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https://www.readbyqxmd.com/read/29165738/alloantibodies-in-von-willebrand-disease
#1
Massimo Franchini, Pier Mannuccio Mannucci
von Willebrand disease (VWD), the most commonly known inherited bleeding disorder, is caused by a partial (type 1) or total (type 3) deficiency or dysfunction (type 2) of von Willebrand factor (VWF). Its management encompasses the prevention or treatment of bleeding by raising endogenous VWF levels using a synthetic agent, such as desmopressin, or providing exogenous VWF concentrates. The development of inhibitory alloantibodies against VWF is a rare but often severe complication encountered during the treatment of type 3 VWD, which is associated with a lack of hemostatic response to infused VWF concentrates and more rarely with allergic, even anaphylactic, reactions...
November 17, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29165644/-the-money-can-be-a-motivator-to-me-a-little-but-mostly-pbf-just-helps-me-to-do-better-in-my-job-an-exploration-of-the-motivational-mechanisms-of-performance-based-financing-for-health-workers-in-malawi
#2
Julia Lohmann, Danielle Wilhelm, Christabel Kambala, Stephan Brenner, Adamson S Muula, Manuela De Allegri
Performance-based financing (PBF) is assumed to improve health care delivery by motivating health workers to enhance their work performance. However, the exact motivational mechanisms through which PBF is assumed to produce such changes are poorly understood to date. Although PBF is increasingly recognized as a complex health systems intervention, its motivational effect for individual health workers is still often reduced to financial 'carrots and sticks' in the literature and discourse. Aiming to contribute to the development of a more comprehensive understanding of the motivational mechanisms, we explored how PBF impacted health worker motivation in the context of the Malawian Results-based Financing for Maternal and Newborn Health (RBF4MNH) Initiative...
November 20, 2017: Health Policy and Planning
https://www.readbyqxmd.com/read/29165056/citrinin-induces-apoptosis-in-human-hct116-colon-cancer-cells-through-endoplasmic-reticulum-stress
#3
Amal Salah, Chayma Bouaziz, Alexandre Prola, Julie Pires Da Silva, Hassen Bacha, Salwa Abid-Essefi, Christophe Lemaire
The mycotoxin citrinin (CTN) is a natural contaminant of various human foods that may produce serious adverse health problems. Several studies demonstrated that citrinin exerts cytotoxic and genotoxic effects in both in vivo and in vitro systems. However, the precise mechanisms of action (MOA), particularly in intestinal cells remain unclear. The aim of the present study was to examine the precise MOA of citrinin in vitro. Data demonstrated that CTN significantly decreased the number of viable human intestinal HCT116 cells and induced apoptotic events including (1) decrease in ΔѰm indicative of mitochondrial membrane permeabilization, (2) activation of caspase 3, (3) elevated production of reactive oxygen species (ROS) and (4) relative persistence of plasma membrane integrity...
2017: Journal of Toxicology and Environmental Health. Part A
https://www.readbyqxmd.com/read/29165051/a-structure-function-analysis-in-patients-with-prekallikrein-deficiency
#4
Antonio Girolami, Silvia Ferrari, Elisabetta Cosi, Anna Maria Lombardi
OBJECTIVE: To investigate the structure-function relation in prekallikrein (PK) deficiency. PK is one of the proteins of the contact phase of blood coagulation which at the present time is the object of a revival of interest. METHODS: All patients with PK deficiency who had been investigated by molecular biology techniques are the object of the present investigation. Details of patients were obtained from personal files and a time-unlimited PubMed search. Only cases with a molecular-biology-based diagnosis were included...
