RESUMEN
BACKGROUNDS: Radiofrequency ablation (RFA) is known to destroy tumoral tissue and activate immune cells. This study aimed to investigate the impact of RFA on peripheral T-cell responses and its relationship with tumor origin and hepatitis status. METHODS: A retrospective analysis was conducted on 62 patients with various types of tumors, including hepatocellular carcinoma, colorectal cancer, lung cancer, breast cancer, and others, who underwent RFA treatment between June 2017 and December 2018. Blood samples were collected before and one day after RFA treatment. The peripheral T-cell subsets were measured by flow cytometry, and their changes were analyzed. RESULTS: The study found a decrease in the CD4+CD8-and CD4-CD8+ T-cell subsets after RFA, but no significant changes were observed in the populations of CD4+CD8+ and the CD4+CD8-/CD4-CD8+ ratio. Furthermore, no significant differences were observed in peripheral T-cell subsets concerning tumor type or hepatitis status. CONCLUSIONS: The study suggests that RFA treatment may have a short-term impact on peripheral T-cell responses, characterized by a decrease in certain T-cell subsets. However, these changes do not seem to be related to the tumor type or hepatitis status of the patients.
Asunto(s)
Carcinoma Hepatocelular , Ablación por Catéter , Hepatitis , Neoplasias Hepáticas , Ablación por Radiofrecuencia , Humanos , Estudios Retrospectivos , Subgrupos de Linfocitos T , Carcinoma Hepatocelular/cirugía , Neoplasias Hepáticas/cirugía , Hepatitis/cirugíaRESUMEN
BACKGROUND: Since January 2022, there has been an increase in reports of cases of acute hepatitis of unknown cause in children. Although cases have been reported across multiple continents, most have been reported in the United Kingdom. Investigations are ongoing to identify the causative agent or agents. METHODS: We conducted a retrospective study involving children referred to a single pediatric liver-transplantation center in the United Kingdom between January 1 and April 11, 2022. These children were 10 years of age or younger and had hepatitis that met the case definition of the U.K. Health Security Agency for confirmed acute hepatitis that was not hepatitis A through E and did not have a metabolic, inherited or genetic, congenital, or mechanical cause, in the context of a serum aminotransferase level greater than 500 IU per liter. We reviewed medical records and documented demographic characteristics, clinical features, and results of liver biochemical, serologic, and molecular tests for hepatotropic and other viruses, as well as radiologic and clinical outcomes. The outcomes were classified as an improving condition, liver transplantation, or death. RESULTS: A total of 44 children had hepatitis that met the confirmed case definition, and most were previously healthy. The median age was 4 years (range, 1 to 7). Common presenting features were jaundice (in 93% of the children), vomiting (in 54%), and diarrhea (in 32%). Among the 30 patients who underwent molecular testing for human adenovirus, 27 (90%) were positive. Fulminant liver failure developed in 6 patients (14%), all of whom received a liver transplant. None of the patients died. All the children, including the 6 who received liver transplants, were discharged home. CONCLUSIONS: In this series involving 44 young children with acute hepatitis of uncertain cause, human adenovirus was isolated in most of the children, but its role in the pathogenesis of this illness has not been established.
Asunto(s)
Hepatitis , Fallo Hepático Agudo , Trasplante de Hígado , Enfermedad Aguda , Niño , Preescolar , Hepatitis/etiología , Hepatitis/cirugía , Humanos , Lactante , Fallo Hepático Agudo/etiología , Fallo Hepático Agudo/cirugía , Trasplante de Hígado/efectos adversos , Estudios RetrospectivosRESUMEN
Patients with hepatitis-associated aplastic anemia (HAA) who undergo living-donor liver transplantation (LDLT) have a poor prognosis with infections and bleeding complications. Rapid recovery of blood cells is critical for preventing these complications and improving the outcome. Immunosuppressive therapy (IST) combined with thrombopoietin receptor agonists is considered effective for aplastic anemia. However, there are no data on the benefits of adding thrombopoietin receptor agonists to IST for HAA. We present the case of a child with severe HAA who underwent LDLT, and who achieved rapid blood cell recovery with IST combined with romiplostim, a thrombopoietin receptor agonist. In addition, despite having undergone LDLT, the patient had no adverse events such as serious liver dysfunction or thrombosis. This case suggests that IST combined with thrombopoietin receptor agonists may be a promising treatment option for HAA patients undergoing LDLT.
