Risk factors for fibrosis progression in non-alcoholic steatohepatitis: Analysis of the European cohort in the real-world GAIN study / Factores de riesgo para la progresión de la fibrosis en la esteatohepatitis no alcohólica: análisis de la cohorte europea en el estudio GAIN del mundo real

Objective To better understand drivers of disease progression in non-alcoholic steatohepatitis (NASH), we assessed clinical and sociodemographic markers of fibrosis progression in adults with NASH.Patients and methodsPhysician-reported patient demographics and clinical characteristics were utilised from the real-world Global Assessment of the Impact of NASH (GAIN) study. Factors associated with likelihood of fibrosis progression since NASH diagnosis were identified using a logistic regression model.ResultsOverall, 2349 patients in Europe from the GAIN study were included; mean age was 54.6 years and 41% were women. Significant covariates included age, years since diagnosis, employment status, fibrosis stage at diagnosis, type 2 diabetes mellitus, hypertension, liver transplant and liver biopsy at diagnosis. Risk of progression was 1.16 (95% confidence interval 1.12–1.20; p<0.001) times higher for each additional year since NASH diagnosis and 5.43 (2.68–11.37; p<0.001) times higher when physicians proposed a liver transplant at diagnosis. Compared with full-time employed patients, risk of progression was 1.77 (1.19–2.60; p=0.004) times higher for unemployed patients and 3.16 (1.30–7.63; p=0.010) times higher for those unable to work due to NASH.ConclusionsDisease duration, NASH severity and presence of other metabolic comorbidities could help to assess risk of progression in patients with NASH. (AU)

Objetivo Para comprender mejor los factores que impulsan la progresión de la enfermedad en la esteatohepatitis no alcohólica (NASH), evaluamos los marcadores clínicos y sociodemográficos de la progresión de la fibrosis en adultos con NASH.Pacientes y métodosSe utilizaron las características demográficas y clínicas de los pacientes informadas por los médicos del estudio de Evaluación Global del Impacto de NASH (GAIN) del mundo real. Los factores asociados con la probabilidad de progresión de la fibrosis desde el diagnóstico de EHNA se identificaron mediante un modelo de regresión logística.ResultadosEn total, se incluyeron 2.349 pacientes en Europa del estudio GAIN; la edad media fue 54,6 años y el 41% eran mujeres. Las covariables significativas incluyeron edad, años desde el diagnóstico, situación laboral, estadio de fibrosis en el momento del diagnóstico, diabetes mellitus tipo 2, hipertensión, trasplante de hígado y biopsia de hígado en el momento del diagnóstico. El riesgo de progresión fue 1,16 (intervalo de confianza del 95% 1,12-1,20; p < 0,001) veces mayor por cada año adicional desde el diagnóstico de EHNA y 5,43 (2,68-11,37; p < 0,001) veces mayor cuando los médicos propusieron un trasplante de hígado. en el momento del diagnóstico. En comparación con los pacientes empleados a tiempo completo, el riesgo de progresión fue 1,77 (1,19-2,60; p = 0,004) veces mayor para los pacientes desempleados y 3,16 (1,30-7,63; p = 0,010) veces mayor para aquellos que no podían trabajar debido a a NASH.ConclusionesLa duración de la enfermedad, la gravedad de NASH y la presencia de otras comorbilidades metabólicas podrían ayudar a evaluar el riesgo de progresión en pacientes con NASH. (AU)

Risk factors for fibrosis progression in non-alcoholic steatohepatitis: Analysis of the European cohort in the real-world GAIN study.

OBJECTIVE: To better understand drivers of disease progression in non-alcoholic steatohepatitis (NASH), we assessed clinical and sociodemographic markers of fibrosis progression in adults with NASH. PATIENTS AND METHODS: Physician-reported patient demographics and clinical characteristics were utilised from the real-world Global Assessment of the Impact of NASH (GAIN) study. Factors associated with likelihood of fibrosis progression since NASH diagnosis were identified using a logistic regression model. RESULTS: Overall, 2349 patients in Europe from the GAIN study were included; mean age was 54.6 years and 41% were women. Significant covariates included age, years since diagnosis, employment status, fibrosis stage at diagnosis, type 2 diabetes mellitus, hypertension, liver transplant and liver biopsy at diagnosis. Risk of progression was 1.16 (95% confidence interval 1.12-1.20; p<0.001) times higher for each additional year since NASH diagnosis and 5.43 (2.68-11.37; p<0.001) times higher when physicians proposed a liver transplant at diagnosis. Compared with full-time employed patients, risk of progression was 1.77 (1.19-2.60; p=0.004) times higher for unemployed patients and 3.16 (1.30-7.63; p=0.010) times higher for those unable to work due to NASH. CONCLUSIONS: Disease duration, NASH severity and presence of other metabolic comorbidities could help to assess risk of progression in patients with NASH.

