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Liver replacement

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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treatment scheme consisted of tacrolimus (FK506) plus mycophenolate mofetil (MMF) and hormone; the latter was withdrawn 1 week after LT...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/29343316/black-soldier-fly-larva-fat-inclusion-in-finisher-broiler-chicken-diet-as-an-alternative-fat-source
#2
A Schiavone, S Dabbou, M De Marco, M Cullere, I Biasato, E Biasibetti, M T Capucchio, S Bergagna, D Dezzutto, M Meneguz, F Gai, A Dalle Zotte, L Gasco
The objective of the present study was to evaluate the effects of partial or total replacement of finisher diet soybean oil with black soldier fly (Hermethia illucens L.; HI) larva fat on the growth performance, carcass traits, blood parameters, intestinal morphology and histological features of broiler chickens. At 21 days of age, a total of 120 male broiler chickens (Ross 308) were randomly allocated to three experimental groups (five replicates and eight birds/pen). To a basal control diet (C; 68.7 g/kg as fed of soybean oil), either 50% or 100% of the soybean oil was replaced with HI larva fat (HI50 and HI100 group, respectively)...
January 18, 2018: Animal: An International Journal of Animal Bioscience
https://www.readbyqxmd.com/read/29343286/diagnosis-and-management-of-transthyretin-familial-amyloid-polyneuropathy-in-japan-red-flag-symptom-clusters-and-treatment-algorithm
#3
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
#4
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
#5
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/29329187/deceased-donor-organ-transplantation-performed-in-the-united-states-for-non-citizens-and-non-residents
#6
Francis L Delmonico, Susan Gunderson, Kishore R Iyer, Gabriel M Danovitch, Timothy L Pruett, Jorge D Reyes, Nancy L Ascher
Since 2012, the Organ Procurement and Transplantation Network (OPTN)/United Network for Organ Sharing (UNOS) has required transplant centers to record the citizenship and residency status of patients undergoing transplantation in the United States. This policy replaced the 5% threshold of the non-US citizen/non-U.S. residents (NC/NR) undergoing organ transplantation that could result in an audit of transplant center activity.We analyzed the frequency of NC/NR deceased donor organ transplants and wait list registrations at all US transplant centers using data provided by UNOS for that purpose to the UNOS Ad Hoc International Relations Committee...
January 11, 2018: Transplantation
https://www.readbyqxmd.com/read/29322599/non-invasive-evaluation-of-portal-hypertension-using-shear-wave-elastography-analysis-of-two-algorithms-combining-liver-and-spleen-stiffness-in-191-patients-with-cirrhosis
#7
L Elkrief, M Ronot, F Andrade, M Dioguardi Burgio, T Issoufaly, M Zappa, O Roux, J Bissonnette, A Payancé, D Lebrec, C Francoz, O Soubrane, D Valla, F Durand, V Vilgrain, L Castera, P-E Rautou
BACKGROUND: Two algorithms based on sequential measurements of liver and spleen stiffness using two-dimensional shearwave elastography (2D-SWE) have been recently proposed to estimate clinically significant portal hypertension (hepatic venous pressure gradient [HVPG] ≥10 mm Hg) in patients with cirrhosis, with excellent diagnostic accuracy. AIM: To validate externally these algorithms in a large cohort of patients with cirrhosis. METHODS: One hundred and ninety-one patients with stable cirrhosis (Child-Pugh class A 39%, B 29% and C 31%) who underwent liver and spleen stiffness measurements using 2D-SWE at the time of HVPG measurement were included...
January 11, 2018: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29315136/renal-replacement-therapy-in-the-critically-ill-child
#8
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/29310402/a-rare-variation-of-celiac-trunk-and-hepatic-artery-complicating-pancreaticoduodenectomy-a-case-report-and-literature-review
#9
Zhou Ye, Song Ye, Dongkai Zhou, Shusen Zheng, Weilin Wang
RATIONALE: Anatomical variations of the celiac trunk and the hepatic artery are of considerable importance in hepatopancreatobiliary surgery, liver transplants, and radiological abdominal interventions. PATIENT CONCERNS: Here, we report a 57-year-old man with 2 weeks of painless progressive jaundice. Preoperative imaging and cytology brush results suggested an ampullary tumor and common hepatic artery anomaly (CTA) was reported. The patient underwent pancreaticoduodenectomy (PD)...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29307055/novel-acute-kidney-injury-biomarkers-their-characteristics-utility-and-concerns
#10
REVIEW
Braian M Beker, Mateo G Corleto, Cecilia Fieiras, Carlos G Musso
Acute kidney injury (AKI) consists of a rapid renal function decline which usually increases serum urea and creatinine levels. Since kidney injury begins by inducing biological and molecular changes which evolve to cellular damage, biomarkers could be used as tools for monitoring early AKI appearance, and predicting its recovery. Among the main AKI biomarkers the neutrophil gelatinase-associated lipocalin, cystatin C, kidney injury molecule-1, monocyte chemotactic peptide-1, N-acetyl-β-D-glucosaminidase, interleukin-18, liver-type fatty acid-binding protein, netrin-1, cycle arrest markers, endogenous ouabain, selenium-binding protein 1, and BPIFA2 marker, have been described...
January 6, 2018: International Urology and Nephrology
https://www.readbyqxmd.com/read/29305734/olipudase-alfa-for-treatment-of-acid-sphingomyelinase-deficiency-asmd-safety-and-efficacy-in-adults-treated-for-30-months
#11
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
#12
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/29304756/medical-treatment-of-an%C3%A2-unusual-cerebral-hydatid-disease
#13
Shu Chen, Ning Li, Feifei Yang, Jiqin Wu, Yuekai Hu, Shenglei Yu, Qi Chen, Xuan Wang, Xinyu Wang, Yuanyuan Liu, Jianming Zheng
BACKGROUND: Hydatid disease is a worldwide zoonosis produced by the larval stage of cestodes of the Echinococcus genus. Hydatid disease primarily involves the liver and lungs. The brain is involved in less than 2% of cases. Surgery has long been the only choice for the treatment, but chemotherapy has been successfully replaced surgery in some special cases. CASE PRESENTATION: We report a rare hydatid disease case which presented with multiple lesions in right frontal lobe, an uncommon site, and in the liver and lungs...
January 5, 2018: BMC Infectious Diseases
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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