Asunto(s)
Anemia Aplásica/tratamiento farmacológico , Anemia Aplásica/etiología , Hepatitis/complicaciones , Hepatitis/cirugía , Trasplante de Hígado , Receptores Fc/administración & dosificación , Proteínas Recombinantes de Fusión/administración & dosificación , Trombopoyetina/administración & dosificación , Anemia Aplásica/sangre , Recuento de Células Sanguíneas , Niño , Manejo de la Enfermedad , Susceptibilidad a Enfermedades , Femenino , Hepatitis/diagnóstico , Humanos , Inmunosupresores/administración & dosificación , Inmunosupresores/uso terapéutico , Trasplante de Hígado/efectos adversos , Trasplante de Hígado/métodos , Resultado del TratamientoRESUMEN
Hepatitis-associated aplastic anemia (HAAA) has been reported in 23% to 33% of patients who received orthotopic liver transplantation (LT) for acute liver disease of unknown origin (nonviral hepatitis). In this situation, hematopoietic stem cell transplantation (HSCT) might be a curative option. Here the authors report on 6 patients who received HSCT after LT for nonviral HAAA hepatitis. The outcomes were interpreted in the context of recently reported immune suppressive therapy (IST) outcomes in 8 patients with HAAA and to HSCT outcomes in patients with HAAA who recovered from hepatitis without undergoing LT. All patients transplanted by using HLA-identical sibling donors (3 of 6) were alive and had normal liver function and hematopoiesis without graft versus host disease. Both patients receiving bone marrow from a matched unrelated donor (MUD) experienced extensive graft versus host disease that was fatal for one patient. Thereby, the authors conclude that HSCT can be considered as a first-choice treatment for this category of patients when HLA-identical donors are available. When no HLA-identical donor is available, IST should be applied as HSCT with other donor sources might be reserved for IST nonresponders or poor responders.
Asunto(s)
Anemia Aplásica/terapia , Trasplante de Células Madre Hematopoyéticas/métodos , Hepatitis/complicaciones , Trasplante de Hígado/efectos adversos , Adolescente , Adulto , Anemia Aplásica/etiología , Anemia Aplásica/patología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Hepatitis/cirugía , Humanos , Masculino , Pronóstico , Estudios Retrospectivos , Sociedades MédicasRESUMEN
BACKGROUND: Good syndrome is a rare condition, manifesting as immunodeficiency due to hypogammaglobulinemia associated with thymoma. Herein, we present a patient with Good syndrome whose thymoma was resected after treatment of cytomegalovirus hepatitis. CASE PRESENTATION: The patient was a 45-year-old woman presenting with fever, cough, and nasal discharge, and was diagnosed with thymoma and hypogammaglobulinemia. She subsequently developed cytomegalovirus hepatitis that was treated by immunoglobulin. After resolution of the hepatitis, she underwent thymectomy through a left anterior thoracotomy. Her postoperative course was uneventful, and while receiving ongoing immunoglobulin therapy, she has been doing well without signs of infection. CONCLUSIONS: Management of infections is important for patients with Good syndrome. To minimize the risk of perioperative infection, we should take care while planning the surgical approach and procedure.
Asunto(s)
Agammaglobulinemia/complicaciones , Infecciones por Citomegalovirus/complicaciones , Enfermedades de Inmunodeficiencia Primaria/complicaciones , Timoma/complicaciones , Timoma/cirugía , Neoplasias del Timo/complicaciones , Neoplasias del Timo/cirugía , Agammaglobulinemia/terapia , Comorbilidad , Citomegalovirus , Femenino , Hepatitis/complicaciones , Hepatitis/cirugía , Humanos , Persona de Mediana Edad , Enfermedades de Inmunodeficiencia Primaria/terapia , TimectomíaRESUMEN
El síndrome de Fitz-Hugh-Curtis (FHCS) es la inflamación de la cápsula hepática sin afectación del parénquima asociada a una enfermedad pélvica inflamatoria. Hay muy pocos casos descritos en varones. El síntoma característico es el dolor abdominal en el cuadrante superior derecho, que hace que se confunda el cuadro con una enfermedad de la vía biliar. Son características las adherencias fuertes entre el diafragma y el hígado en forma de «cuerda de violín¼. Presentamos el caso de un varón de 81 años que se somete a una colecistectomía laparoscópica por pancreatitis de repetición. Durante la cirugía se encuentran las características adherencias en «cuerda de violín¼, que se seccionan. El paciente da positivo para anticuerpos contra Chlamydia trachomatis. Hay nueve casos descritos en la literatura de FHCS en varones. Este síndrome se confunde muchas veces con patología infecciosa biliar, lo que nos obliga a someter al paciente a una cirugía para realizar el diagnóstico cuando se encuentran las características adherencias. Si sospechamos la enfermedad mediante las pruebas complementarias, podemos intentar tratarla con antibióticos.Fitz-Hugh-Curtis syndrome (FHCS) is the inflammation of the hepatic capsule without affecting the parenchyma, which is associated with a pelvic inflammatory disease. There have been very few cases in men. The main symptom is abdominal pain in the right upper quadrant, which can be confused with a bile duct disorder. Strong violin string-like adhesions between the diaphragm and the liver are characteristic. In the study concerned, it is reported the case of an 81 year-old man who undergoes a laparoscopic cholecystectomy for recurrent pancreatitis. During surgery, the typical violin string-like adhesions are found and sectioned. The patient tests positive for Chlamydia trachomatis antibodies. Only nine cases in men have been reported in FHCS literature. This syndrome is frequently confused with infectious biliary tract disease, so the patient should undergo a surgery to diagnose when the characteristic adhesions are found. If the disease is suspected by the additional tests, it can be treated with antibiotics.