Pediatric patients with lysosomal acid lipase deficiency / Pacientes pediátricos con deficiencia de lipasa ácida lisosomal

Lysosomal acid lipase (LAL) deficiency is a rare, autosomal recessive disease caused by mutations in the LIPA gene, which produces cholesteryl ester and triglyceride accumulation predominantly in hepatocytes, adrenal glands, and gastrointestinal tract. We describe two new cases occurring in siblings, aged 5 and 7 years, who presented with hepatomegaly, dyslipidemia, and abnormal liver function. Percutaneous liver biopsy revealed portal inflammation, hypertrophic Kupffer cells with a foamy appearance and microvesicular steatosis with fibrosis. Immunostaining for lysosomal markers, cathepsin D and LAMP1 reflected the lysosomal nature of the lipid vacuoles. After enzymatic confirmation, enzyme replacement therapy was initiated for both siblings. Follow-up transaminase levels and lipid profiles showed a notable decrease in AST and ALT and a slight increase in HDL cholesterol. It is crucial to increase awareness of this rare condition among clinicians and pathologists. The expression of lysosomal markers around the lipid vacuoles might help diagnose LAL deficiency in pediatric patients.(AU)

La deficiencia de lipasa ácida lisosomal (LAL) es una enfermedad autosómica recesiva inusual, causada por mutaciones en el gen LIPA, que genera acumulación de éster de colesterol y triglicéridos predominantemente en hepatocitos, glándulas suprarrenales y tracto gastrointestinal. Describimos 2 casos adicionales que ocurrieron en 2 hermanos, de 5 y 7 años, que presentaron hepatomegalia, dislipidemia y función hepática anormal. La biopsia hepática percutánea reveló inflamación portal leve, células de Kupffer hipertróficas, con un aspecto espumoso y esteatosis microvesicular difusa con fibrosis. La inmunotinción de marcadores lisosomales, catepsina D y LAMP1, reflejó la naturaleza lisosomal de las vacuolas lipídicas. Después de la confirmación enzimática, ambos hermanos iniciaron terapia de reemplazo enzimático. Los niveles de transaminasas y los perfiles lipídicos de seguimiento mostraron una disminución notoria en AST y ALT y un ligero aumento en el colesterol HDL. Es crucial aumentar la conciencia de esta inusual condición entre médicos y patólogos. La expresión de marcadores lisosomales alrededor de las vacuolas lipídicas podría ayudar a diagnosticar la deficiencia de LAL en pacientes pediátricos.(AU)

Pediatric patients with lysosomal acid lipase deficiency.

Lysosomal acid lipase (LAL) deficiency is a rare, autosomal recessive disease caused by mutations in the LIPA gene, which produces cholesteryl ester and triglyceride accumulation predominantly in hepatocytes, adrenal glands, and gastrointestinal tract. We describe two new cases occurring in siblings, aged 5 and 7 years, who presented with hepatomegaly, dyslipidemia, and abnormal liver function. Percutaneous liver biopsy revealed portal inflammation, hypertrophic Kupffer cells with a foamy appearance and microvesicular steatosis with fibrosis. Immunostaining for lysosomal markers, cathepsin D and LAMP1 reflected the lysosomal nature of the lipid vacuoles. After enzymatic confirmation, enzyme replacement therapy was initiated for both siblings. Follow-up transaminase levels and lipid profiles showed a notable decrease in AST and ALT and a slight increase in HDL cholesterol. It is crucial to increase awareness of this rare condition among clinicians and pathologists. The expression of lysosomal markers around the lipid vacuoles might help diagnose LAL deficiency in pediatric patients.

Caracterização clínica e patológica dos linfomas hepáticos: um estudo retrospetivo unicêntrico / Clinical and pathological characterization of hepatic lymphomas: a retrospective single-center study

ABSTRACT Background: The incidence of hepatic lymphoma has been increasing recently and diagnosis can be challenging as clinical presentation and radiological findings are usually variable and non-specific. Objective The aims of this study were to describe their main clinical, pathological and imaging characteristics and identify poor prognostic factors. Methods A retrospective study that included all patients with histological diagnosis of liver lymphoma over a 10-year period at our center was performed. Results A total of 36 patients were identified, with mean age of 56.6 years and male predominance (58%). There were three patients with primary liver lymphoma (8.3%) and 33 with secondary liver lymphoma (91.7%). The most common histological type was diffuse large B-cell lymphoma (33.3%). The most common clinical manifestations included fever, lymphadenopathy, weight loss, night sweats and abdominal discomfort; three patients (11.1%) were asymptomatic. Computed tomography scan revealed heterogenous radiological patterns including a single nodule (26.5%), multiple nodules (41.2%) or diffuse infiltration (32.4%). The mortality rate during follow-up was 55.6%. Higher levels of C-reactive protein (P=0.031) and absence of treatment response (P<0.001) were significantly associated with higher mortality. Conclusion Hepatic lymphoma is a rare disease that may involve liver as part of a systemic disease or, less commonly, be confined to this organ. Clinical presentation and radiological findings are often variable and non-specific. It is associated with high mortality and poor prognostic factors include higher levels of C-reactive protein and absence of response to treatment.

RESUMO Contexto A incidência de linfoma hepático tem aumentando recentemente e o diagnóstico pode ser desafiante, na medida em que a apresentação clínica e os achados imagiológicos são geralmente variáveis e inespecíficos. Objetivo: Os objetivos deste estudo foram descrever suas principais características clínicas, patológicas e de imagem e identificar fatores de mau prognóstico. Métodos: Foi realizado um estudo retrospetivo que incluiu todos os pacientes com diagnóstico histológico de linfoma hepático num período de 10 anos no nosso centro. Resultados: Foram identificados 36 pacientes, com média de idade de 56,6 anos e predomínio de género masculino (58%). Havia três pacientes com linfoma hepático primário (8,3%) e 33 com linfoma hepático secundário (91,7%). O tipo histológico mais comum foi o linfoma difuso de grandes células B (33,3%). As manifestações clínicas mais comuns incluíram febre, linfadenopatia, emagrecimento, hipersudorese noturna e desconforto abdominal; 3 (11,1%) pacientes eram assintomáticos. A tomografia computadorizada revelou padrões radiológicos heterogêneos, incluindo um único nódulo (26,5%), múltiplos nódulos (41,2%) ou infiltração difusa (32,4%). A taxa de mortalidade durante o seguimento foi de 55,6%. Níveis mais elevados de proteína C reativa (P=0,031) e ausência de resposta ao tratamento (P<0,001) foram significativamente associados a maior mortalidade. Conclusão O linfoma hepático é uma doença rara que pode envolver o fígado como parte de uma doença sistêmica ou, menos comumente, estar confinado a este órgão. A apresentação clínica e os achados radiológicos são frequentemente variáveis e inespecíficos. Associa-se a elevada mortalidade e fatores de mau prognóstico incluem níveis mais elevados de proteína C reativa e ausência de resposta ao tratamento.

