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FEATURED Journal article
Efficacy of the pharmacologic chaperone migalastat in a subset of male patients with the classic phenotype of Fabry disease and migalastat-amenable variants: data from the phase 3 randomized, multicenter, double-blind clinical trial and extension studyRead more
Kathleen Nicholls' selected work
Safety of switching to Migalastat from enzyme replacement therapy in Fabry disease: Experi..
Pegunigalsidase alfa, a novel PEGylated enzyme replacement therapy for Fabry disease, prov..
Fatal Enteroviral Encephalitis in a Patient with Common Variable Immunodeficiency Harbouri..
Cardio- Renal Outcomes With Long- Term Agalsidase Alfa Enzyme Replacement Therapy: A 10-Ye..
Displaying the 127 most recent scholarly works by Kathleen Nicholls.
Efficacy of the pharmacologic chaperone migalastat in a subset of male patients with the classic phenotype of Fabry disease and migalastat-amenable variants: data from the phase 3 randomized, multicenter, double-blind clinical trial and extension study
Dominique P Germain, Kathy Nicholls, Roberto Giugliani, Daniel G Bichet, Derralynn A Hughes, Laura M Barisoni, Robert B Colvin, J Charles Jennette, Nina Skuban, Jeffrey P Castelli, Elfrida Benjamin, Jay A Barth, Christopher Viereck
Journal article | 2019 | Genetics in Medicine
PURPOSE: Outcomes in patients with Fabry disease receiving migalastat during the phase 3 FACETS trial (NCT00925301) were evaluated..
Safety of switching to Migalastat from enzyme replacement therapy in Fabry disease: Experience from the Phase 3 ATTRACT study
Derralynn A Hughes, Kathleen Nicholls, Gere Sunder-Plassmann, Ana Jovanovic, Ulla Feldt-Rasmussen, Raphael Schiffmann, Robert Giugliani, Vipul Jain, Chris Viereck, Jeffrey P Castelli, Nina Skuban, Jay A Barth, Daniel G Bichet
Journal article | 2019 | American Journal of Medical Genetics Part A
Pegunigalsidase alfa, a novel PEGylated enzyme replacement therapy for Fabry disease, provides sustained plasma concentrations and favorable pharmacodynamics: A 1-year Phase 1/2 clinical trial
Raphael Schiffmann, Ozlem Goker-Alpan, Myrl Holida, Pilar Giraldo, Laura Barisoni, Robert B Colvin, Charles J Jennette, Gustavo Maegawa, Simeon A Boyadjiev, Derlis Gonzalez, Kathy Nicholls, Ahmad Tuffaha, Mohamed G Atta, Bonita Rup, Martha R Charney, Alona Paz, Mali Szlaifer, Sari Alon, Einat Brill-Almon, Raul Chertkoff, et al.
Journal article | 2019 | Journal of Inherited Metabolic Disease
Fatal Enteroviral Encephalitis in a Patient with Common Variable Immunodeficiency Harbouring a Novel Mutation in NFKB2
Charlotte A Slade, Catriona McLean, Thomas Scerri, Binh Giang Tran, Steven Megaloudis, Alexander Strathmore, Jessica C Tempany, Katherine Nicholls, Colleen D'Arcy, Melanie Bahlo, Philip D Hodgkin, Jo A Douglass, Vanessa L Bryant
Journal article | 2019 | Journal of Clinical Immunology
Cardio- Renal Outcomes With Long- Term Agalsidase Alfa Enzyme Replacement Therapy: A 10-Year Fabry Outcome Survey (FOS) Analysis
Uma Ramaswami, Michael Beck, Derralynn Hughes, Christoph Kampmann, Jaco Botha, Guillem Pintos-Morell, Michael L West, Dau-Ming Niu, Kathy Nicholls, Roberto Giugliani
Journal article | 2019 | Drug Design, Development and Therapy
Critical care usage after major gastrointestinal and liver surgery: a prospective, multicentre observational study.
