Class information for:
Level 1: FABRY DISEASE//ALPHA GALACTOSIDASE A//FABRYS DISEASE

Basic class information

ID Publications Average number
of references
Avg. shr. active
ref. in WoS
4517 1698 29.9 72%



Bar chart of Publication_year

Last years might be incomplete

Classes in level above (level 2)



ID, lev.
above
Publications Label for level above
330 17366 FABRY DISEASE//GAUCHER DISEASE//MUCOPOLYSACCHARIDOSES

Terms with highest relevance score



Rank Term Type of term Relevance score
(tfidf)
Class's shr.
of term's tot.
occurrences
Shr. of publ.
in class containing
term
Num. of
publ. in
class
1 FABRY DISEASE Author keyword 1164 83% 38% 653
2 ALPHA GALACTOSIDASE A Author keyword 335 87% 10% 163
3 FABRYS DISEASE Author keyword 197 82% 7% 113
4 AGALSIDASE ALFA Author keyword 125 97% 2% 35
5 ANDERSON FABRY DISEASE Author keyword 117 89% 3% 54
6 AGALSIDASE BETA Author keyword 81 96% 1% 25
7 GLOBOTRIAOSYLCERAMIDE Author keyword 61 61% 4% 66
8 ENZYME REPLACEMENT THERAPY Author keyword 58 25% 12% 202
9 ALPHA GALACTOSIDASE A DEFICIENCY Author keyword 56 100% 1% 19
10 DEV METAB NEUROL BRANCH Address 31 34% 4% 74

Web of Science journal categories

Author Key Words



Rank Web of Science journal category Relevance score
(tfidf)
Class's shr.
of term's tot.
occurrences
Shr. of publ.
in class containing
term
Num. of
publ. in
class
LCSH search Wikipedia search
1 FABRY DISEASE 1164 83% 38% 653 Search FABRY+DISEASE Search FABRY+DISEASE
2 ALPHA GALACTOSIDASE A 335 87% 10% 163 Search ALPHA+GALACTOSIDASE+A Search ALPHA+GALACTOSIDASE+A
3 FABRYS DISEASE 197 82% 7% 113 Search FABRYS+DISEASE Search FABRYS+DISEASE
4 AGALSIDASE ALFA 125 97% 2% 35 Search AGALSIDASE+ALFA Search AGALSIDASE+ALFA
5 ANDERSON FABRY DISEASE 117 89% 3% 54 Search ANDERSON+FABRY+DISEASE Search ANDERSON+FABRY+DISEASE
6 AGALSIDASE BETA 81 96% 1% 25 Search AGALSIDASE+BETA Search AGALSIDASE+BETA
7 GLOBOTRIAOSYLCERAMIDE 61 61% 4% 66 Search GLOBOTRIAOSYLCERAMIDE Search GLOBOTRIAOSYLCERAMIDE
8 ENZYME REPLACEMENT THERAPY 58 25% 12% 202 Search ENZYME+REPLACEMENT+THERAPY Search ENZYME+REPLACEMENT+THERAPY
9 ALPHA GALACTOSIDASE A DEFICIENCY 56 100% 1% 19 Search ALPHA+GALACTOSIDASE+A+DEFICIENCY Search ALPHA+GALACTOSIDASE+A+DEFICIENCY
10 AGALSIDASE 27 92% 1% 11 Search AGALSIDASE Search AGALSIDASE

Key Words Plus



Rank Web of Science journal category Relevance score
(tfidf)
Class's shr.
of term's tot.
occurrences
Shr. of publ.
in class containing
term
Num. of
publ. in
class
1 OUTCOME SURVEY 311 84% 10% 171
2 ATYPICAL VARIANT 266 90% 7% 116
3 AGALSIDASE BETA THERAPY 210 88% 6% 100
4 ALPHA GALACTOSIDASE A 185 73% 8% 140
5 AGALSIDASE ALPHA 155 81% 6% 95
6 ENZYME REPLACEMENT THERAPY 143 25% 30% 509
7 ALPHA GALACTOSIDASE 140 40% 16% 278
8 HEMIZYGOTES 99 91% 2% 41
9 ONSET HYPERTROPHIC CARDIOMYOPATHY 86 90% 2% 37
10 HUMAN ALPHA GALACTOSIDASE 79 67% 4% 72

