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Liver replacement therapy

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https://www.readbyqxmd.com/read/29344227/sirolimus-treatment-for-cirrhosis-or-hepatocellular-carcinoma-patients-accompanied-by-psoriasis-after-liver-transplantation-a-single-center-experience
#1
Lin Zhou, Guo-Sheng Du, Li-Chao Pan, Yong-Gen Zheng, Zhi-Jia Liu, Hai-Da Shi, Shao-Zhen Yang, Xian-Jie Shi, Meng Xuan, Li-Kui Feng, Zhi-Dong Zhu
There is currently no consensus on the most suitable therapeutic approach for psoriasis (PS) co-existing with posthepatic cirrhosis (PCs) and hepatocellular carcinoma (HCC) following liver transplantation (LT). The present study provides an analysis of the therapeutic experience of such patients. Five LT recipients (two with PC and three with HCC) with accompanying PS were included. The induction program consisted of methylprednisolone plus basiliximab treatment. The initial postoperative treatment scheme consisted of tacrolimus (FK506) plus mycophenolate mofetil (MMF) and hormone; the latter was withdrawn 1 week after LT...
December 2017: Oncology Letters
https://www.readbyqxmd.com/read/29343286/diagnosis-and-management-of-transthyretin-familial-amyloid-polyneuropathy-in-japan-red-flag-symptom-clusters-and-treatment-algorithm
#2
REVIEW
Yoshiki Sekijima, Mitsuharu Ueda, Haruki Koike, Sonoko Misawa, Tomonori Ishii, Yukio Ando
Hereditary ATTR (ATTRm) amyloidosis (also called transthyretin-type familial amyloid polyneuropathy [ATTR-FAP]) is an autosomal-dominant, adult-onset, rare systemic disorder predominantly characterized by irreversible, progressive, and persistent peripheral nerve damage. TTR gene mutations (e.g. replacement of valine with methionine at position 30 [Val30Met (p.Val50Met)]) lead to destabilization and dissociation of TTR tetramers into variant TTR monomers, which form amyloid fibrils that deposit in peripheral nerves and various organs, giving rise to peripheral and autonomic neuropathy and several non-disease specific symptoms...
January 17, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29337842/perioperative-coagulation-management-in-liver-transplant-recipients
#3
Dmitri Bezinover, Daniel Dirkmann, James Findlay, Cosmin Guta, Matthias Hartmann, Ramona Nicolau-Raducu, Ahmed M Mukhtar, Marina Moguilevitch, Evan Pivalizza, David Rosenfeld, Fuat Saner, Christopher Wray, Gebhardt Wagener, James West
We review contemporary coagulation management for patients undergoing liver transplantation. A better understanding of the complex physiologic changes that occur in patients with end-stage liver disease has resulted in significant advances in anesthetic and coagulation management. A group of internationally recognized experts have critically evaluated current approaches for coagulopathy detection and management. Strategies for blood component and factor replacement have been evaluated and recommended therapies proposed...
January 15, 2018: Transplantation
https://www.readbyqxmd.com/read/29336994/agreement-between-the-results-of-meta-analyses-from-case-reports-and-from-clinical-studies-regarding-the-efficacy-of-laronidase-therapy-in-patients-with-mucopolysaccharidosis-type-i-who-initiated-enzyme-replacement-therapy-in-adult-age-an-example-of-case-reports
#4
REVIEW
Miguel Sampayo-Cordero, Bernat Miguel-Huguet, Almudena Pardo-Mateos, Marc Moltó-Abad, Cecilia Muñoz-Delgado, Jordi Pérez-López
BACKGROUND: Case reports might have a prominent role in the rare diseases field, due to the small number of patients affected by one such disease. A previous systematic review regarding the efficacy of laronidase therapy in patients with mucopolysaccharidosis type I (MPS-I) who initiated enzyme replacement therapy (ERT) in adult age has been published. The review included a meta-analysis of 19 clinical studies and the description of eleven case reports. It was of interest to perform a meta-analysis of those case reports to explore the role of such meta-analyses as a tool for evidence-based medicine in rare diseases...
January 5, 2018: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29315136/renal-replacement-therapy-in-the-critically-ill-child
#5
Claire A Westrope, Sarah Fleming, Melpo Kapetanstrataki, Roger C Parslow, Kevin P Morris
OBJECTIVES: Although renal replacement therapy is widely used in critically ill children, there have been few comprehensive population-based studies of its use. This article describes renal replacement therapy use, and associated outcomes, in critically ill children across the United Kingdom in the largest cohort study of this patient group. DESIGN: A retrospective observational study using prospectively collected data. SETTING: Data from the Pediatric Intensive Care Audit Network database which collects data on all children admitted to U...
