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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
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
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
OBJECTIVES: To characterize the presence of bland (non-tumor) 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 five tertiary care medical centers. Using clinicopathologic variables including patient age, BMI, ECOG status, tumor stage, grade, nodal status and histology, nearest-neighbor and multiple propensity score matched cohorts of bland thrombus versus non-bland thrombus patients were assessed...
February 27, 2018: Urology
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
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
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)...
February 26, 2018: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
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...
February 15, 2018: Journal of Rheumatology
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
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)...
February 13, 2018: Journal of Experimental Biology
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...
January 17, 2018: Molecular Therapy: the Journal of the American Society of Gene Therapy
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
INTRODUCTION: We sought to evaluate the discordance between ureteroscopic biopsy and surgical pathology for grading and staging of upper tract urothelial carcinoma (UTUC) and to establish preoperative predictors of aggressive tumors. METHODS: We performed a retrospective review of 314 patients who underwent ureteroscopic biopsy followed by surgical management for UTUC from 2000-2016 at three institutions. Our primary outcomes were muscle-invasive (≥pT2) disease at surgical pathology and upgrading of clinical low-grade (cLG) tumors to pathologic high-grade (pHG)...
February 7, 2018: Journal of Urology
Jennifer Kranz, Laura-Maria Krabbe
No abstract text is available yet for this article.
February 2, 2018: Der Urologe. Ausg. A
Jennifer M Kwon, Dietrich Matern, Joanne Kurtzberg, Lawrence Wrabetz, Michael H Gelb, David A Wenger, Can Ficicioglu, Amy T Waldman, Barbara K Burton, Patrick V Hopkins, Joseph J Orsini
BACKGROUND: Krabbe disease is a rare neurodegenerative genetic disorder caused by deficiency of galactocerebrosidase. Patients with the infantile form of Krabbe disease can be treated at a presymptomatic stage with human stem cell transplantation which improves survival and clinical outcomes. However, without a family history, most cases of infantile Krabbe disease present after onset of symptoms and are ineligible for transplantation. In 2006, New York began screening newborns for Krabbe disease to identify presymptomatic cases...
February 1, 2018: Orphanet Journal of Rare Diseases
R N Alcalay, P Wolf, O A Levy, U J Kang, C Waters, S Fahn, B Ford, S H Kuo, N Vanegas, H Shah, C Liong, S Narayan, M W Pauciulo, W C Nichols, Z Gan-Or, G A Rouleau, W K Chung, P Oliva, J Keutzer, K Marder, X K Zhang
Glucocerebrosidase (GCase, deficient in Gaucher disease) enzymatic activity measured in dried blood spots of Parkinson's Disease (PD) cases is within healthy range but reduced compared to controls. It is not known whether activities of additional lysosomal enzymes are reduced in dried blood spots in PD. To test whether reduction in lysosomal enzymatic activity in PD is specific to GCase, we measured GCase, acid sphingomyelinase (deficient in Niemann-Pick disease types A and B), alpha galactosidase A (deficient in Fabry), acid alpha-glucosidase (deficient in Pompe) and galactosylceramidase (deficient in Krabbe) enzymatic activities in dried blood spots of PD patients (n = 648) and controls (n = 317) recruited from Columbia University...
January 21, 2018: Neurobiology of Disease
Eleonora Cavalca, Martina Cesani, Jennifer Gifford, Miguel Sena Esteves, Maria Rosa Terreni, Giuseppe Leoncini, Marco Peviani, Alessandra Biffi
OBJECTIVE: Lysosomal Storage Disorders (LSDs) are a broad class of inherited metabolic diseases caused by the defective activity of lysosomal enzymes. Central nervous system (CNS) manifestations are present in roughly 50% of LSD patients and represent an unmet medical need for them. We explored the therapeutic potential of Metallothioneins (MTs), a newly identified family of proteins with reported neuroprotective roles, in the murine models of two LSDs with CNS involvement. METHODS: MT-1 over-expressing transgenic mice (MTtg) were crossed with the murine models of Batten and Krabbe diseases...
January 25, 2018: Annals of Neurology
Barbara Buchberger, Laura Krabbe
BACKGROUND: Acupuncture is a non-pharmacological option to relieve pregnancy-related complaints. OBJECTIVES: To critically appraise the best available evidence for the use of acupuncture in outpatient care. SEARCH STRATEGY: The MEDLINE, Cochrane Library, and Centre for Reviews and Dissemination databases were searched for English-language and German-language papers published from January 1980 to March 2017 using search terms related to pregnancy combined with 'acupuncture'...
January 21, 2018: International Journal of Gynaecology and Obstetrics
Jonathan Y Li, Dana M Middleton, Steven Chen, Leonard White, Carley R Corado, Charles Vite, Allison Bradbury, James M Provenzale
Purpose The purpose of this study was to compare quantitative diffusion tensor imaging metrics in dogs affected with a model of Krabbe disease to age-matched normal controls. We hypothesized that fractional anisotropy would be decreased and radial diffusivity would be increased in the Krabbe dogs. Methods We used a highly reproducible region-of-interest interrogation technique to measure fractional anisotropy and radial diffusivity in three different white matter regions within the internal capsule and centrum semiovale in four Krabbe affected brains and three age-matched normal control brains...
January 1, 2018: Neuroradiology Journal
Chris H Hill, Georgia M Cook, Samantha J Spratley, Stuart Fawke, Stephen C Graham, Janet E Deane
Sphingolipids are essential components of cellular membranes and defects in their synthesis or degradation cause severe human diseases. The efficient degradation of sphingolipids in the lysosome requires lipid-binding saposin proteins and hydrolytic enzymes. The glycosphingolipid galactocerebroside is the primary lipid component of the myelin sheath and is degraded by the hydrolase β-galactocerebrosidase (GALC). This enzyme requires the saposin SapA for lipid processing and defects in either of these proteins causes a severe neurodegenerative disorder, Krabbe disease...
January 11, 2018: Nature Communications
Allison M Bradbury, Mohammad A Rafi, Jessica Bagel, Becky K Brisson, Michael S Marshall, Jill Pesayco Salvador, Xuntian Jiang, Gary P Swain, Maria L Prociuk, Patricia O'Donnell, Caitlin Fitzgerald, Daniel S Ory, Ernesto R Bongarzone, G Diane Shelton, David A Wenger, Charles Vite
Globoid cell leukodystrophy (GLD), or Krabbe disease, is an inherited, neurologic disorder that results from deficiency of a lysosomal enzyme, galactosylceramidase (GALC). Most commonly, deficits of GALC result in widespread central and peripheral nervous system (CNS, PNS) demyelination and death in affected infants typically by 2 years of age. Hematopoietic stem cell transplantation is the current standard of care in children diagnosed prior to symptom onset; however, disease correction is incomplete. Herein we present the first adeno-associated virus (AAV) gene therapy experiments in a naturally occurring canine model of GLD that closely recapitulates the clinical disease progression, neuropathological alterations, and biochemical abnormalities observed in human patients...
January 9, 2018: Human Gene Therapy
Anna Selivanova, Paul F M Krabbe
INTRODUCTION: A crucial assumption in health valuation methods is that respondents pay equal attention to all information components presented in the response task. So far, there is no solid evidence that respondents are fulfilling this condition. The aim of our study is to explore the attendance to various information cues presented in the discrete choice (DC) response tasks. METHODS: Eye tracking was used to study the eye movements and fixations on specific information areas...
2018: PloS One
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