Evolución de los pacientes con enfermedad hepática crítica / Prognosis of patients with critical hepatic disease

INTRODUCTION: The acute liver failure on chronic (ACLF), is an entity, whose recognition is increasing. The ACLF and CLIF OF indexes have been recently presented with the objective of predicting mortality in this kind of patients. MATERIAL AND METHODS: All patients admitted to the Ramón y Cajal University Hospital diagnosed of acute liver failure on chronic during 2016 and 2017 were collected. We collect the scores: SOFA, CLIF, APACHE II, SAPS II and ACLF score in patients admitted to the ICU by comparing them with each other and define which stages have worse prognosis. RESULTS: A total of 46 patients were collected. The study presents an intra ICU mortality of 31% (15/46) and a six-month mortality of 59.6% (28/46). Patients classified as death, present ACLF values ​​at admission (49.5 vs 60 p = 0.001), and at three days (46.66 vs 59.4 p = 0.001) higher than survivors. In the analysis of the ROC curve, the area under the curve in relation to six-month mortality is higher in the ACLF index (0.8) compared to the MELD (0.69) SOFA (0.66) SAPS II (0.69) or APACHE II (0.65). Patients with ACLF indexes above 65 had an intra UCI mortality of 54%, however, mortality at 6 months is 90%. Patients with ACLF values ​​greater than 65 present mean values ​​of lactic acid, leukocytes, INR or bilirubin higher than those under 65 in a statistically significant manner. CONCLUSIONS: The data presented in this study suggest that the ACLF index works as an adequate predictor of intra-ICU mortality and at 6 months.

INTRODUCCIÓN: El fallo hepático agudo sobre crónico es una entidad cuyo reconocimiento va en aumento. Los índices ACLF y CLIF OF, han sido presentados recientemente con el objetivo de predecir la mortalidad en este tipo de enfermos. MATERIAL Y MÉTODOS: Se recogen todos los pacientes ingresados en una unidad de cuidados intensivos (UCI) de un hospital terciario universitario, diagnosticados de fallo hepático agudo sobre crónico durante 2016 y 2017. Recogemos los índices SOFA, CLIF, APACHE II, SAPS II Y ACLF en pacientes ingresados en UCI comparándolos entre sí. Definimos que estadios presentan peor pronóstico. RESULTADOS: Se analizan un total de 46 pacientes. El estudio presenta una mortalidad intra-UCI del 31% (15/46) y una mortalidad a los seis meses de 59,6% (28/46). Los pacientes clasificados como éxitus presentan valores ACLF al ingreso (49,5 vs 60 p = 0,001), a los tres días (46,66 vs 59,4 p = 0,001) superiores a los supervivientes. En el análisis de la curva COR, el área bajo la curva en relación a la mortalidad a los seis meses, es superior en el índice ACLF (0,8) en comparación con el MELD (0,69) SOFA (0,66) SAPS II (0,69) o APACHE II (0,65). Los pacientes con índices ACLF superiores a 65 presentaban una mortalidad intra-UCI del 54% sin embargo, la mortalidad a los 6 meses es del 90%. Los pacientes con valores ACLF superiores a 65 presentan a su vez valores medios de láctico, leucocitos, INR o bilirrubina mayores de forma estadísticamente significativa. CONCLUSIONES: Los datos presentados en este estudio sugieren que el índice ACLF funciona como un adecuado predictor de mortalidad intra-UCI y a los 6 meses.

Hepatic Venous Pressure Gradient Predicts Long-Term Mortality in Patients with Decompensated Cirrhosis

PURPOSE: The present study aimed to investigate the role of hepatic venous pressure gradient (HVPG) for prediction of long-term mortality in patients with decompensated cirrhosis. MATERIALS AND METHODS: Clinical data from 97 non-critically-ill cirrhotic patients with HVPG measurements were retrospectively and consecutively collected between 2009 and 2012. Patients were classified according to clinical stages and presence of ascites. The prognostic accuracy of HVPG for death, survival curves, and hazard ratios were analyzed. RESULTS: During a median follow-up of 24 (interquartile range, 13-36) months, 22 patients (22.7%) died. The area under the receiver operating characteristics curves of HVPG for predicting 1-year, 2-year, and overall mortality were 0.801, 0.737, and 0.687, respectively (all p17 mm Hg, respectively (p=0.015). In the ascites group, the mortality rates at 1 and 2 years were 3.9% and 17.6% with HVPG 17 mm Hg, respectively (p=0.044). Regarding the risk factors for mortality, both HVPG and model for end-stage liver disease were positively related with long-term mortality in all patients. Particularly, for the patients with ascites, both prothrombin time and HVPG were independent risk factors for predicting poor outcomes. CONCLUSION: HVPG is useful for predicting the long-term mortality in patients with decompensated cirrhosis, especially in the presence of ascites.

