Preeclampsia de presentación grave asociada a hígado graso agudo del embarazo / Severe preeclampsia associated with acute fatty liver of pregnancy / Pré-eclâmpsia grave associada a fígado gorduroso agudo da gravidez

La preeclampsia se puede asociar a una patología poco frecuente como es el hígado graso agudo del embarazo. Se reporta el caso clínico de una paciente de 35 años, tercigesta, cursando embarazo gemelar que presenta preeclampsia con elementos de gravedad, asociada a hígado graso agudo del embarazo. Se realiza diagnóstico y tratamiento precoz de ambas patologías, presentando buena evolución materno-fetal.

Preeclampsia can be associated with acute fatty liver of pregnancy, a rare disease. This report describes the case of a 35-year-old patient, gravida 3, pregnant with twins, who presented with severe pre-eclampsia associated with acute fatty liver of pregnancy. Early diagnosis and treatment of both pathologies was performed, resulting in good maternal-fetal evolution.

A pré-eclâmpsia pode estar associada a uma patologia rara, como o fígado gorduroso agudo da gravidez. Neste relato, apresentamos uma paciente de 35 anos, terciária, em gestação gemelar, apresentando pré-eclâmpsia grave, associada a esteatose hepática aguda na gestação. É realizado diagnóstico e tratamento precoces de ambas as patologias, apresentando boa evolução materno-fetal.

Hepatitis aguda por virus E. Presentación de dos casos con evolución clínica diferente / Acute hepatitis due to E virus. Presentation of two cases with different clinical evolution

RESUMEN El virus de la hepatitis E tiene una amplia distribución a nivel mundial. Se presentaron dos casos clínicos en la provincia de Matanzas, con diagnóstico confirmado de hepatitis E mediante la determinación del ARN viral en heces fecales congeladas; a pesar de proceder de áreas de salud distantes, coincidieron en el mismo período de tiempo. El primero de ellos, una gestante asintomática diagnosticada fortuitamente a partir de elevación de enzimas hepáticas de citolisis. Evolucionó satisfactoriamente sin repercusión en su bienestar materno, trasmisión fetal, ni complicaciones perinatales. El segundo, una paciente portadora de síndrome metabólico, con evolución tórpida de su cuadro infeccioso viral, que la llevó a la insuficiencia hepática y a la muerte. Con estos casos se reflejó el amplio espectro de esta enfermedad en cuanto a formas clínicas de presentación y evolución. Se demostró que pueden ocurrir complicaciones en cualquier grupo poblacional, de ahí la importancia de considerarla en el diagnóstico diferencial de las enfermedades infecciosas hepáticas (AU).

ABSTRACT Hepatitis E virus is widely distributed around the world. Two clinical cases occurring in the province of Matanzas were presented, both with diagnosis of E hepatitis confirmed through viral RNA determination in frozen stool; although patients came from faraway health areas, they coincided in the same time period. The first patient, a pregnant asymptomatic woman, was incidentally diagnosed due to an increase of cytolysis liver enzymes. Her evolution was satisfactory without repercussion on maternal wellbeing, fetal transmission, nor perinatal complications. The second patient, a metabolic syndrome carrier, had torpid evolution of a viral infectious disease leading her to liver failure and death. These cases highlighted the wide range of this disease according to its clinical forms of presentation and evolution. It was showed that complications may occur in any population group, in consequence it is important to consider this disease when making the differential diagnosis of liver infectious diseases (AU).

[Concerns and preventive interventions in the treatment of pre-hepatic failure].

Liver failure is a rapidly progressive severe condition with very high mortality rate. Therefore, early warning and early intervention in the early stage of liver failure are essential to improve the prognosis of patients. Presently, the diagnostic criteria for pre-hepatic failure are not uniform and the studies are mainly focused on people with hepatitis B-related liver failure. This article discusses the current early diagnosis, warning, and treatment of pre-hepatic failure.

[Research progress of prewarning parameters of pre-hepatic failure].

Liver failure is a common clinical syndrome of severe liver disease, with high short-term mortality. Although there is currently no standardized and unified diagnostic criteria for pre-hepatic failure in the world, so the proposal of its concept is of great significance to further improve the prewarning of liver failure. This article reviews the prewarning parameters of the risk of liver failure related from the perspectives of etiologies, clinical laboratory tests and pathogenesis, and thereby aims to help clinicians improve their understanding of early diagnosis of liver failure and promote related research to further reduce the mortality rate of patients with liver failure.

