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https://www.readbyqxmd.com/read/28921500/growth-hormone-therapy-for-people-with-thalassaemia
#1
REVIEW
Chin Fang Ngim, Nai Ming Lai, Janet Yh Hong, Shir Ley Tan, Amutha Ramadas, Premala Muthukumarasamy, Meow-Keong Thong
BACKGROUND: Thalassaemia is a recessively-inherited blood disorder that leads to anaemia of varying severity. In those affected by the more severe forms, regular blood transfusions are required which may lead to iron overload. Accumulated iron from blood transfusions may be deposited in vital organs including the heart, liver and endocrine organs such as the pituitary glands which can affect growth hormone production. Growth hormone deficiency is one of the factors that can lead to short stature, a common complication in people with thalassaemia...
September 18, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28884960/genotypic-phenotypic-features-and-enzyme-replacement-therapy-outcome-in-patients-with-mucopolysaccharidosis-vi-from-turkey
#2
Mustafa Kılıç, Ali Dursun, Turgay Coşkun, Ayşegül Tokatlı, Rıza K Özgül, Didem Yücel-Yılmaz, Mehmet Karaca, Deniz Doğru, Dursun Alehan, Sibel Kadayıfçılar, Aydan Genç, Handan Turan-Dizdar, Burhanettin Gönüldaş, Sema Savcı, Melda Sağlam, Cemalettin Aksoy, Umut Arslan, Hatice-Serap Sivri
Mucopolysaccharidosis type VI (MPS VI) is a lysosomal storage disorder (LSD) characterized by a chronic, progressive course with multiorgan involvement. In our study, clinical, biochemical, molecular findings, and response to enzyme replacement therapy (ERT) for at least 6 months were evaluated in 20 patients with MPS VI. Treatment effects on clinical findings such as liver and spleen sizes, cardiac and respiratory parameters, visual and auditory changes, joints' range of motions, endurance tests and changes in urinary glycosaminoglycan excretions, before and after ERT were analyzed...
September 8, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28881270/molecular-and-clinical-characterization-of-a-series-of-patients-with-childhood-onset-lysosomal-acid-lipase-deficiency-retrospective-investigations-follow-up-and-detection-of-two-novel-lipa-pathogenic-variants
#3
Livia Pisciotta, Giulia Tozzi, Lorena Travaglini, Roberta Taurisano, Tiziano Lucchi, Giuseppe Indolfi, Francesco Papadia, Maja Di Rocco, Lorenzo D'Antiga, Patricia Crock, Komal Vora, Scott Nightingale, Helen Michelakakis, Anastasia Garoufi, Lilia Lykopoulou, Stefano Bertolini, Sebastiano Calandra
BACKGROUND AND AIMS: Childhood/Adult-onset Lysosomal Acid Lipase Deficiency (LAL-D) is a recessive disorder due to loss of function variants of LAL, the enzyme which hydrolyses cholesteryl esters, derived from internalized apoB containing lipoproteins. The disease is characterized by multi-organ involvement including the liver, spleen, intestine and cardiovascular system. The aim of this study was the clinical and molecular characterization of 14 (13 unrelated) previously unreported patients with childhood-onset LAL-D...
August 26, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28859230/continuous-renal-replacement-therapy-is-associated-with-reduced-serum-ammonia-levels-and-mortality-in-acute-liver-failure
#4
Filipe S Cardoso, Michelle Gottfried, Shannan Tujios, Jody C Olson, Constantine J Karvellas
BACKGROUND: Hyperammonemia has been associated with intracranial hypertension and mortality in patients with acute liver failure (ALF). We evaluated the effect of renal replacement therapy (RRT) on serum ammonia level and outcomes in ALF. METHODS: Multicenter cohort study of consecutive ALF patients from the United States ALF Study Group registry between 01/1998-12/2016. Firstly, we studied the association of ammonia with hepatic encephalopathy (HE) and 21-day transplant-free survival (TFS) (n=1186)...
August 31, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28857064/implementation-of-g6pd-testing-and-primaquine-for-p-vivax-radical-cure-operational-perspectives-from-thailand-and-cambodia
#5
Suravadee Kitchakarn, Dysoley Lek, Sea Thol, Chantheasy Hok, Aungkana Saejeng, Rekol Huy, Nipon Chinanonwait, Krongthong Thimasarn, Chansuda Wongsrichanalai
Following progressive success in reducing the burden of malaria over the past two decades, countries of the Asia Pacific are now aiming for elimination of malaria by 2030. Plasmodium falciparum and Plasmodium vivax are the two main malaria species that are endemic in the region. P. vivax is generally perceived to be less severe but will be harder to eliminate, owing partly to its dormant liver stage (known as a hypnozoite) that can cause multiple relapses following an initial clinical episode caused by a mosquito-borne infection...
