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https://www.readbyqxmd.com/read/28644566/clinical-characteristics-and-outcome-of-liver-transplantation-for-alagille-syndrome-in-children
#1
Tao Zhou, Jianjun Zhang, Yi Luo, Yuan Liu, Sy Zhuang, Feng Xue, Longzhi Han, Qiang Xia
AIM: The aim of this study was to analyze clinical characteristics and outcome of liver transplantation for Alagille syndrome in children. METHOD: By retrospectively reviewing the medical records of 9 Alagille syndrome patients(AGS) with liver transplantation(LT) in Renji Hospital between 2006 and 2015,After operative,the height and weight Z score were compared with preoperative. RESULT: There were 9 patients included in the study; cholestasis and peculiar faces were seen in all of the patients (100%), heart defect in 8 patients (88...
June 23, 2017: Journal of Digestive Diseases
https://www.readbyqxmd.com/read/28644367/oral-cholic-acid-is-efficacious-and-well-tolerated-in-patients-with-bile-acid-synthesis-and-zellweger-spectrum-disorders
#2
James E Heubi, Kevin E Bove, Kenneth D R Setchell
BACKGROUND/AIMS: Patients with bile acid synthesis disorders (BASD) due to single enzyme defects (SED) or Zellweger spectrum disorders (ZSD) accumulate hepatotoxic atypical bile acids resulting in potentially fatal progressive liver disease. We evaluated the efficacy and safety of oral cholic acid in patients with BASD. METHODS: In this phase 3, open-label, single-arm, nonrandomized, noncomparative study conducted over 18 years, patients were administered cholic acid orally 10 to 15 mg/kg/day...
June 21, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28644356/low-hepatic-tissue-copper-in-pediatric-nonalcoholic-fatty-liver-disease
#3
Michael Mendoza, Shelley Caltharp, Ming Song, Lindsay Collin, Juna V Konomi, Craig J McClain, Miriam B Vos
OBJECTIVE: Animal models and studies in adults have demonstrated that copper restriction increases severity of liver injury in nonalcoholic fatty liver disease (NAFLD). This has not been studied in children. We aimed to determine if lower tissue copper is associated with increased NAFLD severity in children. METHODS: This was a retrospective study of pediatric patients who had a liver biopsy including a hepatic copper quantitation. The primary outcome compared hepatic copper concentration in NAFLD versus non-NAFLD...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28644197/preoperative-regional-maximal-removal-rate-of-technetium-99m-galactosyl-human-serum-albumin-is-correlated-with-liver-functional-parameters-but-did-not-predict-postoperative-morbidity
#4
Koichi Yano, Atsushi Nanashima, Yoshiro Fujii, Masahide Hiyoshi, Naoya Imamura, Takeomi Hamada, Yuki Tsuchimochi, Takashi Wada, Yoichi Mizutani, Toshinori Hirai
BACKGROUND: Recently, posthepatectomy complications have been predicted by advances of new liver functional parameters, and the technetium-99m-galactosyl human serum albumin (GSA) liver scintigraphy has been applied widely for this purpose. We evaluated the clinical significance of the regional maximal removal rate (GSA-Rmax) in patients with various liver diseases as predictors of posthepatectomy complications. PATIENTS AND METHODS: Between 2012 and March 2016, we examined 200 patients who underwent hepatectomy for liver and biliary diseases...
June 22, 2017: Nuclear Medicine Communications
https://www.readbyqxmd.com/read/28643460/periodic-limb-movement-in-sleep-and-sickle-cell-disease-a-neglected-association
#5
Ana C Cabañas-Pedro, Suely Roizenblatt, Altay A L de Souza, Sérgio Tufik, Maria S Figueiredo
High frequency of periodic limb movements in sleep (PLMS) has been described among children with sickle cell disease (SCD), but there is little information about PLMS among adults with SCD. We aim to determine the frequency of PLMS among adults with SCD and to identify possible associations with iron status and haemolytic parameters. We analysed polysomnography on 99 adults: 74 with sickle cell anaemia (HbSS), 19 with HbSC (double heterozygosis HbS and HbC) and 6 with HbS-beta thalassaemia. Laboratory data were collected close to the time of the polysomnography examination...
