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https://www.readbyqxmd.com/read/28433114/hepatic-features-of-wilson-disease
#1
Salih Boga, Aftab Ala, Michael L Schilsky
In Wilson disease (WD) defective AT7B function leads to biliary copper excretion and pathologic copper accumulation, particularly in liver and brain, where it induces cellular damage. Liver disease most often precedes neurologic or psychiatric manifestations. In most patients with neurologic or psychiatric symptoms there is some degree of liver disease at the time of disease presentation. Hepatic manifestations of WD can be extremely variable. Patients with clinically asymptomatic WD are often found by family screening or identified on routine laboratory testing...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28288025/coffee-and-cancer-risk-a-summary-overview
#2
Gianfranco Alicandro, Alessandra Tavani, Carlo La Vecchia
We reviewed available evidence on coffee drinking and the risk of all cancers and selected cancers updated to May 2016. Coffee consumption is not associated with overall cancer risk. A meta-analysis reported a pooled relative risk (RR) for an increment of 1 cup of coffee/day of 1.00 [95% confidence interval (CI): 0.99-1.01] for all cancers. Coffee drinking is associated with a reduced risk of liver cancer. A meta-analysis of cohort studies found an RR for an increment of consumption of 1 cup/day of 0.85 (95% CI: 0...
March 10, 2017: European Journal of Cancer Prevention
https://www.readbyqxmd.com/read/28284465/neurological-disorders-in-liver-transplant-candidates-pathophysiology-and-clinical-assessment
#3
REVIEW
Paolo Feltracco, Annachiara Cagnin, Cristiana Carollo, Stefania Barbieri, Carlo Ori
Compromised liver function, as a consequence of acute liver insufficiency or severe chronic liver disease may be associated with various neurological syndromes, which involve both central and peripheral nervous system. Acute and severe hyperammoniemia inducing cellular metabolic alterations, prolonged state of "neuroinflammation", activation of brain microglia, accumulation of manganese and ammonia, and systemic inflammation are the main causative factors of brain damage in liver failure. The most widely recognized neurological complications of serious hepatocellular failure include hepatic encephalopathy, diffuse cerebral edema, Wilson disease, hepatic myelopathy, acquired hepatocerebral degeneration, cirrhosis-related Parkinsonism and osmotic demyelination syndrome...
February 27, 2017: Transplantation Reviews
https://www.readbyqxmd.com/read/28283349/aav-gene-therapy-corrects-otc-deficiency-and-prevents-liver-fibrosis-in-aged-otc-knock-out-heterozygous-mice
#4
Lili Wang, Peter Bell, Hiroki Morizono, Zhenning He, Elena Pumbo, Hongwei Yu, John White, Mark L Batshaw, James M Wilson
Ornithine transcarbamylase (OTC) deficiency is an X-linked disorder of the urea cycle. Hemizygous males and heterozygous females may experience life-threatening elevations of ammonia in blood and brain, leading to irreversible cognitive impairment, coma, and death. Recent evidence of acute liver failure and fibrosis/cirrhosis is also emerging in OTC-deficient patients. Here, we investigated the long-term consequences of abnormal ureagenesis in female mice heterozygous (Het) for a null mutation in the OTC gene...
March 2, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28283328/carnosine-ameliorates-liver-fibrosis-and-hyperammonemia-in-cirrhotic-rats
#5
Akram Jamshidzadeh, Reza Heidari, Zahra Latifpour, Mohammed Mehdi Ommati, Narges Abdoli, Somayeh Mousavi, Negar Azarpira, Azita Zarei, Mahdi Zarei, Behnam Asadi, Mojgan Abasvali, Yasaman Yeganeh, Faezeh Jafari, Arastoo Saeedi, Asma Najibi, Elnaz Mardani
AIM: Chronic liver injury and cirrhosis leads to liver failure. Hyperammonemia is a deleterious consequence of liver failure. On the other hand, oxidative stress seems to play a pivotal role in the pathogenesis of liver fibrosis as well as in the cytotoxic mechanism of ammonia. There is no promising therapeutic agent against ammonia-induced complications. The present study was conducted to evaluate the role of carnosine (CA) administration on liver pathological changes, elevated plasma ammonia, and its consequent events in cirrhotic rats...