[Advances in early warning and treatment of pre-hepatic failure].

Liver function of patients with pre-hepatic failure deteriorates rapidly, and with this there exists a risk of liver failure and high rates of mortality. This paper summarizes the concept of pre-hepatic failure, particularly the advances in early warning and treatment of pre-hepatic failure developing into hepatic failure, with a view to enhance clinicians' concerns to pre-hepatic failure for promoting the advancement of liver failure prevention and treatment, and improving the success rate of liver failure treatment.

Telehealth-Based Evaluation Identifies Patients Who Are Not Candidates for Liver Transplantation.

The burden of chronic liver disease has increased exponentially, driving more patients toward orthotopic liver transplant (OLT) evaluation.1 Because of limited access to transplant centers, patients often travel long distances to be evaluated for OLT.2 Liver transplantation in the VA system is offered at 6 Veterans Affairs transplant centers (VATCs) across the United States, including Richmond. To increase access to specialty care, the VA introduced the Specialty Care Access Network-Extension of Community Healthcare Outcomes (SCAN-ECHO) program,3,4 which was designed to transfer subspecialty knowledge to primary care physicians. In 2011, the Richmond VA introduced SCAN-ECHO for gastroenterology/hepatology providers to facilitate case-based distance learning combined with real-time consultation in hepatology, and the opportunity for an initial triage without completing a formal transplant evaluation. We studied the role of SCAN-ECHO in triaging OLT evaluations and the utility of this health care delivery in the field of transplantation.

Amatoxin-Containing Mushroom Poisonings: Species, Toxidromes, Treatments, and Outcomes.

Amatoxins are produced primarily by 3 species of mushrooms: Amanita, Lepiota, and Galerina. Because amatoxin poisonings are increasing, the objective of this review was to identify all amatoxin-containing mushroom species, present a toxidromic approach to earlier diagnoses, and compare the efficacies and outcomes of therapies. To meet these objectives, Internet search engines were queried with keywords to select peer-reviewed scientific articles on amatoxin-containing mushroom poisoning and management. Descriptive epidemiological analyses have documented that most mushroom poisonings are caused by unknown mushrooms, and most fatal mushroom poisonings are caused by amatoxin-containing mushrooms. Amanita species cause more fatal mushroom poisonings than other amatoxin-containing species, such as Galerina and Lepiota. Amanita phalloides is responsible for most fatalities, followed by Amanita virosa and Amanita verna. The most frequently reported fatal Lepiota ingestions are due to Lepiota brunneoincarnata, and the most frequently reported fatal Galerina species ingestions are due to Galerina marginata. With the exception of liver transplantation, the current treatment strategies for amatoxin poisoning are all supportive and have not been subjected to rigorous efficacy testing in randomized controlled trials. All patients with symptoms of late-appearing gastrointestinal toxicity with or without false recovery or quiescent periods preceding acute liver insufficiency should be referred to centers providing liver transplantation. Patients with amatoxin-induced acute liver insufficiency that does not progress to liver failure will have a more favorable survival profile with supportive care than patients with amatoxin-induced acute liver failure, about half of whom will require liver transplantation.

Optimizing the Management of Abnormal Liver Function Tests after Orthotopic Liver Transplant: A Systems-Based Analysis of Health Care Utilization.

Elevated liver function tests (eLFTs) are a major cause of unplanned readmissions (UR) after orthotopic liver transplantation. Diagnostic workup for eLFTs requires multiple invasive and noninvasive procedures, often done in the inpatient setting to expedite diagnosis, yet consequently resulting in increased costs. In this study, we evaluated eLFT readmissions at a single institution with respect to resource utilization. From 3/2013 to 12/2015, 388 patients underwent orthotopic liver transplantation, resulting in 463 UR totaling 5833 bed days; 87 (18.8%) UR and 929 (15.9%) bed days were for eLFTs. During eLFT-UR all patients underwent repeat laboratory testing, 75 (86.2%) liver ultrasound, 66 (75.8%) liver biopsy, and 17 (19.5%) endoscopic retrograde cholangiopancreatography. Discharge diagnoses were acute cellular rejection (40.2%), transaminitis not otherwise specified (17.2%), biliary complications (16.1%), recurrent hepatitis (11.5%), vascular complications (5.8%), viral hepatitis (5.8%), and steatohepatitis (3.5%). The greatest bed-day utilization was secondary to acute cellular rejection (60.8%) and biliary complications (13.7%). More than 35 per cent of eLFT-UR were due to transaminitis not otherwise specified, steatohepatitis, recurrent or viral hepatitis, none of which necessitate inpatient treatment. In addition, >25 per cent of eLFT-UR bed days were attributed to diagnostic workup. Identifying patients who can undergo expedited outpatient workup and require only outpatient management will result in significantly decreased readmissions, bed days, and hospital costs.