June 23, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28643313/-anastomotic-leakage-after-laparoscopic-assisted-radical-right-hemicolectomy-reason-analysis-and-management
#6
Xin Wu, Guole Lin, Huizhong Qiu, Yi Xiao, Bin Wu
OBJECTIVE: To analyze the clinical features, possible reasons and management of anastomotic leakage after laparoscopic-assisted radical right hemicolectomy. METHODS: Clinical data of 546 patients undergoing laparoscopic-assisted radical right hemicolectomy in Peking Union Medical College Hospital from October 2010 to September 2016 were retrospectively analyzed. The occurrence of anastomotic leakage and its countermeasures were evaluated. RESULTS: Among 546 patients, 8(1...
June 25, 2017: Zhonghua Wei Chang Wai Ke za Zhi, Chinese Journal of Gastrointestinal Surgery
https://www.readbyqxmd.com/read/28642302/residual-erythropoiesis-protects-against-myocardial-hemosiderosis-in-transfusion-dependent-thalassemia-by-lowering-labile-plasma-iron-via-transient-generation-of-apotransferrin
#7
Maciej W Garbowski, Patricia Evans, Evangelia Vlachodimitropoulou, Robert Hider, John B Porter
Cardiosiderosis is a leading cause of mortality in transfusion-dependent thalassemias. Plasma non-transferrin-bound iron and its redox-active component, labile plasma iron, are key sources of iron loading in cardiosiderosis. Risk factors were identified in 73 patients with or without cardiosiderosis. Soluble transferrin receptor-1 levels were significantly lower in patients with cardiosiderosis (odds ratio 21). This risk increased when transfusion-iron loading rates exceeded the erythroid transferrin uptake rate (derived from soluble transferrin receptor-1) by >0...
June 22, 2017: Haematologica
https://www.readbyqxmd.com/read/28641659/-experimental-study-on-neonatal-abo-or-rhd-compatible-blood-transfusion
#8
Wei Liu, Ai-Ping LE, Jing-Han Liu, Jiong-Cai Lan
OBJECTIVE: To investigate the safety and effectiveness of neonatal ABO or Rh(D) by using compatible blood transfusion through retrospective analysis of data from cases received compatible blood transfusion and type matched blood transfusion. METHODS: The clinical data of 26 cases of neonatal compatible blood transfusion in Chinese Nanchang area from January 2014 to October 2016 were collected, and 26 cases of neonatal type-matched blood transfusion were selected according to ratio of 1:1 cases...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28640108/correlation-of-hla-dp-dq-polymorphisms-with-transplant-etiologies-and-prognosis-in-liver-transplant-recipients
#9
Yi Li, Qian Huang, Jiang-Tao Tang, Tian-Tian Wei, Lin Yan, Zhi-Qiang Yang, Yang-Juan Bai, Lan-Lan Wang, Yun-Ying Shi
Previous study has identified that the genetic variants in the human leukocyte antigen (HLA)-DP/DQ region were strongly associated with hepatitis B virus (HBV) infection. But their roles in liver function recovery after hepatic transplantation were still obscure. This study aimed to investigate whether HLA-DP/DQ polymorphisms were associated with post-transplant etiologies and prognosis in Chinese liver transplant recipients.A total of 144 liver transplant recipients were enrolled, which were divided into 2 groups according to the transplant etiology: HBV-related disease and non-HBV-related disease...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28640024/molecular-adsorbent-recirculating-system-can-reduce-short-term-mortality-among-patients-with-acute-on-chronic-liver-failure-a-retrospective-analysis
#10
Hans U Gerth, Michele Pohlen, Gerold Thölking, Hermann Pavenstädt, Marcus Brand, Anna Hüsing-Kabar, Christian Wilms, Miriam Maschmeier, Iyad Kabar, Josep Torner, Marco Pavesi, Vicente Arroyo, Rafael Banares, Hartmut H J Schmidt
OBJECTIVES: Acute-on-chronic liver failure is associated with numerous consecutive organ failures and a high short-term mortality rate. Molecular adsorbent recirculating system therapy has demonstrated beneficial effects on the distinct symptoms, but the associated mortality data remain controversial. DESIGN: Retrospective analysis of acute-on-chronic liver failure patients receiving either standard medical treatment or standard medical treatment and molecular adsorbent recirculating system...
