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https://www.readbyqxmd.com/read/28640238/treatment-of-mucopolysaccharidosis-type-ii-hunter-syndrome-results-from-a-systematic-evidence-review
#1
REVIEW
Linda A Bradley, Hamish R M Haddow, Glenn E Palomaki
PurposeA pilot systematic evidence review to establish methodology utility in rare genetic diseases, support clinical recommendations, and identify important knowledge gaps.MethodsBroad-based published/gray-literature searches through December 2015 for studies of males with confirmed mucopolysaccharidosis type II (any age, phenotype, genotype, family history) treated with enzyme replacement therapy or hematopoietic stem cell transplantation. Preset inclusion criteria employed for abstract and full document selection, and standardized methods for data extraction and assessment of quality and strength of evidence...
June 22, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/28636702/analysis-of-survival-after-initiation-of-continuous-renal-replacement-therapy-in-a-surgical-intensive-care-unit
#2
James M Tatum, Galinos Barmparas, Ara Ko, Navpreet Dhillon, Eric Smith, Daniel R Margulies, Eric J Ley
Importance: Continuous renal replacement therapy (CRRT) benefits patients with renal failure who are too hemodynamically unstable for intermittent hemodialysis. The duration of therapy beyond which continued use is futile, particularly in a population of patients admitted to and primarily cared for by a surgical service (hereinafter referred to as surgical patients), is unclear. Objective: To analyze proportions of and independent risk factors for survival to discharge after initiation of CRRT among patients in a surgical intensive care unit (SICU)...
June 21, 2017: JAMA Surgery
https://www.readbyqxmd.com/read/28635626/adiponectin-a-therapeutic-target-for-obesity-diabetes-and-endothelial-dysfunction
#3
REVIEW
Arunkumar E Achari, Sushil K Jain
Adiponectin is the most abundant peptide secreted by adipocytes, whose reduction plays a central role in obesity-related diseases, including insulin resistance/type 2 diabetes and cardiovascular disease. In addition to adipocytes, other cell types, such as skeletal and cardiac myocytes and endothelial cells, can also produce this adipocytokine. Adiponectin effects are mediated by adiponectin receptors, which occur as two isoforms (AdipoR1 and AdipoR2). Adiponectin has direct actions in liver, skeletal muscle, and the vasculature...
June 21, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28630772/severe-rhabdomyolysis-due-to-presumed-drug-interactions-between-atorvastatin-with-amlodipine-and-ticagrelor
#4
Iouri Banakh, Kavi Haji, Ross Kung, Sachin Gupta, Ravindranath Tiruvoipati
Atorvastatin and ticagrelor combination is a widely accepted therapy for secondary prevention of ischaemic heart disease. However, rhabdomyolysis is a well-known rare side effect of statins which should be considered when treatments are combined with cytochrome P450 3A4 enzyme inhibitors. We report a case of atorvastatin and ticagrelor associated severe rhabdomyolysis that progressed to multiorgan failure requiring renal replacement therapy, inotropes, intubation, and mechanical ventilation. Despite withdrawal of the precipitating cause and the supportive measures including renal replacement therapy, creatinine kinase increased due to ongoing rhabdomyolysis rapidly progressing to upper and lower limbs weakness...
2017: Case Reports in Critical Care
https://www.readbyqxmd.com/read/28624108/modification-of-immunosuppressive-therapy-as-risk-factor-for-complications-after-liver-transplantation
#5
REVIEW
Paolo De Simone, Paola Carrai, Laura Coletti, Davide Ghinolfi, Stefania Petruccelli, Franco Filipponi
Management of complications post-liver transplantation (LT) includes immunosuppressive manipulations with the aim to reduce the overall burden of immunologic suppression and compensate for renal, cardiovascular, metabolic toxicities, and for the increased oncologic risk. Two approaches can be implemented to reduce immunosuppression-related adverse events: upfront schedules tailored to the pretransplant individual patient's risk profile versus downstream modifications in the event of immunosuppression-related complications...
April 2017: Best Practice & Research. Clinical Gastroenterology
https://www.readbyqxmd.com/read/28616151/therapeutic-potential-of-dental-stem-cells
#6
REVIEW
Elna Paul Chalisserry, Seung Yun Nam, Sang Hyug Park, Sukumaran Anil
Stem cell biology has become an important field in regenerative medicine and tissue engineering therapy since the discovery and characterization of mesenchymal stem cells. Stem cell populations have also been isolated from human dental tissues, including dental pulp stem cells, stem cells from human exfoliated deciduous teeth, stem cells from apical papilla, dental follicle progenitor cells, and periodontal ligament stem cells. Dental stem cells are relatively easily obtainable and exhibit high plasticity and multipotential capabilities...