Asunto(s)
Infecciones por Chlamydia/cirugía , Hepatitis/cirugía , Enfermedad Inflamatoria Pélvica/cirugía , Peritonitis/cirugía , Dolor Abdominal/etiología , Anciano de 80 o más Años , Anticuerpos Antibacterianos/sangre , Infecciones por Chlamydia/complicaciones , Infecciones por Chlamydia/microbiología , Chlamydia trachomatis/inmunología , Colecistectomía Laparoscópica , Hepatitis/microbiología , Humanos , Masculino , Pancreatitis/complicaciones , Enfermedad Inflamatoria Pélvica/microbiología , Peritonitis/microbiología , RecurrenciaRESUMEN
Planar xanthomas in children represent rare dermatologic findings associated with abnormalities in lipid metabolism. While planar xanthomas in Alagille's syndrome have been well described in the literature, there have been no cases reported of eruptive xanthomas in pediatric liver transplant patients. Herein we report a case of a 16-month-old boy status post-liver transplantation who presents with planar xanthomas secondary to cholangiopathy. A brief review of xanthomas and the related literature is also provided.
Asunto(s)
Síndrome de Alagille/diagnóstico , Colestasis/complicaciones , Hiperlipidemias/etiología , Trasplante de Hígado/efectos adversos , Xantomatosis/etiología , Síndrome de Alagille/complicaciones , Pie , Mano , Hepatitis/cirugía , Humanos , Lactante , Lípidos/sangre , MasculinoRESUMEN
Peroxiredoxins (Prxs) belong to the superfamily of thiol-dependent peroxidases, and remove reactive oxygen species (ROS) and other oxidative stress products. The expression and activity of Prxs can be substantially affected by stimuli from the microenvironment, and in turn regulate cytokine secretion in the context of inflammation in both peroxidase-dependent and -independent pathways. Prxs translocate to mitochondria and are hyperoxidized during acute liver damage, and attenuate intracellular ROS accumulation through their peroxidase activity. In particularly, Prx1 modulates the microenvironment in liver injuries by reducing adhesion molecule expression in vascular endothelial cells and inhibiting the inflammatory response and adhesion of macrophages. Prxs have potent prosurvival effects against ROS in ischemic/reperfusion (I/R) injury, but Prxs released from necrotic cells increase secretion of inflammatory cytokines by macrophages through TLR2 and 4 activation, which promotes cell death. Prxs can be used as biomarkers to evaluate I/R injury and predict graft survival in liver transplantation. Prxs are modulated in various types of chronic hepatitis and hepatosteatosis, and mediate disease progression. Alcohol administration increases oxidization and inactivation of Prxs in mice because of oxidative stress. In conclusion, Prxs are essential mediators and biomarkers in inflammatory liver diseases and I/R injury.
Asunto(s)
Hepatitis/fisiopatología , Trasplante de Hígado , Peroxirredoxinas/fisiología , Daño por Reperfusión/fisiopatología , Animales , Biomarcadores/metabolismo , Adhesión Celular , Citocinas/metabolismo , Supervivencia de Injerto , Hepatitis/cirugía , Humanos , Mediadores de Inflamación/metabolismo , Macrófagos/citología , Mitocondrias Hepáticas/metabolismo , Peroxirredoxinas/metabolismo , Transporte de Proteínas , Especies Reactivas de Oxígeno/metabolismo , Receptor Toll-Like 2/metabolismo , Receptor Toll-Like 4/metabolismoRESUMEN
BACKGROUND: Acute liver failure is a potentially fatal disease of various etiologies for which liver transplantation is the only known curative treatment. Although the decision-making on transplantation is largely dependent on the severity of liver injury (based on predicting a fatal outcome), a statistical analysis to predict "survival" has not been extensively conducted. In this study, we investigate the medical history of patients in two distinct areas of Japan with the aim of identifying the predictors of survival in patients with acute liver injury (ALI). METHODS: Datasets of 301 patients with ALI in two distinct areas (93 in southern Kyushu and 208 in northern Tohoku) of Japan, who were treated from 2004 to 2014, were included in the analysis. RESULTS: Among the enrolled 301 cases, 263 patients survived without transplantation. A PT-INR of ≥ 1.3 during the clinical course was found to be adequate for predicting a poor prognosis, because all of the fatal cases emerged from this population (hazard ratios: southern Kyushu, 0.2827; northern Tohoku, 0.1862). All surviving patients showed a reduction in their PT-INR during treatment, whereas the PT-INR did not decrease in the patients with a poor prognosis. A PT-INR of < 1.3 on days 7 and 8 efficiently predicted transplant-free survival (log-rank test: southern Kyushu, P = 0.0030; northern Tohoku, P = 0.0022). CONCLUSIONS: A PT-INR of ≥ 1.3 during the clinical course might identify cases with a poor prognosis, while the recovery of the PT-INR to < 1.3 predicts transplant-free survival.
