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#1
Carsten Oliver Schmidt, Christine Krabbe, Janka Schössow, Martin Albers, Dörte Radke, Jörg Henke
Valid scientific inferences from epidemiological and clinical studies require high data quality. Data generating departments therefore aim to detect data irregularities as early as possible in order to guide quality management processes. In addition, after the completion of data collections the obtained data quality must be evaluated. This can be challenging in complex studies due to a wide scope of examinations, numerous study variables, multiple examiners, devices, and examination centers. This paper describes a Java EE web application used to monitor and evaluate data quality in institutions with complex and multiple studies, named Square<sup>2</sup>...
2017: Studies in Health Technology and Informatics
https://www.readbyqxmd.com/read/28412075/vaccine-preferences-and-acceptance-of-older-adults
#2
R Eilers, H E de Melker, J Veldwijk, P F M Krabbe
BACKGROUND: Expanding vaccination programs for the older population might be important as older adults are becoming a larger proportion of the general population. The aim of this study is to determine the relative importance of vaccine and disease specific characteristics and acceptance for Dutch older adults, including pneumococcal disease, herpes zoster, pertussis vaccination, and influenza vaccination. METHODS: A discrete choice experiment was conducted to generate choice data that was analyzed using a mixed multinomial logit statistical model...
April 12, 2017: Vaccine
https://www.readbyqxmd.com/read/28399699/prognostic-and-discriminative-power-of-the-7th-tnm-classification-for-patients-with-surgically-treated-papillary-renal-cell-carcinoma-results-of-a-multi-institutional-validation-study-corona-subtype-project
#3
Matthias May, Cristian Surcel, Umberto Capitanio, Paolo Dell'Oglio, Tobias Klatte, Shahrokh Shariat, Thorsten Ecke, Ingmar Wolff, Daniel Vergho, Nina Wagener, Nina Huck, Sascha Pahernik, Stefan Zastrow, Manfred Wirth, Hendrik Borgmann, Axel Haferkamp, Mireia Musquera, Laura M Krabbe, Edwin Herrmann, Anna Scavuzzo, Cristian Mirvald, Georg Hutterer, Richard Zigeuner, Christian G Stief, Raphaela Waidelich, Luca Cindolo, Krystina Kalusova, Sabine D Brookman-May
OBJECTIVE: Studies on the prognostic reliability of the Union for International Cancer Control tumor, node, metastasis (TNM) staging system for renal cell carcinoma (RCC) predominantly focus on clear-cell RCC. Therefore, the aim of this study was to investigate whether the oncological prognosis of surgically treated papillary RCC (papRCC) patients is reliably given by the current TNM system, by analyzing the largest database reported to date. MATERIALS AND METHODS: Data on 2325 papRCC patients who underwent surgical treatment in 1984- 2015 were collated from 17 international centers (median follow-up 47 months)...
April 12, 2017: Scandinavian Journal of Urology
https://www.readbyqxmd.com/read/28350534/evaluation-of-a-poct-device-for-c-reactive-protein-hematocrit-and-leukocyte-differential
#4
Albert J de Graaf, Sietske W Hiemstra, Evelien W M Kemna, Johannes G Krabbe
No abstract text is available yet for this article.
March 28, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28331342/spotlight-on-atezolizumab-and-its-potential-in-the-treatment-of-advanced-urothelial-bladder-cancer
#5
REVIEW
Ahmet Murat Aydin, Solomon L Woldu, Ryan C Hutchinson, Martin Boegemann, Aditya Bagrodia, Yair Lotan, Vitaly Margulis, Laura-Maria Krabbe
Metastatic urothelial carcinoma of the bladder is an aggressive malignancy with poor prognosis, reflecting a lack of effective systemic therapies. The current standard of care includes multiagent platinum-based chemotherapy; however a majority of patients do not respond to treatment and most eventually succumb to disease. Recently, renewed interest in immunotherapy in the form of immune-checkpoint inhibition has gained widespread attention for a number of malignancies. Atezolizumab, an anti-PDL1 antibody, has been shown to be effective in a subset of patients previously treated with or unfit for platinum-based chemotherapy, and has shown durable responses with a good tolerability profile...
