Results 61 to 70 of about 31,122 (181)
A Dose‐Tailored Anti‐Plasma Cell Regimen Lowers the Mortality of Late‐Stage Cardiac Amyloidosis
The present study was designed to assess the efficacy and safety of a dose‐tailored BD regimen and a dose‐tailored DBD regimen in patients with LCCA at Mayo Stage III. Both the two regimens markedly increased survival rate and time in those patients and the dose‐tailored DBD regimen was superior to the dose‐tailored BD regimen in both efficacy and ...
Yun Ti +8 more
wiley +1 more source
Fabry disease: recent advances in pathology, diagnosis, treatment and monitoring
Background In Fabry disease (α-galactosidase A deficiency) accumulation of Globotriaosylceramide (Gb3) leads to progressive organ failure and premature death.
Hoffmann Björn
doaj +1 more source
Enzyme replacement therapy for Anderson-Fabry disease.
Anderson-Fabry disease is an X-linked defect of glycosphingolipid metabolism. Progressive renal insufficiency is a major source of morbidity, additional complications result from cardio- and cerebro-vascular involvement.
Paulo Nascimento +6 more
core +1 more source
Gene Therapy of Anderson-Fabry Disease
Antonino, Tuttolomondo +2 more
openaire +2 more sources
Abstract The Scientific Committee of the Spanish Agency for Food Safety and Nutrition (AESAN) has carried out an updated review of the scientific evidence on the influence of climate change on the transmission of foodborne pathogens. This global phenomenon represents an emerging threat to food safety and public health, since alterations in weather ...
Antonio Valero Díaz +6 more
wiley +1 more source
Anderson-Fabry, the histrionic disease: from genetics to clinical management
Anderson-Fabry disease (AFD) is an Xlinked lysosomal storage disorder of glycosphingolipid catabolism, due to deficiency or absence of a galactosidase A (α-gal A) enzyme. The disease may affect males and females, the latter with an average 10 years
Franco Cecchi +2 more
doaj +1 more source
Kidneys in Anderson-Fabry Disease
Anderson-Fabry disease is an X-linked recessive glycolipid storage disease caused by deficient activity of the lysosomal enzyme alpha-galactosidase A. Numerous mutations are responsible for development of the disease. Clinical manifestations include acroparesthesia from childhood, corneal dystrophy, angiokeratomas, hypohidrosis, hearing loss and, with ...
Bašić Jukić, Nikolina, Kes, Petar
openaire +1 more source
Abstract Aims Describe patient characteristics, treatment patterns, clinical outcomes, healthcare resource utilization (HCRU) and medical costs associated with patients who were diagnosed with obstructive hypertrophic cardiomyopathy (HCM) in clinical practice in England.
Faizel Osman +9 more
wiley +1 more source
Anderson-Fabry disease: Worthy to in-SPECT the nerves? [PDF]
Massalha, Samia, Slart, Riemer H. J. A.
openaire +3 more sources
Free‐Breathing Ungated Radial Simultaneous Multi‐Slice Cardiac T1 Mapping
Background Modified Look‐Locker imaging (MOLLI) T1 mapping sequences are acquired during breath‐holding and require ECG gating with consistent R‐R intervals, which is problematic for patients with atrial fibrillation (AF). Consequently, there is a need for a free‐breathing and ungated framework for cardiac T1 mapping.
Johnathan V. Le +7 more
wiley +1 more source

