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Hepatopathy

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https://www.readbyqxmd.com/read/28107167/activity-of-the-liver-enzyme-ornithine-carbamoyltransferase-otc-in-blood-lc-ms-ms-assay-for-non-invasive-diagnosis-of-ornithine-carbamoyltransferase-deficiency
#1
Jakub Krijt, Jitka Sokolová, Pavel Ješina, Lenka Dvořáková, Martin Řeboun, Katarína Brennerová, Martin Mistrík, Jiří Zeman, Tomáš Honzík, Viktor Kožich
BACKGROUND: Liver enzymes are released from hepatocytes into circulation and their activity can be measured in the blood. We examined whether the plasma activity of the liver enzyme ornithine carbamoyltransferase, determined by a novel liquid chromatography-mass spectrometry (LC-MS/MS) assay, could be utilized for the detection of OTC deficiency (OTCD), an X-linked inborn error of the urea cycle. METHODS: The plasma ornithine carbamoyltransferase (OTC) activity was assayed in the reverse reaction using isotopically labeled citrulline-d4 as a substrate and by determination of the product, ornithine-d4, by LC-MS/MS analysis...
January 20, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28097681/thromboelastography-in-dogs-with-chronic-hepatopathies
#2
W Fry, C Lester, N M Etedali, S Shaw, A DeLaforcade, C R L Webster
BACKGROUND: The coagulation status of dogs with liver disease is difficult to predict using conventional coagulation testing. HYPOTHESIS/OBJECTIVES: To evaluate thromboelastography (TEG) results and associations with conventional coagulation results and indicators of disease severity and prognosis in dogs with chronic hepatopathies (CH). ANIMALS: Twenty-one client-owned dogs. METHODS: Dogs with CH were prospectively (10 dogs) and retrospectively (11 dogs) enrolled from 2008 to 2014...
January 18, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28043933/clinical-biochemical-and-histopathology-features-of-patients-with-glycogenic-hepatopathy
#3
Saurabh Mukewar, Ayush Sharma, Kandace Lackore, Felicity Enders, Michael Torbenson, Patrick S Kamath, Lewis R Roberts, Yogish C Kudva
BACKGROUND & AIMS: Glycogenic hepatopathy, a syndrome characterized by hepatomegaly and increased liver transaminases in patients with type-1 diabetes, has not been well characterized in adults. We describe the clinical, biochemical, and histo-pathology profile of a cohort of patients with glycogenic hepatopathy. We also examined differences between patients with type-1 diabetes with vs without glycogenic hepatopathy. METHODS: We performed a case-control study of patients with type-1 diabetes diagnosed with glycogenic hepatopathy and patients with type-1 diabetes without glycogenic hepatopathy (controls)...
December 30, 2016: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28042636/glycogenic-hepatopathy-an-underdiagnosed-cause-of-relapsing-hepatitis-in-uncontrolled-type-1-diabetes-mellitus
#4
Ahmed A Al Sarkhy, Zafar A Zaidi, Amir M Babiker
Glycogenic hepatopathy is a rare condition that causes significant hepatomegaly and elevated liver enzyme levels in uncontrolled type 1 diabetic patients. It develops due to excessive accumulation of glycogen in the hepatocytes. It is typically reversible with good glycemic control and rarely progresses to mild fibrosis, but not cirrhosis.
January 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28029208/pulmonary-and-tricuspid-valvuloplasty-in-carcinoid-heart-disease
#5
Ashkan Karimi, Negiin Pourafshar, James C Fudge
A 26-year-old female with carcinoid heart disease consisting of severe pulmonary and tricuspid valve stenosis was admitted with line associated sepsis. She recovered from sepsis with antibiotics and aggressive fluid resuscitation but became grossly volume overloaded with evidence of tense ascites and lower extremity edema. She developed worsening renal and hepatic function due to congestive nephropathy and hepatopathy, which did not respond to intravenous diuretics, and she was deemed too sick for surgical pulmonary and tricuspid valve replacement...