2017: OncoTargets and Therapy
https://www.readbyqxmd.com/read/28302345/newborn-screening-for-six-lysosomal-storage-disorders-in-a-cohort-of-mexican-patients-three-year-findings-from-a-screening-program-in-a-closed-mexican-health-system
#6
Juana Inés Navarrete-Martínez, Ana Elena Limón-Rojas, Maria de Jesús Gaytán-García, Jesús Reyna-Figueroa, Guillermo Wakida-Kusunoki, Ma Del Rocío Delgado-Calvillo, Consuelo Cantú-Reyna, Héctor Cruz-Camino, David Eduardo Cervantes-Barragán
OBJECTIVE: To evaluate the results of a lysosomal newborn screening (NBS) program in a cohort of 20,018 Mexican patients over the course of 3years in a closed Mexican Health System (Petróleos Mexicanos [PEMEX] Health Services). STUDY DESIGN: Using dried blood spots (DBS), we performed a multiplex tandem mass spectrometry enzymatic assay for six lysosomal storage disorders (LSDs) including Pompe disease, Fabry disease, Gaucher disease, mucopolysaccharidosis type I (MPS-I), Niemann-Pick type A/B, and Krabbe disease...
March 9, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28253865/measurement-properties-of-the-eq-5d-across-four-major-geriatric-conditions-findings-from-topics-mds
#7
Jennifer E Lutomski, Paul F M Krabbe, Nienke Bleijenberg, Jeanett Blom, Gertrudis I J M Kempen, Janet MacNeil-Vroomen, Maaike E Muntinga, Ewout Steyerburg, Marcel G M Olde-Rikkert, René J F Melis
BACKGROUND: As populations age, chronic geriatric conditions linked to progressive organ failure jeopardize health-related quality of life (HRQoL). Thus, this research assessed the validity and applicability of the EQ-5D (a common HRQoL instrument) across four major chronic geriatric conditions: hearing issues, joint damage, urinary incontinence, or dizziness with falls. METHODS: The study sample comprised 25,637 community-dwelling persons aged 65 years and older residing in the Netherlands (Data source: TOPICS-MDS, www...
March 3, 2017: Health and Quality of Life Outcomes
https://www.readbyqxmd.com/read/28208664/the-role-of-the-neutrophil-to-lymphocyte-ratio-for-survival-outcomes-in-patients-with-metastatic-castration-resistant-prostate-cancer-treated-with-abiraterone
#8
Martin Boegemann, Katrin Schlack, Stefan Thomes, Julie Steinestel, Kambiz Rahbar, Axel Semjonow, Andres Jan Schrader, Martin Aringer, Laura-Maria Krabbe
The purpose of this study was to examine the prognostic capability of baseline neutrophil-to-lymphocyte-ratio (NLR) and NLR-change under Abiraterone in metastatic castration-resistant prostate cancer patients. The impact of baseline NLR and change after eight weeks of treatment on progression-free survival (PFS) and overall survival (OS) was analyzed using Kaplan-Meier-estimates and Cox-regression. 79 men with baseline NLR <5 and 17 with NLR >5 were analyzed. In baseline analysis of PFS NLR >5 was associated with non-significantly shorter median PFS (five versus 10 months) (HR: 1...
February 11, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28193891/mapping-evaporative-water-loss-in-desert-passerines-reveals-an-expanding-threat-of-lethal-dehydration
#9
Thomas P Albright, Denis Mutiibwa, Alexander R Gerson, Eric Krabbe Smith, William A Talbot, Jacqueline J O'Neill, Andrew E McKechnie, Blair O Wolf
Extreme high environmental temperatures produce a variety of consequences for wildlife, including mass die-offs. Heat waves are increasing in frequency, intensity, and extent, and are projected to increase further under climate change. However, the spatial and temporal dynamics of die-off risk are poorly understood. Here, we examine the effects of heat waves on evaporative water loss (EWL) and survival in five desert passerine birds across the southwestern United States using a combination of physiological data, mechanistically informed models, and hourly geospatial temperature data...
February 28, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28170189/mesenchymal-stem-cells-yield-transient-improvements-in-motor-function-in-an-infant-rhesus-macaque-with-severe-early-onset-krabbe-disease
#10
Irina A Isakova, Kate C Baker, Jason Dufour, Donald G Phinney
Krabbe disease, or globoid cell leukodystrophy, is a rare disorder caused by deficient galactosylceramidase activity and loss of myelin-forming oligodendrocytes, resulting in progressive demyelination and severely impaired motor function. Disease symptoms in humans appear within 3-6 months of age (early infantile) and manifest as marked irritability, spasticity, and seizures. The disease is often fatal by the second year of life, with few effective treatment options. Herein we evaluated the therapeutic potential of mesenchymal stem cells (MSCs) administered intracranially to a 1-month-old rhesus macaque diagnosed with severe early-onset Krabbe disease that displayed neurologic and behavioral symptoms similar to those of human patients...