November 22, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/29164820/dual-ppar%C3%AE-%C3%AE-agonist-saroglitazar-improves-liver-histopathology-and-biochemistry-in-experimental-nash-models
#5
Mukul R Jain, Suresh R Giri, Bibhuti Bhoi, Chitrang Trivedi, Akshyaya Rath, Rohan Rathod, Ramchandra Ranvir, Shekhar Kadam, Hiren Patel, Prabodha Swain, Sib Sankar Roy, Nabanita Das, Eshani Karmakar, Walter Wahli, Pankaj R Patel
BACKGROUND & AIMS: Non-alcoholic fatty liver disease (NAFLD) and non-alcoholic steatohepatitis (NASH) are common clinico-pathological conditions that affect millions of patients worldwide. In this study, the efficacy of saroglitazar, a novel PPARα/γ agonist, was assessed in models of NAFLD/NASH. METHODS & RESULTS: HepG2 cells treated with palmitic acid (PA;0.75 mM) showed decreased expression of various antioxidant biomarkers (SOD1, SOD2, glutathione peroxidase and catalase) and increased expression of inflammatory markers (TNFα,IL1β and IL6)...
November 21, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29163963/capsule-endoscopy-in-young-patients-with-iron-deficiency-anaemia-and-negative-bidirectional-gastrointestinal-endoscopy
#6
Diana E Yung, Emanuele Rondonotti, Andry Giannakou, Tomer Avni, Bruno Rosa, Ervin Toth, Alfredo J Lucendo, Reena Sidhu, Hanneke Beaumont, Pierre Ellul, Lucian Negreanu, Victoria Alejandra Jiménez-Garcia, Deidre McNamara, Uri Kopylov, Luca Elli, Konstantinos Triantafyllou, Fahmi Shibli, Maria Elena Riccioni, Mauro Bruno, Xavier Dray, John N Plevris, A Koulaouzidis, Federico Argüelles-Arias, Aymeric Becq, Federica Branchi, María Ángeles Tejero-Bustos, Jose Cotter, Rami Eliakim, Francesca Ferretti, Ian M Gralnek, Juan Manuel Herrerias-Gutierrez, Mary Hussey, Maarten Jacobs, Gabriele Wurm Johansson, Mark McAlindon, Sara Montiero, Artur Nemeth, Marco Pennazio, Deepa Rattehalli, Ana Stemate, Annalisa Tortora, Georgios Tziatzios
Background: Recent data imply young patients (age ≤50 years) undergoing small-bowel (SB) capsule endoscopy (CE) for iron deficiency anaemia (IDA) show higher diagnostic yield (DY) for sinister pathology. We aimed to investigate DY of CE in a large cohort of young IDA patients, and evaluate factors predicting significant SB pathology. Materials and methods: This was a retrospective, multicentre study (2010-2015) in consecutive, young patients (≤50 years) from 18 centres/12 countries, with negative bidirectional gastrointestinal (GI) endoscopy undergoing SBCE for IDA...
November 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/29163554/isoliquiritigenin-activates-nuclear-factor-erythroid-2-related-factor-2-to-suppress-the-nod-like-receptor-protein-3-inflammasome-and-inhibits-the-nf-%C3%AE%C2%BAb-pathway-in-macrophages-and-in-acute-lung-injury
#7
Qinmei Liu, Hongming Lv, Zhongmei Wen, Xinxin Ci, Liping Peng
Among the cellular response mechanisms, the nuclear factor erythroid-2 related factor 2 (Nrf2) pathway is considered a survival pathway that alleviates oxidative injury, while both the NOD-like receptor protein 3 (NLRP3) and NF-κB pathways are pro-inflammatory pathways that cause damage to cells. These pathways are implicated in the development and resolution of acute lung injury (ALI). Isoliquiritigenin (ISL), a flavonoid from the liquorice compound, is suggested to be a regulator of the above pathways, but the mechanisms of how the NLRP3/NF-κB pathway interacts with Nrf2 and its protective effects in ALI remain unknown...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/29163437/transport-deficiency-is-the-molecular-basis-of-candida-albicans-resistance-to-antifungal-oligopeptides