Anti-Rods and Rings Autoantibodies in a Patient With Hepatitis C Virus Infection

No abstract available.

Acute-on-chronic liver failure

Acute-on-chronic liver failure (ACLF) is an increasingly recognized distinct disease entity encompassing an acute deterioration of liver function in patients with chronic liver disease. Although there are no widely accepted diagnostic criteria for ACLF, the Asia.Pacific Association for the Study of the Liver (APASL) and the American Association for the Study of Liver Disease and the European Association for the Study of the Liver (AASLD/EASL) consensus definitions are commonly used. It is obvious that the APASL and the AASLD/EASL definitions are based on fundamentally different features. Two different definitions in two different parts of the world hamper the comparability of studies. Recently, the EASL-Chronic Liver Failure Consortium proposed new diagnostic criteria for ACLF based on analyses of patients with organ failure. There are areas of uncertainty in defining ACLF, such as heterogeneity of ACLF, ambiguity in qualifying underlying liver disease, argument for infection or sepsis as a precipitating event, etc. Although the exact pathogenesis of ACLF remains to be elucidated, alteration of host response to injury, infection, and unregulated inflammation play important roles. The predisposition, infection/inflammation, response, organ failure (PIRO) concept used for sepsis might be useful in describing the pathophysiology and clinical categories for ACLF. Treatment strategies are limited to organ support but better understanding of the pathophysiology is likely to lead to discovery of novel biomarkers and therapeutic strategies in the future.

Comparison of the Model for End-stage Liver Disease and hepatic venous pressure gradient for predicting survival in patients with decompensated liver cirrhosis / 대한간학회지

BACKGROUND/AIMS: This study compared the prognostic values of the Model for End-stage Liver Disease (MELD) and the hepatic venous pressure gradient (HVPG) in the prediction of death within 3 and 12 months in patients with decompensated liver cirrhosis. METHODS: We used data from 136 consecutive patients with decompensated cirrhosis who underwent HVPG between January 2006 and June 2008. Cox regression analysis was used to investigate the independent relationships with death of MELD and HVPG. The prognostic accuracies of MELD and HVPG were analyzed by calculating the area under the receiver operating characteristic curve (AUROC) for the occurrence of death within 3 and 12 months. RESULTS: Both MELD and HVPG were independent predictors of death [hazard ratio (HR)=1.11 and 1.12, respectively; 95% confidence interval (CI)=1.04~1.20 and 1.08-1.16]. Analysis of the AUROC demonstrated that the prognostic power did not differ between MELD and HVPG for predicting the 3-month survival (HR=0.76 and 0.68, respectively; 95% CI=0.62~0.89 and 0.52~0.84; P=0.22) or the 12-month survival (HR=0.72 and 0.73, 95% CI=0.61~0.83 and CI=0.61~0.84). CONCLUSIONS: Both MELD and HVPG are independent prognostic factors of death within 3 and 12 months in patients with decompensated liver cirrhosis, and their accuracies are similar. However, HVPG has a limited role in the prediction of death in decompensated cirrhosis due to its invasiveness and limited use.

Hemocromatosis Neonatal: una causa de fallo hepático in utero: presentación de dos casos y revisión de la bibliografía / Neonatal Hemochromatosis: a cause of liver failure in utero: report of two cases and review of the literature

La hemocromatosis neonatal es una entidad clínico patológicaque presenta fallo hepático grave, se inicia in útero, asociada a siderosis intrahepática y extrahepática, que respeta el sistema reticulo endotelial.Su causa se desconoce pero se debería a un manejo anormal del hierro fetoplacentario con enfermedad hepática perinatal, relación genética familiar o sería consecuencia de una enfermedad gestacional aloinmunitaria. Es un síndrome con características comunes más que una simple entidad patológica, con transmisión materna y alta recurrencia en la misma progenie. La muerte se produceen los primeros días o semanas de vida por fallomultiorgánico. Presentamos dos recién nacidos con hemocromatosis neonatal. El primero falleció por fallo multiorgánico y el segundo permitió el trasplante hepático. Desde 1993, se emplea un cocktail antioxidantequelantedel hierro, junto al tratamiento de soporteestándar del fallo hepático, pero su uso es polémico. En 2002, una comunicación preliminar sugiere que el tratamiento con inmunoglobulinas endovenosas en la segunda mitad de la gestación, en mujeres con el antecedente de un hijo con hemocromatosis neonatal probada, previene las formas recurrentes letales de la enfermedad. Este trastorno se debe sospechar en todo fallo hepático grave al nacer, acompañado de valores séricos de ferritina elevados; pero debe confirmarse por un aumento de los depósitos hepáticos de hierro y siderosis extrahepática demostrables por resonancia magnética nuclear, en la biopsia de glándulas salivales o en la autopsia. La hemocromatosis neonatal es la indicación específicamás frecuente de trasplante hepático en los primeros 3 meses de vida, que parecería ser el tratamiento de elección por considerar desde el momentoque el soporte médico, incluida la terapia antioxidante-quelante del hierro, sea inefectivo, antes de que surjan complicaciones neurológicas irreversibles

