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https://www.readbyqxmd.com/read/29748098/multi-institutional-evaluation-of-the-prognostic-significance-of-ezh2-expression-in-high-grade-upper-tract-urothelial-carcinoma
#1
Nirmish Singla, Laura-Maria Krabbe, Ahmet M Aydin, Vandana Panwar, Solomon L Woldu, Yuval Freifeld, Christopher G Wood, Jose A Karam, Alon Z Weizer, Jay D Raman, Mesut Remzi, Nathalie Rioux-Leclercq, Andrea Haitel, Marco Roscigno, Christian Bolenz, Karim Bensalah, Arthur I Sagalowsky, Shahrokh F Shariat, Yair Lotan, Aditya Bagrodia, Payal Kapur, Vitaly Margulis
PURPOSE: Enhancer of zeste homolog 2 is a methyltransferase encoded by the EZH2 gene, whose role in upper tract urothelial carcinoma (UTUC) is poorly understood. We sought to evaluate the prognostic value of EZH2 expression in UTUC. METHODS: We reviewed a multi-institutional cohort of patients who underwent radical nephroureterectomy for high-grade UTUC from 1990 to 2008. Immunohistochemistry for EZH2 was performed on tissue microarrays. Percentage of staining was evaluated, and the discriminative value of EZH2 was tested, with EZH2 positivity defined as>20% staining present...
May 7, 2018: Urologic Oncology
https://www.readbyqxmd.com/read/29692687/value-judgment-of-health-interventions-from-different-perspectives-arguments-and-criteria
#2
REVIEW
Karin M Vermeulen, Paul F M Krabbe
Background: The healthcare sector is evolving while life expectancy is increasing. These trends put greater pressure on healthcare resources, prompt healthcare reforms, and demand transparent arguments and criteria to assess the overall value of health interventions. There is no consensus on the core criteria by which to value and prioritize interventions, and individual stakeholders might value specific elements differently. The present study is based on a literature review that retrieved the most widely recognized arguments and criteria used in decision-making...
2018: Cost Effectiveness and Resource Allocation: C/E
https://www.readbyqxmd.com/read/29623914/heterozygous-carriers-of-galactocerebrosidase-mutations-that-cause-krabbe-disease-have-impaired-microglial-function-and-defective-repair-of-myelin-damage
#3
REVIEW
Nicole J Scott-Hewitt, Christopher J Folts, Mark D Noble
This review addresses two puzzling findings related to mutations in galactocerebrosidase (GALC) that cause Krabbe disease (KD), a severe lysosomal storage disorder characterized by extensive myelin damage in children with mutations in both GALC alleles. First, heterozygous carriers of KD-causing mutations, which include the biological parents of children with KD, exhibit increased risk for developing other diseases. Second, variants in the GALC locus increase the risk of developing multiple sclerosis (MS), another disease characterized by extensive myelin damage...
March 2018: Neural Regeneration Research
https://www.readbyqxmd.com/read/29623559/head-to-head-comparison-of-eq-5d-3l-and-eq-5d-5l-health-values
#4
Anna Selivanova, Erik Buskens, Paul F M Krabbe
BACKGROUND: The EQ-5D is a widely used preference-based instrument to measure health-related quality of life. Some methodological drawbacks of its three-level version (EQ-5D-3L) prompted development of a new format (EQ-5D-5L). There is no clear evidence that the new format outperforms the standard version. OBJECTIVE: The objective of this study was to make a head-to-head comparison of the EQ-5D-3L and EQ-5D-5L in a discrete choice model setting giving special attention to the consistency and logical ordering of coefficients for the attribute levels and to the differences in health-state values...
April 6, 2018: PharmacoEconomics
https://www.readbyqxmd.com/read/29622486/autopsy-neuropathology-findings-in-a-child-with-chronic-infantile-krabbe-leukoencephalopathy
#5
Marc R Del Bigio
No abstract text is available yet for this article.
March 9, 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29615819/unfolded-protein-response-is-activated-in-krabbe-disease-in-a-manner-dependent-on-the-mutation-type
#6
Kaori Irahara-Miyana, Takanobu Otomo, Hidehito Kondo, Mohammad Arif Hossain, Keiichi Ozono, Norio Sakai
Krabbe disease, one of the autosomal-recessive lysosomal storage disorders (LSDs), is caused by a deficiency of galactocerebrosidase (GALC) activity, resulting in the intracellular accumulation of psychosine, which is cytotoxic for neuronal cells. Genetically pathogenic mutations result in conformational changes in GALC and disrupt the lysosmal trafficking of cargos, which subsequently accumulate in the endoplasmic reticulum (ER). Recently, ER stress together with the activation of the unfolded protein response (UPR) has been suggested to play a key role in the pathogenesis of LSDs...
