Munirah Alsaleh, Thomas A Barbera, Helen L Reeves, Matthew E Cramp, Stephen Ryder, Hani Gabra, Kathryn Nash, Yi-Liang Shen, Elaine Holmes, Roger Williams, Simon D Taylor-Robinson
{"title":"Characterization of the urinary metabolic profile of cholangiocarcinoma in a United Kingdom population.","authors":"Munirah Alsaleh, Thomas A Barbera, Helen L Reeves, Matthew E Cramp, Stephen Ryder, Hani Gabra, Kathryn Nash, Yi-Liang Shen, Elaine Holmes, Roger Williams, Simon D Taylor-Robinson","doi":"10.2147/HMER.S193996","DOIUrl":"10.2147/HMER.S193996","url":null,"abstract":"<p><p><b>Background:</b> Outside South-East Asia, most cases of cholangiocarcinoma (CCA) have an obscure etiology. There is often diagnostic uncertainty. Metabolomics using ultraperformance liquid chromatography mass spectrometry (UPLC-MS) offers the portent to distinguish disease-specific metabolic signatures. We aimed to define such a urinary metabolic signature in a patient cohort with sporadic CCA and investigate whether there were characteristic differences from those in patients with hepatocellular carcinoma (HCC), metastatic secondary liver cancer, pancreatic cancer and ovarian cancer (OCA). <b>Methods:</b> Spot urine specimens were obtained from 211 subjects in seven participating centers across the UK. Samples were collected from healthy controls and from patients with benign hepatic disease (gallstone, biliary strictures, sphincter of Oddi dysfunction and viral hepatitis) and patients with malignant conditions (HCC, pancreatic cancer, OCA and metastatic cancer in the liver). The spectral metabolite profiles were generated using a UPLC-MS detector and data were analyzed using multivariate and univariate statistical analyses. <b>Results:</b> The greatest class differences were seen between the metabolic profiles of disease-free controls compared to individuals with CCA with altered acylcarnitine, bile acid and purine levels. Individuals with benign strictures showed comparable urine profiles to patients with malignant bile duct lesions. The metabolic signatures of patients with bile duct tumors were distinguishable from patients with hepatocellular and ovarian tumors, but no difference was observed between CCA cases and patients with pancreatic cancer or hepatic secondary metastases. <b>Conclusion:</b> CCA causes subtle but detectable changes in the urine metabolic profiles. The findings point toward potential applications of metabonomics in early tumor detection. However, it is key to utilize both global and targeted metabonomics in a larger cohort for in-depth characterization of the urine metabolome in hepato-pancreato-biliary disease.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"11 ","pages":"47-67"},"PeriodicalIF":2.6,"publicationDate":"2019-05-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6507078/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37266913","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Linda Skibsted Kornerup, Henning Pflugrad, Karin Weissenborn, Hendrik Vilstrup, Gitte Dam
{"title":"Cognitive impairment after liver transplantation: residual hepatic encephalopathy or posttransplant encephalopathy?","authors":"Linda Skibsted Kornerup, Henning Pflugrad, Karin Weissenborn, Hendrik Vilstrup, Gitte Dam","doi":"10.2147/HMER.S144667","DOIUrl":"10.2147/HMER.S144667","url":null,"abstract":"<p><p>Liver transplantation (LT) represents the definitive treatment for end-stage liver disease. Cognitive impairment following LT is frequent, referred to as postliver transplant encephalopathy (PLTE). LT removes the underlying chronic liver disease, and until recently hepatic encephalopathy (HE) was assumed to be fully reversible after LT. However, increasing evidence indicates that some degree of cognitive impairment may be present after LT. To which extent PLTE reflects cognitive impairment caused by residual HE (RHE) or the combined effect of other factors affecting brain function before, during, and after LT is not clarified. None of the available psychometric and neurophysiological tests used for detecting HE is shown to be able to distinguish between etiologies. The available, mostly retrospective, clinical studies indicate a high prevalence of abnormal psychometric tests after LT, and not all seem to recover completely. The patients with earlier HE show the most marked improvements, suggesting that the clinical picture of the early PLTE, in fact, represents RHE. Other early post-LT etiologies for PLTE comprise cerebral ischemia, critical illness encephalopathy, and immunosuppressive therapy. Late-onset etiologies comprise diabetes and hypertension, among others. PLTE regardless of etiology is a worrying issue and needs more attention in the form of mechanistic research, development of diagnostic/discriminative tools, and standardized prospective clinical studies.