adjuvant chemotherapy. The abdominal computed tomography (CT) showed hepatomegaly and ascites. Ascitic fluid had transudate characteristics, with no malignant cells. Percutaneous liver biopsy (LB) showed diffuse liver infiltration of metastatic urothelial carcinoma. The patient rapidly deteriorated and died in a week due to ALF. Discussion History of solid cancer and hepatomegaly and/or liver failure without other obvious explanation should encourage to perform LB. Conclusion LB is warranted to avoid misdiagnosis, prolonged hospital stays, and delay in palliative care.

Falla hepática aguda sobre crónica (ACLF), síndrome subdiagnosticado con alta mortalidad / Acute-on-chronic liver failure (ACLF), an underdiagnosed syndrome with high mortality

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)

Hemophagocytic lymphohistiocytosis as a presentation of inflammatory bowel disease / Linfohistiocitosis hemofagocítica como presentación de la enfermedad inflamatoria intestinal

Abstract Background: Hemophagocytic lymphohistiocytosis (HLH) is considered a medical emergency that should be recognized in patients with fever, splenomegaly, and progressive deterioration of the general condition. Laboratory findings include cytopenia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. For HLH diagnosis, it is essential, although not mandatory, to perform a bone marrow biopsy. Given its nature, secondary causes of HLH should be sought, mainly infections, hemato-oncological disorders, autoimmune diseases, and auto-inflammatory conditions. Case report: We present the case of a female adolescent who presented with fever and lower gastrointestinal bleeding. Upon admission, acute liver failure and pancytopenia were documented. A bone marrow aspirate was performed, which revealed hemophagocytosis; other tests confirmed HLH diagnosis. During the diagnostic approach, inflammatory bowel disease was diagnosed. The patient received first-line treatment with an adequate response. Conclusions: Inflammatory bowel disease can be considered a cause of secondary HLH, particularly in patients with suggestive symptoms, such as digestive bleeding in the absence of other secondary causes of HLH.

Resumen Introducción: La linfohistiocitosis hemofagocítica (LHH) es considerada una urgencia médica que debe reconocerse en pacientes con deterioro progresivo del estado general, fiebre, pancitopenia y esplenomegalia. Los hallazgos de laboratorio incluyen citopenia, hipertrigliceridemia, hipofibrinogenemia e hiperferritinemia. Para su diagnóstico es importante, aunque no obligatoria, la realización de aspirado de médula ósea. Dada su naturaleza, se deben buscar causas secundarias de LHH, principalmente enfermedades infecciosas, hematooncológicas, autoinmunitarias y autoinflamatorias. Caso clínico: Se presenta el caso de una adolescente que inició con fiebre y sangrado digestivo bajo. A su ingreso, se documentó falla hepática aguda y pancitopenia. Se realizó aspirado de médula ósea y se encontró hemofagocitocis; el resto de los exámenes concluyeron LHH. Durante su abordaje se diagnosticó enfermedad inflamatoria intestinal. La paciente recibió tratamiento de primera línea con adecuada respuesta. Conclusiones: La enfermedad inflamatoria intestinal puede considerarse como una causa secundaria de LHH, en particular en pacientes con clínica sugestiva, como es el sangrado digestivo, en ausencia de otras causas secundarias de LHH.

Falla hepática aguda en pediatría / Pediatric acute liver failure

Resumen: La falla hepática aguda (FHA) es poco frecuente en pediatría, amenaza la vida y requiere aborda je multidisciplinario para su diagnóstico y tratamiento tempranos. El presente artículo tiene como objetivo realizar actualización de definiciones, enfoques y tratamientos disponibles. Los artículos se obtuvieron de la revisión de la literatura disponible entre 2000 y 2020 en varias bases de datos (Pub- med, LILACS, BIREME, Google Académico y UpToDate), empleando términos "acute liver failure" en Pubmed, "pediatric acute liver failure", "falla hepática aguda en pediatría" para otros buscadores y aplicando filtros según edad, fecha de publicación y tipo de estudio (ensayos clínicos, artículos de revisión, revisiones sistemáticas, estudios de casos y controles), se eligieron aquellos artículos con mayor número de citaciones y con datos recientes. La FHA requiere soporte en cuidado intensivo pe diátrico y su diagnóstico temprano favorece la instauración del tratamiento. Se recomienda el enfo que de pruebas diagnósticas específicas por edad en pacientes pediátricos con FHA. No hay consenso acerca de las indicaciones de trasplante hepático en FHA en pediatría.