April 3, 2018: Journal of Human Genetics
https://www.readbyqxmd.com/read/29572534/the-effects-of-hydroxyethyl-starch-and-gelatine-on-pulmonary-cytokine-production-and-oedema-formation
#7
Julia Krabbe, Nadine Ruske, Till Braunschweig, Svetlana Kintsler, Jan W Spillner, Thomas Schröder, Sebastian Kalverkamp, Stephanie Kanzler, Annette D Rieg, Stefan Uhlig, Christian Martin
Recently, side effects of plasma expanders like hydroxyethyl starch and gelatine gained considerable attention. Most studies have focused on the kidneys; lungs remain unconsidered. Isolated mouse lungs were perfused for 4 hours with buffer solutions based on hydroxyethyl starch (HES) 130/0.4, HES 200/0.5 or gelatine and ventilated with low or high pressure under physiological pH and alkalosis. Outcome parameters were cytokine levels and the wet-to-dry ratio. For cytokine release, murine and human PCLS were incubated in three different buffers and time points...
March 23, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29556419/inflammatory-and-structural-changes-in-vertebral-bodies-and-posterior-elements-of-the-spine-in-axial-spondyloarthritis-construct-validity-responsiveness-and-discriminatory-ability-of-the-anatomy-based-canden-scoring-system-in-a-randomised-placebo-controlled
#8
Simon Krabbe, Inge J Sørensen, Bente Jensen, Jakob M Møller, Lone Balding, Ole R Madsen, Robert G W Lambert, Walter P Maksymowych, Susanne J Pedersen, Mikkel Østergaard
Background: The Canada-Denmark (CANDEN) definitions of spinal MRI lesions allow a detailed anatomy-based evaluation of inflammatory and structural lesions in vertebral bodies and posterior elements of the spine in patients with axial spondyloarthritis (axSpA). The objective was to examine the reliability, responsiveness and discrimination of scores for spinal inflammation, fat, bone erosion and new bone formation based on the CANDEN system and to describe patterns of inflammatory and structural lesions and their temporal development...
2018: RMD Open
https://www.readbyqxmd.com/read/29551464/the-effects-of-zinc-and-copper-containing-welding-fumes-on-murine-rat-and-human-precision-cut-lung-slices
#9
Julia Krabbe, André Esser, Stephanie Kanzler, Till Braunschweig, Svetlana Kintsler, Jan Spillner, Thomas Schröder, Sebastian Kalverkamp, Galina Balakirski, Benjamin Gerhards, Annette D Rieg, Thomas Kraus, Peter Brand, Christian Martin
Recently, the pro-inflammatory effects of metal inert gas brazing welding fumes containing zinc and copper have been demonstrated in humans. Here, murine, rat and human precision cut lung slices (PCLS) were incubated in welding fume containing media with 0.1, 1, 10 and 100 μg/ml for 24 or 48 h. 24 h incubation were determined either by incubation for the total time or for only 6 h followed by a 18 h post-incubation phase. Cytotoxicity, proliferation and DNA repair rates, and cytokine levels were determined...
March 10, 2018: Journal of Trace Elements in Medicine and Biology
https://www.readbyqxmd.com/read/29516569/a-hilic-ms-ms-method-for-simultaneous-quantification-of-the-lysosomal-disease-markers-galactosylsphingosine-and-glucosylsphingosine-in-mouse-serum
#10
Rohini Sidhu, Christina R Mikulka, Hideji Fujiwara, Mark S Sands, Jean E Schaffer, Daniel S Ory, Xuntian Jiang
Deficiencies of galactosylceramidase and glucocerebrosidase result in the accumulation of galactosylsphingosine (GalSph) and glucosylsphingosine (GluSph) in Krabbe and Gaucher diseases, respectively. GalSph and GluSph are useful biomarkers for both diagnosis and monitoring of treatment effects. We have developed and validated a sensitive, accurate, high-throughput assay for simultaneous determination of the concentration of GalSph and GluSph in mouse serum. GalSph and GluSph and their deuterated internal standards were extracted by protein precipitation in quantitative recoveries, baseline separated by hydrophilic interaction chromatography and detected by positive-ion electrospray mass spectrometry in multiple reaction monitoring mode...