September 2017: WHO South-East Asia Journal of Public Health
https://www.readbyqxmd.com/read/28856732/predictive-model-and-risk-factors-associated-with-a-revised-definition-of-early-allograft-dysfunction-in-liver-transplant-recipients
#6
R Nicolau-Raducu, A J Cohen, A Bokhari, H Bohorquez, D Bruce, I Carmody, E Bugeaud, J Seal, D Sonnier, B Nossaman, G Loss
INTRODUCTION: Early allograft dysfunction (EAD) is a well-defined clinical syndrome that reflects overall graft function within the first week after transplant. The aim of this study was to further refine the definition for EAD. METHOD: In this study, 1124 patients were included for analysis. Logistic regression was performed to identify markers of liver injury associated with 6-month patient and graft failure. RESULTS: Recursive partitioning identified cut-points for ALT/AST >3000/6000IU/dL observed within 1(st) week, with bilirubin ≥10mg/dL and INR ≥1...
August 30, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28836191/human-fibrotic-diseases-current-challenges-in-fibrosis-research
#7
Joel Rosenbloom, Edward Macarak, Sonsoles Piera-Velazquez, Sergio A Jimenez
Human fibrotic diseases constitute a major health problem worldwide owing to the large number of affected individuals, the incomplete knowledge of the fibrotic process pathogenesis, the marked heterogeneity in their etiology and clinical manifestations, the absence of appropriate and fully validated biomarkers, and, most importantly, the current void of effective disease-modifying therapeutic agents. The fibrotic disorders encompass a wide spectrum of clinical entities including systemic fibrotic diseases such as systemic sclerosis (SSc), sclerodermatous graft vs...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28813470/comparison-of-macrophage-migration-inhibitory-factor-and-neutrophil-gelatinase-associated-lipocalin-2-to-predict-acute-kidney-injury-after-liver-transplantation-an-observational-pilot-study
#8
Joanna Baron-Stefaniak, Judith Schiefer, Edmund J Miller, Gabriela A Berlakovich, David M Baron, Peter Faybik
INTRODUCTION: Several biomarkers have been suggested as early predictors of acute kidney injury (AKI) after orthotopic liver transplantation (OLT). Neutrophil gelatinase-associated lipocalin-2 (NGAL) appears to be a promising predictor of AKI after OLT, but the clinical benefit remains to be proven. Recently, systemic macrophage migration inhibitory factor (MIF) has been proposed as early indicator for requirement of renal replacement therapy after OLT. The aim of this prospective, observational pilot study was to compare the predictive values of serum and urinary MIF for severe AKI after OLT to those of serum and urinary NGAL...
2017: PloS One
https://www.readbyqxmd.com/read/28797497/reaching-hepatitis-c-virus-elimination-targets-requires-health-system-interventions-to-enhance-the-care-cascade
#9
Nick Scott, Joseph S Doyle, David P Wilson, Amanda Wade, Jess Howell, Alisa Pedrana, Alexander Thompson, Margaret E Hellard
BACKGROUND: Modelling suggests that achieving the World Health Organization's elimination targets for hepatitis C virus (HCV) is possible by scaling up use of direct-acting antiviral (DAA) therapy. However, poor linkage to health services and retention in care presents a major barrier, in particular among people who inject drugs (PWID). We identify and assess the cost-effectiveness of additional health system interventions required to achieve HCV elimination targets in Australia, a setting where all people living with HCV have access to DAA therapy...
September 2017: International Journal on Drug Policy
https://www.readbyqxmd.com/read/28794844/a-case-series-on-simultaneous-liver-and-kidney-transplantation-do-we-need-intraoperative-renal-replacement-therapy
#10
Wongook Wi, Tae Soo Hahm, Gaab-Soo Kim
Since the implementation of the model for end-stage liver disease (MELD) scoring system in 2002, the liver transplantation (LT) society has observed a substantial increase in the number of recipients with renal dysfunction. Intraoperative renal replacement therapy (ioRRT) has emerged as one of the solutions available to manage high-MELD score recipients; however, its usefulness has not yet been proven. To date, we have experienced five cases of simultaneous liver and kidney transplantation (SLKT). Recipients of SLKT tend to have a lower pre-transplant kidney function and the longer operation time mandates a larger amount of fluid than LT alone...