Biopsia hepática: experiencia en un hospital regional del sur de Chile / Liver biopsy: experience in a regional hospital in southern Chile

Introduction: Liver biopsy is an invasive procedure with a minimal rate of associated complications, which represents a highly useful strategy for the diagnosis of pathologies in the liver, when the etiology cannot be clarified by non-invasive techniques. It provides information that allows determining the progression and prognosis of liver diseases. Objectives: To evaluate the main etiology causes of liver disease in patients undergoing liver biopsy. Material and Methods: Review of the clinical record of 61 patients who underwent this procedure between 2018 and 2020 at the Hernán Henríquez Aravena hospital, demographic variables, diagnosis that motivates its performance, and associated complications. Results: Average age 58 years, of this 66% were female and 34% were male. The diagnoses that motivated this procedure were: autoimmune hepatitis, liver Tumors, and chronic liver damage of unknown etiology. 100% of the case had a satisfactory sample for the analysis and clarification of the cause of liver disease. 91% did not present complications and of the 8% associated with complications, pain was the most common. Conclusion: Liver biopsy is an effective method that allowed establishing etiology, confirming diagnosis suspicions, and evaluating the progression of liver disease with a low rate of complications.

Introducción: La biopsia hepática es un procedimiento invasivo con una tasa mínima de complicaciones asociadas, que representa una estrategia de gran utilidad para el diagnóstico de patologías a nivel del hígado, cuando a través de técnicas no invasivas no se logra esclarecer la etiología. Además, entrega información que permite determinar la progresión y pronóstico de enfermedades hepáticas. Objetivos: Evaluar las principales causas etiológicas de hepatopatías en los pacientes sometidos a biopsia hepática. Material y Método: Revisión de fichas clínicas de 61 pacientes sometidos a este procedimiento entre el año 2018 y 2020 en el hospital Hernán Henríquez Aravena, se analizaron variables demográficas, diagnóstico que motiva su realización, y complicaciones asociadas. Resultados: Edad promedio: 58 años de estos: 66% eran del sexo femenino y 34% al sexo masculino. De los diagnósticos que motivaron a la realización de la biopsia fuero: hepatitis autoinmune, tumores hepáticos y daño hepático crónico de etiología no precisada. El 100% de los casos tuvo una muestra satisfactoria para el análisis y esclarecer la causa de la hepatopatía. El 91% no presentó complicaciones y del 8% de las complicaciones presentadas, el dolor fue la principalmente descrita. Conclusiones: La biopsia hepática es un método eficaz que permitió establecer etiología, confirmar sospechas diagnósticas y evaluar progresión de enfermedades hepáticas con una baja tasa de complicación.

Changes in indications for outpatient percutaneous liver biopsy over 5 years: from hepatitis C to fatty liver disease / Cambios en las indicaciones de la biopsia hepática percutánea ambulatoria durante 5 años: de hepatitis C a enfermedad del hígado graso

Introduction: Although the use of non-invasive methods for assessment of liver fibrosis has reduced the need for biopsy, the diagnosis of liver damage still requires histological evaluation in many patients. We aim to describe the indications for percutaneous liver biopsy (PLB) and the rate of complications in an outpatient setting over 5 years. Methods: This observational, single-center, and retrospective study included patients submitted to real-time ultrasound (US)-guided biopsies from 2015 to 2019. We collected age, gender, coagulation tests, comorbidities, and the number of needle passes. The association between the variables and complications was evaluated using the generalized estimating equations method. Results: We analyzed 532 biopsies in 524 patients (55.3% male) with a median age of 49 years (range 13–74y). An average of 130.3 biopsies per year were performed in the first 3 years of the study versus 70.5 in the other 2y. The main indications were hepatitis C virus (HCV) infection (47.0%), autoimmune and cholestatic liver diseases (12.6%), and metabolic dysfunction-associated fatty liver disease (MAFLD) (12.1%). The number of HCV-related biopsies had a remarkable reduction, while MAFLD-related procedures have progressively raised over time. Around 54% of the patients reported pain, which was significantly associated with females (p=0.0143). Serious complications occurred in 11 patients (2.1%) and hospital admission was necessary in 10 cases (1.9%). No patient required surgical approach and there were no deaths. No significant association was found between the studied variables and biopsy-related complications. Conclusion: The indications for PLB in an outpatient setting have changed from HCV to MAFLD over the years. This procedure is safe and has a low rate of serious complications, but new strategies to prevent the pain are still needed, especially for females.(AU)