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"11 ","pages":"41-46"},"PeriodicalIF":2.6,"publicationDate":"2019-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/83/40/hmer-11-41.PMC6456244.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37201646","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Ombitasvir, paritaprevir, and ritonavir with peginterferon-α2a plus ribavirin in treatment-experienced patients with chronic hepatitis C virus genotype 1 infection.","authors":"David Bernstein, Rakesh Tripathi, Daniel E Cohen","doi":"10.2147/HMER.S189158","DOIUrl":"10.2147/HMER.S189158","url":null,"abstract":"<p><strong>Background: </strong>This international, phase 2, open-label, multicenter study (ClinicalTrials.gov Identifier: NCT01609933) was conducted to evaluate the safety and efficacy of an enhanced regimen consisting of the direct-acting antivirals (DAAs) ombitasvir, paritaprevir, and ritonavir administered for 24 weeks, combined with pegylated interferon-α2a plus ribavirin (pegIFN-α2a/RBV) for 48 weeks, in patients with chronic hepatitis C virus (HCV) genotype 1 infection who had experienced virologic failure with a prior DAA regimen. This study was undertaken at a time when options were limited for the retreatment of patients who had failed prior DAA therapy.</p><p><strong>Methods and results: </strong>Thirty-two patients were enrolled; the majority were male (78%) and White (94%), and the median age was 54.5 years. Twelve weeks after the last dose of study drug, sustained virologic response was achieved in 81.3% of patients. Five patients prematurely discontinued the study drugs and one patient relapsed. Safety and tolerability were similar to prior studies of pegIFN-α2a/RBV alone.</p><p><strong>Conclusion: </strong>Given the availability of highly efficacious DAA regimens that are both IFN- and RBV-free, this regimen is no longer relevant in today's HCV treatment landscape.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"11 ","pages":"35-40"},"PeriodicalIF":2.1,"publicationDate":"2019-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/HMER.S189158","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37045499","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Portosystemic shunts and refractory hepatic encephalopathy: patient selection and current options.","authors":"Cyriac Abby Philips, Sasidharan Rajesh, Philip Augustine, Guruprasad Padsalgi, Rizwan Ahamed","doi":"10.2147/HMER.S169024","DOIUrl":"https://doi.org/10.2147/HMER.S169024","url":null,"abstract":"<p><p>Portosystemic shunt (PS) syndrome encompasses a spectrum of disease manifestations ranging from asymptomatic portal hypertension to recurrent and refractory hepatic encephalopathy, ultimately culminating in progressive hepatic failure in patients of cirrhosis and associated large PSs. PSs commonly seen in cirrhosis include splenorenal, gastrorenal, and dilated paraumbilical veins, all of which can present with recurrent or refractory hepatic encephalopathy. In this exhaustive review, we describe the anatomy of PSs, elucidate new theories on their pathophysiology, discuss the clinical implications of PSs in cirrhosis, provide details on different techniques (classical and novel) of shunt embolization, and explore all the pertinent current literature on shunt embolization for refractory and recurrent hepatic encephalopathy, all of which are enumerated with extensive images and illustrations.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"11 ","pages":"23-34"},"PeriodicalIF":2.1,"publicationDate":"2019-01-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/HMER.S169024","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36976134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Sebastián Marciano, Juan Manuel Díaz, Melisa Dirchwolf, Adrián Gadano
