Guía práctica para el examen del paciente adulto con hipertransaminasemia asintomática / Practical guidelines for examination of adults with asymptomatic hypertransaminasaemia

Las causas de elevación sostenida de las transaminasas séricas en pacientes adultos asintomáticos son muy variadas, tanto hepáticas como extrahepáticas, y hace falta una sistemática estandarizada para alcanzar el diagnóstico etiológico, orientada a descartar en primer lugar las causas más frecuentes, hepatitis crónica, viral o autoinmune, enfermedades metabólicas y hepatopatías tóxicas. Diversos patrones bioquímicos, que tienen en cuenta los valores de cada transaminasa, de los enzimas de colestasis, de los enzimas musculares, de la ferritina y de la ceruloplasmina, así como el resultado de la determinación del proteinograma y de los autoanticuerpos permitirán reconocer la mayoría de causas. En los casos sin diagnóstico mediante métodos no invasivos estará justificada la práctica de una biopsia hepática

The causes of sustained elevation of serum transaminases in asymptomatic adults, both hepatic and extrahepatic, are varied. In order to reach an aetiological diagnosis, a standardized protocol should be applied, aimed firstly at ruling out the most common causes, such as chronic hepatitis (viral or autoimmune), metabolic diseases, and toxic liver diseases. Several biochemical patterns, which take into account transaminase, cholestatic enzyme, muscle enzyme, ferritin and ceruloplasmin levels, as well protein electrophoresis and autoantibody measurement, will identify most causes. In cases in which a diagnosis cannot be reached with the use of these non-invasive methods, a needle liver biopsy will be justified

Practical guidelines for examination of adults with asymptomatic hypertransaminasaemia. / Guía práctica para el examen del paciente adulto con hipertransaminasemia asintomática.

The causes of sustained elevation of serum transaminases in asymptomatic adults, both hepatic and extrahepatic, are varied. In order to reach an aetiological diagnosis, a standardized protocol should be applied, aimed firstly at ruling out the most common causes, such as chronic hepatitis (viral or autoimmune), metabolic diseases, and toxic liver diseases. Several biochemical patterns, which take into account transaminase, cholestatic enzyme, muscle enzyme, ferritin and ceruloplasmin levels, as well protein electrophoresis and autoantibody measurement, will identify most causes. In cases in which a diagnosis cannot be reached with the use of these non-invasive methods, a needle liver biopsy will be justified.

Unidades multidisciplinarias en hospitales de referencia para mejorar la atención de los pacientes con enfermedad de Wilson / Multidisciplinary units in tertiary referral hospitals to improve management of Wilson disease

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Breve historia de los congresos de la Asociación Española para el Estudio del Hígado (1967–2015) / A brief history of the meetings of the Spanish Association for the study of the liver (1967-2015)

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Envejecimiento y enfermedades del hígado / Liver diseases in the elderly

La patología hepática del anciano ha despertado menos interés que la de otros órganos, ya que el hígado juega un escaso papel en el envejecimiento. No existen enfermedades hepáticas específicas de la edad avanzada, pero las modificaciones anatómicas y funcionales del hígado ligadas al envejecimiento justifican cambios en la frecuencia y comportamiento clínico y evolutivo de algunas enfermedades hepáticas, en comparación con el que se observa en pacientes más jóvenes.En esta revisión se examinan los aspectos más destacados del estado del hígado en la población sana de edad avanzada, así como las características de las enfermedades hepáticas más prevalentes en esta edad, especialmente la aproximación diagnóstica ante los problemas hepáticos más comunes en la edad avanzada: la elevación asintomática de las transaminasas y la ictericia. Liver diseases in the elderly have aroused less interest than diseases of other organs, since the liver plays a limited role in aging. There are no specific liver diseases of old age, but age-related anatomical and functional modifications of the liver cause changes in the frequency and clinical behavior of some liver diseases compared with those in younger patients. This review discusses the most important features of liver function in the healthy elderly population, as well as the features of the most prevalent liver diseases in this age group, especially the diagnostic approach to the most common liver problems in the elderly: asymptomatic elevation of serum transaminases and jaundice

Liver diseases in the elderly have aroused less interest than diseases of other organs, since the liver plays a limited role in aging. There are no specific liver diseases of old age, but age-related anatomical and functional modifications of the liver cause changes in the frequency and clinical behavior of some liver diseases compared with those in younger patients. This review discusses the most important features of liver function in the healthy elderly population, as well as the features of the most prevalent liver diseases in this age group, especially the diagnostic approach to the most common liver problems in the elderly: asymptomatic elevation of serum transaminases and jaundice

[Liver diseases in the elderly]. / Envejecimiento y enfermedades del hígado.