December 28, 2016: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/28019064/novel-antidiabetic-medications-for-nonalcoholic-fatty-liver-disease-with-type-2-diabetes
#6
REVIEW
Yoshio Sumida, Yuya Seko, Masashi Yoneda
Liver related diseases are the leading causes of death in type 2 diabetes mellitus (T2DM) in Japan. T2DM is closely associated with nonalcoholic fatty liver disease (NAFLD) which is the most prevalent chronic liver disease worldwide. Nonalcoholic steatohepatitis (NASH), a severe form of NAFLD, can lead to hepatocellular carcinoma (HCC) and hepatic failure. NASH can be called "diabetic hepatopathy". There are no established pharmacotherapies for NAFLD/NASH patients with T2DM. Although metformin is established as the first-line therapy for T2DM, given its relative safety and beneficial effects on glycosylated hemoglobin (HbA1c), weight, and cardiovascular mortality, this agent is not recommended as specific therapy for NASH/NAFLD due to no clinical evidences...
December 26, 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/27995467/a-case-of-gastrojejunocolic-fistula-with-steatohepatitis
#7
Teppei Omori, Katsutoshi Tokushige, Fukiko Kinoshita, Ayumi Ito, Makiko Taniai, Mikiko Taneichi, Bunei Iizuka, Michio Itabashi, Yoji Nagashima, Masakazu Yamamoto, Shinichi Nakamura, Etsuko Hashimoto
A man in his 30s, who had undergone retrocolic Billroth II reconstruction for perforated duodenal ulcer, presented with watery diarrhea for 2 years and suspected fatty liver. He was referred to our hospital for management of chronic diarrhea, weight loss, hepatopathy and hypoalbuminemia. Initial upper and lower gastrointestinal endoscopies were negative. Since a small bowel lesion was suspected, peroral single-balloon enteroscopy was performed, which identified feces-like residue near the Billroth II anastomotic site and a connection to the colon separate from the afferent and efferent loops...
December 19, 2016: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/27914828/oleuropein-protects-l-02-cells-against-h2o2-induced-oxidative-stress-by-increasing-sod1-gpx1-and-cat-expression
#8
Ce Shi, Xiangrong Chen, Zuojia Liu, Rizeng Meng, Xingchen Zhao, Zonghui Liu, Na Guo
Oleuropein (OL), a natural phenolic compound, comprises the major constituent of Olea europea leaves and unprocessed olives, and OL is considered to be the principal components that confer the characteristic taste and stability of olive oil. Oxidative damage induced by H2O2 treatment can irreversibly damage the L-02 cells, resulting in cell death and apoptosis. Whether the effects of oxidative stress could be attenuated in cultured human L-02 cells by incubation with OL is still unknown. In this research, the function of OL in protecting human L-02 cells against H2O2 induced cell death and cell apoptosis was investigated, and the mechanism by which OL underlies the effect was also examed...
January 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/27906428/the-epidemiology-of-non-alcoholic-fatty-liver-disease-and-its-connection-with-cardiovascular-disease-role-of-endothelial-dysfunction
#9
A Federico, M Dallio, M Masarone, M Persico, C Loguercio
The non-alcoholic fatty liver disease is considered a predominant hepatopathy worldwide and a component of metabolic syndrome. It represents a risk factor for the development of cardiovascular diseases, independently of the presence of diabetes mellitus, hypertension and obesity. For this reason, nowadays an epidemiological analysis and a research of the causes that correlate non-alcoholic fatty liver disease and cardiovascular pathologies, are extremely useful. There are important epidemiological variations in relation to various geographical areas, and depending on different population groups, the prevalence of this pathology changes...
November 2016: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/27892863/nutrigenomic-activity-of-plant-derived-compounds-in-health-and-disease-results-of-a-dietary-intervention-study-in-dog
#10
Sandy Sgorlon, Bruno Stefanon, Misa Sandri, Monica Colitti
The study was conducted to investigate the effects of dietary administrations of four nutraceuticals in dogs. Seventy four dogs were enrolled in the trials, 24 healthy dogs were fed with a control diet (CT) and the experimental groups received for 60days the same diet supplemented with nutraceuticals, namely Echinacea angustifolia (EA, 0.10mg/kg live weight as echinacoside; 14 dogs), Vaccinium myrtillus (VM, 0.20mg/kg live weight as anthocyanidin, 13 dogs), Curcuma longa (CL, 6.60mg/kg live weight as curcumin, 18 dogs with arthrosis), and Sylibum marianum (SM, 1...
