scholarly journals Hepatic Encephalopathy: A Review

2021 ◽  
pp. 89-97
Author(s):  
Savan Kabaria ◽  
Ishita Dalal ◽  
Kapil Gupta ◽  
Abhishek Bhurwal ◽  
Minacapelli Carlos D. ◽  
...  
Keyword(s):  
Amino Acids ◽  
Liver Disease ◽  
Hepatic Encephalopathy ◽  
Hospital Costs ◽  
Branched Chain Amino Acids ◽  
Review Article ◽  
Branched Chain ◽  
New Research ◽  
The Brain ◽  
Recurrence Frequency

Hepatic encephalopathy (HE) is a reversible syndrome observed in patients with liver disease. The syndrome is characterised by a spectrum of neuropsychiatric abnormalities resulting from the accumulation of neurotoxic substances in the bloodstream and ultimately in the brain. HE is a huge burden to patients, caregivers, and the healthcare system. Common treatments for HE, including rifaximin and lactulose, have been shown to reduce the risk of recurrence, frequency of hospitalisations, hospital costs, and mortality. New research and therapeutics exist, including faecal transplants and small-molecule therapies such as branched-chain amino acids. This review article provides a general overview of the current understanding of HE.

Branched-chain amino acids for people with hepatic encephalopathy

2015 ◽  
Author(s):  
Lise Lotte Gluud ◽  
Gitte Dam ◽  
Iñigo Les ◽  
Juan Córdoba ◽  
Giulio Marchesini ◽  
...  
Keyword(s):  
Amino Acids ◽  
Hepatic Encephalopathy ◽  
Branched Chain Amino Acids ◽  
Branched Chain

scholarly journals Brain Branched-Chain Amino Acids in Maple Syrup Urine Disease: Implications for Neurological Disorders

2020 ◽  
Vol 21 (20) ◽  
pp. 7490
Author(s):  
Jing Xu ◽  
Youseff Jakher ◽  
Rebecca C. Ahrens-Nicklas
Keyword(s):  
Amino Acids ◽  
Maple Syrup Urine Disease ◽  
Attention Deficit Disorder ◽  
Neurological Disorders ◽  
Survival Rates ◽  
Branched Chain Amino Acids ◽  
Maple Syrup ◽  
Urine Disease ◽  
Branched Chain ◽  
The Brain

Maple syrup urine disease (MSUD) is an autosomal recessive disorder caused by decreased activity of the branched-chain α-ketoacid dehydrogenase complex (BCKDC), which catalyzes the irreversible catabolism of branched-chain amino acids (BCAAs). Current management of this BCAA dyshomeostasis consists of dietary restriction of BCAAs and liver transplantation, which aims to partially restore functional BCKDC activity in the periphery. These treatments improve the circulating levels of BCAAs and significantly increase survival rates in MSUD patients. However, significant cognitive and psychiatric morbidities remain. Specifically, patients are at a higher lifetime risk for cognitive impairments, mood and anxiety disorders (depression, anxiety, and panic disorder), and attention deficit disorder. Recent literature suggests that the neurological sequelae may be due to the brain-specific roles of BCAAs. This review will focus on the derangements of BCAAs observed in the brain of MSUD patients and will explore the potential mechanisms driving neurologic dysfunction. Finally, we will discuss recent evidence that implicates the relevance of BCAA metabolism in other neurological disorders. An understanding of the role of BCAAs in the central nervous system may facilitate future identification of novel therapeutic approaches in MSUD and a broad range of neurological disorders.

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