Introducción: Aunque el uso de métodos no invasivos para evaluar la fibrosis hepática ha reducido la necesidad de una biopsia, el diagnóstico de daño hepático aún requiere una evaluación histológica en muchos pacientes. Nuestro objetivo es describir las indicaciones de la biopsia hepática percutánea ambulatoria y la tasa de complicaciones durante cinco años. Métodos: Este estudio observacional, retrospectivo y unicéntrico incluyó pacientes sometidos a biopsias guiadas por ecografía en tiempo real desde 2015 hasta 2019. Recogimos información sobre edad, sexo, pruebas de coagulación, comorbilidades y número de pasadas de aguja. La asociación entre variables y complicaciones se evaluó mediante el método de ecuaciones de estimación generalizada. Resultados: Analizamos 532 biopsias en 524 pacientes (55,3% hombres) con una edad media de 49 años (rango de 13 a 74 años). Se realizó una media de 130,3 biopsias por año en los primeros tres años del estudio frente a 70,5 en los otros dos años. Las principales indicaciones fueron la infección por el virus de la hepatitis C (HCV) (47,0%), las enfermedades hepáticas autoinmunes y colestásicas (12,6%) y la enfermedad del hígado graso asociada a disfunción metabólica (MAFLD) (12,1%). El número de biopsias relacionadas con la HCV tuvo una reducción notable, mientras que los procedimientos relacionados con MAFLD han aumentado progresivamente con el tiempo. Alrededor del 54% de los pacientes informaron dolor, que se asoció significativamente con las mujeres (p = 0,0143). Se produjeron complicaciones graves en 11 pacientes (2,1%) y el ingreso hospitalario fue necesario en 10 casos (1,9%). Ningún paciente requirió abordaje quirúrgico y no hubo muertes. No se encontró asociación significativa entre las variables estudiadas y las complicaciones relacionadas con la biopsia. Conclusión: Las indicaciones para la biopsia hepática percutánea ambulatoria han cambiado de HCV a MAFLD con el pasar de los años.(AU)

Enfermedad de Wilson: consideraciones para optimizar el seguimiento a largo plazo / Considerations for optimizing Wilson's disease patients’ long-term follow-up

La enfermedad de Wilson es una patología genética multiorgánica causada por la acumulación excesiva de cobre en el organismo. La afectación hepática es la inicial y principal, y puede suponer desde una hepatitis leve y transitoria, hasta el debut en forma de cirrosis o insuficiencia hepática aguda grave. En el seguimiento, hasta un 20-30% de estos pacientes evolucionan a cirrosis hepática. En la práctica clínica, la monitorización de la fibrosis hepática se realiza mayoritariamente mediante métodos indirectos y no invasivos (analíticas, elastografía hepática, ecografías) a semejanza de otras hepatopatías crónicas más prevalentes. No obstante, a pesar de que la elastografía constituye una herramienta de gran valor, la evidencia de su utilidad en Wilson es muy limitada. En esta revisión se repasa la información disponible y sus limitaciones para el seguimiento de la enfermedad de Wilson.

Wilson's disease is a sistemic genetic disease caused by the excessive accumulation of copper. The first and main involvement is in the liver, which can range from mild and transient elevation of transaminases to the onset of an overt cirrhosis or acute liver failure. It is known that up to 20-30% of these patients may evolve to liver cirrhosis during follow-up. In clinical practice, liver fibrosis is assessed mainly by using indirect and non-invasive tools (laboratory tests, liver elastography, ultrasound), similar to other prevalent chronic liver diseases. However, despite the fact that liver elastography is a valuable tool in general hepatology, the evidence of its usefulness and accuracy in Wilsońs disease is scarce. This review summarizes the available scientific data and their limitations in Wilson's disease.

Considerations for optimizing Wilson's disease patients' long-term follow-up. / Enfermedad de Wilson: consideraciones para optimizar el seguimiento a largo plazo.

Wilson's disease is a sistemic genetic disease caused by the excessive accumulation of copper. The first and main involvement is in the liver, which can range from mild and transient elevation of transaminases to the onset of an overt cirrhosis or acute liver failure. It is known that up to 20-30% of these patients may evolve to liver cirrhosis during follow-up. In clinical practice, liver fibrosis is assessed mainly by using indirect and non-invasive tools (laboratory tests, liver elastography, ultrasound), similar to other prevalent chronic liver diseases. However, despite the fact that liver elastography is a valuable tool in general hepatology, the evidence of its usefulness and accuracy in Wilsons disease is scarce. This review summarizes the available scientific data and their limitations in Wilson's disease.

Ultrasound-guided percutaneous liver biopsy in children. Five-year experience at a tertiary care center.

INTRODUCTION AND AIMS: Percutaneous liver biopsy with histopathologic analysis is a valuable tool for the diagnosis, prognosis, and treatment evaluation of liver diseases. Its ultrasound-guided performance is useful, making the procedure safer and reducing the risk for complications and hospital stay. Our aim was to describe the indications, histopathologic study, and complications associated with the performance of ultrasound-guided percutaneous liver biopsy in pediatric patients. MATERIAL AND METHODS: The study included 102 ultrasound-guided percutaneous liver biopsies performed on patients <16 years of age, within the time frame of January 2014 and December 2019. The information was obtained from electronic files and histopathologic studies and the data were analyzed through descriptive statistics. RESULTS: A total of 102 procedures were carried out on 99 patients. Mean patient age was 72 months and 58.8% of the patients were female. Over 65% of the indications for liver biopsy included autoimmune hepatitis (23.5%), elevated liver enzymes (21.5%), and chronic liver disease (20.5%). Four patients presented with immediate complications (3.9%), three of which were major (2.9%), concurring with that reported in the international literature. CONCLUSIONS: Our study corroborates the importance of ultrasound-guided liver biopsy in the diagnosis and follow-up of pediatric patients. The procedure also had a low complication rate of only 3.9%.

Changes in indications for outpatient percutaneous liver biopsy over 5 years: from hepatitis C to fatty liver disease.

