The Rate of Hepatic Fibrosis Progression in Patients Post-Fontan.

This investigation analyzed the rate of hepatic fibrosis progression in post-Fontan patients that underwent hepatic biopsy. The study cohort comprised post-Fontan patients that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2019. We identified 126 patients that met inclusion criteria. Of the 126, 27 (21%) had a lateral tunnel Fontan, and 99 (79%) had an extracardiac Fontan. For the 27 lateral tunnel Fontan patients, age at Fontan was 4 ± 2 years, and for the 99 extracardiac Fontan patients age at Fontan was 4 ± 2 years (p = 0.98). For the 27 lateral tunnel Fontan patients, the average total fibrosis score was 3.0 ± 1.5; and for the 99 extracardiac Fontan patients, the average total fibrosis was 2.7 ± 1.7 (p = 0.48). For the lateral tunnel Fontan patients, the average Fontan duration was 20 ± 6 years; and for the 99 extracardiac Fontan patients, the average Fontan duration was 11 ± 5 years (p < 0.001). For the 27 lateral tunnel Fontan patients, the average rate of fibrosis progression was 0.16 ± 0.10 total fibrosis score/year; and for the 99 extracardiac Fontan patients, the average rate of fibrosis progression was 0.30 ± 0.23 total fibrosis score/year (p < 0.001). In conclusion, our findings suggest that those with extracardiac Fontans have a faster rate of hepatic fibrosis progression than those with lateral tunnel Fontans. More extensive or multi-institutional studies will be needed to confirm these findings and define the clinical significance of discrepant rates of hepatic fibrosis in post-Fontan patients.

Primary hepatic mucosa-associated lymphoid tissue lymphoma in a patient with no chronic liver disease: Case report.

Extramarginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) is a non-Hodgkin lymphoma of low-grade malignancy. The most common localization is the stomach, and the common nongastric sites are salivary glands, the skin, orbits, the conjunctiva, the lung, breasts, upper airways, other gastrointestinal sites, and the liver. Primary hepatic MALT lymphoma is a rare disease and the diagnostic can be challenging. The clinical presentation is nonspecific and may range from no symptoms to end-stage liver disease. The radiological aspect of hepatic lymphoma may indicate this diagnosis; however, the final diagnosis is made by hepatic biopsy. We report the case of a 47-year-old woman with no chronic liver disease, incidentally found with a focal liver mass at ultrasound examination. The only clinical symptom was fatigue. The blood tests were normal and tumoral markers were negative. Computed tomography and magnetic resonance imaging were performed. However, because the hepatic lesion was first described as a benign entity and, at second opinion, the suspicion of lymphoma was raised, the patient decided to undergo surgery first, without prior biopsy. The histopathologic analysis confirmed the diagnosis: hepatic MALT lymphoma positive for CD 20 and negative for CD 5, BCL6, cyclin D1, and CD 23. No lymph node involvement was noted and follow-up imaging with positron emission tomography-computed tomography did not show any other site of disease, thus confirming the diagnosis of primary hepatic MALT lymphoma. The aim of this paper was to highlight the imagistic features of primary hepatic lymphoma to contribute to the early diagnosis of this rare disease entity.

Minimally Invasive Hepatobiliary Surgery.

Comparative studies and large-scale case series that confirm the advantages of laparoscopy in children with hepatobiliary diseases are scarce, and the use of laparoscopy remains a matter of debate. This article reviews the current literature on the role of laparoscopic and robotic surgery in pediatric patients with choledochal cyst, biliary atresia, gallbladder diseases, and hepatobiliary malignancies. Studies were identified through a search of the MEDLINE database. Laparoscopy may be beneficial for resection of choledochal cyst and cholecystectomy. However, more data are required before recommendations on the use of minimally invasive techniques for other hepatobiliary conditions can be published.

Vitamin D deficiency is associated with severity of liver disease in HIV/HCV coinfected patients.