August 2017: Korean Journal of Anesthesiology
https://www.readbyqxmd.com/read/28792509/regional-citrate-anticoagulation-for-continuous-renal-replacement-therapy-in-pediatric-patients-with-liver-failure
#11
Keila Rodriguez, Poyyapakkam R Srivaths, Leyat Tal, Mary N Watson, Alyssa A Riley, Ryan W Himes, Moreshwar S Desai, Michael C Braun, Ayse Akcan Arikan
Pediatric liver failure patients frequently develop multiple organ failure and require continuous renal replacement therapy (CRRT) as part of supportive therapy in the pediatric intensive care unit. While many centers employ no anticoagulation for fear of bleeding complications, balanced coagulation disturbance predisposes these patients to clotting as well as bleeding, making maintenance of longer circuit life to deliver adequate dialysis clearance challenging. Regional citrate anticoagulation (RCA) is an attractive option as it avoids systemic anticoagulation, but since citrate metabolism is impaired in liver failure, concerns about toxicity has limited its use...
2017: PloS One
https://www.readbyqxmd.com/read/28779790/management-of-renal-dysfunction-in-patients-with-liver-cirrhosis-role-of-pretransplantation-hemodialysis-and-outcomes-after-liver-transplantation
#12
REVIEW
Ashok Thorat, Long-Bin Jeng
Patients with end-stage liver disease (ESLD) who develop hepatorenal syndrome (HRS) have very high mortality rates. For patients with HRS type I, median survival without specific therapy is only 2 weeks. Due to worsening clinical condition in such patients secondary to uremia and hepatic disease, some form of renal replacement therapy (RRT), either intermittent hemodialysis IHD or continuous veno-venous hemodialysis (CVVHD), must be instituted. However, the literature regarding the survival benefits of the hemodialysis for the worsening renal failure in liver cirrhotic patients remains limited...
December 2016: Seminars in Vascular Surgery
https://www.readbyqxmd.com/read/28763689/investigation-of-novel-pharmacological-chaperones-for-gaucher-disease
#13
Buge Yilmazer, Z Begum Yagci, Emre Bakar, Burcu Ozden, Kutlu Ulgen, Elif Ozkirimli
Beta-Glucocerebrosidase (GBA) is a lysosomal protein that is responsible for the hydrolysis of glycosylceramide into glucose and ceramide. Mutations in GBA lead to the accumulation of glycosylceramide in the lysosome causing an enlargement of the spleen and the liver and skeletal deformations. This disease is called Gaucher Disease. Enzyme replacement therapies and substrate reduction methods that are used to treat Gaucher Disease fail when the disease is neuropathic because they fail to pass the blood brain barrier...
July 20, 2017: Journal of Molecular Graphics & Modelling
https://www.readbyqxmd.com/read/28760474/impact-of-postoperative-liver-dysfunction-on-survival-after-left-ventricular-assist-device-implantation
#14
Kaustav Majumder, John R Spratt, Christopher T Holley, Samit S Roy, Rebecca J Cogswell, Kenneth Liao, Ranjit John
BACKGROUND: Liver dysfunction in left ventricular assist device (LVAD) recipients is common both before and after implantation. Postoperative liver dysfunction (PLD) develops in some LVAD recipients without preoperative liver dysfunction. The aim of this study was to assess clinical outcomes in such patients. METHODS: Records of all patients undergoing implantation of a HeartMate II (HM II, St. Jude Medical, Inc, Minneapolis, MN) LVAD at a single center at the University of Minnesota from January 2005 through June 2014 were analyzed...