Neonatal hemochromatosis is a rare clinical pathologic entity, defined by severe neonatal liver failure of intrauterine onset associated with intra-and extra hepatic siderosis that spares reticuloendothelial system. It is the most frequently recognized cause of liver failure in neonates. The cause is unknown but it may develop secondary to abnormal fetoplacental iron handling or perinatal liver disease or be familial or as a consequence of gestational alloimmune disease. It’s a syndrome with a common feature rather than a single pathologic entity, with maternal transmission and a high recurrence in the sibship. Death from multisystem organ failure usually occurs in the first few days or weeks of life. We report two newborn with neonatal hemochromatosis. The first died for multiorgan failure, despite aggressive support. The second underwent to liver transplantation. Since 1993, an antioxidant-chelator cocktail has been used in addition to standard supportive care, but this remains controversial. By 2002, a preliminary report suggested that treatment with weekly intravenous immunoglobulin during the later half of pregnancy, for woman whose most recent gestation was affected with proven NH. The diagnosis is suspected in the presence of severely impaired hepatic synthetic function accompanied by high serum ferritin levels, but is confirmed only by demonstration of increased hepatic iron stores, and extra-hepatic siderosis shown by autopsy or in vivo, which can be achieved by biopsy of the minor salivary glands or magnetic resonance imaging. Neonatal hemochromatosis is the most common specific indication for liver transplantation in the first three months of life and appears to be the treatment of choice, and must as well be considered as soon as it becomes apparent that medical support, which should include chelation-antioxidant treatment, is ineffective, before irreversible neurological complications appear.

Capecitabine and Oxaliplatin (XELOX) for the Treatment of Patients with Metastatic Gastric Cancer and Severe Liver Dysfunction

Gastric cancer patients with severe liver dysfunction secondary to hepatic metastases have limited treatment options. Most cytotoxic drugs have a narrow therapeutic index. Although both capecitabine and oxaliplatin have been well tolerated as single agents for patients with severe hepatic dysfunction, the combination of these drugs has not been investigated. We report here on a case of successful treatment of a patient suffering with severe liver dysfunction and metastatic gastric cancer; the patient was treated with a combination of capecitabine and oxaliplatin (XELOX). The initial bilirubin level of the patient was 10.9 mg/dL. After two cycles of treatment, his bilirubin level decreased to 2.1 mg/dL. He has experienced an excellent radiological response and he has received six cycles of XELOX chemotherapy. XELOX chemotherapy is feasible and it can be associated with positive outcomes for the patients suffering with metastatic gastric cancer and severe liver dysfunction.

A pilot study of polyunsaturated phosphatidyl choline in fulminant and subacute hepatic failure.

Present pilot study was conducted to evaluate efficacy and safety of polyunsaturated phosphatidyl choline (PPC) in a phase III clinical trial in patients of fulminant and subacute hepatic failure over one year period in a prospective randomised blinded controlled design. We found that in patients of fulminant hepatic failure, recovery period from encephalopathy was faster and mortality rate lower in the test group of patients who received PPC in a dose of 350 mg thrice daily for 6 to 8 weeks as compared to the control groups who did not receive it. In the patients of subacute hepatic failure, recovery from encephalopathy was faster, mortality rate lower and regression of ascites was significantly higher (P = 0.0022) in test group of patients who received PPC as compared to the control group. However, as the number of patients in the present pilot study is small, we propose that larger clinical trials are warranted in this direction to prove the efficacy and safety of PPC in fulminant and subacute hepatic failure.

experience of fulminant hepatic failure in Lahore

To critically evaluate the prevalence, clinical presentation, associated factors, diagnosis and prognosis of Fulminant Hepatic Failure in a hospital setting. Design: A prospective study of patients with fulminant hepatic failure and hepatocellular jaundice. Setting: Department of Medicine, Mayo Hospital/K.E. Medical College, Lahore-Pakistan. Subjects: Nine hundred ninety eight consecutive patients with hepatocellular jaundice admitted to Mayo Hospital from 1989-92. Out of a total of 998, 30 patients [3%] had acute fulminant hepatic failure. Majority of these patients were young females [24/30] with F: M ratio 4: 1 and most of these were pregnant in their third trimester common cause was viral hepatitis followed by drug induced hepatic injury. Twenty five [83.33%] of the patients died within one week of admission and 5 [16.67%] who survived were those who had relatively prolonged incubation period. Acute Fulminant Hepatic Failure [FHF] is rare but very serious complication of Acute Viral hepatitis [AVH]. Liver transplantation is the treatment of choice. Future treatment possibilities are discussed

Aetiology and prognostic factors in hepatic failure in central India.