March 8, 2018: Biomedical Chromatography: BMC
https://www.readbyqxmd.com/read/29499258/the-adverse-survival-implications-of-bland-thrombus-in-renal-cell-carcinoma-with-venous-tumor-thrombus
#11
Ryan Hutchinson, Charles Rew, Gong Chen, Solomon Woldu, Laura-Maria Krabbe, Matthew Meissner, Kunj Sheth, Nirmish Singla, Nabeel Shakir, Viraj A Master, Jose A Karam, Surena F Matin, Leonardo D Borregales, Christopher Wood, Timothy Masterson, R Houston Thompson, Stephen A Boorjian, Bradley C Leibovich, E Jason Abel, Aditya Bagrodia, Vitaly Margulis
OBJECTIVE: To characterize the presence of bland (nontumor) thrombus in advanced renal cell carcinoma and assess the impact of this finding on cancer-specific survival. METHODS: A multi-institutional database of patients treated with nephrectomy with caval thrombectomy for locally-advanced renal tumors was assembled from 5 tertiary care medical centers. Using clinicopathologic variables including patient age, body mass index, Eastern Cooperative Oncology Group performance status, tumor stage, grade, nodal status and histology, and nearest-neighbor and multiple-matching propensity score matched cohorts of bland thrombus vs nonbland thrombus patients were assessed...
February 27, 2018: Urology
https://www.readbyqxmd.com/read/29495920/multifocal-vertebral-sclerosing-bone-changes-and-soft-tissue-masses-caused-by-hodgkin-s-lymphoma-in-a-patient-with-systemic-lupus-erythematosus-a-case-report
#12
S Krabbe, J Helweg-Larsen, A Loft, S Jacobsen
No abstract text is available yet for this article.
March 2, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29481565/analysis-of-age-related-changes-in-psychosine-metabolism-in-the-human-brain
#13
Michael S Marshall, Benas Jakubauskas, Wil Bogue, Monika Stoskute, Zane Hauck, Emily Rue, Matthew Nichols, Lisa L DiAntonio, Richard B van Breemen, Jeffrey H Kordower, Carlos A Saavedra-Matiz, Ernesto R Bongarzone
α-Synuclein aggregation has been linked to Gaucher's disease (GD) and Krabbe's disease (KD), lysosomal conditions affecting glycosphingolipid metabolism. α-Synuclein pathology has been directly attributed to the dysregulation of glycosphingolipids in both conditions, specifically to increased galactosylsphingosine (psychosine) content in the context of KD. Furthermore, the gene (GALC) coding for the psychosine degrading enzyme galactosylceramidase (GALC), has recently been identified as a risk loci for Parkinson's disease...
2018: PloS One
https://www.readbyqxmd.com/read/29479774/hematopoietic-stem-cell-transplantation-in-late-onset-krabbe-disease-no-evidence-of-worsening-demyelination-and-axonal-loss-4-years-post-allograft
#14
Cornelia Laule, Irene M Vavasour, Elham Shahinfard, Burkhard Mädler, Jing Zhang, David K B Li, Alex L MacKay, Sandra M Sirrs
BACKGROUND AND PURPOSE: Late-onset adult Krabbe disease is a very rare demyelinating leukodystrophy, affecting less than 1 in a million people. Hematopoietic stem cell transplantation (HSCT) strategies can stop the accumulation of toxic metabolites that damage myelin-producing cells. We used quantitative advanced imaging metrics to longitudinally assess the impact of HSCT on brain abnormalities in adult-onset Krabbe disease. METHODS: A 42-year-old female with late-onset Krabbe disease and an age/sex-matched healthy control underwent annual 3T MRI (baseline was immediately prior to HSCT for the Krabbe subject)...
May 2018: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
https://www.readbyqxmd.com/read/29449506/whole-body-magnetic-resonance-imaging-in-axial-spondyloarthritis-reduction-of-sacroiliac-spinal-and-entheseal-inflammation-in-a-placebo-controlled-trial-of-adalimumab
#15
Simon Krabbe, Mikkel Østergaard, Iris Eshed, Inge J Sørensen, Bente Jensen, Jakob M Møller, Lone Balding, Ole R Madsen, Karsten Asmussen, Grith Eng, Niklas R Jørgensen, Susanne J Pedersen
OBJECTIVE: To investigate whether adalimumab (ADA) reduces whole-body (WB-) magnetic resonance imaging (MRI) indices for inflammation in the entheses, peripheral joints, sacroiliac joints, spine, and the entire body in patients with axial spondyloarthritis (axSpA). METHODS: An investigator-initiated, randomized, placebo-controlled, double-blinded 48-week followup trial included 49 patients with axSpA, who had Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) ≥ 4...