January 2017: Journal of Tissue Engineering
https://www.readbyqxmd.com/read/28614908/adenoviral-production-of-interleukin-2-at-the-tumor-site-removes-the-need-for-systemic-postconditioning-in-adoptive-cell-therapy
#7
Joao Manuel Santos, Riikka Havunen, Mikko Siurala, Víctor Cervera-Carrascon, Siri Tähtinen, Suvi Sorsa, Marjukka Anttila, Pauliina Karell, Anna Kanerva, Akseli Hemminki
Systemic high-dose interleukin-2 (IL-2) postconditioning has long been utilized in boosting the efficacy of T cells in adoptive cell therapy (ACT) of solid tumors. The resulting severe off-target toxicity of these regimens renders local production at the tumor an attractive concept with possible safety gains. We evaluated the efficacy and safety of intratumorally administered IL-2-coding adenoviruses in combination with tumor-infiltrating lymphocyte therapy in syngeneic Syrian hamsters bearing HapT1 pancreatic tumors and with T cell receptor transgenic ACT in B16...
June 14, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/28611949/pseudomalabsorption-of-levothyroxine-a-challenge-for-the-endocrinologist-in-the-treatment-of-hypothyroidism
#8
Nancy Van Wilder, Bert Bravenboer, Sarah Herremans, Nathalie Vanderbruggen, Brigitte Velkeniers
BACKGROUND: Hypothyroidism due to non-compliance with levothyroxine therapy (pseudomalabsorption) is rare. The diagnosis is considered in patients with persistent severe hypothyroidism despite treatment with large doses of levothyroxine. Intestinal malabsorption, drug and dietary interference with levothyroxine absorption and nephrotic syndrome should be excluded. The diagnosis of pseudomalabsorption can be demonstrated by using "an oral 1,000 µg of levothyroxine test" showing a rapid decrease in thyroid-stimulating hormone and increase in thyroxine...
February 2017: European Thyroid Journal
https://www.readbyqxmd.com/read/28602971/new-developments-in-hepatorenal-syndrome
#9
Ayse L Mindikoglu, Stephen C Pappas
Hepatorenal Syndrome (HRS) continues to be one of the major complications of decompensated cirrhosis leading to death in the absence of liver transplantation. Challenges in precisely evaluating renal function in the patient with cirrhosis remain due to the limitations of serum creatinine (Cr) alone in estimating glomerular filtration rate (GFR); current GFR estimating models appear to underestimate renal function. Newer models incorporating renal biomarkers, such as the Cr-Cystatin C GFR Equation for Cirrhosis appear to more accurately estimate true GFR...
June 7, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28587951/treatment-na%C3%A3-ve-spontaneous-tumor-lysis-syndrome-in-metastatic-prostate-adenocarcinoma-an-unusual-suspect
#10
Maya Ignaszewski, Patrick Kohlitz
Tumor lysis syndrome (TLS) is a potentially fatal condition defined both by laboratory and clinical criteria. It is caused by the catabolism of tumor cells which leads to considerable release and elevated levels of phosphate, potassium and uric acid in the bloodstream. These electrolyte derangements predispose patients to renal tubule uric acid precipitation, acute kidney injury, arrhythmias, neuromuscular irritability and even seizures. Although this phenomenon is well described with hematological malignancies, it is also known to occur among solid tumors...
May 26, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28585336/liver-directed-human-amniotic-epithelial-cell-transplantation-improves-systemic-disease-phenotype-in-hurler-syndrome-mouse-model
#11
Natalie S Rodriguez, Lisa Yanuaria, Kevin Murphy R Parducho, Irving M Garcia, Bino A Varghese, Brendan H Grubbs, Toshio Miki
Mucopolysaccharidosis type 1 (MPS1) is an inherited lysosomal storage disorder caused by a deficiency in the glycosaminoglycan (GAG)-degrading enzyme α-l-iduronidase (IDUA). In affected patients, the systemic accumulation of GAGs results in skeletal dysplasia, neurological degeneration, multiple organ dysfunction, and early death. Current therapies, including enzyme replacement and bone marrow transplant, improve life expectancy but the benefits to skeletal and neurological phenotypes are limited. In this study, we tested the therapeutic efficacy of liver-directed transplantation of a placental stem cell, which possesses multilineage differentiation potential, low immunogenicity, and high lysosomal enzyme activity...