Asunto(s)
Hepatitis/mortalidad , Relación Normalizada Internacional , Fallo Hepático Agudo/mortalidad , Trasplante de Hígado , Tiempo de Protrombina , Enfermedad Aguda , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Niño , Toma de Decisiones Clínicas , Femenino , Hepatitis/complicaciones , Hepatitis/cirugía , Hospitales Universitarios , Humanos , Japón , Estimación de Kaplan-Meier , Fallo Hepático Agudo/etiología , Fallo Hepático Agudo/cirugía , Masculino , Persona de Mediana Edad , Pronóstico , Curva ROC , Tasa de Supervivencia , Adulto JovenRESUMEN
To explore the effects of propofol post-conditioning (PPC) on hepatic ischaemia/reperfusion injury (HIRI) and the potential mechanisms that might be involved in the interaction of Brahma-related gene1(BRG1) and Nuclear-related factor 2(Nrf2). Patients were randomized into PPC(n = 16) and non-PPC(NPC)( n = 21) groups. Propofol(2 mg/kg) was infused within 10 min. of the onset of liver reperfusion during liver transplantation in the PPC group. Liver function tests, as well as Brg1, Nrf2, Heme oxygenase-1(HO-1) and NADPH:quinone oxidoreductase1(NQO1) expression levels were evaluated. CMV-Brg1 mice were designed to investigate the role of Brg1 overexpression during HIRI. Brg1 and Nrf2 siRNA were used to examine the relationship between Brg1 and Nrf2/HO-1 pathways in propofol-mediated effects in a human hepatocyte(L02) hypoxia/reoxygenation(H/R) model. In patients, PPC attenuated both donor liver pathological and function injury, and reducing oxidative stress markers, compared to the NPC group, 24 hrs after surgery. PPC increased liver Brg1, Nrf2, HO-1 and NQO1 expression. In mice, PPC reduced HIRI by decreasing liver oxidative stress and activating Nrf2/HO-1 pathway, accompanied by up-regulation of BRG1 expression. BRG1 overexpression activated Nrf2/HO-1 transcription in CMV-BRG1 mice during HIRI. In vitro, PPC significantly elevated expression of Nrf2, HO-1 and NQO1, resulting in a reduction of cell DCFH-DA and 8-isoprostane levels and decreased lactate dehydrogenase levels, leading to an overall increase in cell viability. Moreover, the protective effects of propofol were partially abrogated in Nrf2-knock-down or BRG1-knock-down hepatocytes. Nrf2-knock-down drastically reduced protein expression of HO-1 and NQO1, while Brg1-knock-down decreased HO-1 expression. Propofol post-conditioning alleviates HIRI through BRG1-mediated Nrf2/HO-1 transcriptional activation.
