Abstract:
:Over the past 35 years, the outlook for a patient presenting with acute liver failure (ALF) has changed beyond all recognition. A patient presenting in 1984 had an 80 % likelihood of succumbing to intracranial hypertension. Today due to dramatic improvements in intensive care in dedicated liver transplant units, this has been reduced to just 20 %. Prompt fluid resuscitation, empirical treatment for sepsis and standardised management protocols that include early intubation and high flow hemofiltration for ammonia removal, limit the numbers of patients who die from the sequelae of cerebral edema and ALF. With the evolution and development of bedside prognostic markers that will include personalised genomic, metabonomic and immune profiling, rationalisation of grafts to those who are not predicted to survive is likely to further minimise the number of grafts utilised. Furthermore, in those patients with a dismal prognosis, the use of plasmapheresis, immunomodulatory therapies, biological liver support systems and hepatocyte transplantation offer a potential bridge until the injured liver can begin to regenerate avoiding transplantation and life-long immunosuppressant therapy.
journal_name
Metab Brain Disjournal_title
Metabolic brain diseaseauthors
Ryan JM,Tranah T,Mitry RR,Wendon JA,Shawcross DLdoi
10.1007/s11011-012-9363-1subject
Has Abstractpub_date
2013-03-01 00:00:00pages
7-10issue
1eissn
0885-7490issn
1573-7365journal_volume
28pub_type
杂志文章,评审abstract::This study was performed to analyze the mutational spectrum of the phenylalanine hydroxylase (PAH) gene in phenylketonuria (PKU) patients in Northwest China, to identify mutational hot spots, and to determine the correlation between variants and clinical phenotypes of PKU. A large cohort of 475 PKU families in Northwe...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-019-0387-7
更新日期:2019-06-01 00:00:00
abstract::The effect of factors released from N2A neuroblastoma cells on the expression of myelin protein genes in glioma C6 cells, i.e., proteolipid protein (PLP) and myelin-associated glycoprotein (MAG), was studied. Both cells lines were propagated in serum-free DMEM-F10 (1:1) medium. The addition of 50% N2A conditioned medi...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/BF01000160
更新日期:1992-09-01 00:00:00
abstract::It has been suggested that alterations of GABAergic neurotransmission are implicated in the pathophysiology of hepatic encephalopathy (HE). Increased concentrations of endogenous benzodiazepines with positive allosteric modulatory properties at the GABA-A receptor complex were proposed as a pathophysiological mechanis...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1023/b:mebr.0000043974.89820.22
更新日期:2004-12-01 00:00:00
abstract::Transient bilateral common carotid artery occlusion (tBCCAO), followed by reperfusion, is a model of transient global hypoperfusion. In the present study we aimed to investigate the probable effects of Vanillic acid (VA) on some physiological parameters including cerebral hyperemia, blood-brain barrier (BBB) disruptio...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-018-0187-5
更新日期:2018-06-01 00:00:00
abstract::Sevoflurane, one of the most commonly used volatile anesthetics in clinical treatment, has been shown to induce a widespread increase in brain apoptosis. However, the underlying mechanism is still unknown. Sestrin 2 has been recently shown to regulate intracellular reactive oxygen species (ROS) levels and play a cruci...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-015-9673-1
更新日期:2015-10-01 00:00:00
abstract::The central nervous system is one of the most vulnerable organs affected by mercury toxicity. Both acute and chronic exposure to mercury is also known to cause a variety of neurological or psychiatric disorders. Here, the neuroprotective effect of berberine (BN; 100 mg/kg bwt) on mercuric chloride (HgCl2; 0.4 mg/kg bw...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-015-9652-6
更新日期:2015-08-01 00:00:00
abstract::Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism. Usually diagnosed within the first month of birth, it is essential that the patient strictly follow the dietary restriction of natural protein intake. Otherwise, PKU impacts the development of the brain severely and may result in microceph...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-019-00472-7
更新日期:2019-12-01 00:00:00
abstract::The synthesis, storage and release of acetylcholine (ACh) requires the expression of several specialized proteins, including choline acetyltransferase (ChAT) and the vesicular ACh transporter (VAChT). The VAChT gene is located within the first intron of the ChAT gene. This unique genomic organization permits coordinat...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/BF02680013