January 8, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29305734/olipudase-alfa-for-treatment-of-acid-sphingomyelinase-deficiency-asmd-safety-and-efficacy-in-adults-treated-for-30-months
#6
Melissa P Wasserstein, George A Diaz, Robin H Lachmann, Marie-Hélène Jouvin, Indrani Nandy, Allena J Ji, Ana Cristina Puga
Olipudase alfa, a recombinant human acid sphingomyelinase (ASM), is an enzyme replacement therapy for the treatment of nonneurologic manifestations of acid sphingomyelinase deficiency (ASMD). This ongoing, open-label, long-term study (NCT02004704) assessed safety and efficacy of olipudase alfa following 30 months of treatment in five adult patients with ASMD. There were no deaths, serious or severe events, or discontinuations during 30 months of treatment. The majority of adverse events were mild and included headache, nausea, and abdominal pain...
January 5, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29305416/involvement-of-hepcidin-in-iron-metabolism-dysregulation-in-gaucher-disease
#7
Thibaud Lefebvre, Niloofar Reihani, Raed Daher, Thierry Billette de Villemeur, Nadia Belmatoug, Christian Rose, Yves Colin-Aronovicz, Hervé Puy, Caroline Le Van Kim, Mélanie Franco, Zoubida Karim
Gaucher disease is an inherited deficiency of glucocerebrosidase leading to accumulation of glucosylceramide in tissues such as the spleen, liver, and bone marrow. The resulting lipid-laden macrophages lead to the appearance of Gaucher cells. Anemia associated with an unexplained hyperferritinemia is a frequent finding in Gaucher disease, but whether this pathogenesis is related to an iron metabolism disorder has remained unclear. To investigate this issue, we explored the iron status of a large cohort of 90 type I Gaucher disease patients, including 66 patients treated with enzyme replacement therapy...
January 5, 2018: Haematologica
https://www.readbyqxmd.com/read/29304513/nonvitamin-k-antagonist-oral-anticoagulants-use-in-patients-with-atrial-fibrillation-and-bioprosthetic-heart-valves-prior-surgical-valve-repair-a-multicenter-clinical-practice-experience
#8
Vincenzo Russo, Emilio Attena, Carmine Mazzone, Francesca Esposito, Valentina Parisi, Ciro Bancone, Anna Rago, Gerardo Nigro, Raffaele Sangiuolo, Antonio D' Onofrio
This is an observational study to investigate the efficacy and safety of nonvitamin K antagonist oral anticoagulants (NOACs) in atrial fibrillation (AF) patients with bioprosthetic valves or prior surgical valve repair in clinical practice. A total of 122 patients (mean age: 74.1 ± 13.2; 54 females) with bioprosthetic heart valve or surgical valve repair and AF treated with NOACs were included in the analysis. The mean CHA2DS2-VASc (Congestive heart failure, Hypertension, Age >75 years, Diabetes mellitus, prior Stroke or transient ischemic attack, Vascular disease) and HAS-BLED (Hypertension, Abnormal renal and liver function, Stroke, Bleeding, Labile INR [international normalized ratio], Elderly, Drugs or alcohol) score values were 3...
January 5, 2018: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29302509/a-novel-homozygous-lipa-mutation-in-a-korean-child-with-lysosomal-acid-lipase-deficiency
#9
Kwang Yeon Kim, Ju Whi Kim, Kyung Jae Lee, Eunhyang Park, Gyeong Hoon Kang, Young Hun Choi, Woo Sun Kim, Jung Min Ko, Jin Soo Moon, Jae Sung Ko
Patients with lysosomal acid lipase (LAL) deficiency and glycogen storage disease (GSD) demonstrated hepatomegaly and dyslipidemia. In our case, a 6-year-old boy presented with hepatosplenomegaly. At 3 years of age, GSD had been diagnosed by liver biopsy at another hospital. He showed elevated serum liver enzymes and dyslipidemia. Liver biopsy revealed diffuse microvesicular fatty changes in hepatocytes, septal fibrosis and foamy macrophages. Ultrastructural examination demonstrated numerous lysosomes that contained lipid material and intracytoplasmic cholesterol clefts...
December 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29295737/immunomodulatory-liver-depot-gene-therapy-for-pompe-disease
#10
J E Bond, P S Kishnani, D D Koeberl
Pompe disease is caused by mutations in acid alpha glucosidase (GAA) that causes accumulation of lysosomal glycogen affecting the heart and skeletal muscles, and can be fatal. Enzyme replacement therapy (ERT) with recombinant human GAA (rhGAA) improves muscle function by reducing glycogen accumulation. Limitations of ERT include a short half-life and the formation of antibodies that result in reduced efficacy. By harnessing the immune tolerance induction properties of the liver, liver-targeted gene delivery (with an adeno-associated virus vector containing a liver specific promoter), suppresses immunity against the GAA introduced by gene therapy...