Asunto(s)
Antioxidantes/uso terapéutico , ADN Helicasas/genética , Hemo-Oxigenasa 1/genética , Trasplante de Hígado/métodos , Factor 2 Relacionado con NF-E2/genética , Proteínas Nucleares/genética , Propofol/uso terapéutico , Daño por Reperfusión/prevención & control , Factores de Transcripción/genética , Adolescente , Adulto , Anciano , Animales , Línea Celular , ADN Helicasas/metabolismo , Reposicionamiento de Medicamentos , Femenino , Regulación de la Expresión Génica , Hemo-Oxigenasa 1/metabolismo , Hepatitis/metabolismo , Hepatitis/patología , Hepatitis/cirugía , Hepatocitos/efectos de los fármacos , Hepatocitos/metabolismo , Hepatocitos/patología , Humanos , Hipnóticos y Sedantes/uso terapéutico , Hígado/metabolismo , Hígado/patología , Hígado/cirugía , Neoplasias Hepáticas/metabolismo , Neoplasias Hepáticas/patología , Neoplasias Hepáticas/cirugía , Masculino , Ratones , Ratones Endogámicos C57BL , Persona de Mediana Edad , Factor 2 Relacionado con NF-E2/metabolismo , Proteínas Nucleares/metabolismo , Estrés Oxidativo/efectos de los fármacos , Estudios Prospectivos , Daño por Reperfusión/genética , Daño por Reperfusión/metabolismo , Daño por Reperfusión/patología , Factores de Transcripción/metabolismoRESUMEN
BACKGROUND & AIMS: Acute liver failure after ingestion of toxic mushrooms is a significant medical problem. Most exposures to toxic mushrooms produce no symptoms or only mild gastroenteritis, but some lead to severe hepatic necrosis and fulminant hepatic failure requiring liver transplantation. We aimed to assess mortality from mushroom poisoning and identify variables associated with survival and liver transplantation. METHODS: We collected information from 27 patients (13 male; median age, 47 years) admitted to the emergency department within 24 hours of ingesting wild mushrooms. They developed severe liver injury (serum levels of transaminases greater than 400 IU/L) and were treated with activated charcoal and N-acetylcysteine at a tertiary medical center in San Francisco, California from January 1997 through December 2014. Viral hepatitis, autoimmune liver disease, acetaminophen, salicylate toxicity, and chronic liver diseases were ruled out for all patients. We analyzed patient demographics, time since ingestion, presenting symptoms, laboratory values, and therapies administered. A good outcome was defined as survival without need for liver transplant. A poor outcome was defined as death or liver transplant. Positive predictive values were calculated, and the χ2 test was used to analyze dichotomous variables. RESULTS: Liver injury was attributed to ingestion of Amanita phalloides in 24 patients and Amanita ocreata in 3 patients. Twenty-four of the patients ingested mushrooms with meals and 3 patients for hallucinogenic purpose. At 24-48 hours after ingestion, all patients had serum levels of alanine aminotransferase ranging from 554 to 4546 IU/L (median, 2185 IU/L). Acute renal impairment developed in 5 patients. Twenty-three patients survived without liver transplantation, and 4 patients had poor outcomes (1 woman underwent liver transplantation on day 20 after mushroom ingestion, and 3 women died of hepatic failure). Of the 23 patients with peak levels of total bilirubin of 2 mg/dL or more during hospitalization, only 4 had a poor outcome. Peak serum level of aspartate aminotransferase less than 4000 IU/L, peak international normalized ratio less than 2, and a value of serum factor V greater than 30% identified patients with good outcomes with 100% positive predictive value; if these peak values were used as a cutoff, 10 of 27 patients (37%), 7 of 27 patients (26%), and 6 of 12 patients (50%), respectively, could have avoided transfer to a transplant center. CONCLUSIONS: In an analysis of 27 patients with hepatocellular damage due to mushroom (Amanita) poisoning and peak levels of total bilirubin greater than 2 mg/dL, the probability of liver transplantation or death is 17%, fulfilling Hy's law. Patients with peak levels of aspartate aminotransferase less than 4000 IU/L can be monitored in a local hospital, whereas patients with higher levels should be transferred to liver transplant centers. Women and older patients were more likely to have a poor outcome than men and younger patients.
Asunto(s)
Hepatitis/complicaciones , Hepatitis/patología , Fallo Hepático/mortalidad , Intoxicación por Setas/mortalidad , Intoxicación por Setas/patología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Hepatitis/etiología , Hepatitis/cirugía , Humanos , Fallo Hepático/etiología , Trasplante de Hígado , Masculino , Persona de Mediana Edad , Intoxicación por Setas/complicaciones , Estudios Retrospectivos , San Francisco , Análisis de Supervivencia , Resultado del Tratamiento , Adulto JovenRESUMEN
This report describes the case of an 85-year-old man who underwent left hemicolectomy for descending colon cancer and hepatic segmentectomy for metastatic liver cancer. Seven years after the liver surgery, an abdominal CT scan revealed a tumor in the remnant liver. He also presented with colon diverticula in the ascending colon, which was located close to the liver tumor. With a preoperative diagnosis of metastatic liver cancer, the tumor was resected with S8 segmentectomy. Histopathological examination of the resected tumor revealed a hepatic inflammatory pseudotumor, not metastatic liver cancer. The present case showed the difficulty of preoperatively diagnosing inflammatory pseudotumors. Furthermore, it is noted in the case that since the liver tumor was located close to the colon diverticula in the ascending colon, the diverticula was potentially associated with the formation of hepatic inflammatory pseudotumor.