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Journal article | 2019 | British Journal of Anaesthesia
BACKGROUND: Patient selection for critical care admission must balance patient safety with optimal resource allocation. This study..
Association between peri-operative angiotensin-converting enzyme inhibitors and angiotensin-2 receptor blockers and acute kidney injury in major elective non-cardiac surgery: a multicentre, prospective cohort study.
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Journal article | 2018 | Anaesthesia
The peri-operative use of angiotensin-converting enzyme inhibitors or angiotensin-2 receptor blockers is thought to be associated ..
Phenotypic characteristics of the p.Asn215Ser (p.N215S) GLA mutation in male and female patients with Fabry disease: A multicenter Fabry Registry study
Dominique P Germain, Eva Brand, Alessandro Burlina, Franco Cecchi, Scott C Garman, Judy Kempf, Dawn A Laney, Ales Linhart, Laszlo Marodi, Kathy Nicholls, Alberto Ortiz, Federico Pieruzzi, Suma P Shankar, Stephen Waldek, Christoph Wanner, Ana Jovanovic
Journal article | 2018 | Molecular Genetics & Genomic Medicine
Delayed Diagnosis and Complications of Predominantly Antibody Deficiencies in a Cohort of Australian Adults
Charlotte A Slade, Julian J Bosco, Tran Binh Giang, Elizabeth Kruse, Robert G Stirling, Paul U Cameron, Fiona Hore-Lacy, Michael F Sutherland, Sara L Barnes, Stephen Holdsworth, Samar Ojaimi, Gary A Unglik, Joseph De Luca, Mittal Patel, Jeremy McComish, Kymble Spriggs, Yang Tran, Priscilla Auyeung, Katherine Nicholls, Robyn E O'Hehir, et al.
Journal article | 2018 | Frontiers in Immunology
Migalastat improves diarrhea in patients with Fabry disease: clinical-biomarker correlations from the phase 3 FACETS trial
Raphael Schiffmann, Daniel G Bichet, Ana Jovanovic, Derralynn A Hughes, Roberto Giugliani, Ulla Feldt-Rasmussen, Suma P Shankar, Laura Barisoni, Robert B Colvin, J Charles Jennette, Fred Holdbrook, Andrew Mulberg, Jeffrey P Castelli, Nina Skuban, Jay A Barth, Kathleen Nicholls
Journal article | 2018 | Orphanet Journal of Rare Diseases
Improvement of Fabry Disease-Related Gastrointestinal Symptoms in a Significant Proportion of Female Patients Treated with Agalsidase Beta: Data from the Fabry Registry
William R Wilcox, Ulla Feldt-Rasmussen, Ana Maria Martins, Alberto Ortiz, Roberta M Lemay, Ana Jovanovic, Dominique P Germain, Carmen Varas, Katherine Nicholls, Frank Weidemann, Robert J Hopkin
Journal article | 2018 | JIMD reports
Oral pharmacological chaperone migalastat compared with enzyme replacement therapy in Fabry disease: 18-month results from the randomised phase III ATTRACT study
Derralynn A Hughes, Kathleen Nicholls, Suma P Shankar, Gere Sunder-Plassmann, David Koeller, Khan Nedd, Gerard Vockley, Takashi Hamazaki, Robin Lachmann, Toya Ohashi, Iacopo Olivotto, Norio Sakai, Patrick Deegan, David Dimmock, Francois Eyskens, Dominique P Germain, Ozlem Goker-Alpan, Eric Hachulla, Ana Jovanovic, Charles M Lourenco, et al.
Journal article | 2017 | Journal of Medical Genetics
Safety of Nonsteroidal Anti-inflammatory Drugs in Major Gastrointestinal Surgery: A Prospective, Multicenter Cohort Study.
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Journal article | 2017 | World Journal of Surgery
BACKGROUND: Significant safety concerns remain surrounding the use of nonsteroidal anti-inflammatory drugs (NSAIDs) following gast..