December 2016: Research in Veterinary Science
https://www.readbyqxmd.com/read/27875839/severe-metabolic-acidosis-and-hepatopathy-due-to-leukoencephalopathy-with-thalamus-and-brainstem-involvement-and-high-lactate
#11
Elizabeth A Sellars, Tonya Balmakund, Katherine Bosanko, Brandi L Nichols, Stephen G Kahler, Yuri A Zarate
Leukoencephalopathy with thalamus and brainstem involvement and high lactate (LTBL) is a recently described autosomal recessive mitochondrial disease characterized by early onset of neurological symptoms, a biphasic clinical course, and distinctive neuroimaging. Pathogenic variants in the EARS2 gene that encode for mitochondrial glutamyl-tRNA synthetase are responsible for LTBL. Here, we describe the clinical course of an infant diagnosed with an acute crisis of LTBL and severe liver disease. This article illustrates the utility of blood lactate quantification in addition to basic metabolic testing and brain imaging in a child with low tone and poor growth...
November 22, 2016: Neuropediatrics
https://www.readbyqxmd.com/read/27866557/-52-acute-toxic-hepatopathy-cases-induced-by-tetrachloromethane-combined-organic-solvent
#12
Y L Kong, Y W Chen, C Y Min
No abstract text is available yet for this article.
September 20, 2016: Chinese Journal of Industrial Hygiene and Occupational Diseases
https://www.readbyqxmd.com/read/27854233/cysteine-supplementation-may-be-beneficial-in-a-subgroup-of-mitochondrial-translation-deficiencies
#13
Marina Bartsakoulia, Juliane S Mϋller, Aurora Gomez-Duran, Patrick Yu Wai Man, Veronika Boczonadi, Rita Horvath
BACKGROUND: Mitochondrial encephalomyopathies are severe, relentlessly progressive conditions and there are very few effective therapies available to date. We have previously suggested that in two rare forms of reversible mitochondrial disease (reversible infantile respiratory chain deficiency and reversible infantile hepatopathy) supplementation with L-cysteine can improve mitochondrial protein synthesis, since cysteine is required for the 2-thiomodification of mitochondrial tRNAs. OBJECTIVES: We studied whether supplementation with L-cysteine or N-acetyl-cysteine (NAC) results in any improvement of the mitochondrial function in vitro in fibroblasts of patients with different genetic forms of abnormal mitochondrial translation...
August 30, 2016: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/27853829/-hepatopancreaticobiliary-diseases-in-igg4-associated-autoimmune-diseases
#14
L Grenacher
BACKGROUND: IgG4-related diseases are rare systemic multiorgan diseases and can thus affect any organ system. The incidence of diagnosis has significantly increased due to increasing awareness. OBJECTIVE: In the abdomen the hepatopancreaticobiliary system provides an essential organ system for the expression of IgG4-associated autoimmune diseases. The focus here is autoimmune pancreatitis type 1 but IgG4-associated sclerosing cholangitis and IgG4-associated hepatopathy, which can also occur in combination are less well-known...
December 2016: Der Radiologe
https://www.readbyqxmd.com/read/27809283/effect-of-high-carbohydrate-diet-on-plasma-metabolome-in-mice-with-mitochondrial-respiratory-chain-complex-iii-deficiency
#15
Jayasimman Rajendran, Nikica Tomašić, Heike Kotarsky, Eva Hansson, Vidya Velagapudi, Jukka Kallijärvi, Vineta Fellman
Mitochondrial disorders cause energy failure and metabolic derangements. Metabolome profiling in patients and animal models may identify affected metabolic pathways and reveal new biomarkers of disease progression. Using liver metabolomics we have shown a starvation-like condition in a knock-in (Bcs1l(c.232A>G)) mouse model of GRACILE syndrome, a neonatal lethal respiratory chain complex III dysfunction with hepatopathy. Here, we hypothesized that a high-carbohydrate diet (HCD, 60% dextrose) will alleviate the hypoglycemia and promote survival of the sick mice...