January 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28168127/krabbe-disease-report-of-a-rare-lipid-storage-and-neurodegenerative-disorder
#11
Pratyusha Pavuluri, Sabitha Vadakedath, Rajkumar Gundu, Sushmitha Uppulety, Venkataramana Kandi
Krabbe disease is a rare (one in 100,000 births) autosomal recessive condition, usually noticed among children. It causes sphingolipidosis (dysfunctional metabolism of sphingolipids) and leads to fatal degenerative changes affecting the myelin sheath of the nervous system. We report a case of a six-year-old male child who presented with symptoms of muscle spasticity and irritability. Diagnosis of this disease can only be made with clinical suspicion. Laboratory diagnosis includes brain magnetic resonance imaging (MRI), magnetic resonance (MR) spectroscopy, biochemical analysis of cerebrospinal fluid, and genetic analysis for detecting mutation in genes coding for galactosyl cerebroside (GALC)...
January 1, 2017: Curēus
https://www.readbyqxmd.com/read/28145830/corrigendum
#12
(no author information available yet)
Hinderink JB, Krabbe PFM, van den Broek P. Development and application of a health-related quality-of-life instrument for adults with cochlear implants: The Nijmegen Cochlear Implant Questionnaire. Otolaryngol Head Neck Surg. 2000;123:756-765. (Original DOI: 10.1067/mhn.2000.108203 ).
February 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/28117439/a-competitive-inhibitory-circuit-for-selection-of-active-and-passive-fear-responses
#13
Jonathan P Fadok, Sabine Krabbe, Milica Markovic, Julien Courtin, Chun Xu, Lema Massi, Paolo Botta, Kristine Bylund, Christian Müller, Aleksandar Kovacevic, Philip Tovote, Andreas Lüthi
When faced with threat, the survival of an organism is contingent upon the selection of appropriate active or passive behavioural responses. Freezing is an evolutionarily conserved passive fear response that has been used extensively to study the neuronal mechanisms of fear and fear conditioning in rodents. However, rodents also exhibit active responses such as flight under natural conditions. The central amygdala (CEA) is a forebrain structure vital for the acquisition and expression of conditioned fear responses, and the role of specific neuronal sub-populations of the CEA in freezing behaviour is well-established...
January 25, 2017: Nature
https://www.readbyqxmd.com/read/28109651/late-onset-krabbe-disease-due-to-the-new-galc-p-ala543pro-mutation-with-intriguingly-high-residual-galc-activity-in%C3%A2-vitro
#14
Inge Krägeloh-Mann, Klaus Harzer, Kevin Rostásy, Stefanie Beck-Wödl, Antje Bornemann, Judith Böhringer, Andrea Bevot, Verena Beck, Gisela Merkel, Maria Pechan
BACKGROUND: Krabbe disease (KD) is an inherited leukodystrophy due to a defect in the GALC gene which encodes the lysosomal galactosylceramide β-galactosidase (GALC). About two thirds of patients show the early onset form of KD dominated by cerebral demyelination leading to death in early infancy. Late onset forms include a spectrum of late infantile, juvenile and adult clinical courses. The deficiency of GALC leads to a galactosylceramide lipidosis in which lysosomal storage phenomena are seen almost only at the ultrastructural level...
January 6, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28103109/impaired-spermatogenesis-in-the-twitcher-mouse-a-morphological-evaluation-from-the-seminiferous-tubules-to-epididymal-transit
#15
Alice Luddi, Martina Gori, Laura Crifasi, Camilla Marrocco, Giuseppe Belmonte, Elvira Costantino-Ceccarini, Paola Piomboni
Spermatogenesis is a complex process of proliferation and differentiation during male germ cell development whereby undifferentiated spermatogonial germ cells evolve into maturing spermatozoa. In this developmental process the interactions between different cell types are finely regulated, hence any disruption in these relationships leads to male infertility. The twitcher mouse, the murine model of Krabbe disease, is characterized by deficiency of galactosylceramidase, an enzyme also involved in the metabolism of the galactosyl-alkyl-acyl-glycerol, the precursor of sulfogalactosyl-alkyl-acyl-glycerol, the most abundant glycolipid in spermatozoa...