INTRODUCTION: Although the use of non-invasive methods for assessment of liver fibrosis has reduced the need for biopsy, the diagnosis of liver damage still requires histological evaluation in many patients. We aim to describe the indications for percutaneous liver biopsy (PLB) and the rate of complications in an outpatient setting over 5 years. METHODS: This observational, single-center, and retrospective study included patients submitted to real-time ultrasound (US)-guided biopsies from 2015 to 2019. We collected age, gender, coagulation tests, comorbidities, and the number of needle passes. The association between the variables and complications was evaluated using the generalized estimating equations method. RESULTS: We analyzed 532 biopsies in 524 patients (55.3% male) with a median age of 49 years (range 13-74y). An average of 130.3 biopsies per year were performed in the first 3 years of the study versus 70.5 in the other 2y. The main indications were hepatitis C virus (HCV) infection (47.0%), autoimmune and cholestatic liver diseases (12.6%), and metabolic dysfunction-associated fatty liver disease (MAFLD) (12.1%). The number of HCV-related biopsies had a remarkable reduction, while MAFLD-related procedures have progressively raised over time. Around 54% of the patients reported pain, which was significantly associated with females (p=0.0143). Serious complications occurred in 11 patients (2.1%) and hospital admission was necessary in 10 cases (1.9%). No patient required surgical approach and there were no deaths. No significant association was found between the studied variables and biopsy-related complications. CONCLUSION: The indications for PLB in an outpatient setting have changed from HCV to MAFLD over the years. This procedure is safe and has a low rate of serious complications, but new strategies to prevent the pain are still needed, especially for females.

Hamartomatosis biliar múltiple / Multiple biliary hamartomatosis

Introducción: La hamartomatosis biliar múltiple o también llamada enfermedad de los complejos de von Meyenburg fue descrita por este autor en 1955. Tiene un origen disembriogénico con un curso evolutivo benigno y asintomático, con pruebas funcionales hepáticas normales. Por los estudios de imágenes se puede confirmar el diagnóstico, pero igualmente ante un hígado multinodular pueden diagnosticar una hepatopatía crónica sin precisar su etiología, por lo que es imprescindible el diagnóstico histológico con biopsia hepática translaparoscópica dirigida. No se necesita ningún tratamiento específico y su seguimiento es ecográfico semestral o anual. Objetivo: Presentar el valor de la biopsia hepática dirigida por laparoscopia a las lesiones por hamartomatosis biliar múltiple. Desarrollo: Se presenta un paciente de 53 años con antecedentes de ser un bebedor social con frecuencia semanal. Ingresa por fiebre asociada a una sepsis urinaria, en el que aparece un fortuito hallazgo ecográfico de un hígado multinodular, sin precisar un diagnóstico etiológico por otros estudios de imágenes. Esto motivó a realizarle una laparoscopia con toma de biopsia hepática dirigida a las lesiones observadas. Se confirma el diagnóstico histológico de esta entidad. Conclusiones: Se demostró la importancia y vigencia del valor diagnóstico de la laparoscopia, al igual que la biopsia hepática dirigida para lograr el diagnóstico histológico de certeza en esta entidad(AU)

Introduction: Multiple biliary hamartomatosis or von Meyenburg complex disease was described by this author in 1955. Its origin is dysembryogenic with a benign and asymptomatic evolutionary course, with normal liver function tests. Imaging studies can confirm the diagnosis, but likewise, when it is a multinodular liver, chronic liver disease can be diagnosed without specifying its etiology, which is why it is essential a histological diagnosis with a directed overlaparoscopic liver biopsy. No specific treatment is needed and its follow-up is semi-annual or annual ultrasound. Objective: To present the value of laparoscopically directed liver biopsy for multiple biliary hamartomatosis lesions. Case report: A 53-year-old patient with a history of being a social drinker with a weekly frequency is reported. He was admitted for fever associated with urinary sepsis, in which a fortuitous ultrasound finding of a multinodular liver appeared, without requiring an etiological diagnosis by other imaging studies. This led to a laparoscopy with a liver biopsy aimed at the observed lesions. The histological diagnosis of this entity is confirmed. Conclusions: The importance and validity of the diagnostic value of laparoscopy, as well as directed liver biopsy to achieve a certain histological diagnosis in this entity, was demonstrated(AU)

Concordancia entre el resultado de la biopsia hepática y el índice APRI (Ast to Platelet Ratio Index) en el diagnóstico de cirrosis en pacientes con enfermedad hepática autoinmune / Concordance between hepatic biopsy and the APRI index (Ast to Platelet Ratio Index) for the diagnosis of cirrhosis in patients with autoimmune liver disease

Introducción y objetivos: Se ha propuesto que métodos no invasivos pueden remplazar la biopsia hepática en el diagnóstico del daño tisular en pacientes con hepatopatía autoinmune (EHA). Este estudio evalúa el rendimiento diagnóstico y grado de concordancia entre el índice Ast to Platelet Ratio Index (APRI) y la biopsia hepática en el diagnóstico de cirrosis en estos pacientes. Material y métodos: En una cohorte de pacientes con EHA se determinó el valor del índice APRI y los resultados de la biopsia hepática según la escala METAVIR. Se evaluó el área bajo la curva (AUC) y la concordancia entre un valor de APRI > 2 y un puntaje METAVIR F4 como marcadores de la presencia de cirrosis hepática mediante un estadístico de kappa. Resultados: Se incluyeron 70 pacientes (51 ± 13 años). Las hepatopatías autoinmunes más frecuentes fueron la cirrosis biliar primaria (CBP) (40%), Hepatitis autoinmune (HAI) (24,3%) y el síndrome de sobreposición HAI–CBP (32,9%). Se confirmó cirrosis por biopsia en 16 pacientes (22,9%); 15 pacientes (21,4%) presentaron índice APRI > 2 (cirrosis) y solo seis cumplieron ambos criterios. El AUC del APRI fue de 0,77 (IC 95% 0,65-0,88). La concordancia entre las pruebas fue baja para un punto de corte APRI > 2 (kappa 0,213; IC 95% 0,094 - 0,332), o para puntos de corte > 1,5, > 1 o > 0,5 (kappa 0,213, 0,255, 0,257, respectivamente). Conclusiones: Nuestros resultados sugieren que existe un pobre acuerdo entre el resultado del APRI y la biopsia hepática en el diagnóstico de cirrosis en pacientes con EHA, por lo tanto, no se debe utilizar como método diagnóstico único para determinar la presencia de cirrosis.(AU)

