Effects of enzyme replacement therapy on the cardiomyopathy of Anderson–Fabry disease: a randomised, double-blind, placebo-controlled clinical trial of agalsidase …
DA Hughes, PM Elliott, J Shah, J Zuckerman… - Heart, 2008 - heart.bmj.com
Background: Anderson–Fabry disease is an X-linked glycosphingolipid storage disorder
caused by deficient activity of the lysosomal enzyme α-galactosidase A. This leads to a …
caused by deficient activity of the lysosomal enzyme α-galactosidase A. This leads to a …
Onset and progression of the Anderson–Fabry disease related cardiomyopathy
C Kampmann, A Linhart, F Baehner, T Palecek… - International journal of …, 2008 - Elsevier
BACKGROUND: Cardiac involvement is responsible for substantial morbidity and mortality
in Anderson–Fabry disease (AFD). We sought to document its onset and progression in a …
in Anderson–Fabry disease (AFD). We sought to document its onset and progression in a …
Cardiovascular testing in Fabry disease: exercise capacity reduction, chronotropic incompetence and improved anaerobic threshold after enzyme replacement
T Lobo, J Morgan, A Bjorksten, K Nicholls… - Internal Medicine …, 2008 - Wiley Online Library
Aim: The aim of this study was to document exercise capacity and serial electrocardiogram
and echocardiograph findings in a cohort of Australian patients with Fabry disease, in …
and echocardiograph findings in a cohort of Australian patients with Fabry disease, in …
Twenty‐four‐month α‐galactosidase A replacement therapy in Fabry disease has only minimal effects on symptoms and cardiovascular parameters
JW Koskenvuo, JJ Hartiala, P Nuutila… - Journal of Inherited …, 2008 - Wiley Online Library
Fabry disease is an X‐linked lysosomal storage disease caused by deficiency of α‐
galactosidase A enzyme activity. Decreased enzyme activity leads to accumulation of …
galactosidase A enzyme activity. Decreased enzyme activity leads to accumulation of …
Anderson-Fabry disease: long-term echocardiographic follow-up under enzyme replacement therapy
T Kovacevic-Preradovic, M Zuber… - European journal of …, 2008 - academic.oup.com
Abstract Aims Anderson-Fabry disease affects various organ systems due to
glycosphingolipid accumulation. Enzyme replacement therapy (ERT) has been reported to …
glycosphingolipid accumulation. Enzyme replacement therapy (ERT) has been reported to …
[HTML][HTML] Rescue of mutant α-galactosidase A in the endoplasmic reticulum by 1-deoxygalactonojirimycin leads to trafficking to lysosomes
R Hamanaka, T Shinohara, S Yano… - … et Biophysica Acta (BBA …, 2008 - Elsevier
Active-site-specific chaperone therapy for Fabry disease is a genotype-specific therapy
using a competitive inhibitor, 1-deoxygalactonojirimycin (DGJ). To elucidate the mechanism …
using a competitive inhibitor, 1-deoxygalactonojirimycin (DGJ). To elucidate the mechanism …
Cardiac performance during exercise in patients with Fabry's disease
Background Fatigability and dyspnoea on effort are present in many patients with Fabry's
disease. We assessed the determinants of cardiac performance during exercise in patients …
disease. We assessed the determinants of cardiac performance during exercise in patients …
[HTML][HTML] Compromiso cardiovascular en la enfermedad de Fabry
AM Barón, A Barón - Revista Colombiana de Cardiología, 2008 - scielo.org.co
Compromiso cardiovascular en la enfermedad de Fabry SciELO - Scientific Electronic
Library Online vol.15 issue3 Treatment of refractory chest angina with spinal electrical …
Library Online vol.15 issue3 Treatment of refractory chest angina with spinal electrical …
Cardiovascular involvement in Fabry disease
AM Barón O, A Barón C - Revista Colombiana de Cardiología, 2008 - scielo.org.co
Cardiovascular involvement in Fabry disease SciELO - Scientific Electronic Library Online
vol.15 issue3 Treatment of refractory chest angina with spinal electrical stimulator: literature …
vol.15 issue3 Treatment of refractory chest angina with spinal electrical stimulator: literature …