April 2017: Systems Biology in Reproductive Medicine
https://www.readbyqxmd.com/read/28095876/multi-criteria-decision-analysis-mcda-testing-a-proposed-mcda-framework-for-orphan-drugs
#16
C Schey, P F M Krabbe, M J Postma, M P Connolly
BACKGROUND: Since the introduction of the orphan drugs legislation in Europe, it has been suggested that the general method of assessing drugs for reimbursement is not necessarily suitable for orphan drugs. The National Institute for Health and Clinical Excellence indicated that several criteria other than cost and efficacy could be considered in reimbursement decisions for orphan drugs. This study sought to explore the multi-criteria decision analysis (MCDA) framework proposed by (Orphanet J Rare Dis 7:74, 2012) to a range of orphan drugs, with a view to comparing the aggregate scores to the average annual cost per patient for each product, and thus establishing the merit of MCDA as a tool for assessing the value of orphan drugs in relation to their pricings...
January 17, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28075543/prognostic-value-of-tissue-based-biomarker-signature-in-clear-cell-renal-cell-carcinoma
#17
Ahmed Q Haddad, Jun-Hang Luo, Laura-Maria Krabbe, Oussama Darwish, Bishoy Gayed, Ramy Youssef, Payal Kapur, Dinesh Rakheja, Yair Lotan, Arthur Sagalowsky, Vitaly Margulis
OBJECTIVE: To improve risk stratification for recurrence prognostication in patients with localised clear cell renal cell carcinoma (ccRCC). PATIENTS AND METHODS: In all, 367 patients with non-metastatic ccRCC were included. The cohort was divided into a training and validation set. Using tissue microarrays, immunostaining was performed for 24 biomarkers representative of key pathways in ccRCC. Using Least Absolute Shrinkage and Selection Operator (LASSO) Cox regression, we identified several markers that were used to construct a risk classifier for risk of disease recurrence...
January 11, 2017: BJU International
https://www.readbyqxmd.com/read/28052886/intestinal-rehabilitation-for-children-with-intestinal-failure-is-cost-effective-a-simulation-study
#18
Henk Groen, Esther G Neelis, Marten J Poley, Joanne F Olieman, René Scheenstra, Paul Fm Krabbe, Gerard Dijkstra, Edmond Hhm Rings
BACKGROUND: Children with intestinal failure (IF) depend on parenteral nutrition (PN). The goal in the treatment of IF is to wean children off PN through intestinal rehabilitation (IR). Although the healthcare burden of IF is enormous, to our knowledge there has been no previous cost-effectiveness analysis in pediatric IF including IR. OBJECTIVE: We sought to determine the cost-effectiveness of IR in terms of costs and life-years. DESIGN: We simulated the treatment of IF in children in a discrete-event model...
February 2017: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28050682/competition-and-quality-indicators-in-the-health-care-sector-empirical-evidence-from-the-dutch-hospital-sector
#19
R R Croes, Y J F M Krabbe-Alkemade, M C Mikkers
There is much debate about the effect of competition in healthcare and especially the effect of competition on the quality of healthcare, although empirical evidence on this subject is mixed. The Netherlands provides an interesting case in this debate. The Dutch system could be characterized as a system involving managed competition and mandatory healthcare insurance. Information about the quality of care provided by hospitals has been publicly available since 2008. In this paper, we evaluate the relationship between quality scores for three diagnosis groups and the market power indicators of hospitals...
January 3, 2017: European Journal of Health Economics: HEPAC: Health Economics in Prevention and Care
https://www.readbyqxmd.com/read/28045982/fecal-bile-salts-and-the-development-of-necrotizing-enterocolitis-in-preterm-infants
#20
Christian V Hulzebos, Anne G J F van Zoonen, Jan B F Hulscher, Trijntje E Schat, Elisabeth M W Kooi, Martijn Koehorst, Renze Boverhof, Paul F M Krabbe, Albert K Groen, Henkjan J Verkade
BACKGROUND: Intestinal bile salts (BSs) may be implicated in NEC development. We hypothesized that fecal BS levels are higher in preterm infants at risk for NEC. METHODS: We compared the composition and concentration of fecal BSs in ten preterm infants who developed NEC (Bell's Stage ≥ II) with twenty matched control infants without NEC. Conjugated and unconjugated fecal BSs were measured after birth (T1) and twice prior to NEC (T2, T3). Data are presented as medians and interquartile ranges...
2017: PloS One
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