December 29, 2017: Cellular Immunology
https://www.readbyqxmd.com/read/29286074/codonopis%C3%A2-bulleynana-forest-ex-diels-inhibits-autophagy-and-induces-apoptosis-of-colon-cancer-cells-by-activating-the-nf-%C3%AE%C2%BAb-signaling-pathway
#11
Yunpeng Luan, Yanmei Li, Lina Zhu, Shuangqing Zheng, Dechang Mao, Zhuxue Chen, Yong Cao
Despite its favorable clinical efficacy, oxaliplatin‑based chemotherapy frequently results in treatment withdrawal and induces liver damage in colon cancer. Therefore, it is important to develop novel drugs, which can safely and effectively complement or replace the therapeutic effects of oxaliplatin. Codonopis bulleynana Forest ex Diels (cbFeD) has wide range of pharmacological effects, including anticancer effects. In the present study, the anticancer activity of cbFeD and its potential molecular mechanisms were investigated...
December 21, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/29285688/pancreatoblastoma-an-unusual-diagnosis-in-an-adult-patient
#12
Gonçalo Nunes, Hélder Coelho, Marta Patita, Rita Barosa, Pedro Pinto Marques, Lídia Roque Ramos, Maria José Brito, Aurora Tomaz, Jorge Fonseca
The authors describe a 31-year-old man admitted due to progressive weight loss, diarrhea and massive hepatomegaly. Laboratory data showed anemia (haemoglobin 11.7 g/dl), abnormal liver tests (total bilirubin 1.4 g/dl, aspartate aminotransferase 70 U/l, alanine aminotransferase 37 U/l and alkaline phosphatase 520 U/l). Abdominal ultrasound (US) displayed a large heterogeneous liver with a segment IV 25 mm nodule. Magnetic resonance revealed a 4 cm pancreatic tail mass and several liver nodules consistent with metastasis...
December 28, 2017: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29285318/comparison-of-hydrocortisone-and-prednisone-in-the-glucocorticoid-replacement-therapy-post-adrenalectomy-of-cushing-s-syndrome
#13
Kunlong Tang, Liang Wang, Zhongyuan Yang, Yingying Sui, Liming Li, Yuting Huang, Peng Gao
Cushing's syndrome requires glucocorticoid replacement following adrenalectomy. Based on a simplified glucocorticoid therapy scheme and the peri-operative observation, we investigated its efficacy and safety up to 6 months post-adrenalectomy in this cohort study. We found the adrenocorticotropic hormone (ACTH) levels were normal post-adrenalectomy, and sufficient to stimulate the recovery of the dystrophic adrenal cortex, thus exogenous supplemental ACTH might not be necessary. Patients were grouped by oral reception of either hydrocortisone or prednisone since day 2 post-adrenalectomy...
December 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/29284322/maximizing-nutrition-support-practice-and-measuring-adherence-to-nutrition-support-guidelines-in-a-canadian-tertiary-care-icu
#14
Michele E McCall, Alice Adamo, Katherine Latko, Ashley K Rieder, Nicole Durand, Tova Nathanson
OBJECTIVE: New comprehensive guidelines for nutrition support (NS) in the intensive care unit (ICU) can be used to improve quality of care and benchmark current practice. The objective of this study was to (a) compare NS practices in our medical/surgical ICU (MSICU) to 18 recommendations described in the Canadian Clinical Practice Guidelines and Society of Critical Care Medicine/American Society of Parenteral and Enteral Nutrition guidelines, (b) determine the percentage of goal calories and protein delivered, and (c) identify the barriers to successful NS delivery...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/29262333/systemic-messenger-rna-therapy-as-a-treatment-for-methylmalonic-acidemia
#15
Ding An, Jessica L Schneller, Andrea Frassetto, Shi Liang, Xuling Zhu, Ji-Sun Park, Matt Theisen, Sue-Jean Hong, Jenny Zhou, Raj Rajendran, Becca Levy, Rebecca Howell, Gilles Besin, Vladimir Presnyak, Staci Sabnis, Kerry E Murphy-Benenato, E Sathyajith Kumarasinghe, Timothy Salerno, Cosmin Mihai, Christine M Lukacs, Randy J Chandler, Lin T Guey, Charles P Venditti, Paolo G V Martini
Isolated methylmalonic acidemia/aciduria (MMA) is a devastating metabolic disorder with poor outcomes despite current medical treatments. Like other mitochondrial enzymopathies, enzyme replacement therapy (ERT) is not available, and although promising, AAV gene therapy can be limited by pre-existing immunity and has been associated with genotoxicity in mice. To develop a new class of therapy for MMA, we generated a pseudoU-modified codon-optimized mRNA encoding human methylmalonyl-CoA mutase (hMUT), the enzyme most frequently mutated in MMA, and encapsulated it into biodegradable lipid nanoparticles (LNPs)...