Introduction and objectives: It has been proposed that non-invasive methods may replace liver biopsy for the diagnosis of tissue damage in patients with autoimmune liver disease (ALD). The aim of this study was to determine diagnostic performance and degree of concordance between the APRI index and liver biopsy for diagnosing cirrhosis in these patients. Material and methods: In a cohort of patients with ALD, the value of the APRI index and liver biopsy results were determined according to the METAVIR score. The AUC and the degree of concordance between an APRI value >2 and a METAVIR score of F4 were evaluated as markers of liver cirrhosis, through a kappa statistic. Results: In total, 70 patients (age 51 ± 13 years) were included. The most common autoimmune liver diseases were primary biliary cirrhosis (PBC) (40%), autoimmune hepatitis (AIH) (24.3%) and AIH-PBC overlap syndrome (32.9%). Cirrhosis was confirmed by biopsy in 16 patients (22.9%). 15 patients (21.4%) had an APRI index >2 (Cirrhosis) and only six met both criteria. The AUC of the APRI was 0.77 (95% CI 0.65-0.88). The degree of concordance between the tests was low for an APRI cut-off point >2 (kappa 0.213; 95% CI 0.094-0.332), as well as for cut-off points >1.5, >1 and >0.5 (kappa 0.213, 0.255, 0.257, respectively). Conclusion: Our results suggest that there is little concordance between APRI and liver biopsy for the diagnosis of cirrhosis in patients with ALD. It should therefore not be used as a single diagnostic method to determine cirrhosis.(AU)

Calidad de la biopsia hepática en 10 instituciones hospitalarias de Bogotá / Quality of liver biopsies taken in ten hospitals of Bogotá

Resumen Introducción: La biopsia hepática es la prueba de oro para el diagnóstico de las enfermedades que comprometen el hígado, una muestra adecuada y una muy buena lectura son elementos que determinan la utilidad de la prueba y el impacto en la toma de decisiones. Objetivo: Evaluar la calidad de las biopsias hepáticas a partir de la frecuencia de un diagnóstico definitivo en la lectura de las mismas y su relación con el número de espacios porta y su longitud informada. Materiales y métodos: Estudio observacional retrospectivo basado en registros, entre el 1 de enero de 2010 y el 30 de julio de 2017. Se realizó la revisión de las historias clínicas de los pacientes a quienes se les realizó biopsia hepática y se evaluó el resultado de la patología. Resultados: Se incluyeron 659 informes de patología de 10 instituciones. El porcentaje de reporte de espacios porta varió entre un 15 % y un 87,4 %, entre las instituciones. La mediana de longitud de la biopsia fue 15 mm (rango intercuartílico [RIC]: 10-20) con el valor más bajo de 1,3 (1-1,5) y el más alto de 1,8 (1,4-2) y la del número de espacios porta fue de 10 (RIC: 7-15), con el valor más bajo de 5 (1-8) y el más alto de 13 (10-17). Los diagnósticos definitivos se presentaron entre 35 % y 69 %, diagnósticos probables entre 25 % y 63 %, y sin diagnóstico entre un 5 % y 31,8 %. En el resultado de la regresión logística del diagnóstico y análisis univariado, se encontró que el número de espacios porta presentó un Odds ratio (OR) de 1,12 (intervalo de confianza [IC] 95 %: 1,05-1,19) y la longitud, OR: 1,74 (1,06-2,87); con el análisis multivariado, el número de espacios porta sigue siendo significativo (OR: 1,12 [1,02 a 1,22], p = 0,011). Conclusiones: En Bogotá existen 3 instituciones hospitalarias con adecuada calidad preanalítica en la toma de biopsias hepáticas y diagnósticos definitivos por encima del 60 %, asociados en esta serie con la presencia de un cilindro de tejido hepático de longitud y número de espacios porta adecuados. Con el análisis multivariado, el número de espacios porta presentó significancia. Se insiste en la importancia de la experiencia y entrenamiento del patólogo que evalúa la biopsia.

Abstract Introduction: Liver biopsy is the gold-standard test for the diagnosis of diseases involving the liver. An adequate sample and an accurate reading of the report are key to determine the usefulness of the test and its impact on decision-making. Objective: To assess the quality of liver biopsies based on the frequency of a "definitive diagnosis" in their report and their association with the number of portal spaces and reported length. Materials and methods: Record-based retrospective observational study, from January 1, 2010, to July 30, 2017. A review of the medical records of patients who underwent liver biopsy was performed, and the pathology result was evaluated. Results: 659 pathology reports from 10 hospitals were included. The percentage of portal space reporting varied between 15% and 87.4%. The median biopsy length was 15mm (IQR: 10-20) and the median number of portal spaces was 10 (IQR: 7-15). Definitive diagnoses were between 35% and 69%, probable diagnoses between 25% and 63%, and no diagnosis between 5% and 31.8%. The logistic regression of the diagnosis and a univariate analysis found that the number of portal spaces had an OR of 1.12 (95%CI: 1.05-1.19), while length had an OR of 1.74 (95%CI: 1.06-2.87). The multivariate analysis showed that the number of portal spaces is significant [OR: 1.12 (95%CI:1.02 to 1.22), p = 0.011]. Conclusions: In Bogotá, there are 3 hospitals with adequate pre-analytical quality of liver biopsies and definitive diagnoses above 60%, which in this series is associated with the presence of a cylinder of liver tissue of adequate length and the number of portal spaces. Multivariate analysis showed that the number of portal spaces is significant. The importance of the experience and training of the pathologist who evaluates the biopsy is stressed.