Virulence and resistance profiles of MRSA isolates in pre- and post-liver transplantation patients using microarray.

Methicillin-resistant Staphylococcus aureus (MRSA) screening plays a great role in preventing infections in surgical patients. This study aims to evaluate clonality, virulence and resistance of MRSA in pre- and post-liver transplantation (LT) patients. Nasal and groin swabs of 190 patients were collected. PCR for virulence genes and staphylococcal cassette chromosome mec (SCCmec) types, microarray, PFGE, multilocus sequence typing and MIC were performed. MRSA carriers were detected in 20.5 % (39/190) of the patients. However, only three colonized patients developed infections post-LT. Sixty-nine MRSA isolates were identified, and the most frequent SCCmec type was type II (29/69; 42.0 %). Most isolates (57/69; 82.6 %) were susceptible to trimethoprim-sulfamethoxazole (TMP/SMX) and harboured the lukD, lukE, clf and fnbA genes as determined by PCR. Five sequence types (ST) were identified among nine clones; 36.2 % (25/69) isolates belonged to a predominant clone (ST105 and SCCmec type II) that was susceptible to TMP/SMX, mupirocin and chlorhexidine, which had 87.9 % similarity with the New York/Japan clone. The array showed virulence difference in isolates of the same clone and patients and that colonized isolates (pre-LT patients) were less virulent than those post-LT and those infected. Therefore, despite the high frequency of MRSA colonization, infection due to MRSA was uncommon in our LT unit. MRSA isolates presented great diversity. Isolates of the same clone expressed different virulence factors by array. Colonizing isolates pre-LT expressed less virulent factors than post-LT and infecting isolates.

Impact of MELD Allocation System on Waiting List and Early Post-Liver Transplant Mortality.

BACKGROUND AND AIMS: MELD allocation system has changed the clinical consequences on waiting list (WL) for LT, but its impact on mortality has been seldom studied. We aimed to assess the ability of MELD and other prognostic scores to predict mortality after LT. METHODS: 301 consecutive patients enlisted for LT were included, and prioritized within WL by using the MELD-score according to: hepatic insufficiency (HI), refractory ascites (RA) and hepatocellular carcinoma (HCC). The analysis was performed to predict early mortality after LT (8 weeks). RESULTS: Patients were enlisted as HI (44.9%), RA (19.3%) and HCC (35.9%). The major aetiologies of liver disease were HCV (45.5%). Ninety-four patients (31.3%) were excluded from WL, with no differences among the three groups (p = 0.23). The remaining 207 patients (68.7%) underwent LT, being HI the most frequent indication (42.5%). HI patients had the shortest length within WL (113.6 days vs 215.8 and 308.9 respectively; p<0.001), but the highest early post-LT mortality rates (18.2% vs 6.8% and 6.7% respectively; p<0.001). The independent predictors of early post-LT mortality in the HI group were higher bilirubin (OR = 1.08; p = 0.038), increased iMELD (OR = 1.06; p = 0.046) and non-alcoholic cirrhosis (OR = 4.13; p = 0.017). Among the prognostic scores the iMELD had the best predictive accuracy (AUC = 0.66), which was strengthened in non-alcoholic cirrhosis (AUC = 0.77). CONCLUSION: Patients enlisted due to HI had the highest early post-LT mortality rates despite of the shortest length within WL. The iMELD had the best accuracy to predict early post-LT mortality in patients with HI, and thus it may benefit the WL management.

Lipid emulsions in the treatment and prevention of parenteral nutrition-associated liver disease in infants and children.

Long-term parenteral nutrition (PN) carries the risk of progressive liver disease in infants with intestinal failure. Although PN-associated liver disease (PNALD) is multifactorial in etiology, components of soybean oil lipid emulsions have been implicated in the disease's pathogenesis. Historically, infants with PNALD who were unable to wean from PN to full enteral feeding developed cirrhosis and end-stage liver disease, which require liver transplantation to survive. Over the past 2 decades, novel strategies for the management of parenteral lipids have improved morbidity and mortality from PNALD in infants with intestinal failure. Current strategies for the treatment of PNALD include restricting the dose of parenteral soybean oil lipid emulsion and/or replacing the soybean oil with a parenteral fish-oil lipid emulsion or emulsions of mixed-lipid sources. The purpose of this report is to review published data that evaluate these strategies in parenteral lipid management for the treatment and prevention of PNALD.