#8
Marta Schielmann, Piotr Szweda, Katarzyna Gucwa, Marcin Kawczyński, Maria J Milewska, Dorota Martynow, Joachim Morschhäuser, Sławomir Milewski
Oligopeptides incorporating N(3)-(4-methoxyfumaroyl)-L-2,3-diaminopropanoic acid (FMDP), an inhibitor of glucosamine-6-phosphate synthase, exhibited growth inhibitory activity against Candida albicans, with minimal inhibitory concentration values in the 0.05-50 μg mL(-1) range. Uptake by the peptide permeases was found to be the main factor limiting an anticandidal activity of these compounds. Di- and tripeptide containing FMDP (F2 and F3) were transported by Ptr2p/Ptr22p peptide transporters (PTR) and FMDP-containing hexa-, hepta-, and undecapeptide (F6, F7, and F11) were taken up by the oligopeptide transporters (OPT) oligopeptide permeases, preferably by Opt2p/Opt3p...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/29163168/atheroregressive-potential-of-the-treatment-with-a-chimeric-monoclonal-antibody-against-sulfated-glycosaminoglycans-on-pre-existing-lesions-in-apolipoprotein-e-deficient-mice
#9
Victor Brito, Katia Mellal, Karina F Zoccal, Yosdel Soto, Liliane Ménard, Roger Sarduy, Lucia H Faccioli, Huy Ong, Ana M Vázquez, Sylvie Marleau
The retention of lipoprotein particles in the intima, in particular to glycosaminoglycan side chains of proteoglycans, is a critical step in atherosclerosis initiation. Administration of chP3R99, a chimeric mouse/human monoclonal antibody inducing an anti-idiotypic network response against glycosaminoglycans was previously shown to prevent atherosclerotic lesion progression, yet its effect in the late-stage progression of lesions remains unknown. This study investigated the effect of chP3R99 at a late stage of disease development in apolipoprotein E-deficient mice and the vascular mechanisms involved...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29162954/analyses-of-nutrients-and-body-mass-index-as-risk-factor-for-preeclampsia
#10
Yusrawati, Nicko Pisceski Kusika Saputra, Nur Indrawati Lipoeto, Rizanda Machmud
Background: Little is known about the pathogenesis of preeclampsia. Many factors are identified as risk factors for preeclampsia including nutrients and obesity. The aim of this study was to assess whether nutrients and body mass index (BMI) are risk factors for preeclampsia. Methods: This was a case-control study at the Department of Obstetric and Gynecology in Dr. M. Djamil Hospital, Padang, Indonesia. A total of 140 patients were enrolled in this study with 70 cases and 70 controls...
December 2017: Journal of Obstetrics and Gynaecology of India
https://www.readbyqxmd.com/read/29161904/consequences-of-testing-for-mismatch-repair-deficiency-of-colorectal-cancer-in-clinical-practice
#11
L W Leicher, M H A Lammertink, S R Offerman, H Morreau, M M de Jong, J W B de Groot, H L van Westreenen, H F A Vasen, W H de Vos Tot Nederveen Cappel
INTRODUCTION: Mismatch repair deficiency (dMMR) can be found in Lynch syndrome (LS)-associated colorectal carcinoma and in 15% of sporadic colorectal cancer (CRC). Outcome of MMR-deficiency testing is important for surgical decisions as extended colectomy is recommended in young LS-patients with CRC. Moreover, the finding of a dMMR tumour has consequences for the choices of adjuvant chemotherapy as MMR-deficient CRC is resistant to 5-fluorouracil (5-FU) monotherapy. Aims of our study are to evaluate whether MMR-deficiency testing leads to (1) identification of LS, (2) change in surgical treatment and (3) adjustment of systemic therapy in patients with dMMR CRC...