Ultrasound-guided percutaneous liver biopsy in children. Five-year experience at a tertiary care center. / Biopsia hepática percutánea guiada por ultrasonido en pediatría. Experiencia de 5 años en un centro de tercer nivel.

INTRODUCTION AND AIMS: Percutaneous liver biopsy with histopathologic analysis is a valuable tool for the diagnosis, prognosis, and treatment evaluation of liver diseases. Its ultrasound-guided performance is useful, making the procedure safer and reducing the risk for complications and hospital stay. Our aim was to describe the indications, histopathologic study, and complications associated with the performance of ultrasound-guided percutaneous liver biopsy in pediatric patients. MATERIAL AND METHODS: The study included 102 ultrasound-guided percutaneous liver biopsies performed on patients <16 years of age, within the time frame of January 2014 and December 2019. The information was obtained from electronic files and histopathologic studies and the data were analyzed through descriptive statistics. RESULTS: A total of 102 procedures were carried out on 99 patients. Mean patient age was 72 months and 58.8% of the patients were female. Over 65% of the indications for liver biopsy included autoimmune hepatitis (23.5%), elevated liver enzymes (21.5%), and chronic liver disease (20.5%). Four patients presented with immediate complications (3.9%), three of which were major (2.9%), concurring with that reported in the international literature. CONCLUSIONS: Our study corroborates the importance of ultrasound-guided liver biopsy in the diagnosis and follow-up of pediatric patients. The procedure also had a low complication rate of only 3.9%.

Concordance between hepatic biopsy and the APRI index (Ast to Platelet Ratio Index) for the diagnosis of cirrhosis in patients with autoimmune liver disease. / Concordancia entre el resultado de la biopsia hepática y el índice APRI (Ast to Platelet Ratio Index) en el diagnóstico de cirrosis en pacientes con enfermedad hepática autoinmune.

INTRODUCTION AND OBJECTIVES: It has been proposed that non-invasive methods may replace liver biopsy for the diagnosis of tissue damage in patients with autoimmune liver disease (ALD). The aim of this study was to determine diagnostic performance and degree of concordance between the APRI index and liver biopsy for diagnosing cirrhosis in these patients. MATERIAL AND METHODS: In a cohort of patients with ALD, the value of the APRI index and liver biopsy results were determined according to the METAVIR score. The AUC and the degree of concordance between an APRI value >2 and a METAVIR score of F4 were evaluated as markers of liver cirrhosis, through a kappa statistic. RESULTS: In total, 70 patients (age 51 ± 13 years) were included. The most common autoimmune liver diseases were primary biliary cirrhosis (PBC) (40%), autoimmune hepatitis (AIH) (24.3%) and AIH-PBC overlap syndrome (32.9%). Cirrhosis was confirmed by biopsy in 16 patients (22.9%). 15 patients (21.4%) had an APRI index >2 (Cirrhosis) and only six met both criteria. The AUC of the APRI was 0.77 (95% CI 0.65-0.88). The degree of concordance between the tests was low for an APRI cut-off point >2 (kappa 0.213; 95% CI 0.094-0.332), as well as for cut-off points >1.5, >1 and >0.5 (kappa 0.213, 0.255, 0.257, respectively) CONCLUSION: Our results suggest that there is little concordance between APRI and liver biopsy for the diagnosis of cirrhosis in patients with ALD. It should therefore not be used as a single diagnostic method to determine cirrhosis.

Markers of hepatic fibrosis. / Marcadores de fibrosis hepática.

Chronic liver diseases constitute a major health problem. Chronic liver inflammation, defined by the degree of hepatic fibrosis, is asymptomatic in a significant percentage of patients; hence, the disease often remains undiagnosed until it has reached very advanced phases and, frequently, when the damage is irreversible. Ideally, patients should be screened during the initial phases of chronic inflammation, thus allowing for the effective management of the natural evolution of the disease by stopping or delaying its course. Standard diagnostic methods (transaminase determination or abdominal ultrasonography) do not allow for the early diagnosis of the degree of fibrosis. A liver biopsy is the invasive method of choice to screen for fibrosis, however, due to its limitations, non-invasive diagnostic methods such as elastography or serological markers are increasingly used as a good alternative for the early diagnosis of the degree of fibrosis.