Abstract: Acute liver failure (ALF) is a rare and life-threatening entity in pediatrics which requires a multidis ciplinary approach for early diagnosis and treatment. The objective of this article is to update defi nitions, management, and available treatments. We obtained the articles by reviewing the literature available between 2000 and 2020 in different databases (Pubmed, LILACS, BIREME, Google Scholar, and UpToDate), using terms such as "acute liver failure" in Pubmed, and in other databases "pedia tric acute liver failure" and "falla hepática aguda en pediatría" using filters such as age, publication date, and types of study (clinical trials, review articles, systematic reviews, and case-control studies). We chose those articles with the highest number of citations and with recent data. The ALF requires support in the pediatric intensive care unit and its early diagnosis favors the beginning of treatment. In pediatric patients with ALF, it is recommended to focus on age-specific diagnostic testing. There is no agreement regarding the liver transplantation in pediatric cases of ALF.

Effects of therapeutic plasma exchange on liver function test and coagulation parameters in acute liver failure patients

ABSTRACT Background: Currently the treatment of choice for critical liver failure is liver transplantation. Liver failure is treated conservatively until a matching liver donor becomes available. The therapeutic plasma exchange (TPE) plays an important role as a bridge to transplantation by removing accumulated toxins from patient plasma, as well as restoring the coagulation profile. Method: This was a retrospective study on critically ill liver disease patients who underwent TPE from January 2012 to September 2015. The data were collected for the analyses of coagulation parameters, liver function tests, renal function tests, model for end-stage liver disease (MELD) scores, mortality, and hospital stay. Results: In the study duration, a total of 45 patients with critical liver disease underwent therapeutic plasma exchange. The TPE resulted in a statistically significant reduction in the bilirubin level, aspartate aminotransferase (AST), alanine aminotransferase (ALT), prothrombin time (PT), international normalized ratio (INR), serum ferritin level and MELD scores. Higher MELD scores in both pre- and post-TPE were associated with higher mortality during the hospital stay. Conclusion: The TPE is safe and well-tolerated, and it improves coagulation profile and liver function tests in critically ill liver disease patients, but the overall survival remains low.

ACG Clinical Guideline: disorders of the hepatic and mesenteric circulation

Disorders of the mesenteric, portal, and hepatic veins and mesenteric and hepatic arteries have important clinical consequences and may lead to acute liver failure, chronic liver disease, noncirrhotic portal hypertension, cirrhosis, and hepatocellular carcinoma. Although literature in the field of vascular liver disorders is scant, these disorders are common in clinical practice, and general practitioners, gastroenterologists, and hepatologists may benefit from expert guidance and recommendations for management of these conditions. These guidelines represent the official practice recommendations of the American College of Gastroenterology. Key concept statements based on author expert opinion and review of literature and specific recommendations based on PICO/GRADE analysis have been developed to aid in the management of vascular liver disorders. These recommendations and guidelines should be tailored to individual patients and circumstances in routine clinical practice.

Falla hepática aguda / Acute liver failure

La falla hepática aguda es la pérdida súbita de la función hepática en un corto plazo en un paciente sin enfermedad hepática previa, que se acompaña de coagulopatía y encefalopatía. Es una entidad rara con una incidencia muy baja que afecta especialmente a personas jóvenes. La principal causa en países desarrollados es la toxicidad por acetaminofén, mientras que en los países subdesarrollados son las hepatitis virales. El curso natural de la enfermedad es la progresión rápida a muerte por falla orgánica multisistémica, sepsis o edema cerebral. Después del diagnóstico, los pacientes deben remitirse tempranamente a la unidad de cuidado intensivo y a centros que ofrezcan trasplante hepático. La supervivencia sin trasplante hepático hasta hace pocos años era menor al 15%; sin embargo, en la actualidad puede ser hasta del 50%, dependiendo de la causa, y está relacionada con tratamientos específicos, la disponibilidad de trasplante hepático y una atención óptima en las unidades de cuidados intensivos. El trasplante hepático se constituye en el tratamiento de elección para los pacientes con falla hepática aguda y criterios de mal pronóstico del King's College.