December 19, 2017: Cell Reports
https://www.readbyqxmd.com/read/29261831/evaluation-of-early-allograft-function-using-the-liver-graft-assessment-following-transplantation-risk-score-model
#16
Vatche G Agopian, Michael P Harlander-Locke, Daniela Markovic, Wethit Dumronggittigule, Victor Xia, Fady M Kaldas, Ali Zarrinpar, Hasan Yersiz, Douglas G Farmer, Jonathan R Hiatt, Ronald W Busuttil
Importance: Early allograft dysfunction (EAD) following a liver transplant (LT) unequivocally portends adverse graft and patient outcomes, but a widely accepted classification or grading system is lacking. Objective: To develop a model for individualized risk estimation of graft failure after LT and then compare the model's prognostic performance with the existing binary EAD definition (bilirubin level of ≥10 mg/dL on postoperative day 7, international normalized ratio of ≥1...
December 20, 2017: JAMA Surgery
https://www.readbyqxmd.com/read/29246491/impact-of-loss-of-soat2-function-on-disease-progression-in-the-lysosomal-acid-lipase-deficient-mouse
#17
Adam M Lopez, Jen-Chieh Chuang, Stephen D Turley
Although only a small proportion of cholesterol in the body is esterified, in several diseases marked expansion of the esterified cholesterol (EC) pool occurs. These include Wolman disease (WD) and Cholesteryl Ester Storage Disease (CESD) which both result from mutations in LIPA, the gene that encodes lysosomal acid lipase (LAL). The respective contributions that our three cholesterol esterifying enzymes make to EC production, especially in disorders like CESD, are not well defined. The current studies represent a detailed exploration of our earlier findings in young male LAL-deficient mice also missing sterol-O-acyltransferase 2 (SOAT2, also called ACAT2)...
December 12, 2017: Steroids
https://www.readbyqxmd.com/read/29246152/linking-physiologically-based-pharmacokinetic-and-genome-scale-metabolic-networks-to-understand-estradiol-biology
#18
Joanna H Sier, Alfred E Thumser, Nick J Plant
BACKGROUND: Estrogen is a vital hormone that regulates many biological functions within the body. These include roles in the development of the secondary sexual organs in both sexes, plus uterine angiogenesis and proliferation during the menstrual cycle and pregnancy in women. The varied biological roles of estrogens in human health also make them a therapeutic target for contraception, mitigation of the adverse effects of the menopause, and treatment of estrogen-responsive tumours. In addition, endogenous (e...
December 15, 2017: BMC Systems Biology
https://www.readbyqxmd.com/read/29234937/-lysosomal-acid-lipase-deficiency-lal-d-diagnostic-and-therapeutic-options-in-an-underdiagnosed-disease
#19
REVIEW
S Synoracki, S Kathemann, K W Schmid, H Jastrow, H A Baba
BACKGROUND AND CLINICAL SETTING: Lysosomal acid lipase deficiency is an autosomal recessive storage disease caused by mutations in the LIPA gene. The accumulation of cholesteryl esters and triglycerides in hepatocytes lead to hepatomegaly with progressive fibrosis and liver cirrhosis. Characteristically, patients have a hepatomegaly combined with high serum levels of cholesterol, LDL-cholesterol and in some cases triglyceride, whereas HDL-cholesterol is decreased. Histologically, hepatocytes show a microvesicular steatosis with typically ballooned Kupffer cells...
December 12, 2017: Der Pathologe
https://www.readbyqxmd.com/read/29221978/influence-of-6-hydroxyethyl-starch-130-0-4-versus-crystalloid-solution-on-structural-renal-damage-markers-after-coronary-artery-bypass-grafting-a-post-hoc-subgroup-analysis-of-a-prospective-trial
#20
Thomas Datzmann, Markus Hoenicka, Helmut Reinelt, Andreas Liebold, Hagen Gorki
OBJECTIVE: The restriction of hydroxyethyl starch (HES) has mandated changes in volume management based on data of critically ill patients. Reliable data of structural renal damage after HES treatment in cardiac surgical patients are lacking. The influence of 6% HES 130/0.4 was investigated in this study. DESIGN: An exploratory post hoc subgroup analysis of a prospective trial was performed. SETTING: The study was carried out at a university hospital...
May 25, 2017: Journal of Cardiothoracic and Vascular Anesthesia
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