November 21, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/29161317/evaluating-architecture-impact-on-system-energy-efficiency
#12
Shijie Yu, Hailong Yang, Rui Wang, Zhongzhi Luan, Depei Qian
As the energy consumption has been surging in an unsustainable way, it is important to understand the impact of existing architecture designs from energy efficiency perspective, which is especially valuable for High Performance Computing (HPC) and datacenter environment hosting tens of thousands of servers. One obstacle hindering the advance of comprehensive evaluation on energy efficiency is the deficient power measuring approach. Most of the energy study relies on either external power meters or power models, both of these two methods contain intrinsic drawbacks in their practical adoption and measuring accuracy...
2017: PloS One
https://www.readbyqxmd.com/read/29159972/vitamin-b12-deficiency-and-impaired-expression-of-amnionless-during-aging
#13
Alice Pannérec, Eugenia Migliavacca, Antonio De Castro, Joris Michaud, Sonia Karaz, Laurence Goulet, Serge Rezzi, Tze Pin Ng, Nabil Bosco, Anis Larbi, Jerome N Feige
BACKGROUND: Physical frailty and loss of mobility in elderly individuals lead to reduced independence, quality of life, and increased mortality. Vitamin B12 deficiency has been linked to several age-related chronic diseases, including in the musculo-skeletal system, where vitamin B12 deficiency is generally believed to be linked to poor nutritional intake. In the present study, we asked whether aging and frailty associate with altered vitamin B12 homeostasis in humans and investigated the underlying molecular mechanisms using preclinical models...
November 21, 2017: Journal of Cachexia, Sarcopenia and Muscle
https://www.readbyqxmd.com/read/29159707/propionyl-coa-carboxylase-pcca-1-and-pccb-1-gene-deletions-in-caenorhabditis-elegans-globally-impair-mitochondrial-energy-metabolism
#14
Kimberly A Chapman, Julian Ostrovsky, Meera Rao, Stephen D Dingley, Erzsebet Polyak, Marc Yudkoff, Rui Xiao, Michael J Bennett, Marni J Falk
Propionic acidemia (PA) is a classical inborn error of metabolism with high morbidity that results from the inability of the propionyl-CoA carboxylase (PCC) enzyme to convert propionyl-CoA to methylmalonyl-CoA. PA is inherited in an autosomal recessive fashion due to functional loss of both alleles of either PCCA or PCCB. These genes are highly conserved across evolutionarily diverse species and share extensive similarity with pcca-1 and pccb-1 in the nematode, Caenorhabditis elegans. Here, we report the global metabolic effects of deletion in a single PCC gene, either pcca-1 or pccb-1, in C...
November 20, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29158361/prochemerin-cleavage-by-factor-xia-links-coagulation-and-inflammation
#15
Xiaomei Ge, Yasuto Yamaguchi, Lei Zhao, Loredana Bury, Paolo Gresele, Caroline Berube, Lawrence L Leung, John Morser
Chemerin is a chemoattractant and adipokine that circulates in blood as inactive prochemerin (chem163S). Chem163S is activated by a series of C-terminal proteolytic cleavages resulting in diverse chemerin forms with different levels of activity. We screened a panel of proteases in the coagulation, fibrinolytic and inflammatory cascades to identify those that process prochemerin in plasma. Factor XIa (FXIa) cleaved chem163S, generating a novel chemerin form, chem162R, as an intermediate product, and chem158K, as the final product...
November 20, 2017: Blood
https://www.readbyqxmd.com/read/29157486/nouveaut%C3%A3-s-dans-l%C3%A2-hyperplasie-cong%C3%A3-nitale-des-surr%C3%A3-nales-new-insights-in-congenital-adrenal-hyperplasia
#16
L Dumeige, C Bouvattier, M Lombès
Congenital adrenal hyperplasia is an autosomal recessive disease due to functional abnormalities of adrenal steroid enzymes. The most common form of the disease is due to a 21-hydroxylase deficiency. The classical forms (most severe) are characterized by a deficiency in cortisol and sometimes in aldosterone, which may compromise the vital prognosis of neonates, and by an increase in androgen synthesis, leading to the virilization of girls' external genitalia at birth, followed by clinical signs of hyperandrogenism during childhood and adolescence...