Acute liver failure is the severe short-term liver function impairment in a patient without previous liver disease, which is accompanied by coagulopathy and encephalopathy. It is a rare condition with a very low incidence that affects young people. The leading cause in developed countries is acetaminophen toxicity, while in developing countries is mainly caused by viral hepatitis. The natural course is characterized by a rapid progression to death due to multisystemic organ failure, sepsis, or cerebral edema. After diagnosis, patients must be transferred to the intensive care unit and liver transplantation centers. Survival without liver transplantation until a few years ago was less than 15%; however, currently it can be up to 50% depending on the cause, and it is related to specific treatments, availability of liver transplantation and optimal care in the intensive care units. Liver transplantation is the treatment of choice for patients with acute liver failure and King's College criteria for poor prognosis.

Fulminant acute hepatitis in pediatrics in Latin America and the Caribbean

SUMMARY OBJECTIVE: The objective of this review was to investigate the epidemiology of Fulminant Acute Hepatitis in Latin America and the Caribbean and identify possible measures aimed at a better understanding and improvement of patient support. METHODS: We used 3 different researchers to investigate the topic of Fulminant Acute Hepatitis in pediatrics in papers published by Latin American and Caribbean authors in the PubMed and SciELO databases from 2000 to 2016. RESULTS: We found 2,879 articles in the databases searched. After selecting and excluding articles according to the study protocol, 68 remaining studies were obtained for analysis. A total of 1,265 cases of acute fulminant hepatitis were detected, with a predominance of females (42.9%), followed by males (39.4%), with no description of sex in 17.7% of the cases. The main cause was viral hepatitis, representing 45.1% of the cases. The hepatitis A virus was responsible for 34.7% of the total cases and 76.9% of the infectious causes. Of the total number of patients, 26.9% were described as idiopathic, and 11.5% had no cause. CONCLUSION: The preventable causes of Fulminant Acute Hepatitis include hepatitis viruses - primarily the hepatitis A virus - and poisoning. Active vaccination, basic sanitation, and public awareness can reduce the number of patients and, consequently, the costs of liver transplantation due to these causes.

RESUMO OBJETIVO: O objetivo desta revisão foi investigar a epidemiologia da hepatite aguda fulminante na América Latina e Caribe e identificar possíveis ações objetivando melhor compreensão e melhora do suporte desses pacientes. MÉTODOS: Pesquisou-se o tema hepatite aguda fulminante em pediatria, publicado por autores da América Latina e Caribe nas bases de dados PubMed e SciELO por três diferentes investigadores no período de 2000 a 2016. RESULTADOS: Foram encontrados 2.879 artigos nos bancos de dados pesquisados. Após seleção e exclusão de artigos de acordo com o protocolo de pesquisa, resultaram 68 estudos remanescentes para análise. Foram avaliados 1.265 casos com hepatite aguda fulminante, havendo predominância no sexo feminino (42,9%), seguido do masculino (39,4%), sendo que não houve a descrição de sexo em 17,7% dos casos. A principal causa foram as hepatites virais (HV), representando 45,1% dos casos. O vírus da hepatite A foi responsável por 34,7% do total de casos e por 76,9% das causas infecciosas. Do total de pacientes, 26,9% foram descritos como idiopáticos e 11,5% não tiveram causa descrita. CONCLUSÃO: As causas evitáveis de hepatite aguda fulminante incluem os vírus da hepatite — principalmente o vírus da hepatite A — e intoxicações. A vacinação ativa, o saneamento básico e a conscientização pública podem reduzir o número de doentes e, consequentemente, os custos com transplante hepático por essas causas.

Sudden onset acute liver failure in a patient with clinically occult small cell lung carcinoma: autopsy report and review of the medical literature

Liver metastases are commonly found in advanced cancer patients; however, acute liver failure secondary to diffuse liver infiltration is rare. Small cell lung carcinoma accounts for 15% of lung carcinomas. We describe the ninth case of small cell lung carcinoma massively metastatic to the liver, reported in the scientific literature, with sudden clinical onset and death after a few days. An autopsy was performed to understand the cause of death.