November 1, 2016: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/27801339/-the-prevalence-and-clinical-features-of-fulminant-type-1-diabetes
#16
Z Q Li, J P Zhang
Objective: To characterize the clinic features of fulminant type 1 diabetes mellitus(FT1DM). Methods: It was a retrospective study analyzing the clinical and laboratory data of patients diagnosed as FT1DM and type 1 diabetes in the Department of Endocrinology in China-Japan Friendship Hospital from January 2011 to November 2015. Results: A total of 125 newly diagnosed type 1 diabetic patients were included in the study, 14 of them (11.2%) met with the criteria of FT1DM. The age at onset was (35.8±8.3) years...
November 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/27796022/-treatment-of-autosomal-dominant-polycystic-kidney-disease-adpkd-tolvaptan
#17
Massimo Cirillo
The European Medicines Agency approved tolvaptan to slow cyst growth and renal failure progression in adults with ADPKD, glomerular filtration 60 mL/min x 1.73 m2 and rapidly progressive disease. In a multicenter 3-year study, conducted on 1,445 patients with non-genotyped ADPKD, ages 18-50 years, predicted creatinine clearance 60 mL/min and kidney total volume 750 mL, tolvaptan slowed kidney failure progression (-23%-46% for different objectives) and reduced kidney volume increase and pain without effects on hypertension and albuminuria...
September 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/27790307/primary-signet-ring-cell-carcinoma-of-the-prostate-treated-by-radical-cystoprostatectomy-and-chemoradiotherapy
#18
Sun Wook Kim, Woohyun Kim, Yong-Hyun Cho, Tae-Jung Kim, Insuk Woo, Dong Wan Sohn
Primary signet ring cell carcinoma (SRCC) of the prostate is very rare. Although SRCC is primarily found in the stomach and colon, it can also be found in the pancreas, breast, thyroid, bladder, and prostate. We recently diagnosed and treated a case of primary SRCC of the prostate. A 56-year-old Korean man was referred to our institution for evaluation of a one-month history of hematuria and recently identified bladder mass. Transurethral resection of the bladder tumour was performed and histological and immunohistochemical evaluation revealed a diagnosis of SRCC with tumour invading into the outer half of the deep muscularis propria...
May 2016: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/27742550/transient-elastography-as-a-noninvasive-assessment-tool-for-hepatopathies-of-different-etiology-in-pediatric-type-1-diabetes-mellitus
#19
Zeinab A Elkabbany, Nancy S Elbarbary, Eman A Ismail, Nesrine A Mohamed, Dina Ragab, Shereen Abdel Alem, Yasmine M Ezzat, Sarah S Maurice, Noha U Hashem
AIM: To identify the prevalence and effect of hepatopathies of different etiologies among pediatric patients with type 1 diabetes mellitus (T1DM) using transient elastography (TE) and its relation to glycemic control. METHODS: One hundred T1DM patients were studied focusing on liver functions, fasting lipid profile, hemoglobin A1c (HbA1c), hepatitis C virus (HCV), serum immunoglobulins, autoimmune antibodies; anti-nuclear antibody (ANA), anti-smooth muscle antibody (ASMA), and anti-liver kidney microsomal antibody (anti-LKM)...
January 2017: Journal of Diabetes and its Complications
https://www.readbyqxmd.com/read/27737396/transient-hyperphosphatemia-a-benign-laboratory-disorder-in-a-boy-with-gitelman-syndrome
#20
Sylva Skalova, Stepan Kutilek
Transient hyperphosphatasemia of infancy and early childhood (THI) is characterized by transiently increased activity of serum alkaline phosphatase (S-ALP), predominantly its bone or liver isoform, in children under five years of age. There are no signs of metabolic bone disease or hepatopathy corresponding with the increased S-ALP. THI is benign disorder, rather laboratory than clinical disorder, which is usually accidentally detected in both healthy and sick children. When encountered in a child with either chronic bone, liver or kidney disease, it might concern the physician...
July 2016: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
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