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在“
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”中,
命中:
1,075
条,耗时:小于0.01 秒
在所有数据库中总计命中:
4,095
条
1.
A comparison of central nervous system involvement in patients with classical
Fabry
disease or the later-onset subtype with the IVS4+919G>A mutation
作者:
Han-Jui Lee
;
Ting-Rong Hsu
;
Sheng-Che Hung
;
Wen-Chung Yu
;
Tzu-Hung Chu…
关键词:
Agalsidase alfa
;
Central nervous system manifestations
;
Fabry
Outcome Survey
;
Later
;
onset
Fabry
disease
;
Magnetic resonance imaging
刊名:BMC Neurology
出版年:2017
2.
Metabolic progression to clinical phenotype in classic
Fabry
disease
作者:
Marco Spada
;
David Kasper
;
Veronica Pagliardini…
关键词:
Fabry
disease
;
Globotriaosylsphingosine
;
Lysosomal storage disorders
刊名:Italian Journal of Pediatrics
出版年:2017
3.
On-line monitoring of methanol and methyl formate in the exhaust gas of an industrial formaldehyde production plant by a mid-IR gas sensor based on tunable
Fabry
-Pérot filter technology
作者:
Andreas Genner
;
Christoph Gasser
;
Harald Moser…
关键词:
Formaldehyde production
;
Fabry
;
Pérot detector
;
Mid
;
infrared
;
Process analytical chemistry
;
Methyl formate
;
Methanol
刊名:Analytical and Bioanalytical Chemistry
出版年:2017
4.
Development of a saturated absorption spectroscopy setup at IGISOL for characterisation of
Fabry
-Pérot interferometers
作者:
S. Geldhof
;
S. El Youbi
;
I. D. Moore
;
I. Pohjalainen…
关键词:
Fabry
;
Pérot interferometer
;
Saturation spectroscopy
;
Rubidium
刊名:Hyperfine Interactions
出版年:2017
5.
Cardiovascular magnetic resonance demonstration of the spectrum of morphological phenotypes and patterns of myocardial scarring in Anderson-
Fabry
disease
作者:
Djeven Parameshvara Deva
;
Kate Hanneman…
关键词:
Cardiomyopathy
;
Hypertrophy
;
Cardiovascular magnetic resonance
;
Anderson
;
Fabry
disease
;
Left ventricular morphology
;
Myocardial scar
;
Late gadolinium enhancement
刊名:Journal of Cardiovascular Magnetic Resonance
出版年:2017
6.
Orphan drug policies and use in pediatric nephrology
作者:
Diana Karpman
;
Peter Höglund
关键词:
Orphan drug
;
Rare disease
;
Quality
;
adjusted life
;
year
;
Fabry
disease
;
Atypical hemolytic uremic syndrome
;
Cystinosis
刊名:Pediatric Nephrology
出版年:2017
7.
Prevalence of
Fabry
disease in dialysis patients: Japan Fabry disease screening study (J-FAST)
作者:
Osamu Saito
;
Eiji Kusano
;
Tetsu Akimoto…
关键词:Α ;
galactosidase activity
;
Fabry
disease
;
Dialysis patient
;
Screening
;
X
;
linked recessive inheritance
;
E66Q variation
刊名:Clinical and Experimental Nephrology
出版年:2016
8.
Experiences of Being Heterozygous for
Fabry
Disease: a Qualitative Study
作者:
Charlotte von der Lippe
;
Jan C. Frich
;
Anna Harris…
关键词:
Experiences
;
Heterozygous
;
Fabry
disease
;
Psychosocial
X ;
linked
;
Genetic counseling
刊名:Journal of Genetic Counseling
出版年:2016
9.
Surges in proteinuria are associated with plasma GL-3 elevations in a young patient with classic
Fabry
disease
作者:
Takahiro Kanai
;
Takane Ito
;
Jun Odaka
;
Takashi Saito…
关键词:
Enzyme replacement therapy
;
Fabry
disease
;
Podocyte
;
Foot process effacement
;
Proteinuria
;
Renal pathology
刊名:European Journal of Pediatrics
出版年:2016
10.
α-Galactosidase delivery using 30Kc19-human serum albumin nanoparticles for effective treatment of
Fabry
disease
作者:
Hong Jai Lee
;
Hee Ho Park
;
Youngsoo Sohn…
关键词:
Fabry
disease
;
Enzyme replacement therapy
;
Protein nanoparticle
;
Drug delivery
;
Enzyme stability
刊名:Applied Microbiology and Biotechnology
出版年:2016
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