March 7, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/28266292/two-and-a-half-hours-of-cardiopulmonary-resuscitation-in-a-deceased-brain-dead-donor-before-liver-transplantation-a-good-idea-to-accept
#6
Dieter P Hoyer, Gernot M Kaiser, Andreas Paul, Nikolaos Machairas, Ernesto P Molmenti, Georgios C Sotiropoulos
The sequelae of cardiac arrest (CA) and cardiopulmonary resuscitation (CPR) in organ donors potentially results in ischemic organ injury and graft dysfunction after transplantation. Thresholds of resuscitation times in brain dead liver donors have not been established so far. We report the case of a brain dead liver donor who experienced 2.5 hours of CPR whose liver was successfully transplanted. A 75-year-old male experienced CA and was treated by CPR with streptokinase application for 2.5 hours until stabilization of cardiac function...
January 2017: Chirurgia
https://www.readbyqxmd.com/read/28212619/brain-and-liver-pathology-amyloid-deposition-and-interferon-responses-among-older-hiv-positive-patients-in-the-late-haart-era
#7
Isaac H Solomon, Umberto De Girolami, Sukrutha Chettimada, Vikas Misra, Elyse J Singer, Dana Gabuzda
BACKGROUND: HIV+ patients on highly active antiretroviral therapy (HAART) with suppressed viral loads have a low incidence of HIV-associated dementia, but increased prevalence of milder forms of HIV-associated neurocognitive disorders (HAND). These milder forms of HAND are often associated with minimal histological abnormalities, and their pathophysiology is unclear. Comorbidities, altered amyloid metabolism, accelerated brain aging, and activated interferon responses are suspected to play a role in HAND pathogenesis in HAART-treated persons...
February 17, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28123500/effects-of-low-dose-tolvaptan-on-electrolyte-abnormality-and-hemodynamic-parameters-in-a-liver-cirrhosis-associated-portopulmonary-hypertension-patient-a-case-report
#8
Yoshito Ogihara, Norikazu Yamada, Kaoru Dohi, Akimasa Matsuda, Satoshi Ota, Ken Ishikura, Mashio Nakamura, Masaaki Ito
The present study reported a case of portopulmonary hypertension (POPH) that was secondary to underlying liver cirrhosis in a 58-year-old woman, who was successfully treated with low-dose tolvaptan. The patient had suffered from refractory peripheral edema and electrolyte abnormalities, including severe hypokalemia, under the combination therapy of sildenafil, ambrisentan, furosemide and spironolactone. Subsequent to the initiation of low-dose tolvaptan at 3.75 mg/day with concurrent de-escalation of the dose of furosemide, the daily urine volume increased, peripheral edema improved and the serum potassium level increased immediately...
January 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28108838/clinical-impact-of-preoperative-brain-mr-angiography-and-mr-imaging-in-candidates-for-liver-transplantation-a-propensity-score-matching-study-in-a-single-institution
#9
Mi Sun Chung, Ho Sung Kim, Young-Suk Lim, Sang-Beom Jeon, Seon-Ok Kim, Hwa Jung Kim, Shin Hwang, Seung Chai Jung, Choong Gon Choi, Sang Joon Kim
OBJECTIVES: To investigate the prevalence of cerebrovascular stenosis and white matter lesions on preoperative magnetic resonance angiography (MRA) and magnetic resonance imaging (MRI) in liver transplantation candidates. METHODS: This retrospective study included 1,460 consecutive patients with liver cirrhosis (LC) who underwent MRA with/without brain MRI for pretransplantation evaluation. These patients were matched with 5,331 controls using propensity scores, and the prevalences of significant cerebrovascular stenosis and white matter lesions were compared...
January 20, 2017: European Radiology
https://www.readbyqxmd.com/read/28092846/status-epilepticus-in-patients-with-cirrhosis-how-to-avoid-misdiagnosis-in-patients-with-hepatic-encephalopathy
#10
Marika Rudler, Clémence Marois, Nicolas Weiss, Dominique Thabut, Vincent Navarro
PURPOSE: Status epilepticus (SE) in patients with cirrhosis is a rare but serious situation. Diagnosis may be difficult in emergency presentation, especially when patients present with hepatic encephalopathy (HE). Misdiagnosis must be avoided since some anti-epileptic drugs aggravate HE. In this retrospective study, we therefore assessed the frequency of SE in patients with cirrhosis, evaluated the accuracy of diagnosis and determined rates of mortality. METHOD: We reviewed data from all patients hospitalized from 2005 to 2013 in the Hepatology ICU for complications of cirrhosis with an initial diagnosis of SE...