Journals

Reviews



Title Publ. year Cit. Active references % act. ref.
to same field
A systematic review on screening for Fabry disease: prevalence of individuals with genetic variants of unknown significance 2014 19 64 86%
Lysosomal storage disease 3 - Fabry's disease 2008 185 91 98%
Fabry disease 2010 81 335 93%
Natural course of Fabry disease and the effectiveness of enzyme replacement therapy: a systematic review and meta-analysis 2014 8 89 94%
Fabry disease: a review of current management strategies 2010 49 143 94%
Screening for Fabry disease in high-risk populations: a systematic review 2010 45 42 98%
Fabry disease 2009 78 165 85%
Prevalence of Fabry Disease in Stroke Patients-A Systematic Review and Meta-analysis 2014 7 36 78%
Fabry disease: Guidelines for the evaluation and management of multi-organ system involvement 2006 111 69 96%
Vasculopathy in patients with Fabry disease: Current controversies and research directions 2010 35 95 91%

Address terms



Rank Address term Relevance score
(tfidf)
Class's shr.
of term's tot.
occurrences
Shr. of publ.
in class containing
term
Num. of
publ. in
class
1 DEV METAB NEUROL BRANCH 31 34% 4.4% 74
2 CARDIAC REPAIR REGENERAT 15 82% 0.5% 9
3 REFERENCE MALAD FABRY MALAD HEREDITAI TI 12 75% 0.5% 9
4 LYSOSOMAL STORAGE DISORDERS UNIT 12 50% 1.0% 17
5 LYSOSOMAL STORAGE DISORDERS 11 69% 0.5% 9
6 LYSOSOMAL STORAGE DIS 9 52% 0.7% 12
7 CLIN NEPHROSCI 4 47% 0.4% 7
8 INTERNAL MED CLIN HAEMATOL 4 75% 0.2% 3
9 SECT NEWBORN SCREENING 4 75% 0.2% 3
10 CHARLES DENT METAB UNIT 4 20% 1.1% 18

Related classes at same level (level 1)



Rank Relatedness score Related classes
1 0.0000098836 ECCRINE ANGIOMATOUS HAMARTOMA//ANGIOKERATOMA//SOLITARY ANGIOKERATOMA
2 0.0000067106 ALPHA GALACTOSIDASE//STREPTOMYCES GRISEOLOALBUS//DEBARYOMYCES HANSENII UFV 1
3 0.0000063389 MUCOPOLYSACCHARIDOSES//HUNTER SYNDROME//MAROTEAUX LAMY SYNDROME
4 0.0000052620 FUCOSIDOSIS//ALPHA L FUCOSIDASE//FUCOSE DEHYDROGENASE
5 0.0000050192 GAUCHER DISEASE//GLUCOCEREBROSIDASE//GAUCHERS DISEASE
6 0.0000037879 POMPE DISEASE//GLYCOGEN STORAGE DISEASE TYPE II//ACID ALPHA GLUCOSIDASE
7 0.0000036893 FARBER DISEASE//ASAH1//FARBER LIPOGRANULOMATOSIS
8 0.0000032644 ASPARTYLGLUCOSAMINURIA//GLYCOSYLASPARAGINASE//ASPARTYLGLUCOSAMINIDASE
9 0.0000029685 GALACTOSIALIDOSIS//GM1 GANGLIOSIDOSIS//SIALIDASE
10 0.0000026285 DANON DISEASE//LAMP 2 DEFICIENCY//LAMP 2