June 6, 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28584444/quantitative-structure-activity-relationship-study-of-the-anti-hepatitis-peptides-employing-random-forests-and-extra-trees-regressors
#12
Gunjan Mishra, Deepak Sehgal, Jayaraman K Valadi
Antimicrobial peptides are host defense peptides being viewed as replacement to broad-spectrum antibiotics due to varied advantages. Hepatitis is the commonest infectious disease of liver, affecting 500 million globally with reported adverse side effects in treatment therapy. Antimicrobial peptides active against hepatitis are called as anti-hepatitis peptides (AHP). In current work, we present Extratrees and Random Forests based Quantitative Structure Activity Relationship (QSAR) regression modeling using extracted sequence based descriptors for prediction of the anti-hepatitis activity...
2017: Bioinformation
https://www.readbyqxmd.com/read/28581881/prevalence-and-clinical-intentions-of-antithrombotic-therapy-on-discharge-to-hospice-care
#13
Christina A Kowalewska, Brie N Noble, Erik K Fromme, Mary Lynn McPherson, Kristi N Grace, Jon P Furuno
BACKGROUND: There are no guidelines for antithrombotic therapy on admission to hospice care. Antithrombotic therapy may offer some benefit in these patients, but is also associated with well-described risks. OBJECTIVE: We quantified the frequency and characteristics of patients prescribed antithrombotic therapy on discharge from acute care to hospice care. DESIGN: Retrospective cohort study. Settings/Subjects: Adult (age> = 21 years) patients discharged from acute care to hospice care between January 1, 2010 and June 30, 2014...
June 5, 2017: Journal of Palliative Medicine
https://www.readbyqxmd.com/read/28579679/yttrium-90-radioembolization-for-metastatic-colorectal-cancer-outcomes-by-number-of-lines-of-therapy
#14
REVIEW
Andrew Marsala, Edward W Lee, Siddharth A Padia
Metastatic colorectal cancer represents the most common liver malignancy, and imparts a very poor prognosis for those who develop this disease. Unlike primary liver tumors such as hepatocellular carcinoma, which largely develops in patients with underlying cirrhosis, most metastatic liver tumor patients have normal underlying liver function. Owing to this, most will succumb to tumoral replacement of the liver rather than from underlying liver dysfunction. Radioembolization represents a treatment modality that can be used in multiple fashions to treat one or both lobes of the liver...
June 2017: Seminars in Interventional Radiology
https://www.readbyqxmd.com/read/28579181/estrogen-replacement-reduces-risk-and-increases-survival-times-of-women-with-hepatocellular-carcinoma
#15
Manal M Hassan, Gehan Botrus, Reham Abdel-Wahab, Robert A Wolff, Donghui Li, David Tweardy, Alexandria T Phan, Ernest Hawk, Milind Javle, Ju-Seog Lee, Harrys A Torres, Asif Rashid, Renato Lenzi, Hesham M Hassabo, Yasmin Abaza, Ahmed S Shalaby, Sahin Lacin, Jeffrey Morris, Yehuda Z Patt, Christopher I Amos, Saira A Khaderi, John A Goss, Prasun K Jalal, Ahmed O Kaseb
BACKGROUND & AIMS: Environmental factors have been identified that affect risk of hepatocellular carcinoma (HCC), but little is known about the effects of sex hormones on liver cancer development or outcome. We investigated whether menopause hormone therapy (MHT) affects risk, age at onset, or outcome of HCC. METHODS: We performed a case-control study of 234 female patients treated for HCC at a tertiary medical center and with 282 healthy women (controls), from January 1, 2004 through May 31, 2015...
