Results 61 to 70 of about 31,072 (153)
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
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
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
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
Clinical prodromes of neurodegeneration in Anderson-Fabry disease [PDF]
To estimate the prevalence of prodromal clinical features of neurodegeneration in patients with Anderson-Fabry disease (AFD) in comparison to age-matched ...
Hughes, D +6 more
core
Prevalence of Anderson-Fabry disease in male patients with late onset hypertrophic cardiomyopathy
Background-Although studies have suggested that "late-onset" hypertrophic cardiomyopathy (HCM) may be caused by sarcomeric protein gene mutations, the cause of HCM in the majority of patients is unknown.
Tei, C +13 more
core
When Should Cardiologists Suspect Anderson-Fabry Disease?
Anderson-Fabry disease is a lysosomal storage disorder caused by α-galactosidase defects and progressive intracellular accumulation of globotriaosylceramide. The disease can be specifically treated with enzyme replacement therapy.
Eliana Disabella +21 more
core +1 more source
Otological aspects of Fabry disease in patients with normal hearing [PDF]
2019-08We investigated the otological aspects of Fabry disease (FD) in patients with normal hearing. Forty-one patients (21 men, 20 women) with bilaterally normal hearing were recruited, and their otological symptoms and hearing evaluations, which ...
Sone, Michihiko +6 more
core +1 more source
Cutaneous manifestations of Fabry disease: A systematic review
Abstract Fabry disease (FD) is a rare X‐linked lysosomal storage disorder resulting in potential debilitating accumulation of glycosphingolipids in organs such as skin, nerves, heart, kidneys, lungs, and the central nervous system. Skin is easily investigated and can guide clinicians to diagnose FD, minimizing delay of enzyme substitution therapy. This
Rami Nabil Al‐Chaer +4 more
wiley +1 more source