February 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28057091/-resting-state-functional-magnetic-resonance-study-of-brain-function-changes-after-tips-operation-in-patients-with-liver-cirrhosis
#11
C Liu, H B Wang, Y Q Yu, M Q Wang, G B Zhang, L Y Xu, J M Wu
Objective: To investigate the brain function changes in cirrhosis patients after transjugular intrahepatic portosystemic shunt (TIPS), resting-state functional MRI (rs-fMRI) performed and fractional amplitude of low frequency fluctuation (fALFF) was analyzed. Methods: From January 2014 to February 2016, a total of 96 cirrhotic patients from invasive technology department and infection department in the First Affiliated Hospital of Anhui Medical University were selected , the blood ammonia data of 96 cirrhotic patients with TIPS operation in four groups were collected after 1, 3, 6 and 12 month, and all subjects performed rs-fMRI scans...
December 20, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28025030/apelin-apj-system-a-novel-promising-therapy-target-for-pathological-angiogenesis
#12
REVIEW
Lele Wu, Linxi Chen, Lanfang Li
Apelin is the endogenous ligand of the G protein-coupled receptor APJ. Both Apelin and APJ receptor are widely distributed in various tissues such as heart, brain, limbs, retina and liver. Recent research indicates that the Apelin/APJ system plays an important role in pathological angiogenesis which is a progress of new blood branches developing from preexisting vessels via sprouting. In this paper, we review the important role of the Apelin/APJ system in pathological angiogenesis. The Apelin/APJ system promotes angiogenesis in myocardial infarction, ischemic stroke, critical limb ischemia, tumor, retinal angiogenesis diseases, cirrhosis, obesity, diabetes and other related diseases...
March 2017: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/28002032/hyponatremia-unfavourable-prognostic-factor-in-hepatic-cirrhosis
#13
Aurelia Enescu, F Petrescu, P Mitruţ, V Pădureanu, Octavia Ileana Petrescu, Valeria Carmen Albu, Andreea Larisa Moraru, Anca Ştefania Enescu
Hyponatremia is defined by a level of Na in serum below or equal to 136 mEq/L while in hepatic cirrhosis it is classically considered as relevant only at a level of Na below 130 mEq/L. Hyponatremia frequently occurs in patients with end-stage hepatic disease. The frequency and severity are variable but it has been estimated that it occurs with a frequency of 57% in hospitalized patients with cirrhosis and in those on waiting lists for hepatic transplants. Signs and symptoms of hyponatremia are related to dysfunctions of the central nervous system, due to migration of the water from intravascular space to the brain cells, resulting in the occurrence of cerebral edema...
December 1, 2016: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/27995280/dietary-fructose-as-a-risk-factor-for-non-alcoholic-fatty-liver-disease-nafld
#14
REVIEW
Salamah Mohammad Alwahsh, Rolf Gebhardt
Glucose is a major energy source for the entire body, while fructose metabolism occurs mainly in the liver. Fructose consumption has increased over the last decade globally and is suspected to contribute to the increased incidence of non-alcoholic fatty liver disease (NAFLD). NAFLD is a manifestation of metabolic syndrome affecting about one-third of the population worldwide and has progressive pathological potential for liver cirrhosis and cancer through non-alcoholic steatohepatitis (NASH). Here we have reviewed the possible contribution of fructose to the pathophysiology of NAFLD...
April 2017: Archives of Toxicology
https://www.readbyqxmd.com/read/27981407/the-bile-duct-ligated-rat-a-relevant-model-to-study-muscle-mass-loss-in-cirrhosis
#15
Cristina R Bosoi, Mariana M Oliveira, Rafael Ochoa-Sanchez, Mélanie Tremblay, Gabriella A Ten Have, Nicolaas E Deutz, Christopher F Rose, Chantal Bemeur
Muscle mass loss and hepatic encephalopathy (complex neuropsychiatric disorder) are serious complications of chronic liver disease (cirrhosis) which impact negatively on clinical outcome and quality of life and increase mortality. Liver disease leads to hyperammonemia and ammonia toxicity is believed to play a major role in the pathogenesis of hepatic encephalopathy. However, the effects of ammonia are not brain-specific and therefore may also affect other organs and tissues including muscle. The precise pathophysiological mechanisms underlying muscle wasting in chronic liver disease remains to be elucidated...
April 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/27923694/neuropsychiatric-performance-in-patients-with-cirrhosis-who-is-normal
#16
REVIEW
Sara Montagnese, Michele De Rui, Paolo Angeli, Piero Amodio
In patients with cirrhosis a normal neuropsychiatric performance has been traditionally defined by the absence of any degree of hepatic encephalopathy and/or the absence of psychometric or neurophysiological abnormalities, compared with data from the healthy population. As the understanding and management of end-stage liver disease continues to change, it is our impression that the concept of normal neuropsychiatric performance also needs updating. This review explores novel and more pragmatic interpretations of neuropsychiatric "normality" compared with top personal performance, in terms of risk of overt hepatic encephalopathy or brain failure and in relation with events such as liver transplantation, decompensation, acute-on-chronic liver failure and transjugular intrahepatic portosystemic shunt placement...