Asunto(s)
Colon Ascendente/cirugía , Neoplasias del Colon/cirugía , Diagnóstico Diferencial , Divertículo del Colon/complicaciones , Hepatitis/diagnóstico , Neoplasias Hepáticas/diagnóstico , Anciano de 80 o más Años , Colon Ascendente/patología , Hepatectomía , Hepatitis/cirugía , Humanos , Neoplasias Hepáticas/secundario , MasculinoAsunto(s)
Albúminas/administración & dosificación , Hepatectomía , Hepatitis/terapia , Fallo Hepático Agudo/terapia , Trasplante de Hígado , Diálisis Renal/métodos , Adulto , Femenino , Hepatitis/complicaciones , Hepatitis/cirugía , Humanos , Fallo Hepático Agudo/complicaciones , Masculino , Persona de Mediana Edad , Insuficiencia Multiorgánica/prevención & controlRESUMEN
A content of a biliary ways microflora and its correlation with clinical form and severity of inflammatory-destructive processes in hepatopancreatobiliary zone were studied up. The investigation objective was the optimization of a treatment--diagnostic tactics for the complications and remote recurrences rate reduction in a complicated biliary calculous disease. There was established, that a standard bacteriological investigation do not give a possibility to estimate some causes of a biliary ducts affection, a chlamidial infection in particular. So on, for the individual antibacterial therapy choosing a more detailed and modern examination of patients is needed.
Asunto(s)
Anticuerpos Antibacterianos/sangre , Infecciones por Chlamydia/diagnóstico , Chlamydia/aislamiento & purificación , Colecistitis/diagnóstico , Hepatitis/diagnóstico , Pancreatitis/diagnóstico , Enfermedad Inflamatoria Pélvica/diagnóstico , Peritonitis/diagnóstico , Adulto , Antibacterianos/uso terapéutico , Sistema Biliar/microbiología , Sistema Biliar/patología , Procedimientos Quirúrgicos del Sistema Biliar , Chlamydia/crecimiento & desarrollo , Chlamydia/patogenicidad , Infecciones por Chlamydia/tratamiento farmacológico , Infecciones por Chlamydia/microbiología , Infecciones por Chlamydia/cirugía , Colecistitis/tratamiento farmacológico , Colecistitis/microbiología , Colecistitis/cirugía , Diagnóstico Diferencial , Femenino , Hepatitis/tratamiento farmacológico , Hepatitis/microbiología , Hepatitis/cirugía , Humanos , Inmunoglobulina G/sangre , Inmunoglobulina M/sangre , Hígado/microbiología , Hígado/patología , Masculino , Persona de Mediana Edad , Páncreas/microbiología , Páncreas/patología , Páncreas/cirugía , Pancreatitis/tratamiento farmacológico , Pancreatitis/microbiología , Pancreatitis/cirugía , Enfermedad Inflamatoria Pélvica/tratamiento farmacológico , Enfermedad Inflamatoria Pélvica/microbiología , Enfermedad Inflamatoria Pélvica/cirugía , Peritonitis/tratamiento farmacológico , Peritonitis/microbiología , Peritonitis/cirugía , Índice de Severidad de la EnfermedadRESUMEN
Alcohol-related liver disease is the second most frequent indication for liver transplantation (LT), yet as many as 90% to 95% of patients with alcohol-related end-stage liver disease are never formally evaluated for LT. Furthermore, despite its significance as a cause of chronic liver disease and indication for LT, it has received little attention in recent years for several reasons, including the good posttransplant short-term results, and the lack of specific "drugs" used for this disease. A writing group, endorsed by the International Liver Transplant Society, was convened to write guidelines on Liver Transplantation for Alcoholic Liver Disease to summarize current knowledge and provide answers to controversial and delicate ethical as well as clinical problems. We report here a short version of the guidelines (long version available at www.ilts.org) with the final recommendations graded for level of evidence. The writing group membership is expected to remain active for 5 years, reviewing the guideline annually, and updating the online version when appropriate.
Asunto(s)
Hepatopatías Alcohólicas/cirugía , Trasplante de Hígado , Abstinencia de Alcohol , Consumo de Bebidas Alcohólicas/efectos adversos , Comorbilidad , Hepatitis/cirugía , Humanos , Terapia de Inmunosupresión , Hepatopatías Alcohólicas/mortalidad , Hepatopatías Alcohólicas/psicología , Guías de Práctica Clínica como Asunto , Calidad de Vida , Resultado del TratamientoRESUMEN
BACKGROUND: There are a lot of different causes of abdominal pain; in this case, a young woman suffers from three diseases with similar symptoms. Adult intestinal mal-rotation is a rare condition of deviation from the normal 270° counter clockwise rotation of the midgut resulting in, not only mal-position of the small intestine, but also mal-fixation of the mesentery. Fitz-Hugh-Curtis syndrome is a rare complication of pelvic inflammatory disease; it involves liver capsule inflammation associated with genital tract infection, which is usually caused by Neisseria gonorrhoea and Chlamydia trachomatis. Neuroendocrine tumors are enterochromaffin cell neoplasms that arise from cells of the endocrine (hormonal) and nervous systems; the appendicular one is the most common primary malignant lesion of these tumors, it's incidence is about 0.3 - 0.9% of appendectomies done. Just for knowledge, this is the first described case of concomitant presence of all these diseases with clinical symptoms attributable to each one. CASE PRESENTATION: 40-years-old woman suffers from acute abdominal pain, predominantly on the right quadrants, without abdominal distension, no guarding nor rigidity and normal intestinal peristalsis. She has a long history of abdominal intermittent pain, with cramps every 30-40 min, resolving spontaneously. She was diagnosed as intestinal mal-rotation through computed tomography scan which has evidenced a mobilized intra--peritoneal duodenum with cecum/ascending colon predominately lying on the left side and the small intestine almost entirely lying on the right side of abdomen, without evidence of effusion, edema or signs of intestinal ischemia or infarction. Exploratory laparoscopy demonstrated an inflammatory process in the hepatic-renal space, with bloody adhesions above the liver capsule; this is additional to the typical pelvic inflammatory disease signs (Fitz-Hugh-Curtis syndrome). Appendectomy was performed with histological analysis resulting in appendicular neuroendocrine tumor. CONCLUSIONS: Although the patient has an intestinal mal-rotation which could explain the abdominal painful symptoms, it is not possible to exclude other concomitant causes, such as perihepatitis on pelvic inflammatory disease or neuroendocrine tumors. Even if all these diseases are rarely seen in daily clinical practice, they should be considered in the differential diagnosis of chronic intermittent abdominal pain in a young woman.