October 2017: Annales D'endocrinologie
https://www.readbyqxmd.com/read/29156507/liver-transplantation-in-adults-with-liver-disease-due-to-common-variable-immunodeficiency-leads-to-early-recurrent-disease-and-poor-outcome
#17
Vian Azzu, Joshua E Elias, Adam Duckworth, Susan Davies, Rebecca Brais, Dinakantha S Kumararatne, Alexander E S Gimson, William J H Griffiths
Common variable immunodeficiency (CVID) is the commonest from of primary immunodeficiency characterized by antibody deficiency, recurrent bacterial infections and autoimmunity. Advanced chronic liver disease occurs in a subset of patients with CVID and manifests with various histological features such as nodular regenerative hyperplasia, inflammation, fibrosis and cholangiopathy. We present a case series characterizing the outcomes in adult patients transplanted for primary CVID-related liver disease. We discuss the unique transplantation challenges faced in this primary immunodeficiency group including susceptibility to infections and early disease recurrence...
November 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29155436/enzyme-replacement-therapy-for-infantile-onset-pompe-disease
#18
REVIEW
Min Chen, Lingli Zhang, Shuyan Quan
BACKGROUND: Infantile-onset Pompe disease is a rare and progressive autosomal-recessive disorder caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). Current treatment involves enzyme replacement therapy (with recombinant human alglucosidase alfa) and symptomatic therapies (e.g. to control secretions). Children who are cross-reactive immunological material (CRIM)-negative require immunomodulation prior to commencing enzyme replacement therapy.Enzyme replacement therapy was developed as the most promising therapeutic approach for Pompe disease; however, the evidence is lacking, especially regarding the optimal dose and dose frequency...
November 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29153638/%C3%AE-1-anti-trypsin-improves-function-of-porcine-donor-lungs-during-ex-vivo-lung-perfusion
#19
Huiqing Lin, Manyin Chen, Feng Tian, Jussi Tikkanen, Lei Ding, Hei Yu Andrew Cheung, Daisuke Nakajima, Zhe Wang, Andrea Mariscal, David Hwang, Marcelo Cypel, Shaf Keshavjee, Mingyao Liu
BACKGROUND: Ex-vivo lung perfusion (EVLP), a technique for donor lung assessment, also represents a platform for donor lung repair and immunomodulation. α1-Anti-trypsin (A1AT), a medication used to treat emphysema in A1AT-deficient patients, has anti-inflammatory properties and has been shown to attenuate ischemia-reperfusion injury in rat and pig lung transplants. The objective of this study was to determine whether administration of A1AT during EVLP can improve donor lung quality after prolonged hypothermic preservation...
October 2, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29153568/maternal-obesity-and-cardiac-development-in-the-offspring-study-in-human-neonates-and-minipigs
#20
Maria Angela Guzzardi, Tiziana Liistro, Luna Gargani, Lamia Ait Ali, Gennaro D'Angelo, Silvia Rocchiccioli, Federica La Rosa, Alessandra Kemeny, Elena Sanguinetti, Nadia Ucciferri, Mariarosaria De Simone, Antonietta Bartoli, Pierluigi Festa, Piero A Salvadori, Silvia Burchielli, Rosa Sicari, Patricia Iozzo
OBJECTIVES: The aim of this study was to investigate the consequences of maternal overweight on cardiac development in offspring in infants (short term) and minipigs (short and longer term). BACKGROUND: The epidemic of overweight involves pregnant women. The uterine environment affects organ development, modulating disease susceptibility. Offspring of obese mothers have higher rates of cardiovascular events and mortality. METHODS: Echocardiography was performed in infants born to lean and overweight mothers at birth and at 3, 6, and 12 months of age...
November 10, 2017: JACC. Cardiovascular Imaging
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