The present study was undertaken to establish the aetiology and prognostic factors of liver failure in central India. Of the 122 cases of hepatic failure 95 (78%), 19 (15.5%) and 8 (6.5%) were labelled as fulminant hepatic failure (FHF), chronic hepatic failure (CHF) and subacute hepatic failure (SAHF) respectively. Hepatitis E virus (HEV) and hepatitis B virus (HBV) were aetiological agents amongst 41% (n = 39) and 37% (n = 35) patients with FHF respectively. Mixed infection among such cases even though observed was infrequent and 15% (n = 14) of FHF did not have any serological markers. They were presumed to be due to non A-E viral infection. Thirty-one (33%) of the FHF patients were pregnant and 29 (94%) of them were due to HEV. Amongst patients with SAHF and CHF, HBV and HCV were important aetiological agents. The static prognostic risk factors noted in the present study are age above 40 years, presence of identifiable viral aetiology (A to E), alcoholic status in males and pregnancy particularly in the third trimester or postpartum state. Among the dynamic factors, bilirubin level above 20 mg/dl and prothrombin time over 20 seconds appeared to be the risk factors.

Serum ammonia level in hepatic encephalopathy a clinical study of 35 cases

1. To determine the diagnostic significance of Serum Ammonia in Hepatic encephalopathy. 2. To correlate serum Ammonia level with clinical severity of hepatic encephalopathy. Design: A descriptive study of 23 patients of either sex between ages 13-80 years. Settings: Medical Unit IV, Civil Hospital Karachi. Subjects and Methodology: Patients suffering from hepatic encephalopathy and having an underlying acute of chronic liver disease. The diagnosis was made on history, clinical findings and laboratory parameters. Serum Ammonia was estimated by sigma method [N range 17-80 ug/dl]. Main outcome measures: Hyperammonemia in relation to level of consciousness and its impact on final outcome of treatment. Majority of our patients, [82.85%] had hyperammonemia. Patients with chronic liver disease, with ascites and with hypoalbuminemia had higher levels. No age related effect was noted but females had higher serum ammonia levels. However S. Ammonia level did not correlate well with the severity and final outcome of encephalopathy. 1. Serum Ammonia estimation is helpful in the diagnosis of hepatic encephalopathy and differential diagnosis of unexplained coma. 2. It's level had no correlation with clinical severity of hepatic encephalopathy

Fulminant hepatitis in Kala-azar.

One hundred and fifty cases of Kala-azar were studied for evidence of hepatic involvement. The hepatic function was mildly affected in 25 cases and 3 cases had fulminate hepatitis. Most of the cases were cured after anti-Kala-azar therapy except 2 cases, who died of hepatic failure. This study suggests that fulminant hepatitis may be the outcome of Kala-azar, itself.

Hepatic manifestations in typhoid fever.

Thirty one children with typhoid fever aged 2 months to 12 years and blood culture positive for multidrug resistant S. typhi were prospectively studied for their hepatic functions at the time of hospitalization and 2-3 weeks after completion of antibiotic therapy. Hepatic manifestations included hepatomegaly (51.6%); jaundice (16.1%); raised levels of serum glutamic oxaloacetic transaminase (SGOT) (61.3%), serum glutamic pyruvic transaminase (SGPT) (48.4%), alkaline phosphatase (AP) (22.6%) and serum bilirubin (SB) (6.1%); reduced levels of serum albumin (SA) (41.9%); prolonged prothrombin time (PT) (9.7%) and abnormal ultrasound abdomen (19.3%). Hepatic dysfunction was a notable feature even in those cases without hepatomegaly, with raised levels of SGOT (60%), SGPT (40%), AP (20%), SB (6.7%), decreased SA (53.3%) and prolonged PT (6.7%). There was no correlation between the degree of hepatic enlargement or hyperbilirubinemia with abnormalities in liver functions. Hepatic dysfunction was noticed to be transient, as all these parameters returned to normal within 2-3 weeks after successful antibiotic therapy.