May 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/29444164/evolution-of-nodule-stiffness-might-predict-response-to-local-ablative-therapy-a-series-of-patients-with-hepatocellular-carcinoma
#16
Michael Praktiknjo, Viktoria Krabbe, Alessandra Pohlmann, Matthias Sampels, Christian Jansen, Carsten Meyer, Christian P Strassburg, Jonel Trebicka, Maria A Gonzalez Carmona
BACKGROUND: Early information on treatment response of HCC to local ablative therapy is crucial. Elastography as a non-invasive method has recently been shown to play a potential role in distinguishing between benign and malignant liver lesions. Elastography of hepatocellular carcinoma (HCC) in early response to local ablative therapy has not been studied to date. METHODS: We prospectively included a cohort of 14 patients with diagnosis of HCC who were treated with local ablative therapy (transarterial chemoembolization, TACE and/or radiofrequency ablation, RFA)...
2018: PloS One
https://www.readbyqxmd.com/read/29440360/avian-thermoregulation-in-the-heat-evaporative-cooling-capacity-and-thermal-tolerance-in-two-australian-parrots
#17
Todd J McWhorter, Alexander R Gerson, William A Talbot, Eric Krabbe Smith, Andrew E McKechnie, Blair O Wolf
Avian orders differ in their thermoregulatory capabilities and tolerance of high environmental temperatures. Evaporative heat loss, and the primary avenue whereby it occurs, differs amongst taxa. Although Australian parrots (Psittaciformes) have been impacted by mass mortality events associated with extreme weather events (heat waves), their thermoregulatory physiology has not been well characterized. We quantified the upper limits to thermoregulation under extremely hot conditions in two Australian parrots: the mulga parrot ( Psephotellus varius ; ∼55 g) and the galah ( Eolophus roseicapilla ; ∼265 g)...
March 23, 2018: Journal of Experimental Biology
https://www.readbyqxmd.com/read/29433937/long-term-improvement-of-neurological-signs-and-metabolic-dysfunction-in-a-mouse-model-of-krabbe-s-disease-after-global-gene-therapy
#18
Michael S Marshall, Yazan Issa, Benas Jakubauskas, Monika Stoskute, Vince Elackattu, Jeffrey N Marshall, Wil Bogue, Duc Nguyen, Zane Hauck, Emily Rue, Subha Karumuthil-Melethil, Violeta Zaric, Maarten Bosland, Richard B van Breemen, Maria I Givogri, Steven J Gray, Stephen J Crocker, Ernesto R Bongarzone
We report a global adeno-associated virus (AAV)9-based gene therapy protocol to deliver therapeutic galactosylceramidase (GALC), a lysosomal enzyme that is deficient in Krabbe's disease. When globally administered via intrathecal, intracranial, and intravenous injections to newborn mice affected with GALC deficiency (twitcher mice), this approach largely surpassed prior published benchmarks of survival and metabolic correction, showing long-term protection of demyelination, neuroinflammation, and motor function...
March 7, 2018: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/29427584/discordance-between-ureteroscopic-biopsy-and-final-pathology-for-upper-tract-urothelial-carcinoma
#19
Ezra J Margolin, Justin T Matulay, Gen Li, Xiaosong Meng, Brian Chao, Varun Vijay, Hayley Silver, Timothy N Clinton, Laura-Maria Krabbe, Solomon L Woldu, Nirmish Singla, Aditya Bagrodia, Vitaly Margulis, William C Huang, Marc A Bjurlin, Ojas Shah, Christopher B Anderson
PURPOSE: We evaluated the discordance between ureteroscopic biopsy and surgical pathology findings for grading and staging upper tract urothelial carcinoma. We also sought to establish preoperative predictors of aggressive tumors. MATERIALS AND METHODS: We retrospectively reviewed the records of 314 patients who underwent ureteroscopic biopsy followed by surgical management of upper tract urothelial carcinoma from 2000 to 2016 at a total of 3 institutions. Our primary outcomes were muscle invasive (pT2 or greater) disease at surgical pathology and upgrading of clinical low grade tumors to pathological high grade...
February 7, 2018: Journal of Urology
https://www.readbyqxmd.com/read/29396627/-alvimopan-for-recovery-of-bowel-function-after-radical-cystectomy
#20
REVIEW
Jennifer Kranz, Laura-Maria Krabbe
No abstract text is available yet for this article.
February 2018: Der Urologe. Ausg. A
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