Algoritmos útiles para el diagnóstico histopatológico de la enfermedad hepática con base en los patrones de daño hepático / Useful Algorithms for Histopathological Diagnosis of Liver Disease Based on Patterns of Liver Damage

En los anteriores artículos, se revisaron las patologías hepáticas más frecuentes desde el punto de vista morfológico y la importancia de una adecuada correlación, para lograr un mejor entendimiento entre clínicos y patólogos. El ejercicio que se hará a continuación, se basa en establecer una aproximación al diagnóstico histopatológico de algunas de las patologías hepáticas a las que se les realiza biopsias con mayor frecuencia, teniendo en cuenta algoritmos basados en patrones de daño hepático

Previous articles have reviewed the most frequent liver pathologies from the morphological point of view and looked at the importance of adequate correlation for obtaining better understanding between clinicians and pathologists. The next exercise is directed toward histopathological diagnosis of some of the liver diseases for which biopsies are performed most frequently. It takes into account algorithms based on patterns of liver damage

Post-reperfusion liver biopsy and its value in predicting mortality and graft dysfunction after liver transplantation / Biópsia hepática pós-reperfusão e sua utilização na predição da mortalidade e da disfunção hepática pós-transplante

ABSTRACT Background: The outcome of the patients after liver transplant is complex and to characterize the risk for complications is not always easy. In this context, the hepatic post-reperfusion biopsy is capable of portraying alterations of prognostic importance. Aim: To compare the results of liver transplantation, correlating the different histologic features of the hepatic post-reperfusion biopsy with graft dysfunction, primary non-function and patient survival in the first year after transplantation. Method: From the 377 transplants performed from 1996 to 2008, 164 patients were selected. Medical records were reviewed and the following clinical outcomes were registered: mortality in 1, 3, 6 and 12 months, graft dysfunction in varied degrees and primary graft non-function. The post-reperfusion biopsies had been examined by a blinded pathologist for the outcomes. The following histological variables had been evaluated: ischemic alterations, congestion, steatosis, neutrophilic exudate, monomorphonuclear infiltrate and necrosis. Results: The variables associated with increased mortality were: steatosis (p=0.02209), monomorphonuclear infiltrate (p=0.03935) and necrosis (p<0.00001). The neutrophilic exudate reduced mortality in this study (p=0.00659). The primary non-function showed significant association (p<0.05) with the necrosis, steatosis and the monomorphonuclear infiltrate. Conclusion: Post-reperfusion biopsy is useful tool to foresee complications after liver transplant.

RESUMO Racional: A evolução dos pacientes após transplante hepático é complexa e caracterizar o risco para complicações nem sempre é fácil. Nesse contexto, a biópsia hepática pós-reperfusão é capaz de retratar alterações de importância prognóstica. Objetivo: Avaliar os resultados no primeiro ano após transplante hepático, correlacionando as alterações histológicas à biópsia hepática pós-reperfusão com a sobrevida, a disfunção e o não-funcionamento primário do enxerto. Método: Dos 377 transplantes ocorridos de 1996 a 2008, 164 pacientes foram selecionados para estudo. Os seguintes desfechos clínicos foram registrados: mortalidade em 1, 3, 6 e 12 meses, disfunção do enxerto em graus variados e o não-funcionamento primário do enxerto. As biópsias pós-reperfusão foram examinadas por um patologista sem conhecimento dos resultados. As seguintes variáveis histológicas foram avaliadas: alterações isquêmicas, congestão, esteatose, exsudato neutrofílico, infiltrado monomorfonuclear e necrose. Resultados: As variáveis associadas com aumento da mortalidade foram: esteatose (p=0.02209), infiltrado monomorfonuclear (p=0.03935) e necrose (p<0.00001). O infiltrado neutrofílico reduziu a mortalidade neste estudo (p=0.00659). O não-funcionamento primário do enxerto mostrou associação significativa (p<0.05) com a necrose, a esteatose e com o infiltrado monomorfonuclear. Conclusão: A biópsia hepática pós-reperfusão é ferramenta útil em prever complicações após o transplante hepático.

Patología del trasplante hepático, aspectos más relevantes del período postrasplante tardío / Most Relevant Pathology Issues in the Late Post Liver Transplant Period

La sobrevida de los pacientes postrasplante hepático supera el 90% al año y el 75% a los 5 años. Entender las causas de pérdida del injerto, o inclusive la muerte del paciente, es esencial para mejorar aún más los resultados a largo plazo. La evaluación de las biopsias hepáticas tiene un papel importante en la explicación y manejo de la disfunción del injerto de hígado, que ocurre después del primer año del trasplante. La interpretación de estas biopsias puede ser muy difícil, en especial por la alta incidencia de enfermedades recurrentes que pueden mostrar un cuadro clínico y unas características histopatológicas que semejan varias condiciones, especialmente cuando el rechazo agudo o crónico pueden sobreponerse a una patología ya existente o presentarse de manera simultánea y contribuir a la disfunción tardía del injerto, por lo que el análisis de la biopsia puede ayudar a determinar el componente principal de la lesión. Es indispensable la correlación clínico patológica, teniendo en cuenta la enfermedad original, el tipo de inmunosupresión, las pruebas de función hepática, las serologías virales, los autoanticuerpos y los hallazgos radiológicos. En este artículo comentaré las patologías más frecuentes y las que causan más problemas en su diagnóstico durante el período postrasplante tardío

One year survival rates of liver transplant patients exceed 90% while five year survival rates exceed 75%. Understanding the causes of graft losses and patient deaths is essential for further improvement of long-term results. Evaluation of liver biopsies has an important role in explaining liver graft dysfunction that occurs more than one year after transplantation, and thus is key for post-transplant patient management. The interpretation of these biopsies can be very difficult especially because of the high incidence of recurrent diseases that sometimes have clinical and histopathological features that resemble various other conditions. This is especially true for acute and chronic rejection which can overwhelm an existing condition and which can develop simultaneously with other conditions that contribute to late graft dysfunction. Analysis of the biopsy can help determine the main component of a lesion. Clinical findings must be correlated to pathological findings, and the correlation must take into account the original disease, the type of immunosuppression, liver function tests, viral serology, autoantibodies and radiological findings. In this article I will discuss the most common diseases and those that cause the most problems for diagnosis during the late post-transplant period