{"title":"Spontaneous bacterial peritonitis in patients with cirrhosis: incidence, outcomes, and treatment strategies.","authors":"Sebastián Marciano, Juan Manuel Díaz, Melisa Dirchwolf, Adrián Gadano","doi":"10.2147/HMER.S164250","DOIUrl":"10.2147/HMER.S164250","url":null,"abstract":"<p><p>Spontaneous bacterial peritonitis is the most frequent bacterial infection in patients with cirrhosis. The reported incidence varies between 7% and 30% in hospitalized patients with cirrhosis and ascites, representing one of their main complications. Outcomes in patients with spontaneous bacterial peritonitis are poor since acute kidney injury, acute-on-chronic liver failure, and death occur in as much as 54%, 60%, and 40% of the patients, respectively, at midterm. Early antibiotic treatment of spontaneous bacterial peritonitis is crucial. However, the landscape of microbiological resistance is continuously changing, with an increasing spread of multidrug-resistant organisms that make its current management more challenging. Thus, the selection of the empirical antibiotic treatment should be guided by the severity and location where the infection was acquired, the risk factors for multidrug-resistant organisms, and the available information on the local expected bacteriology. The use of albumin as a complementary therapy for selected high-risk patients with spontaneous bacterial peritonitis is recommended in addition to antibiotics. Even though antibiotic prophylaxis has proven to be effective to prevent spontaneous bacterial peritonitis, a careful selection of high-risk candidates is crucial to avoid antibiotic overuse. In this article we review the pathogenesis, risk factors, and prognosis of spontaneous bacterial peritonitis, as well as the current evidence regarding its treatment and prophylaxis.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"11 ","pages":"13-22"},"PeriodicalIF":2.1,"publicationDate":"2019-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/2b/54/hmer-11-013.PMC6336019.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36926481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Carlos Roncero, Pablo Ryan, Richard Littlewood, Juan Macías, Juan Ruiz, Pedro Seijo, Raúl Felipe Palma-Álvarez, Pablo Vega
{"title":"Practical steps to improve chronic hepatitis C treatment in people with opioid use disorder.","authors":"Carlos Roncero, Pablo Ryan, Richard Littlewood, Juan Macías, Juan Ruiz, Pedro Seijo, Raúl Felipe Palma-Álvarez, Pablo Vega","doi":"10.2147/HMER.S187133","DOIUrl":"10.2147/HMER.S187133","url":null,"abstract":"<p><strong>Objectives: </strong>People with a history of injecting drugs have high prevalence of hepatitis C virus (HCV) infection, and many have opioid use disorder (OUD). Modern HCV therapies with improved efficacy and tolerability are available, but access is often limited for this group, who may be underserved for health care and face social inequity. This work develops practical steps to improve HCV care in this population.</p><p><strong>Methods: </strong>Practical steps to improve HCV care in OUD populations were developed based on clinical experience from Spain, structured assessment of published evidence.</p><p><strong>Results: </strong>Options for improving care at engagement/screening stages include patient education programs, strong provider-patient relationship, peer support, and adoption of rapid effective screening tools. To facilitate work up/treatment, start options include simplified work up process, integration of HCV and OUD care, and continuous psychosocial support prior, during, and after HCV treatment.</p><p><strong>Conclusion: </strong>It is important to plan on local basis to set up a joint integrated approach between specific drug treatment services and local points of HCV care. The elements for a specific integrated program should be chosen from options identified, including education services, peer input, organization to make HCV screening and treatment easier by co-location of services, and wider access to prescribing direct-acting antiviral (DAA) therapy.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"11 ","pages":"1-11"},"PeriodicalIF":2.1,"publicationDate":"2018-12-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c2/c9/hmer-11-001.PMC6307489.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36839011","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zhongping Duan, Shaojie Xin, Jing Zhang, Shaoli You, Yu Chen, Hongling Liu, Sujun Zheng, Zheng Li, Robert Ashley, Michael Millis