June 1, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28574999/predictors-of-renal-function-recovery-among-patients-undergoing-renal-replacement-therapy-following-orthotopic-liver-transplantation
#16
Maria Claudia Cruz Andreoli, Nádia Karina Guimarães de Souza, Adriano Luiz Ammirati, Thais Nemoto Matsui, Fabiana Dias Carneiro, Ana Claudia Mallet de Souza Ramos, Ilson Jorge Iizuca, Maria Paula Vilela Coelho, Rogério Carballo Afonso, Ben-Hur Ferraz-Neto, Marcio Dias de Almeida, Marcelino Durão, Marcelo Costa Batista, Julio Cesar Monte, Virgílio Gonçalves Pereira, Oscar Pavão Dos Santos, Bento Cardoso Dos Santos
Renal dysfunction frequently occurs during the periods preceding and following orthotopic liver transplantation (OLT), and in many cases, renal replacement therapy (RRT) is required. Information regarding the duration of RRT and the rate of kidney function recovery after OLT is crucial for transplant program management. We evaluated a sample of 155 stable patients undergoing post-intensive care hemodialysis (HD) from a patient population of 908 adults who underwent OLT. We investigated the average time to renal function recovery (duration of RRT required) and determined the risk factors for remaining on dialysis > 90 days after OLT...
2017: PloS One
https://www.readbyqxmd.com/read/28574107/continuous-renal-replacement-therapy-forty-year-anniversary
#17
Claudio Ronco
In 1977 Peter Kramer performed the first CAVH (continuous arteriovenous hemofiltration) treatment in Gottingen, Germany. CAVH soon became a reliable alternative to hemo- or peritoneal dialysis in critically ill patients. The limitations of CAVH spurred new research and the discovery of new treatments such as CVVH and CVVHD (continuous veno-venous hemofiltration and continuous veno-venous hemodialysis). The alliance with industry led to development of new specialized equipment with improved accuracy and performance in delivering continuous renal replacement therapies (CRRTs)...
May 31, 2017: International Journal of Artificial Organs
https://www.readbyqxmd.com/read/28570275/long-term-culture-of-human-liver-tissue-with-advanced-hepatic-functions
#18
Soon Seng Ng, Anming Xiong, Khanh Nguyen, Marilyn Masek, Da Yoon No, Menashe Elazar, Eyal Shteyer, Mark A Winters, Amy Voedisch, Kate Shaw, Sheikh Tamir Rashid, Curtis W Frank, Nam Joon Cho, Jeffrey S Glenn
A major challenge for studying authentic liver cell function and cell replacement therapies is that primary human hepatocytes rapidly lose their advanced function in conventional, 2-dimensional culture platforms. Here, we describe the fabrication of 3-dimensional hexagonally arrayed lobular human liver tissues inspired by the liver's natural architecture. The engineered liver tissues exhibit key features of advanced differentiation, such as human-specific cytochrome P450-mediated drug metabolism and the ability to support efficient infection with patient-derived inoculums of hepatitis C virus...
June 2, 2017: JCI Insight
https://www.readbyqxmd.com/read/28567317/open-label-fosmetpantotenate-a-phosphopantothenate-replacement-therapy-in-a-single-patient-with-atypical-pkan
#19
Yiolanda-Panayiota Christou, George A Tanteles, Elena Kkolou, Annita Ormiston, Kostas Konstantopoulos, Maria Beconi, Randall D Marshall, Horacio Plotkin, Kleopas A Kleopa
Objective. Pantothenate kinase-associated neurodegeneration (PKAN) is an autosomal recessive disorder with variable onset, rate of progression, and phenotypic expression. Later-onset, more slowly progressive PKAN often presents with neuropsychiatric as well as motor manifestations that include speech difficulties, progressive dystonia, rigidity, and parkinsonism. PKAN is caused by biallelic PANK2 mutations, a gene that encodes pantothenate kinase 2, a regulatory enzyme in coenzyme A biosynthesis. Current therapeutic strategies rely on symptomatic relief...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28557005/sarcopenia-in-alcoholic-liver-disease-clinical-and-molecular-advances
#20
Jaividhya Dasarathy, Arthur J McCullough, Srinivasan Dasarathy
Despite advances in treatment of alcohol use disorders that focus on increasing abstinence and reducing recidivism, alcoholic liver disease is projected to be the major cause of cirrhosis and its complications. Malnutrition is recognized as the most frequent complication in alcoholic liver disease (ALD) and despite the high clinical significance, there are no effective therapies to reverse malnutrition in ALD. Malnutrition is a relatively imprecise term and sarcopenia or skeletal muscle loss, the major component of malnutrition, is primarily responsible for the adverse clinical consequences in patients with liver disease...
May 29, 2017: Alcoholism, Clinical and Experimental Research
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