Falla hepática aguda fulminante como manifestación de leucemia de células T del adulto en paciente con infección por HTLV-1 / Acute Liver Failure as Presentation of Adult T-cell Leukemia in an HTLV-1 Patient

La falla hepática aguda es el desarrollo de injuria hepática severa con deterioro de la función de síntesis y encefalopatía. Dentro de la variedad de causas, las infiltraciones neoplásicas representan menos del 0,5%. Presentamos el caso de un paciente varón HTLV1 positivo que debuta con una clínica de hepatitis aguda, siendo posteriormente diagnosticado con diseminación de linfoma/leucemia de células T del adulto. Desafortunadamente el paciente presentó deterioro rápido y progresivo de la función hepática, falleciendo a los pocos días de la hospitalización

Acute liver failure is the development of severe hepatic injury with deterioration of liver synthesis function and encephalopathy. Among all the variety of causes, neoplastic infiltration represents less than 0.5%. We present the case of a male patient with a past medical history of HTLV-1 infection, who reports symptoms of acute hepatitis, being diagnosed with Adult T-cell leukemia/ lymphoma. Unfortunately, the patient had a rapid deterioration and passed away a few days after admission

SOFA nas primeiras 24 horas como preditor de desfecho em insuficiência hepática aguda / SOFA in the first 24 hours as an outcome predictor of acute liver failure

RESUMO Objetivo: Descrever uma coorte de doentes com insuficiência hepática aguda, e analisar os fatores demográficos e clínicos associados à mortalidade. Métodos: Estudo de coorte retrospectivo em que todos os pacientes admitidos por insuficiência hepática aguda foram incluídos no período de 28 de julho de 2012 a 31 de agosto de 2017. Dados clínicos e demográficos foram coletados via Sistema Epimed. Foram mensurados SAPS 3, SOFA e MELD. Estimaram-se as OR e seus IC95%. Foram obtidas as curvas Características de Operação do Receptor para os escores de prognóstico, assim como a curva Kaplan-Meier de sobrevida para o escore com melhor predição de mortalidade. Resultados: A maioria dos 40 doentes era do sexo feminino (77,5%), e a etiologia mais frequente foi hepatite pelo vírus B (n = 13). Apenas 35% dos doentes foram submetidos ao transplante hepático. A mortalidade hospitalar foi de 57,5% (IC95%: 41,5 - 73,5). Dentre os escores investigados, apenas o SOFA se manteve associado ao risco de morte (OR = 1,37; IC95% 1,11 - 1,69; p < 0,001). Após a estratificação do SOFA em < 12 e ≥ 12 pontos, a sobrevida foi maior nos pacientes com SOFA < 12 (Log-rank p < 0,001). Conclusão: SOFA nas primeiras 24 horas foi o maior preditor de desfecho fatal.

ABSTRACT Objective: To describe a cohort of patients with acute liver failure and to analyze the demographic and clinical factors associated with mortality. Methods: Retrospective cohort study in which all patients admitted for acute liver failure from July 28, 2012, to August 31, 2017, were included. Clinical and demographic data were collected using the Epimed System. The SAPS 3, SOFA, and MELD scores were measured. The odds ratios and 95% confidence intervals were estimated. Receiver operating characteristics curves were obtained for the prognostic scores, along with the Kaplan-Meier survival curve for the score best predicting mortality. Results: The majority of the 40 patients were female (77.5%), and the most frequent etiology was hepatitis B (n = 13). Only 35% of the patients underwent liver transplantation. The in-hospital mortality rate was 57.5% (95%CI: 41.5 - 73.5). Among the scores investigated, only SOFA remained associated with risk of death (OR = 1.37; 95%CI 1.11 - 1.69; p < 0.001). After SOFA stratification into < 12 and ≥ 12 points, survival was higher in patients with SOFA <12 (log-rank p < 0.001). Conclusion: SOFA score in the first 24 hours was the best predictor of fatal outcome.

Fulminant hepatic failure caused by herbal supplies

The consumption of botanicals for therapeutic purposes has increased significantly in recent years. Drug-induced liver disease (DILI) is a frequent cause of acute liver injury, around 50% in the United States, and about 1% is secondary to the use of phytotherapeuticals and herbal supplies. Ruellia bahiensis, a plant species of the Acanthaceae family, is a tropical plant distributed in Northeastern Brazil. In folk medicine in the state of Bahia, the species is known as "mãe-boa" and is commonly used. L.S.S, a 23-year old, female, patient was admitted at University Hospital of Bahia-Brazil with signs and symptoms of acute hepatitis. She had made daily use of an herbal supply popularly known as "mãe-boa" for at least two years prescribed by a physician. Diagnostic investigation was negative for viral and autoimmune hepatitis, leptospirosis, dengue, and CMV (cytomegalovirus). The patient had to undergo liver transplantation. Explant revealed massive hepatic necrosis. According to histological findings, and after exclusion of other etiologies, liver damage was assigned to herbal supply. The prolonged use of Ruellia bahiensis infusions may have caused the liver dysfunction.