OBJECTIVE: To study the association of plasma 25-hydroxy vitamin D (25(OH)D) levels in HIV/HCV coinfected patients with severity of liver disease and virological response to hepatitis C virus (HCV) therapy with pegylated-interferon-alpha plus ribavirin (pegIFNα/RBV). METHODS: A cross-sectional study in 174 HIV/HCV coinfected patients that underwent a liver biopsy previously to start HCV therapy and a retrospective study of 125 of them. Plasma 25(OH)D levels were quantified by enzyme immunoassay. Liver biopsies were evaluated by METAVIR score. A sustained virological response (SVR) was defined as an undetectable serum HCV viral load (<10 IU/mL) up through 24 weeks after the end of HCV treatment. RESULTS: The median of plasma 25(OH)D level was 48 nmol/L (p25th: 32.5; p75th: 56.1) and 27 (15.5%) had 25(OH)D deficiency (<25 nmol/L). The percentage of 25(OH)D deficiency was higher in patients with significant fibrosis (F ≥ 2) (92.6% vs. 57.1%; p = 0.010) and moderate necroinflammatory activity grade (A ≥ 2) (85.2% vs. 60%; p = 0.043). However, adjusted logistic regression analyses showed that 25(OH)D deficiency was only associated with severity of liver disease [F ≥ 2 (OR = 8.47 (95% of confidence interval (CI) = 1.88; 38.3); p = 0.005) and A ≥ 2 (OR = 3.25 (95%CI = 1.06; 10.1); p = 0.040)]. Moreover, any significant relationship was found between 25(OH)D deficiency and SVR after HCV therapy. CONCLUSION: Plasma 25(OH)D deficiency was associated with liver disease severity in HIV/HCV coinfected patients, but it was not associated with HCV treatment failure.

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.

Hallazgos clínicos e histomorfológicos en pacientes con esteatosis hepática no alcohólica / Clinical and hystomorphological findings in patients with non alcoholic hepatic steatosis

Se realizó un estudio observacional y descriptivo de 32 pacientes con esteatosis hepática no alcohólica, diagnosticados en el Hospital Provincial Docente Clinicoquirúrgico "Saturnino Lora Torres" de Santiago de Cuba, de enero de 2011 a junio de 2013, a fin de caracterizarles epidemiológica, clínica e histomorfológicamente. En la investigación se obtuvo un incremento de la esteatosis hepática no alcohólica entre las hepatopatías crónicas, con predominio en personas de los grupos etarios de 35-44 y 45-54 años y del sexo masculino, y una mayor asociación a la hipertrigliceridemia y la hipertransaminasemia; igualmente, la dislipidemia y el sobrepeso -- según índice de masa corporal -- representaron los factores de riesgo más frecuentes en los afectados. No se encontró significación estadística al correlacionar los hallazgos ecográficos con los obtenidos por biopsia, de manera que quedó reafirmado que la laparoscopia constituye el medio de mayor sensibilidad y especificidad para diagnosticar la enfermedad en cuestión.

An observational and descriptive study of 32 patients with hepatic alcoholic steatosis, diagnosed in "Saturnino Lora Torres" Teaching Clinical Surgical Provincial Hospital in Santiago de Cuba was carried out from January, 2011 to June, 2013, in order to characterize them epidemiologically, clinically and hystomorphologically. In the investigation an increment of the non alcoholic hepatic steatosis was obtained among the chronic hepatopathies, with prevalence in people of the age groups 35-44 and 45-54 years and of the male sex, and a higher association to hypertriglyceridemia and hypertransaminasemia; equally, the dyslipidemia and overweight -- according to index of body mass -- represented the most frequent risk factors in those affected. There was no statistical significance when correlating the echographical with the biopsy findings, so that it was reaffirmed that laparoscopy constitutes the most sensitive and specific mean to diagnose the disease.