DIRECTED CYTOKINE THERAPY IN COMPLEX TREATMENT OF PATIENTS WITH OBSTRUCTIVE JAUNDICE OF CHOLELITHIC GENESIS.

The article analyzed the results of surgical treatment of 137 patients with obstructive jaundice of benign genesis. An immune status was studied in serum in dynamics before surgery. The rates of CD3, CD4, CD8, CD19, Ig A, M, G were determined on the first, third, seventh and fourteenth days after operation. The levels of TNFα, IFNγ, IL-2, IL-6, IL-4, IL-10 were investigated in serum and at the same time TNFα, IL-4, IL-6 were noted in the bile duct and IL-6 - in urine. Obstructive jaundice of cholelithiс genesis is characterized by disbalance of immune and cytokine status. The depth of disbalance depends on the degree of hepatic dysfunction and presence of purulent cholangitis. The directed cytokine therapy by ronkoleykin influenced positively on elimination of disbalance in immune and cytokine status and this therapy improved results of surgery in postoperative period.

Engraftment Syndrome after Autologous Stem Cell Transplantation: An Update Unifying the Definition and Management Approach.

Engraftment syndrome (ES) encompasses a continuum of periengraftment complications after autologous hematopoietic stem cell transplantation. ES may include noninfectious fever, skin rash, diarrhea, hepatic dysfunction, renal dysfunction, transient encephalopathy, and capillary leak features, such as noncardiogenic pulmonary infiltrates, hypoxia, and weight gain with no alternative etiologic basis other than engraftment. Given its pleiotropic clinical presentation, the transplant field has struggled to clearly define ES and related syndromes. Here, we present a comprehensive review of ES in all documented disease settings. Furthermore, we discuss the proposed risk factors, etiology, and clinical relevance of ES. Finally, our current approach to ES is included along with a proposed treatment algorithm for the management of this complication.

Support of hepatic regeneration by trophic factors from liver-derived mesenchymal stromal/stem cells.

Mesenchymal stromal/stem cells (MSCs) have multilineage differentiation potential and as such are known to promote regeneration in response to tissue injury. However, accumulating evidence indicates that the regenerative capacity of MSCs is not via transdifferentiation but mediated by their production of trophic and other factors that promote endogenous regeneration pathways of the tissue cells. In this chapter, we provide a detailed description on how to obtain trophic factors secreted by cultured MSCs and how they can be used in small animal models. More specific, in vivo models to study the paracrine effects of MSCs on regeneration of the liver after surgical resection and/or ischemia and reperfusion injury are described.

[Extracorporeal methods of hematological correction in patients with acute liver insufficiency after cardiac surgery].

BACKGROUND & AIMS: Acute liver failure (ALF) usually develops in multiple organ dysfunction syndrome (MODS) and carries a high mortality risk in patients after cardiac surgery. Artificial liver support devices aim to remove albumin-bound and water-soluble toxins arising as a result of liver failure. The currently most used devices combine haemodialysis with albumin dialysis (MARS) or plasma separation and adsorption (Prometheus). The aim of this study was to assess safety and efficacy of use MARS or Prometheus in elderly patients with ALF have been operated for heart diseases. METHOD: We studied 26 elder patients with ALF and MODS as postoperative complication after cardiac surgery. Patients were assigned to groups, given a combination of MARS and standard medical therapy (SMT) (MARS-group, n=9) or Prometheus and SMT (Prometheus-group, n=17). Inclusion criteria were clinical and laboratory signs of ALF: serum total bilirubin level>180 mkmol/L, 2-fold increasing serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT), low serum cholinesterase and high serum ammonia levels. A variety of clinical and biochemical parameters were assessed. Primary endpoint was survival probabilities at day 28. RESULTS: MARS was used to provide 1 to 2 rounds (minimum of 6 hours each) and Prometheus was used to provide 2 to 14 rounds (minimum of 6 hours each). There were amelioration of haemodinamic instability, especially in MARS-group (increase in ADmean was 17% in MARS (p=0.005) and 10% in Prometheus-group (p=0.001)), increase in P/F ratio (12% in Prometheus-group (p=0.07)), decrease in serum total bilirubin (8.6% in MARS-group (p=0.028) and 33% in Prometheus-group (p<0.001)) and unconjugated bilirubin levels (29% in Prometheus-group (p=0.003)), also we had decreasing in serum aminotransferase levels and trend to increasing in serum cholinesterase level (12% in MARS-group (p=0.87) and 8% in Prometheus-group (p=0.86)). There were no side effects of extracorporeal liver support in both patients groups. Survival of patients with ALF, treated with MARS was 22%, in Prometheus group--35%. CONCLUSIONS: MARS and Prometheus are found to be safe and effective in patients with ALF after cardiac surgery. Further studies are needed to assess whether therapy might be beneficial in specific sublets of patients.