Liver transplantation for acute liver failure due to antitubercular drugs - a single-center experience

OBJECTIVES: Patients receiving treatment for tuberculosis are at risk of developing acute liver failure due to the hepatotoxicity of antitubercular drugs. We aimed to describe our experience with liver transplantation from deceased donors in this situation. METHODS: We identified patients undergoing transplantation for acute liver failure due to antitubercular drugs in our prospectively maintained database. RESULTS: Of 81 patients undergoing transplantation for acute liver failure, 8 cases were attributed to antitubercular drugs during the period of 2006-2016. Regarding the time of tuberculosis treatment until the onset of jaundice, patients were on antitubercular drugs for a mean of 64.7 days (21-155 days). The model for end-stage liver disease (MELD) score of patients ranged from 32 to 47 (median 38), and seven patients underwent transplantation under vasopressors. The 1-year survival was 50%. Three patients died during the week following transplantation due to septic shock (including a patient with acute liver failure due to hepatic/disseminated tuberculosis), and the remaining patient died 2 months after transplantation due to pulmonary infection. There were 2 cases of mild rejection and 1 case of moderate rejection. Of the surviving patients, all were considered cured of tuberculosis after alternative drugs were given. CONCLUSION: Patients arrived very sick and displayed poor survival after deceased donor transplantation.

Hepatitis autoinmune en la edad pediátrica / Autoimmune hepatitis in the pediatric age

Resumen: En pediatría, la hepatitis autoinmune y la colangitis esclerosante son patologías de afección hepática cuyo mecanismo de daño es inmunológico. La hepatitis autoinmune es una enfermedad de etiología desconocida, caracterizada por hepatitis de interfase, hipergammaglobulinemia, autoanticuerpos circulantes y una respuesta favorable a la inmunosupresión. Es una enfermedad eminentemente pediátrica, con una afección prevalente hacia mujeres jóvenes. La terapia debe ser instituida con prontitud para prevenir el deterioro rápido, promover la remisión de la enfermedad y la supervivencia a largo plazo. La falta persistente de respuesta o la falta de adherencia al tratamiento dan como resultado una enfermedad hepática terminal. Los pacientes que desarrollan esta patología, y aquellos con insuficiencia hepática fulminante en el momento del diagnóstico, requerirán trasplante hepático.

Abstract: In pediatrics, autoimmune hepatitis and sclerosing cholangitis are liver disorders with an immunological damage mechanism. Autoimmune hepatitis is a disease of unknown etiology characterized by interface hepatitis, hypergammaglobulinemia, circulating autoantibodies and a favorable response to immunosuppression. It is an eminently pediatric disease with a prevalent condition in young women. Therapy should be instituted promptly to prevent rapid deterioration, promote remission of disease and long-term survival. The persistent lack of response or lack of adherence to treatment results in terminal liver failure; these patients, and those with fulminant hepatic insufficiency at the time of diagnosis, will require liver transplantation.

Síndrome inflamatória de reconstituição imune como causa de hepatite autoimune e insuficiência hepática aguda / Immune reconstitution inflammatory syndrome as a cause of autoimmune hepatitis and acute liver failure

RESUMO A insuficiência hepática aguda é uma síndrome rara com elevada mortalidade e frequentemente reconhecida de forma tardia. Os médicos intensivistas desempenham um papel fundamental na suspeição diagnóstica e no manejo das disfunções múltiplo-orgânicas características desta entidade. A síndrome inflamatória de reconstituição imune é uma entidade que se caracteriza pela piora paradoxal do quadro prévio do paciente, após o início de antirretrovirais, desencadeada contra patógenos presentes no hospedeiro ou autoantígenos. A hepatite autoimune tem sido recentemente descrita como uma destas manifestações autoimunes. Os autores relatam o primeiro caso com evolução à insuficiência hepática aguda e óbito em poucos dias após o desenvolvimento de encefalopatia, revisam os casos de hepatite autoimune descritos e tecem comentários sobre as possibilidades terapêuticas neste contexto.

ABSTRACT Acute liver failure is a rare syndrome with high mortality and is often diagnosed late. Intensivist physicians play fundamental roles in the diagnostic suspicion and the management of the multiple-organic dysfunctions characteristic of this entity. Immune reconstitution inflammatory syndrome is an entity that is characterized by the paradoxical worsening of the patient's previous condition, after the initiation of antiretrovirals, triggered against either pathogens present in the host or autoantigens. Autoimmune hepatitis has recently been described as one of these autoimmune manifestations. The authors report the first case with evolution to acute liver failure and death within a few days after the development of encephalopathy, review the cases of autoimmune hepatitis described and comment on the therapeutic possibilities in this context.