{"title":"Comparison of extracorporeal cellular therapy (ELAD<sup>®</sup>) vs standard of care in a randomized controlled clinical trial in treating Chinese subjects with acute-on-chronic liver failure.","authors":"Zhongping Duan, Shaojie Xin, Jing Zhang, Shaoli You, Yu Chen, Hongling Liu, Sujun Zheng, Zheng Li, Robert Ashley, Michael Millis","doi":"10.2147/HMER.S180246","DOIUrl":"https://doi.org/10.2147/HMER.S180246","url":null,"abstract":"<p><strong>Background: </strong>Preliminary evidence of safety and efficacy of an extracorporeal cellular therapy (ELAD<sup>®</sup>) has been demonstrated in subjects with acute forms of liver failure. This study compared ELAD with standard of care in Chinese subjects with acute-on-chronic liver failure (ACLF), predominantly secondary to chronic viral hepatitis.</p><p><strong>Subjects and methods: </strong>Subjects meeting eligibility criteria were randomized to either the ELAD group or the control group. All subjects received plasma exchange and venovenous hemofiltration and either ELAD treatment for 3-5 days, unless terminated early, along with standard of care or standard of care alone (control) and were then followed up for 12 weeks.</p><p><strong>Results: </strong>Forty-nine subjects (ELAD subjects, 32; controls, 17) were randomized under this protocol. Kaplan-Meier analysis of transplant-free survival (TFS) revealed a significant difference in favor of ELAD vs control (<i>P</i>=0.049, Wilcoxon signed-rank test). There was a significant difference in TFS on day 28 in ELAD vs control (<i>P</i>=0.022). In a multiple regression model, the relationship between group assignment and outcome was significant (<i>P</i>=0.031) when changes in food intake and Model for End-Stage Liver Disease (MELD) scores at screening were included as additional independent variables. The duration of ELAD treatment alone was a significant predictor of TFS (<i>P</i>=0.043). Median time to a 5-point increase in MELD, transplant, or death was longer than 72 days with ELAD vs 26 days for control (<i>P</i>=0.036). Total bilirubin level decreased by 25% during ELAD treatment vs 37% increase in the control group (<i>P</i><0.001) over an equivalent period. Adverse events attributed to the ELAD system were expected and could be managed conservatively. Intergroup differences in certain vital signs and laboratory parameters were noted during treatment and generally resolved posttreatment.</p><p><strong>Conclusion: </strong>ELAD treatment was well tolerated by Chinese subjects with ACLF, predominately secondary to chronic viral hepatitis. Results demonstrate a significant improvement in TFS in ELAD vs control groups in association with significant improvements in serum bilirubin levels presumably related to improvement in hepatic function.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"10 ","pages":"139-152"},"PeriodicalIF":2.1,"publicationDate":"2018-11-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/HMER.S180246","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36756204","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zohair Ahmed, Jinma Ren, Adam Gonzalez, Umair Ahmed, Saqib Walayat, Daniel K Martin, Harsha Moole, Sherri Yong, Sean Koppe, Sonu Dhillon
{"title":"Universal Index for Cirrhosis (UIC index): The development and validation of a novel index to predict advanced liver disease.","authors":"Zohair Ahmed, Jinma Ren, Adam Gonzalez, Umair Ahmed, Saqib Walayat, Daniel K Martin, Harsha Moole, Sherri Yong, Sean Koppe, Sonu Dhillon","doi":"10.2147/HMER.S160616","DOIUrl":"https://doi.org/10.2147/HMER.S160616","url":null,"abstract":"<p><strong>Aim: </strong>The purpose of this study was to create and validate a novel serological diagnostic index to predict cirrhosis of all etiologies.</p><p><strong>Methods: </strong>This was a retrospective observational study of 771 patients, age >18 years, who underwent a liver biopsy. The stage of fibrosis and routine laboratory values were recorded. The data were randomly separated into 2 datasets (training 50% and testing 50%). A stepwise logistic regression model was used to develop the novel index. The area under the curve of receiver operating characteristic (AUROC) was applied to compare the new index to existing ones (Fibro-Q, FIB4, APRI, AAR), which was also validated in the testing dataset.</p><p><strong>Results: </strong>Variables associated with the presence of cirrhosis were first assessed by univariate analysis then by multivariable analysis, which indicated serum glutamic-oxaloacetic acid transaminase, serum glutamic-pyruvic transaminase, international normalized ratio, albumin, blood urea nitrogen, glucose, platelet count, total protein, age, and race were the independent predictors of cirrhosis (<i>P</i><0.05). Regression formula for prediction of cirrhosis was generated and a novel index was subsequently created. The diagnostic performance of the novel index for predicting cirrhosis was assessed using the receiver operating characteristic curve. The new index had significantly higher AUROC (0.83, 95% CI: 0.79-0.87) than Fibro-Q (0.80, 95% CI: 0.76-0.85), FIB4 (0.79, 95% CI: 0.74-0.83), APRI (0.74, 95% CI: 0.69-0.78), and AAR (0.72, 95% CI: 0.67-0.78).