July 28, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28755198/remaining-challenges-in-the-treatment-of-tyrosinemia-from-the-clinician-s-viewpoint
#15
Grant A Mitchell, Hao Yang
This chapter provides a clinical perspective on the challenges that stand between current clinical practice and a cure for hepatorenal tyrosinemia (HT1). HT1 has been transformed in the last 50 years from an aggressive often undiagnosed childhood disease causing liver failure or liver cancer, with infant death in most patients, to a condition that is detectable at birth, and for which treatment with nitisinone (NTBC) and diet can prevent detectable liver or kidney abnormalities. What challenges remain? The properties of the affected metabolic pathway and the broad spectrum of severity seen in untreated patients are incompletely understood but potentially important for patients...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28754776/the-hlf-il-6-stat3-feedforward-circuit-drives-hepatic-stellate-cell-activation-to-promote-liver-fibrosis
#16
Dai-Min Xiang, Wen Sun, Bei-Fang Ning, Teng-Fei Zhou, Xiao-Feng Li, Wei Zhong, Zhuo Cheng, Ming-Yang Xia, Xue Wang, Xing Deng, Wei Wang, Heng-Yu Li, Xiu-Liang Cui, Shi-Chao Li, Bin Wu, Wei-Fen Xie, Hong-Yang Wang, Jin Ding
BACKGROUND AND AIMS: Liver fibrosis is a wound-healing response that disrupts the liver architecture and function by replacing functional parenchyma with scar tissue. Recent progress has advanced our knowledge of this scarring process, but the detailed mechanism of liver fibrosis is far from clear. METHODS: The fibrotic specimens of patients and HLF (hepatic leukemia factor)(PB/PB) mice were used to assess the expression and role of HLF in liver fibrosis. Primary murine hepatic stellate cells (HSCs) and human HSC line Lx2 were used to investigate the impact of HLF on HSC activation and the underlying mechanism...
July 28, 2017: Gut
https://www.readbyqxmd.com/read/28742776/venovenous-bypass-is-associated-with-a-lower-incidence-of-acute-kidney-injury-after-liver-transplantation-in-patients-with-compromised-pretransplant-renal-function
#17
Kai Sun, Fu Hong, Yun Wang, Vatche G Agopian, Min Yan, Ronald W Busuttil, Randolph H Steadman, Victor W Xia
BACKGROUND: Although the hemodynamic benefits of venovenous bypass (VVB) during liver transplantation (LT) are well appreciated, the impact of VVB on posttransplant renal function is uncertain. The aim of this study was to determine if VVB was associated with a lower incidence of posttransplant acute kidney injury (AKI). METHODS: Medical records of adult (≥18 years) patients who underwent primary LT between 2004 and 2014 at a tertiary hospital were reviewed. Patients who required pretransplant renal replacement therapy and intraoperative piggyback technique were excluded...
July 21, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28738402/biodistribution-of-liver-derived-mesenchymal-stem-cells-after-peripheral-injection-in-a-hemophilia-a-patient
#18
Etienne M Sokal, Catherine Anne Lombard, Véronique Roelants, Mustapha Najimi, Sharat Varma, Camillo Sargiacomo, Joachim Ravau, Giuseppe Mazza, François Jamar, Julia Versavau, Vanessa Jacobs, Marc Jacquemin, Stéphane Eeckhoudt, Catherine Lambert, Xavier Stéphenne, Françoise Smets, Cédric Hermans
BACKGROUND: With the exception of liver transplantation, there is no cure for hemophilia, which is currently managed by preemptive replacement therapy. Liver-derived stem cells are in clinical development for inborn and acquired liver diseases and could represent a curative treatment for hemophilia A. The liver is a major factor VIII (FVIII) synthesis site, and mesenchymal stem cells have been shown to control joint bleeding in animal models of hemophilia. Adult-derived human liver stem cells (ADHLSCs) have mesenchymal characteristics and have been shown able to engraft in and repopulate both animal and human livers...
August 2017: Transplantation
https://www.readbyqxmd.com/read/28736005/benefits-of-intraoperative-continuous-renal-replacement-therapy-during-liver-transplantation-in-patients-with-renal-dysfunction
#19
S D Baek, M Jang, W Kim, H Yu, S Hwang, S-G Lee, G-S Hwang, E K Lee, S M Kim, J W Chang
BACKGROUND: Comparative outcomes of continuous renal replacement therapy during liver transplantation have not been investigated. We retrospectively compared the outcomes of intraoperative continuous renal replacement therapy with those of non-dialytic conservative treatment in patients with pretransplant renal dysfunction. METHODS: We analyzed 240 transplantation patients with preoperative renal dysfunction (estimated glomerular filtration rate <60 mL/min/1...
July 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28735098/cholangiocytes-cell-transplantation
#20
REVIEW
Lorenzo Ridola, Maria Consiglia Bragazzi, Vincenzo Cardinale, Guido Carpino, Eugenio Gaudio, Domenico Alvaro
BACKGROUND: Due to significant limitations to the access to orthotropic liver transplantation, cell therapies for liver diseases have gained large interest worldwide. SCOPE OF REVIEW: To revise current literature dealing with cell therapy for liver diseases. We discussed the advantages and pitfalls of the different cell sources tested so far in clinical trials and the rationale underlying the potential benefits of transplantation of human biliary tree stem cells (hBTSCs)...
July 19, 2017: Biochimica et Biophysica Acta
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