Resultados preliminares de hipotermia tópica do fígado em modelo de isquemia/reperfusão hepática em ratos / Preliminary results of topical hepatic hypothermia in a model of liver ischemia/reperfusion injury in rats

ABSTRACT BACKGROUND Ischemia/reperfusion causes organ damage but it is mandatory in hepatic transplantation, trauma and other complex liver surgeries, when Pringle maneuver is applied to minimize bleeding during these procedures. It is well known that liver ischemia/reperfusion leads to microcirculatory disturbance and cellular injury. In this setting hypothermia is known to reduce oxygen demand, lowering intracellular metabolism. OBJECTIVE: To evaluate the effects of hypothermia in liver ischemia/reperfusion injury, using a new model of topic isolated liver hypothermia. METHODS We used male Wistar rats weighting about 250 grams, kept in ad libitum feeding regime and randomly divided into two groups of nine animals: 1) Normothermic group, rats were submitted to normothermic ischemia of the median and left hepatic lobes, with subsequent resection of right and caudate lobes during liver reperfusion; and 2) Hypothermic group, rats were submitted to liver ischemia under hypothermia at 10°C. Liver ischemia was performed for 45 minutes. The animals were euthanized 48 hours after liver reperfusion for blood and liver tissue sampling. RESULTS The transaminases analyses showed a significant decrease of AST and ALT in Hypothermic group (P<0.01) compared to Normothermic group (1403±1234 x 454±213 and 730±680 x 271±211 U/L, respectively). Histology showed severe necrosis in 50% and mild necrosis in 50% of cases in Normothermic group, but severe necrosis in 10% and mild or absent necrosis 90% of the cases in hypothermic group. CONCLUSION: A simplified model of liver ischemia/reperfusion that simulates orthotopic liver autotransplantion was demonstrated. Topical hypothermia of isolated hepatic lobules showed liver protection, being a viable and practical method for any kind of in vivo liver preservation study.

RESUMO CONTEXTO: A isquemia/reperfusão leva a grave lesão de órgãos, mas ocorre obrigatoriamente no transplante hepático, no trauma e em outras cirurgias hepáticas complexas, quando a manobra de Pringle é aplicada com o intuito de minimizar o sangramento durante os procedimentos. É bem conhecido que a isquemia/reperfusão do fígado leva a distúrbios microcirculatórios e lesões celulares. Neste cenário, a hipotermia é conhecida por reduzir a demanda de oxigênio, diminuindo o metabolismo intracelular. OBJETIVO: Avaliar os efeitos da hipotermia na lesão de isquemia/reperfusão hepática utilizando-se um novo modelo de hipotermia isolada do fígado. MÉTODOS: Utilizaram-se ratos Wistar do sexo masculino com peso aproximado de 250 gramas, mantidos em regime de alimentação ad libitum e divididos aleatoriamente em dois grupos de nove animais: 1) Grupo Normotérmico - os ratos foram submetidos a isquemia normotérmica dos lobos hepáticos mediano e esquerdo, com posterior ressecção dos lobos direito e caudado durante a reperfusão hepática; e 2) Grupo Hipotérmico - os ratos foram submetidos a isquemia hepática sob hipotermia a 10°C. A isquemia hepática foi realizada durante 45 minutos. Os animais foram sacrificados 48 horas após a reperfusão hepática para coleta de sangue e tecido hepático para análise. RESULTADOS: As transaminases AST e ALT apresentaram diminuição significativa no grupo Hipotérmico (P<0,01) em relação ao grupo Normotérmico (1403±1234 x 454±213 e 730±680 x 271±211 U/L, respectivamente). A histologia mostrou necrose grave em 50% e necrose leve em 50% dos casos no grupo Normotérmico, porém, necrose grave em 10% e necrose leve ou ausente em 90% dos casos no grupo Hipotérmico. CONCLUSÃO: Foi demonstrado modelo simplificado de isquemia/reperfusão do fígado que simula o autotrasplante de fígado. A hipotermia tópica dos lóbulos hepáticos isolados mostrou proteção do fígado a ischemia/reperfusão, sendo um método viável e prático para qualquer tipo de estudo de preservação hepática in vivo.