Asunto(s)
Dolor Abdominal/complicaciones , Infecciones por Chlamydia/complicaciones , Dolor Crónico/etiología , Anomalías del Sistema Digestivo/complicaciones , Hepatitis/complicaciones , Vólvulo Intestinal/complicaciones , Tumores Neuroendocrinos/complicaciones , Enfermedad Inflamatoria Pélvica/complicaciones , Peritonitis/complicaciones , Dolor Abdominal/cirugía , Adulto , Neoplasias del Apéndice/cirugía , Infecciones por Chlamydia/cirugía , Dolor Crónico/cirugía , Anomalías del Sistema Digestivo/cirugía , Femenino , Hepatitis/cirugía , Humanos , Vólvulo Intestinal/cirugía , Tumores Neuroendocrinos/cirugía , Enfermedad Inflamatoria Pélvica/cirugía , Peritonitis/cirugía , Adherencias Tisulares/complicacionesAsunto(s)
Aneurisma Falso/diagnóstico , Arteria Hepática/patología , Hepatitis/cirugía , Hepatopatías/cirugía , Trasplante de Hígado/efectos adversos , Hemorragia Posoperatoria/diagnóstico , Adulto , Oclusión con Balón/efectos adversos , Diagnóstico Diferencial , Humanos , Donadores Vivos , Masculino , Hemorragia Posoperatoria/patología , Trombosis/etiologíaRESUMEN
Biliary atresia (BA) is a severe chronic cholestasis disorder of infants that leads to death if not treated on time. Neonatal hepatitis syndrome (NHS) is another leading cause of neonatal cholestasis confounding the diagnosis of BA. Recent studies indicate that altered bile acid metabolism is closely associated with liver injury and cholestasis. In this study, we systematically measured the bile acid metabolome in plasma of BA, NHS, and healthy controls. Liver bile acids were also measured using biopsy samples from 48 BA and 16 NHS infants undergoing operative cholangiography as well as 5 normal adjacent nontumor liver tissues taken from hepatoblastoma patients as controls. Both BA and NHS samples had significantly elevated bile acid levels in plasma compared to normal controls. BA patients showed a distinct bile acid profile characterized by the higher taurochenodeoxycholic acid (TCDCA) level and lower chenodeoxycholic acid (CDCA) level than those in NHS patients. The ratio of TCDCA to CDCA in plasma was significantly higher in BA compared to healthy infants (p < 0.001) or NHS (p < 0.001). The area under receiver operating characteristic curve for TCDCA/CDCA to differentiate BA from NHS was 0.923 (95% CI: 0.862-0.984). These findings were supported by significantly altered expression levels of bile acid transporters and nuclear receptors in liver including farnesoid X receptor (FXR), small heterodimer partner (SHP), bile salt export pump (BSEP), and multidrug resistant protein 3 (MDR3) in BA compared to NHS. Taken together, the plasma bile acid profiles are distinct in BA, NHS, and normal infants, as characterized by the ratio of TCDCA/CDCA differentially distributed among the three groups of infants.