Treatment of Fabry's Disease with the Pharmacologic Chaperone Migalastat
DP Germain, DA Hughes, K Nicholls, DG Bichet, R Giugliani, WR Wilcox, C Feliciani, SP Shankar, F Ezgu, H Amartino, D Bratkovic, U Feldt-Rasmussen, K Nedd, U Sharaf El Din, CM Lourenco, M Banikazemi, J Charrow, M Dasouki, D Finegold, P Giraldo, et al.
Journal article | 2016 | New England Journal of Medicine
Multicentre prospective cohort study of body mass index and postoperative complications following gastrointestinal surgery.
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Journal article | 2016 | British Journal of Surgery
BACKGROUND: There is currently conflicting evidence surrounding the effects of obesity on postoperative outcomes. Previous studies..
Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active alpha-Galactosidase A in Fabry Patients when Co-Administered with Infused Agalsidase
David G Warnock, Daniel G Bichet, Myrl Holida, Ozlem Goker-Alpan, Kathy Nicholls, Mark Thomas, Francois Eyskens, Suma Shankar, Mathews Adera, Sheela Sitaraman, Richie Khanna, John J Flanagan, Brandon A Wustman, Jay Barth, Carrolee Barlow, Kenneth J Valenzano, David J Lockhart, Pol Boudes, Franklin K Johnson
Journal article | 2015 | PLOS ONE
HLA-DQA1 and PLCG2 Are Candidate Risk Loci for Childhood-Onset Steroid-Sensitive Nephrotic Syndrome
Rasheed A Gbadegesin, Adebowale Adeyemo, Nicholas JA Webb, Larry A Greenbaum, Asiri Abeyagunawardena, Shenal Thalgahagoda, Arundhati Kale, Debbie Gipson, Tarak Srivastava, Jen-Jar Lin, Deepa Chand, Tracy E Hunley, Patrick D Brophy, Arvind Bagga, Aditi Sinha, Michelle N Rheault, Joanna Ghali, Kathy Nicholls, Elizabeth Abraham, Halima S Janjua, et al.
Journal article | 2015 | Journal of the American Society of Nephrology
Adoptive T Cell Immunotherapy for Treatment of Ganciclovir-Resistant Cytomegalovirus Disease in a Renal Transplant Recipient
N Macesic, D Langsford, K Nicholls, P Hughes, DJ Gottlieb, L Clancy, E Blyth, K Micklethwaite, B Withers, S Majumdar, S Fleming, J Sasadeusz
Journal article | 2015 | American Journal of Transplantation
Cardiovascular outcomes in Fabry disease are linked to severity of chronic kidney disease.
Andrew S Talbot, Nigel T Lewis, Kathy M Nicholls
Journal article | 2015 | Heart
OBJECTIVES: Assess the impact of end-stage renal disease (chronic kidney disease stage 5 (CKD5)) on cardiovascular outcomes in pat..
