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Liver disorders
Published in Rachel U Sidwell, Mike A Thomson, Concise Paediatrics, 2020
Rachel U Sidwell, Mike A Thomson
Incidence 1:15 000–20 000 live births. A condition of progressive obliteration of part or all of the extrahepatic biliary ducts (an obliterative cholangiopathy). This leads to chronic liver failure and death. It should be suspected if there is prolonged jaundice beyond 14 days.
Hyperfibrinolysis in Liver Cirrhosis
Published in Pia Glas-Greenwalt, Fibrinolysis in Disease Molecular and Hemovascular Aspects of Fibrinolysis, 2019
The major role of the liver in the regulation of fibrinolysis ensures that a decreased liver function in liver disease will result in changes in fibrinolysis, often manifesting as enhanced fibrinolysis. Most studies have been performed in patients with chronic liver failure, as will be reviewed below. Changes in fibrinolysis in other types of liver disease, such as acute hepatic failure and obstructive jaundice, are different and are outside the scope of this chapter.
Transplantation
Published in Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie, Bailey & Love's Short Practice of Surgery, 2018
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie
The most common indication for transplantation is chronic liver failure. In adults the most common causes are alcoholic liver disease, viral liver disease (hepatitis C in Europe and the USA, and hepatitis B in some other countries), non-alcoholic steatohepatitis, primary biliary cirrhosis and sclerosing cholangitis. In children, who account for around 10–15% of all liver transplantations, biliary atresia is the most common indication for transplantation. Acute fulminant liver failure requiring transplantation on an urgent basis accounts for approximately 10% of liver transplant activity and is usually viral or drug induced (e.g. paracetamol overdose in the UK). There are a variety of metabolic diseases for which transplantation offers the prospect of cure, including Wilson’s disease, oxalosis and familial amyloid polyneuropathy. Primary hepatic malignancy is more common in patients with cirrhosis, especially virally induced disease, and may be best treated by transplantation because the field changes in the cirrhotic liver predispose to further primary malignancies. Cholangiocarcinoma has a high recurrence rate and is seldom an indication for liver transplantation.
Prognostic value of decline in model for end-stage liver disease score and hepatic encephalopathy in hepatitis B-related acute-on-chronic liver failure patients treated with plasma exchange
Published in Scandinavian Journal of Gastroenterology, 2022
Lu Wang, Shu Zhu, Ying Liu, Lihua Zheng, Wenxiong Xu, Qiumin Luo, Yeqiong Zhang, Hong Deng, Xinhua Li, Chan Xie, Liang Peng
Acute-on-chronic liver failure (ACLF) is a clinical syndrome involving acute deterioration in liver function and progression to extrahepatic organ failure in a short time interval in patients with chronic liver disease and associated with high short-term mortality [1,2]. Hepatitis B virus (HBV) is widely acknowledged as the leading cause of ACLF in China [3]. The artificial liver assistant system (ALSS) can reportedly improve the clinical presentation and short-term outcomes of patients with hepatitis B virus-related acute-on-chronic liver failure (HBV-ACLF). It has been established that ALSS is an effective perioperative treatment for liver transplantation (LT) in patients with advanced liver failure [4]. Plasma exchange (PE) brings several advantages including simplicity of operation, convenient operation and low cost, and is the most widely used ALSS in China [5].
Novel drug discovery strategies for the treatment of decompensated cirrhosis
Published in Expert Opinion on Drug Discovery, 2022
Sven Lamatsch, Richard Sittner, Frank Tacke, Cornelius Engelmann
Chronic liver diseases have a significant impact on global health. Liver cirrhosis causes globally more than 1.3 million deaths per year, which accounts for about 2.4% of all deaths in 2017 [1]. Every year, there are 10 million cases of decompensated cirrhosis with a constant increase of age-standardized rates of decompensation between 1990 and 2017 [1]. This rising trend is partially related to an increasing NASH prevalence and also worsening of alcohol abuse in some regions of the world, including Finland and United Kingdom [1]. In cirrhosis, liver-related deaths mostly occur after sudden decompensation characterized by bacterial infection, hepatic encephalopathy, ascites or variceal bleeding. Further progression leads to acute-on-chronic liver failure (ACLF) where multiple organ failures account for the poor prognosis [2]. Whilst the 28-day mortality in patients with ‘sole’ decompensated cirrhosis is approximately 5%, it dramatically increases to 40% with ACLF and even up to almost 90% in patients with high-grade ALCF and four to six organ failure [3] (Table 1).
Safety of treating acute liver injury and failure
Published in Expert Opinion on Drug Safety, 2022
Miren García-Cortés, Aida Ortega-Alonso, Raúl J. Andrade
Acute liver injury (ALI) for the aim of this review is defined by acute liver damage (aminotransferases elevation reflecting hepatic necrosis) commonly associated with impaired liver function (coagulopathy and jaundice), without encephalopathy [1]. The time course differentiates ALI from chronic liver injury, where a commonly used cutoff is duration of illness greater than 26 weeks for chronic cases (interval between first symptoms and onset of encephalopathy). This syndrome may antedate acute liver failure (ALF), which is defined as an acute liver injury with hepatic encephalopathy in a patient without preexisting liver disease [2]. Acute liver failure may also be a sudden presentation in patients with previously undiagnosed Wilson disease, hepatitis B virus infection, or autoimmune hepatitis, in whom underlying cirrhosis may be already present. However, patients with acute severe alcoholic hepatitis, even if recognized for <26 weeks, are considered to have acute-on-chronic liver failure since most have a long history of heavy drinking and ongoing alcoholic liver disease. Furthermore, acute liver failure can be subcategorized by the O´Gradys system (based on jaundice-encephalopathy time) as hyperacute (<7 days), acute (7–21 days), or subacute (>21 days and <26 weeks). In patients with hyperacute or acute liver failure, cerebral edema is common, whereas it is rare in the subacute form [3,4].