June 21, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28639379/the-in-vitro-and-in-vivo-biological-activities-of-the-leaf-of-cape-myrtle-myrsine-africana-l
#11
Hina Gul, Mushtaq Ahmad, Muhammad Zafar, M Shareez Ahmad, AmbreenAbid, Syeda Hira, Imam Shah, Muhammad Gulfraz
The cape myrtle, Myrsine africana L., is a widely used medicinal plant, which has not been well investigated. We assessed the in vivo hepatoprotective and in vitro antiproliferative and antioxidant effects of leaf extracts of M. africana chemically profiled using high-performance liquid chromatography. Three flavonoids were quantified, and gas chromatography-mass spectrometry analysis revealed the presence of common fatty acids. The animal study was conducted on mice treated with CCl4, using three doses each of the methanol and chloroform extract (100, 200 and 300 mg/kg b...
June 22, 2017: Phytotherapy Research: PTR
https://www.readbyqxmd.com/read/28639200/treatment-of-liver-cancer-by-c-ebpa-sarna
#12
Xiaoyang Zhao, Jon Voutila, Stephanos Ghobrial, Nagy A Habib, Vikash Reebye
The prognosis for hepatocellular carcinoma (HCC) remains poor and has not improved in over two decades. Most patients with advanced HCC who are not eligible for surgery have limited treatment options due to poor liver function or large, unresectable tumors. Although sorafenib is the standard-of-care treatment for these patients, only a small number respond. For the remaining, the outlook remains bleak. A better approach to target "undruggable" molecular pathways that reverse HCC is therefore urgently needed...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28638943/elevated-serum-heat-shock-protein-70-and-liver-stiffness-reflect-hepatic-dysfunction-and-severity-in-postoperative-biliary-atresia
#13
Sittisak Honsawek, Wanvisa Udomsinprasert, Napaphat Jirathanathornnukul, Voranush Chongsrisawat, Yong Poovorawan
BACKGROUND: Biliary atresia (BA) is a severe chronic liver disease characterized by progressive obstructive cholangiopathy of biliary tract. Heat shock protein 70 (HSP70) is involved in protecting cells against a wide variety of stress and plays a protective role in tissue damage. The purpose of this study was to investigate serum HSP70 and liver stiffness in BA and determine the association of serum HSP70, liver stiffness, and outcome parameters in post-Kasai BA patients. METHODS: One hundred post-Kasai BA patients and 40 controls were enrolled...
June 21, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28638221/prevalence-of-ifnl3-rs4803217-single-nucleotide-polymorphism-and-clinical-course-of-chronic-hepatitis-c
#14
Bogna Świątek-Kościelna, Ewelina Kałużna, Ewa Strauss, Jerzy Nowak, Iwona Bereszyńska, Ewelina Gowin, Jacek Wysocki, Jolanta Rembowska, Dominika Barcińska, Iwona Mozer-Lisewska, Danuta Januszkiewicz-Lewandowska
AIM: To evaluate the association of IFNL3 (IL28B) SNP rs4803217 with severity of disease and treatment outcome in chronic hepatitis C (CHC). METHODS: The study enrolled 196 CHC Polish patients (82 women and 114 men in age 20-64) infected with hepatitis C virus (HCV) genotype 1. They were treatment naïve and qualified to pegylated interferon alpha (PEG-IFN-α) and ribavirin (RBV) therapy. The analyzed baseline parameters included: degree of inflammation, stage of fibrosis, viral load as well as alanine aminotransferase (ALT), asparagine aminotransferase (AST) and total bilirubin (TBIL)...
June 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28637874/direct-demonstration-of-a-neonatal-fc-receptor-fcrn-driven-endosomal-sorting-pathway-for-cellular-recycling-of-albumin
#15
Esben G W Schmidt, Michael L Hvam, Filipa Antunes, Jason Cameron, Dorthe Viuff, Birgitte Andersen, Nanna N Kristensen, Kenneth A Howard
Albumin is the most abundant plasma protein involved in the transport of many compounds, such as fatty acids, bilirubin, and heme. The endothelial cellular neonatal Fc receptor (FcRn) has been suggested to play a central role in maintaining the high albumin plasma levels through a cellular recycling pathway. However, direct mapping of this process is still lacking. This work presents the use of wild type and engineered recombinant albumins with either decreased or increased FcRn affinity in combination with a low or high FcRn-expressing endothelium cell line to clearly define the FcRn involvement, intracellular pathway and kinetics of albumin trafficking by flow cytometry, quantitative confocal microscopy, and an albumin-recycling assay...