Efficacy and Safety of Abatacept in Lupus Nephritis A Twelve-Month, Randomized, Double-Blind Study
Richard Furie, Kathy Nicholls, Tien-Tsai Cheng, Frederic Houssiau, Ruben Burgos-Vargas, Shun-Le Chen, Jan L Hillson, Stephanie Meadows-Shropshire, Michael Kinaszczuk, Joan T Merrill
Journal article | 2014 | Arthritis & Rheumatology
Changes in plasma and urine globotriaosylceramide levels do not predict Fabry disease progression over 1 year of agalsidase alfa
Raphael Schiffmann, Markus Ries, Derek Blankenship, Kathy Nicholls, Atul Mehta, Joe TR Clarke, Robert D Steiner, Michael Beck, Bruce A Barshop, William Rhead, Michael West, Rick Martin, David Amato, Nitin Nair, Pedro Huertas
Journal article | 2013 | Genetics in Medicine
Analysis of left ventricular mass in untreated men and in men treated with agalsidase-beta: data from the Fabry Registry
Dominique P Germain, Frank Weidemann, Ademola Abiose, Manesh R Patel, Marta Cizmarik, J Alexander Cole, Dana Beitner-Johnson, Karelle Benistan, Gustavo Cabrera, Joel Charrow, Ilkka Kantola, Ales Linhart, Kathy Nicholls, Markus Niemann, C Ronald Scott, Katherine Sims, Stephen Waldek, David G Warnock, Joerg Strotmann
Journal article | 2013 | Genetics in Medicine
A Phase 2 study of migalastat hydrochloride in females with Fabry disease: Selection of population, safety and pharmacodynamic effects
R Giugliani, S Waldek, DP Germain, K Nicholls, DG Bichet, JK Simosky, AC Bragat, JP Castelli, ER Benjamin, PF Boudes
Journal article | 2013 | Molecular Genetics and Metabolism
Migalastat HCl Reduces Globotriaosylsphingosine (Lyso-Gb(3)) in Fabry Transgenic Mice and in the Plasma of Fabry Patients
Brandy Young-Gqamana, Nastry Brignol, Hui-Hwa Chang, Richie Khanna, Rebecca Soska, Maria Fuller, Sheela A Sitaraman, Dominique P Germain, Roberto Giugliani, Derralynn A Hughes, Atul Mehta, Kathy Nicholls, Pol Boudes, David J Lockhart, Kenneth J Valenzano, Elfrida R Benjamin
Journal article | 2013 | PLoS One
Safety and pharmacodynamic effects of a pharmacological chaperone on alpha-galactosidase A activity and globotriaosylceramide clearance in Fabry disease: report from two phase 2 clinical studies
Dominique P Germain, Roberto Giugliani, Derralynn A Hughes, Atul Mehta, Kathy Nicholls, Laura Barisoni, Charles J Jennette, Alexander Bragat, Jeff Castelli, Sheela Sitaraman, David J Lockhart, Pol F Boudes
Journal article | 2012 | Orphanet Journal of Rare Diseases
Cardiovascular Events in Patients With Fabry Disease Natural History Data From the Fabry Registry
Manesh R Patel, Franco Cecchi, Marta Cizmarik, Ilkka Kantola, Ales Linhart, Kathy Nicholls, Joerg Strotmann, Jose Tallaj, Chien Tran Thi, Michael L West, Dana Beitner-Johnson, Ademola Abiose
Journal article | 2011 | Journal of the American College of Cardiology
Effects of enzyme replacement therapy in Fabry disease-A comprehensive review of the medical literature
Olivier Lidove, Michael L West, Guillem Pintos-Morell, Ricardo Reisin, Kathy Nicholls, Luis E Figuera, Rossella Parini, Luiz R Carvalho, Christoph Kampmann, Gregory M Pastores, Atul Mehta
Journal article | 2010 | Genetics in Medicine
Cost-effectiveness and socioeconomic implications of prevention and management of chronic kidney disease in type 2 diabetes
S Chadban, M Howell, S Twigg, M Thomas, G Jerums, A Cass, D Campbell, K Nicholls, A Tong, G Mangos, A Stack, RJ MacIsaac, S Girgis, R Colagiuri, S Colagiuri, J Craig
Journal article | 2010 | Nephrology
Mycophenolate mofetil versus cyclophosphamide for induction treatment of lupus nephritis.
Gerald B Appel, Gabriel Contreras, Mary Anne Dooley, Ellen M Ginzler, David Isenberg, David Jayne, Lei-Shi Li, Eduardo Mysler, Jorge Sánchez-Guerrero, Neil Solomons, David Wofsy, undefined Aspreva Lupus Management Study Group
Journal article | 2009 | Journal of the American Society of Nephrology
Recent studies have suggested that mycophenolate mofetil (MMF) may offer advantages over intravenous cyclophosphamide (IVC) for th..