Extracorporeal continuous portal diversion plus temporal plasmapheresis for "small-for-size" syndrome.

AIM: To investigate the effect of plasmapheresis via the portal vein for "small-for-size" syndrome (SFSS) aided by extracorporeal continuous portal diversion (ECPD). METHODS: Extensive or total hepatectomy in the pig is usually adopted as a postoperative liver failure (PLF) or SFSS model. In this study, animals which underwent 85%-90% hepatectomy were randomized into either the Systemic group (n = 7) or the Portal group (n = 7). In the Systemic group, all pigs received temporal plasmapheresis (PP) via the extracorporeal catheter circuit (systemic to systemic circulation) from 24 to 30 h post-hepatectomy (PH); in the Portal group, all pigs received ECPD to divert partial portal vein flow (PVF) to the systemic circulation after hepatectomy, then converted to temporal PP from 24 to 30 h PH, and subsequently converted to ECPD again until 48 h PH. In the Portal group, the PVF was preserved at 3.0-3.3 times that of the baseline value, similar to that following 70% hepatectomy, which was regarded as the optimal PVF to the hypertrophic liver remnant. At 48 h PH, all pigs were re-opened and the portal vein pressure (PVP), PVF, and HAF (hepatic artery flow) were measured, and then diversion of the portal venous flow was terminated. After 1 h the PVP, PVF, and HAF were re-measured. The portal hemodynamic changes, liver injury, liver regeneration and bacterial/lipopolysaccharide (LPS) translocation were evaluated in the two groups. RESULTS: The PVP in the Portal group was significantly lower than that in the Systemic group during the time period from 2 to 49 h PH (P < 0.05). Serum alanine aminotransferase (ALT), total bilirubin (TB) and ammonia were significantly reduced in the Portal group compared with the Systemic group from 24 to 48 h PH (P < 0.05). The Portal group may have attenuated sinusoidal endothelial injury and decreased the level of HA compared with the Systemic group. In the Systemic group, there was significant sinusoidal dilation, hydropic changes in hepatocytes and hemorrhage into the hepatic parenchyma, and the sinusoidal endothelial lining was partially destroyed and detached into the sinusoidal space. CD31 immunostaining revealed significant destruction of the endothelial lining. In the Portal group, there was no intraparenchymal hemorrhage and the sinusoidal endothelial cells and hepatocytes were well preserved. CD31 immunostaining was mild which indicated less destruction of the endothelial lining. HA was significantly decreased in the Portal group compared with the Systemic group from 2 to 48 h PH. The rate of liver remnant regeneration was elevated, while apoptosis was attenuated in the Portal group compared with the Systemic group. Thymidine kinase activity was much higher in the Portal group than in the Systemic group at 48 h PH. The PCNA index was significantly increased and the apoptotic index was significantly decreased in the Portal group compared with the Systemic group. Bacterial translocation and endotoxin, as well as the inflammatory response, were significantly attenuated in the Portal group compared with the Systemic group. LPS, tumor necrosis factor-α and interleukin-6 levels were all significantly decreased in the Portal group compared with the Systemic group from 24 to 48 h PH, while bacterial DNA level was significantly decreased from 2 to 48 h PH. CONCLUSION: PP plus ECPD via the portal vein can attenuate toxic load and hyperperfusion injury, and should be undertaken instead of PP via the systemic circulation in SFSS or PLF.

Coagulopathies in the PICU: DIC and liver disease.

Bleeding in patients in pediatric intensive care units is associated with an increased risk of mortality. Fortunately, most patients with an abnormal coagulation profile do not bleed because this is generally secondary to liver disease or dietary-induced vitamin K deficiency. When the laboratory markers of coagulopathy are the result of disseminated intravascular coagulation, bleeding is common and the risk of mortality extreme. Although interventions directed toward correcting the abnormal coagulation test results are generally initiated, they are also generally either not warranted or not fully successful.