December 5, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27922089/elderly-patients-have-an-altered-gut-brain-axis-regardless-of-the-presence-of-cirrhosis
#17
Jasmohan S Bajaj, Vishwadeep Ahluwalia, Joel L Steinberg, Sarah Hobgood, Peter A Boling, Michael Godschalk, Saima Habib, Melanie B White, Andrew Fagan, Edith A Gavis, Dinesh Ganapathy, Phillip B Hylemon, Karen E Stewart, Raffi Keradman, Eric J Liu, Jessica Wang, Patrick M Gillevet, Masoumeh Sikaroodi, F Gerard Moeller, James B Wade
Cognitive difficulties manifested by the growing elderly population with cirrhosis could be amnestic (memory-related) or non-amnestic (memory-unrelated). The underlying neuro-biological and gut-brain changes are unclear in this population. We aimed to define gut-brain axis alterations in elderly cirrhotics compared to non-cirrhotic individuals based on presence of cirrhosis and on neuropsychological performance. Age-matched outpatients with/without cirrhosis underwent cognitive testing (amnestic/non-amnestic domains), quality of life (HRQOL), multi-modal MRI (fMRI go/no-go task, volumetry and MR spectroscopy), blood (inflammatory cytokines) and stool collection (for microbiota)...
December 6, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27919127/-deceased-donor-kidney-transplantation-from-a-liver-transplantation-recipient
#18
Shuhei Koike, Takashi Kobayashi, Yoshiyuki Okada, Shinsuke Shibuya, Kaoru Sakai, Yukari Tanaka, Shusuke Akamatsu, Hiromitsu Negoro, Naoki Terada, Toshinari Yamasaki, Yoshiyuki Matsui, Takahiro Inoue, Tomomi Kamba, Yumi Umeya, Toshimi Kaido, Osamu Ogawa
We report a 40-year-old man with end-stage renal disease due to IgA nephropathy who underwent deceased donor kidney transplantation. The donor was diagnosed to be brain-dead due to cerebral hemorrhage after her second liver transplantation for non-viral liver cirrhosis. Intraoperative 1-hour biopsy of the graft kidney revealed moderate global glomerular sclerosis (22%) and interstitial fibrosis (40%) consistent with underlying nephrosclerosis or calcineurin inhibitor nephrotoxicity. Although hemodialysis was needed until the graft began functioning several days after the kidney transplantation, the postoperative clinical course thereafter was uneventful and the graft functioned well with stable serum creatinine levels around 2...
October 2016: Hinyokika Kiyo. Acta Urologica Japonica
https://www.readbyqxmd.com/read/27914003/n-terminal-pro-b-type-natriuretic-peptide-levels-are-linked-with-modified-child-pugh-classification-in-patients-with-nonalcoholic-cirrhosis-nt-probnp-and-liver-cirrhosis
#19
Abdulbaki Kumbasar, Mehtap Navdar, Esra Ataoglu, Isil Uzunhasan, Kadir Ergen, Sule Poturoglu, Filiz Basinoglu, Fatih Yilmaz, Mustafa Yenigun, Fuat Sar, Ozgur Tanriverdi
Excess N-terminal pro-brain natriuretic peptide secretion has been linked to cirrhosis in previously studies. The relationship of plasma N-terminal pro-brain natriuretic peptide levels and cardiac dysfunction determined by echocardiography were investigated in patients with nonalcoholic cirrhosis and a control group of chronic hepatitis. This study was designed as a cross-sectional study. Thirty-two men and thirty-three women who gave informed consent who were followed-up for chronic liver failure were enrolled...
March 2017: Cell Biochemistry and Biophysics
https://www.readbyqxmd.com/read/27895414/hepatic-encephalopathy-ever-closer-to-its-big-bang
#20
REVIEW
Pablo A Souto, Ariel R Marcotegui, Lisandro Orbea, Juan Skerl, Juan Carlos Perazzo
Hepatic encephalopathy (HE) is a neuropsychiatric disorder that commonly complicates the course of patients with liver disease. Despite the fact that the syndrome was probably first recognized hundreds of years ago, the exact pathogenesis still remains unclear. Minimal hepatic encephalopathy (MHE) is the earliest form of HE and is estimated to affect more that 75% of patients with liver cirrhosis. It is characterized by cognitive impairment predominantly attention, reactiveness and integrative function with very subtle clinical manifestations...
November 14, 2016: World Journal of Gastroenterology: WJG
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