更新日期:2000-03-01 00:00:00
abstract::The integrity of the blood-brain barrier (BBB) is an important aspect of normal central nervous system (CNS) function. Recently, it was shown that the BBB breakdown is one of the predisposing factors in the pathogenesis of thiamine-deficiency encephalopathy. The result is discussed along with some reviews on previous ...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/BF02080931
更新日期:1996-03-01 00:00:00
abstract::The present study analyses the potential role of MTHFR gene polymorphism, folate supplementation and dietary pattern among the mothers of NTD neonates and controls in heterogeneous populations of North India, with the special focus on their ethnic labels. Results indicated significant increased risk for neural tube de...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-011-9256-8
更新日期:2011-09-01 00:00:00
abstract::Hepatic encephalopathy (HE) is a neuropsychiatric syndrome which develops in patients with severe liver diseases and/or portal-systemic shunting. Minimal HE, the earliest manifestation of HE, has drawn increasing attention in the last decade. Minimal HE is associated with a series of brain functional changes, such as ...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-014-9504-9
更新日期:2014-09-01 00:00:00
abstract::Minimal hepatic encephalopathy (MHE) is the earliest form of hepatic encephalopathy (HE) and affects up to 80 % of patients with liver cirrhosis. By definition, MHE is characterized by psychomotor slowing and subtle cognitive deficits, but obvious clinical manifestations are lacking. Given its covert nature, MHE is o...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9848-4
更新日期:2016-10-01 00:00:00
abstract::The availability of an animal model is crucial in studying the pathophysiological mechanisms of disease and to test possible therapies. Now, there are several models for the study of liver diseases, but there still remains a lack of a satisfactory animal model of chronic liver disease with hepatic encephalopathy (HE) ...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-005-7925-1
更新日期:2005-12-01 00:00:00
abstract::Mutations in NADH dehydrogenase (ND) subunits of complex I lead to mitochondrial encephalomyopathies associated with various phenotypes. This report aims to present the patient's clinical symptomatology in the context of a very rare 13042G>A de novo mutation and with an emphasis on changing phenotypic expression and p...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9645-x
更新日期:2015-08-01 00:00:00
abstract::Studies have shown that oxidative stress is involved in the pathophysiology of bipolar disorder (BD). It is suggested that omega-3 (ω3) fatty acids are fundamental to maintaining the functional integrity of the central nervous system. The animal model used in this study displayed fenproporex-induced hyperactivity, a s...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-016-9942-7
更新日期:2017-04-01 00:00:00
abstract::Iron is an important micronutrient for neuronal function and survival. It plays an essential role in DNA and protein synthesis, neurotransmission and electron transport chain due to its dual redox states. On the contrary, iron also catalyses the production of free radicals and hence, causes oxidative stress. Therefore...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-014-9522-7
更新日期:2014-09-01 00:00:00
abstract::Effects of sertraline, haloperidol or olanzapine administration on ketamine-induced behaviours in mice were examined. The aim was to ascertain the degree of reversal of such behaviours by sertraline, and compare its effectiveness to haloperidol and olanzapine. Ten-week old mice (N = 120) were equally divided into main...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0031-3
更新日期:2017-10-01 00:00:00
abstract::Thiamine triphosphate (ThTP) was discovered over 60 years ago and it was long thought to be a specifically neuroactive compound. Its presence in most cell types, from bacteria to mammals, would suggest a more general role but this remains undefined. In contrast to thiamine diphosphate (ThDP), ThTP is not a coenzyme. I...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-014-9509-4
更新日期:2014-12-01 00:00:00
abstract::Menkes disease is a very rare X-linked copper metabolism disorder that results from an ATP7A gene mutation. With the advent of subcutaneous copper-histidine therapy, the early diagnosis of Menkes disease becomes of utmost importance for patients' prognosis. In the present study, the clinical characteristics of 12 Kore...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9569-5