Conversion of cyclosporine to tacrolimus in stable renal allograft recipients: Quantification of effects on the severity of gingival enlargement and hirsutism and patient-reported outcomes
Rowan G Walker, Stephen Cottrell, Kathleen Sharp, Rosamaria Tripodi, Kathy M Nicholls, Ian Fraser, George A Varigos, Belinda E Butcher
Journal article | 2007 | Nephrology
Fabry disease: Baseline medical characteristics of a cohort of 1765 males and females in the Fabry Registry
CM Eng, J Fletcher, WR Wilcox, S Waldek, CR Scott, DO Sillence, F Breunig, J Charrow, DP Germain, K Nicholls, M Banikazemi
Conference Proceedings | 2007 | Journal of Inherited Metabolic Disease
A longitudinal molecular surveillance study of human polyomavirus viremia in heart, kidney, liver, and pancreas transplant patients.
Raymund R Razonable, Robert A Brown, Atul Humar, Emma Covington, Emma Alecock, Carlos V Paya, undefined PV16000 Study Group
Journal article | 2005 | The Journal of Infectious Diseases
In this study of 263 heart, kidney, liver, and pancreas transplant patients, BK virus (BKV) and JC virus (JCV) DNAemia were observ..
Glomerular size and global glomerulosclerosis in normal Caucasian donor kidneys: effects of aging and gender.
Mingxi Li, Kathy M Nicholls, Gavin J Becker
Journal article | 2002 | Journal of Nephrology
BACKGROUND: To assess the effects of aging and gender on glomerular size and global glomerulosclerosis (GS) and evaluate the relat..
[IgA nephropathy in Chinese and Australian patients: a comparison between clinical and pathological features].
L Li, K Tao, K Nicholls, P Kincaid-Smith
Journal article | 1991 | Zhonghua yi xue za zhi
The clinical and pathological data were compared between 88 Chinese and 88 Australian patients with IgA nephropathy, whose age, se..
Factors determining renal response to water immersion in non-excretor cirrhotic patients.
KM Nicholls, MD Shapiro, BS Groves, RW Schrier
Journal article | 1986 | Kidney International
Non-excretor cirrhotic patients, defined by their inability to normally excrete a standard water load, display variable responses ..
Role of glomerular filtration rate in the impaired sodium and water excretion of patients with the nephrotic syndrome.
MD Shapiro, KM Nicholls, BM Groves, RW Schrier
Journal article | 1986 | American Journal of Kidney Diseases
Hemodynamic and hormonal factors were monitored in nine patients with nephrotic syndrome who were evaluated relative to their capa..
Sodium excretion in advanced cirrhosis: effect of expansion of central blood volume and suppression of plasma aldosterone.
KM Nicholls, MD Shapiro, R Kluge, HM Chung, DG Bichet, RW Schrier
Journal article | 1986 | Hepatology
Sodium excretion in 13 patients with decompensated cirrhosis was measured under baseline conditions of water loading (n = 13) and ..
Interrelationship between cardiac output and vascular resistance as determinants of effective arterial blood volume in cirrhotic patients.
MD Shapiro, KM Nicholls, BM Groves, R Kluge, HM Chung, DG Bichet, RW Schrier
Journal article | 1985 | Kidney International
The effect of head-out water immersion (HWI) in decompensated cirrhotic patients to correct sodium and water excretion has been fo..
Elevated plasma norepinephrine concentrations in decompensated cirrhosis. Association with increased secretion rates, normal clearance rates, and suppressibility by central blood volume expansion.
KM Nicholls, MD Shapiro, VJ Van Putten, R Kluge, HM Chung, DG Bichet, RW Schrier
Journal article | 1985 | Circulation Research
Plasma norepinephrine concentrations are elevated in patients with decompensated cirrhosis, and correlate inversely with urinary s..
The clinical course of mesangial IgA associated nephropathy in adults.
KM Nicholls, KF Fairley, JP Dowling, P Kincaid-Smith
Journal article | 1984 | Quarterly Journal of Medicine
Clinical data in 244 patients with IgA nephropathy and biopsy findings in 519 biopsies (107 patients had at least two biopsies) we..
Medicine - Royal Melbourne Hospital