Liver diseases in the elderly have aroused less interest than diseases of other organs, since the liver plays a limited role in aging. There are no specific liver diseases of old age, but age-related anatomical and functional modifications of the liver cause changes in the frequency and clinical behavior of some liver diseases compared with those in younger patients. This review discusses the most important features of liver function in the healthy elderly population, as well as the features of the most prevalent liver diseases in this age group, especially the diagnostic approach to the most common liver problems in the elderly: asymptomatic elevation of serum transaminases and jaundice.

Sistemática diagnóstica en la hiperferritinemia / Systematic approach to the diagnosis of hyperferritinemia

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Use of Hy's law and a new composite algorithm to predict acute liver failure in patients with drug-induced liver injury.

BACKGROUND & AIMS: Hy's Law, which states that hepatocellular drug-induced liver injury (DILI) with jaundice indicates a serious reaction, is used widely to determine risk for acute liver failure (ALF). We aimed to optimize the definition of Hy's Law and to develop a model for predicting ALF in patients with DILI. METHODS: We collected data from 771 patients with DILI (805 episodes) from the Spanish DILI registry, from April 1994 through August 2012. We analyzed data collected at DILI recognition and at the time of peak levels of alanine aminotransferase (ALT) and total bilirubin (TBL). RESULTS: Of the 771 patients with DILI, 32 developed ALF. Hepatocellular injury, female sex, high levels of TBL, and a high ratio of aspartate aminotransferase (AST):ALT were independent risk factors for ALF. We compared 3 ways to use Hy's Law to predict which patients would develop ALF; all included TBL greater than 2-fold the upper limit of normal (×ULN) and either ALT level greater than 3 × ULN, a ratio (R) value (ALT × ULN/alkaline phosphatase × ULN) of 5 or greater, or a new ratio (nR) value (ALT or AST, whichever produced the highest ×ULN/ alkaline phosphatase × ULN value) of 5 or greater. At recognition of DILI, the R- and nR-based models identified patients who developed ALF with 67% and 63% specificity, respectively, whereas use of only ALT level identified them with 44% specificity. However, the level of ALT and the nR model each identified patients who developed ALF with 90% sensitivity, whereas the R criteria identified them with 83% sensitivity. An equal number of patients who did and did not develop ALF had alkaline phosphatase levels greater than 2 × ULN. An algorithm based on AST level greater than 17.3 × ULN, TBL greater than 6.6 × ULN, and AST:ALT greater than 1.5 identified patients who developed ALF with 82% specificity and 80% sensitivity. CONCLUSIONS: When applied at DILI recognition, the nR criteria for Hy's Law provides the best balance of sensitivity and specificity whereas our new composite algorithm provides additional specificity in predicting the ultimate development of ALF.

Bezafibrate normalizes alkaline phosphatase in primary biliary cirrhosis patients with incomplete response to ursodeoxycholic acid.