June 21, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28635609/gastroprotective-effect-of-ghrelin-against-indomethacin-induced-gastric-injury-in-rats-possible-role-of-heme-oxygenase-1-pathway
#16
Mona M Allam, Ola A El-Gohary
Ghrelin has been shown to ameliorate gastric injury by several mechanisms in experimental animal models. The present study aimed to investigate the effect of pretreatment with ghrelin on indomethacin-induced gastric injury in rats and the role of heme oxygenase-1(HO-1) pathway as a novel mechanism underlying the gastroprotective effect of ghrelin. In all groups studied, ulcer score (U.S), ulcer index (U.I) and preventive index (P.I) were evaluated and the gastric inflammatory biomarkers including levels of tumor necrosis factor-α (TNF-α), interleukin-1β (IL-1β) and myeloperoxidase (MPO) activity as well as prostaglandin E2 (PGE2), malondialdehyde (MDA), glutathione (GSH), superoxide dismutase (SOD), HO-1 and bilirubin as an indicator of heme oxygenase activity were measured...
July 2017: General Physiology and Biophysics
https://www.readbyqxmd.com/read/28633630/clinical-characteristics-of-acute-hepatitis-a-outbreak-in-taiwan-2015-2016-observations-from-a-tertiary-medical-center
#17
Nan-Yu Chen, Zhuo-Hao Liu, Shian-Sen Shie, Tsung-Hsing Chen, Ting-Shu Wu
BACKGROUND: Acute hepatitis A is a fecal-oral transmitted disease related to inadequate sanitary conditions. In addition to its traditional classification, several outbreaks in the men who have sex with men (MSM) population have resulted in acute hepatitis A being recognized as a sexually transmitted disease. However, few studies have clarified the clinical manifestations in these outbreaks involving the MSM population. METHODS: Beginning in June 2015, there was an outbreak of acute hepatitis A involving the MSM population in Northern Taiwan...
June 20, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28630631/urinalysis-and-clinical-correlations-in-patients-with-p-vivax-or-p-falciparum-malaria-from-colombia
#18
Alberto Tobón-Castaño, Sebastián Barrera Escobar, Cecilia Giraldo Castro
BACKGROUND: Urinalysis is a poorly reviewed diagnostic tool in malaria patients; its application can show the presence of severe malaria. METHODS: Urinalysis was performed in a total of 620 patients diagnosed with malaria by thick blood smear; complications were classified according to WHO major criteria for severity and minor criteria according to the Colombian malaria guideline. RESULTS: Severe or moderate clinical complications were diagnosed in 31...
2017: Journal of Tropical Medicine
https://www.readbyqxmd.com/read/28629212/a-study-of-the-modulating-action-of-quercetin-on-biochemical-and-histological-alterations-induced-by-lead-exposure-in-the-liver-and-kidney-of-rats
#19
Azza Sedky, Hany Elsawy
Lead is a highly toxic metal and a very potent poison. Lead poisoning is a serious condition but can be treated. Quercetin is a flavonoid with many beneficial uses. The aim of the present study was to investigate the possible modulating action of quercetin as a model of an antioxidant against the toxic effects of lead acetate on liver and kidneys of rats. Rats were randomly divided into four groups: (i) saline group (control); (ii) lead group received i.p. lead acetate (20 mg/kg b.w.); (iii) quercetin group received i...
June 30, 2017: Chinese Journal of Physiology
https://www.readbyqxmd.com/read/28628718/a-better-method-for-assessment-of-hepatic-function-in-hcc-patients-who-underwent-radiofrequency-ablation-usefulness-of-albumin-bilirubin-grade
#20
Atsushi Hiraoka, Takashi Kumada, Masashi Hirooka, Kunihiko Tsuji, Ei Itobayashi, Kazuya Kariyama, Toru Ishikawa, Kazuto Tajiri, Hirofumi Ochi, Toshifumi Tada, Hidenori Toyoda, Kazuhiro Nouso, Kouji Joko, Hideki Kawasaki, Yoichi Hiasa, Kojiro Michitaka
AIM/BACKGROUND: To evaluate the efficacy of the newly proposed albumin-bilirubin (ALBI)-grade for therapy selection, clinical features of patients treated with radiofrequency ablation (RFA) were elucidated. MATERIALS/METHODS: From 2000 to 2015, 1101 patients with HCC (<3 cm, ≤3 tumors) treated with RFA were enrolled [males 734, females 367; HCV:HBV:HCV&HBV:others=779:153:5:164; Child-Pugh classification (CP) A:B=842:259]. Liver damage classification (LD) using with ICG-R15 and ALBI-grade were compared in regard to the prognoses of those patients...
June 19, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
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