更新日期:2015-02-01 00:00:00
abstract::The combination of Parkinson syndrome (PS) and prolonged visually evoked potentials (VEPs) in a single patient with hepatic encephalopathy (HE) has not been reported. A 52-year-old male with chronic HE developed PS in early 2001. Treatment with L-DOPA was only of minimal effect. In August 2001 he was admitted because ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-005-2476-z
更新日期:2005-03-01 00:00:00
abstract::Traumatic brain injury (TBI) is accompanied with enhanced matrix metalloproteinase-9 (MMP-9) activity and elevated levels of plasma fibrinogen (Fg), which is a known inflammatory agent. Activation of MMP-9 and increase in blood content of Fg (i.e. hyperfibrinogenemia, HFg) both contribute to cerebrovascular disorders ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-014-9550-3
更新日期:2015-04-01 00:00:00
abstract::This study presents further research into the spectrum of variants in genes responsible for the development of phenylketonuria (PKU) and hyperphenylalaninemia (HPA) in patients in Russia. After a study of 25 frequent variants, 293 patients (327 chromosomes without detected variants) from among 1265 probands still had ...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-019-00461-w
更新日期:2019-12-01 00:00:00
abstract::Hepatic encephalopathy (HE) is an important cause of morbidity and mortality in patients with severe liver disease. Although the mechanisms responsible for HE remain elusive, ammonia is generally considered to be involved in its pathogenesis, and astrocytes are thought to be the principal target of ammonia neurotoxici...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1023/a:1023858902184
更新日期:2003-06-01 00:00:00
abstract::The enzyme nitric oxide synthase has been associated with suicide behavior. NOS1, NOS2 and NOS3 genes are implicated in the production of nitric oxide. However, the association between NOS genes and suicide behavior has not yet been established. To assess the association of Nitric Oxide Synthase (NOS) genes and suicid...
journal_title:Metabolic brain disease
pub_type: 杂志文章,meta分析
doi:10.1007/s11011-019-00406-3
更新日期:2019-08-01 00:00:00
abstract::Sleep disturbances are prevalent among patients with Alzheimer's disease (AD) and often precede the onset and progression of dementia. However, there are no reliable animal models for investigating sleep disturbances in patients with sporadic AD (sAD), which accounts for more than 90% of all AD cases. In the present s...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0125-y
更新日期:2018-02-01 00:00:00
abstract::Tyrosinemia type II is a genetic disorder characterized by elevated blood levels of the amino acid tyrosine caused by the deficiency of tyrosine aminotransferase enzyme, resulting in neurologic and developmental difficulties in the patients. Although neurological sequelae are common in Tyrosinemia type II patients, th...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-019-00511-3
更新日期:2020-01-01 00:00:00
abstract::Parkinson's disease is a neurodegenerative disease whose hallmark pathological features include a selective loss of dopaminergic neurons in the midbrain. Ciclooxygenase-2 activity induction and oxidative stress have been implicated in the aetiology of Parkinson's disease and in the 1-methyl-4-phenyl-1,2,3,6-tetrahydro...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-013-9416-0
更新日期:2013-12-01 00:00:00
abstract::Stroke is the leading cause of disability among adults as well as the 2nd leading cause of death globally. Ischemic stroke accounts for about 85% of strokes, and currently, tissue plasminogen activator (tPA), whose therapeutic window is limited to up to 4.5 h for the appropriate population, is the only FDA approved dr...
journal_title:Metabolic brain disease
pub_type: 杂志文章,评审
doi:10.1007/s11011-020-00573-8
更新日期:2020-08-01 00:00:00
abstract::Oxidative stress is strongly implicated in the pathogenesis of stroke. Strategies using antioxidants to improve neurological functions after stroke have, thus, gained significant attention. Ocimum basilicum L. is used traditionally to treat CNS disorders. Its antioxidant capacity is well established. Our laboratory ha...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-018-0215-5
更新日期:2018-08-01 00:00:00
abstract::The radiological spectrum of neuromyelitis optica has become broader since the detection of aquaporin4 antibodies. We report a case of neuromyelitis optica patient with pseudotumoral encephalic lesion. A 66 year-old woman presented with sudden left lateral homonymous hemianopsia. A brain MRI showed an isolated and ext...
journal_title:Metabolic brain disease
pub_type: 杂志文章
doi:10.1007/s11011-017-0141-y
更新日期:2018-02-01 00:00:00