BACKGROUND & AIMS: Ursodeoxycholic acid (UDCA) is the standard treatment for primary biliary cirrhosis (PBC) but excellent response is not observed in all cases. Since potential favourable effects of fibrates have been reported in short series with inconclusive results, we have carried out a pilot study to analyse the effects of bezafibrate in patients with suboptimal response to UDCA. METHODS: Thirty women (age 52.3 ± 2.3 years) treated with UDCA and abnormal alkaline phosphatase (AP) levels received bezafibrate (400 mg/d) for 1 year. Changes were measured every 3 months during the study period of 12 months, 3 months after discontinuation and 3 months after resuming bezafibrate. RESULTS: Two patients discontinued the treatment after few days, three at 6 and one at 9 months. Bezafibrate treatment resulted in a significant decrease in AP as early as 3 months. Normalization or decrease of AP below 1.5 times normal levels was observed in 13 and 4 patients respectively. There was also a significant decrease in γ-glutamyl transferase and alanine aminotransferase, cholesterol and triglyceride levels. Bezafibrate treatment resulted in significant improvement of pruritus. A rebound in liver biochemistries and pruritus occurred upon drug discontinuation, changes which improved again after resuming bezafibrate. Response to bezafibrate was associated with lower liver stiffness and severity of cholestasis. No severe adverse effects were observed. CONCLUSIONS: Combination treatment of bezafibrate and UDCA is associated with marked decrease or normalization of alkaline phosphatase as early as 3 months in patients with PBC. Better biochemical response was observed in patients with early disease and lower cholestasis.

Un modelo integral y de gestión directa del aseguramiento de la responsabilidad profesional médica en Cataluña / An integral and direct management model of medical profesional responsibility insurance in Catalonia

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Hepatotoxicity associated with glucosamine and chondroitin sulfate in patients with chronic liver disease.

Glucosamine and chondroitin sulfate are molecules involved in the formation of articular cartilage and are frequently used for symptom relief in patients with arthrosis. These molecules are well tolerated with scarce secondary effects. Very few cases of possible hepatotoxicity due to these substances have been described. The aim of this paper is to report the frequency of presumed glucosamine hepatotoxicity in patients with liver disease. A questionnaire was given to 151 consecutive patients with chronic liver disease of different etiology (mean age 59 years, 56.9% women) attended in an outpatient clinic with the aim of evaluating the frequency of consumption of these drugs and determine whether their use coincided with a worsening in liver function test results. Twenty-three patients (15.2%) recognized having taken products containing glucosamine or chondroitin sulfate previously or at the time of the questionnaire. Review of the clinical records and liver function tests identified 2 patients presenting an elevation in aminotransferase values temporarily associated with glucosamine treatment; one of the cases simultaneously presented a skin rash attributed to the drug. Review of these two patients and the cases described in the literature suggest toxicity of glucosamine and chondroitin sulfate. The clinical spectrum is variable, and the mechanism of toxicity is not clear but may involve reactions of hypersensitivity. The consumption of products containing glucosamine and/or chondroitin sulfate is frequent among patients with chronic liver diseases and should be taken into account on the appearance of alterations in liver function tests not explained by the underlying disease.

Problemas frecuentes en el diagnóstico y tratamiento de la enfermedad de Wilson / Common problems in the diagnosis and treatment of Wilson's disease

Este trabajo tiene como objetivo dar respuesta a preguntas que con frecuencia se hacen médicos que atienden a pacientes con enfermedad de Wilson (EW) o pacientes en quienes se plantea la sospecha diagnóstica de que se trate de una EW. Tiene 2 partes, una con respuestas a preguntas relacionadas con el diagnóstico de la enfermedad y otra con respuestas a preguntas relacionadas con el tratamiento. Al final se incluye un breve apéndice con respuestas a preguntas que no se pueden incluir en las 2 anteriores categorías (AU)

The present article aims to provide answers to questions frequently asked by physicians attending patients with Wilson's disease (WD) or those with a suspected diagnosis of WD. The article is divided into 2 parts: a first part with answers to questions relating to the diagnosis of this entity and a second with answers to questions concerning treatment. A brief appendix is included with responses to questions not falling into either of these 2 categories (AU)

[Common problems in the diagnosis and treatment of Wilson's disease]. / Problemas frecuentes en el diagnóstico y tratamiento de la enfermedad de Wilson.

The present article aims to provide answers to questions frequently asked by physicians attending patients with Wilson's disease (WD) or those with a suspected diagnosis of WD. The article is divided into 2 parts: a first part with answers to questions relating to the diagnosis of this entity and a second with answers to questions concerning treatment. A brief appendix is included with responses to questions not falling into either of these 2 categories.