</p><p><strong>Conclusion: </strong>The novel index had the highest AUROC curve when compared with current indices and can be applied to all etiologies of chronic liver disease.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"10 ","pages":"133-138"},"PeriodicalIF":2.1,"publicationDate":"2018-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/HMER.S160616","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36781760","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Expert opinion on the management of hepatitis C infection in Kuwait.","authors":"Motaz Fathy Saad, Saleh Alenezi, Haifaa Asker","doi":"10.2147/HMER.S154842","DOIUrl":"https://doi.org/10.2147/HMER.S154842","url":null,"abstract":"Chronic hepatitis C virus (HCV) infection is a leading cause of death, especially in immunocompromised patients. The lack of clear prevalence data in the Middle East makes it difficult to estimate the true morbidity and mortality burden of HCV. In Kuwait, estimating the burden of disease is complicated by the constant flow of expatriates, many of whom are from HCV-endemic areas. The development of new and revolutionary treatments for HCV necessitates the standardization of clinical practice across all healthcare institutions. While international guidelines from the American Association for the Study of Liver Diseases (AASLD) and European Association for the Study of the Liver (EASL) do address this evolving treatment landscape, the cost-driven treatment prioritization of patients by these guidelines and unique HCV genotype presentation in the Kuwaiti population prompted the development of a more tailored approach. The predominant HCV genotypes prevalent in Kuwait are genotypes 4 and 1. The Kuwait Hepatology Club (KHC), comprising hepatologists across all major institutions in Kuwait, conducted several consensus meetings to develop the scoring criteria, evaluate all current evidence, and propose screening, diagnosis, and treatment suggestions for the management of HCV in this population. While these treatment suggestions were largely consistent with the 2016 AASLD and 2015 EASL guidelines, they also addressed gaps in the unmet needs of the Kuwaiti population with HCV.","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"10 ","pages":"117-132"},"PeriodicalIF":2.1,"publicationDate":"2018-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/HMER.S154842","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36628197","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Nadia Savy, David Brossier, Catherine Brunel-Guitton, Laurence Ducharme-Crevier, Geneviève Du Pont-Thibodeau, Philippe Jouvet
{"title":"Acute pediatric hyperammonemia: current diagnosis and management strategies.","authors":"Nadia Savy, David Brossier, Catherine Brunel-Guitton, Laurence Ducharme-Crevier, Geneviève Du Pont-Thibodeau, Philippe Jouvet","doi":"10.2147/HMER.S140711","DOIUrl":"https://doi.org/10.2147/HMER.S140711","url":null,"abstract":"<p><p>Acute hyperammonemia may induce a neurologic impairment leading to an acute life-threatening condition. Coma duration, ammonia peak level, and hyperammonemia duration are the main risk factors of hyperammonemia-related neurologic deficits and death. In children, hyperammonemia is mainly caused by severe liver failure and inborn errors of metabolism. In an acute setting, obtaining reliable plasma ammonia levels can be challenging because of the preanalytical difficulties that need to be addressed carefully. The management of hyperammonemia includes 1) identification of precipitating factors and cerebral edema presence, 2) a decrease in ammonia production by reducing protein intake and reversing catabolism, and 3) ammonia removal with pharmacologic treatment and, in the most severe cases, with extracorporeal therapies. In case of severe coma, transcranial Doppler ultrasound could be the method of choice to noninvasively monitor cerebral blood flow and titrate therapies.</p>","PeriodicalId":12917,"journal":{"name":"Hepatic Medicine : Evidence and Research","volume":"10 ","pages":"105-115"},"PeriodicalIF":2.1,"publicationDate":"2018-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/HMER.S140711","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36522482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}