Histología de la biopsia hepática, enfoque para el clínico / Histopathology of the liver biopsy, clinical approach

La biopsia hepática es necesaria para la evaluación de la hepatitis crónica. Su utilidad reside en la valoración del grado de inflamación y fibrosis del paciente con hepatitis crónica. La utilización de un sistema semicuantitativo para la clasificación de las biopsias de hepatitis crónicas es necesaria con el fin de tener parámetros menos subjetivos, y para comparar la evolución ante una potencial terapia. Se establece una puntuación semicuantitativa que determina la graduación y el estadiaje. Se considera importante tener como mínimo para la valoración de una biopsia hepática, la presencia al menos de 3-5 espacios porta y realizar tinciones de rutina como hematoxilina eosina, tricromico, reticulita y orceina. Se considera básico para la definición de los tratamientos la actividad inflamatoria y el estadio de la fibrosis, apoyando a la parte clínica, bioquímica y de biología molecular del virus de hepatitis B.

The hepatic biopsy is necessary for the evaluation of chronic hepatitis. Its usefulness is relies on the assessment of the inflammation degree and fibrosis of the patient with chronic hepatitis. The use of a semi quantitative system for the classification of chronic hepatitis biopsies is necessary to keep less subjective parameters and to compare its evolution for a potential therapy. A semi quantitative score is established to determine the degree and staging. For the assessment of a hepatic biopsy, it is important to get at least 3-5 porta spaces and perform routine tinctions as hematoxylin-eosin, trichromic, reticulite and orcein. This is essential for the definition of treatments, inflammatory activity and fibrosis stage, supporting the clinical, biochemical, and molecular biology parts of the viral burden of the hepatitis B virus.

Hepatopatías: Hallazgos histológicos, Clínicos y Bioquímicos

Introducción: la biopsia hepática es uno de los parámetros a evaluar al determinar la etiología y la severidad de la enfermedad hepática. El objetivo general: conocer los datos epidemiológicos de la patología hepática de los pacientes que consultan al Servicio de Gastroenterología del hospital. Método: se realizo un estudio prospectivo con los pacientes que ingresaron a la consulta de hepatología, en el lapso comprendido entre marzo a octubre de 2004. Resultados: se estudió un total de 25 pacientes, doce hombres (48% ) y trece mujeres ( 52% ). Acorde al resultado de las biopsias el 44% se correspondieron a esteatosis hepática, 24% esteatohepatitis, 20% hepatitis crónica, 8% cirrosis y 4% fibrosis hepática. Sólo el 36% de los pacientes presentaban valores de AST por encima del su valor superior y el 56% de los pacientes tenían valores de ALT por encima de su valor normal, con una relación AST / ALT > 1. Conclusiones: 1.-El ecosonograma tiene una sensibilidad del 100% y una especificidad 75%. 2.- Los datos clínicos presentan una sensibilidad del 88% y una especificidad del 100%. 3.- El 44% de los pacientes tenían valores de aminotransferasas dentro del límite de la normalidad, con hallazgos patológicos a la biopsia hepática. Recomendaciones: se debe realizar una biopsia hepática a todos los pacientes con sospecha de hepatopatía, independiente de la alteración de la aminotransferasas.

Introduction: hepatic biopsy is necessary to evaluate the etiology and severity of hepatic diseases. General Objective: to know the epidemiologic facts of patients with hepatic disease attending our hospital. Methods: a prospective study was made with patients who attended the outpatient hepatology consultation at our hospital, from march to October of 2004. Results: We studied a total of 25 patients, twelve male (48%) and thirteen female (52%). According to the results of the hepatic biopsy 44% had Hepatic Steatosis, 24% steatohepatitis, 20% Chronic Hepatitis, 8% Cirrhosis and 4% Hepatic Fibrosis. Conclusion: 1.- Echosonography has a sensitivity of 100% and specificity of 75% 2.- The clinical data represent a sensibility of 88% and a specificity of 100% 3.- 44% of the patients, have normal aminotranferases, with abnormal histology findings at biopsy. Recommendations: We recommend a hepatic biopsy in all patients with suspicious of hepatic disease, regardless of aminotranferases values.