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Ácidos y Sales Biliares/sangre , Atresia Biliar/diagnóstico , Ácido Quenodesoxicólico/sangre , Colestasis/diagnóstico , Hepatitis/diagnóstico , Ácido Tauroquenodesoxicólico/sangre , Subfamilia B de Transportador de Casetes de Unión a ATP/sangre , Subfamilia B de Transportador de Casetes de Unión a ATP/genética , Miembro 11 de la Subfamilia B de Transportador de Casetes de Unión al ATP , Transportadoras de Casetes de Unión a ATP/sangre , Transportadoras de Casetes de Unión a ATP/genética , Alanina Transaminasa/sangre , Alanina Transaminasa/genética , Área Bajo la Curva , Aspartato Aminotransferasas/sangre , Aspartato Aminotransferasas/genética , Ácidos y Sales Biliares/clasificación , Atresia Biliar/sangre , Atresia Biliar/patología , Atresia Biliar/cirugía , Estudios de Casos y Controles , Colangiografía , Colestasis/sangre , Colestasis/patología , Colestasis/cirugía , Femenino , Regulación de la Expresión Génica , Hepatitis/sangre , Hepatitis/patología , Hepatitis/cirugía , Humanos , Lactante , Recién Nacido , Masculino , Metaboloma , Receptores Citoplasmáticos y Nucleares/sangre , Receptores Citoplasmáticos y Nucleares/genética , gamma-Glutamiltransferasa/sangre , gamma-Glutamiltransferasa/genéticaRESUMEN
BACKGROUND: Wolcott-Rallison syndrome (WRS) is caused by recessive EIF2AK3 mutations and characterized by early-onset diabetes and skeletal dysplasia. Hepatic dysfunction has been reported in 60% of patients. AIMS: To describe a cohort of WRS patients and discuss the pattern and management of their liver disease. METHODS: Detailed phenotyping and direct sequencing of EIF2AK3 gene were conducted in all patients. RESULTS: Twenty-eight genetically confirmed patients (67% male; mean age 4.6 years) were identified. 17 different EIF2AK3 mutations were detected, of which 2 were novel. The p.S991N mutation was associated with prolonged survival and p.I650T with delayed onset. All patients presented before 25 months with diabetes with variation in the frequency and severity of 10 other features. Liver disease, first manifested as non-autoimmune hepatitis, was the commonest extra-pancreatic feature identified in 85.7% (24/28). 22/24 had at least one episode of acute hepatic failure which was the cause of death in all deceased patients (13/28). One child was treated by liver transplantation and had no liver disease and better diabetes control for the following 6 years. CONCLUSIONS: Liver disease in WRS is more frequent than previously described and carries high mortality. The first experience with liver transplantation in WRS is encouraging.
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Diabetes Mellitus Tipo 1 , Epífisis/anomalías , Hepatitis , Fallo Hepático , Trasplante de Hígado , Osteocondrodisplasias , eIF-2 Quinasa/genética , Preescolar , Estudios de Cohortes , Comorbilidad , Diabetes Mellitus Tipo 1/genética , Diabetes Mellitus Tipo 1/mortalidad , Diabetes Mellitus Tipo 1/cirugía , Epífisis/cirugía , Femenino , Hepatitis/genética , Hepatitis/mortalidad , Hepatitis/cirugía , Humanos , Fallo Hepático/genética , Fallo Hepático/mortalidad , Fallo Hepático/cirugía , Masculino , Mutación , Osteocondrodisplasias/genética , Osteocondrodisplasias/mortalidad , Osteocondrodisplasias/cirugíaRESUMEN
INTRODUCTION: Co-infected HIV and hepatitis subjects are candidates for a liver transplantation because of progressive liver disease. Chronic liver disease, co-infected or not, requires assessment of respiratory function before liver transplantation. The respiratory evaluation of these 2 groups compared with healthy individuals can define deficits, and this can impair a full recovery after transplant surgery. OBJECTIVE: This study sought to compare the respiratory profile in co-infected patients with chronic liver disease who are candidates for liver transplantation with that of healthy subjects. METHODS: Through respiratory evaluation of flows and lung volumes (spirometry), muscle activity (surface electromyography), and maximum pressure (manovacuometer), 250 people were distributed into 3 groups: 14 patients with HIV and liver disease, 65 healthy subjects, and 171 patients with chronic liver disease. The mean age (years) was respectively 47.5 ± 6.2, 48.3 ± 14.1, and 52.9 ± 8.5. The average body mass index (kg/m(2)) of the groups was 24.6 ± 4.5, 26.0 ± 3.2, and 28.5 ± 5.3, respectively. RESULTS: There was a statistical difference among the groups in the root means square (RMS) rectus abdominis (µV) (P = .0016), RMS diaphragm (µV) (P = .0001), maximal inspiratory pressure (cmH2O) (P = .001), forced exhaled volume at the end of first second (%) (P = .002), and maximal mid expiratory flow 25% to 75% (%) (P = .0001) for the Kruskal-Wallis test. The multivariate analysis among the groups showed that the RMS diaphragm had a tendency to discriminate the co-infected subjects. CONCLUSIONS: The co-infected HIV group showed a muscle deficit of diaphragm and rectus abdominis activity, and the liver disease group showed lower indexes in volumes and respiratory flows.