Class information for:
Level 1: CYSTINOSIS//SALLA DISEASE//NEPHROPATHIC CYSTINOSIS

Basic class information
ID Publications Average number
of references		 Avg. shr. active
ref. in WoS
13328 780 26.9 63%


Bar chart of Publication_year

Last years might be incomplete

Classes in level above (level 2)


ID, lev.
above		 Publications Label for level above
2859 2400 CYSTINOSIS//PANTOTHENATE KINASE//PKAN

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 CYSTINOSIS Author keyword 330 86% 22% 171
2 SALLA DISEASE Author keyword 45 83% 3% 25
3 NEPHROPATHIC CYSTINOSIS Author keyword 32 80% 3% 20
4 CYSTINOSIN Author keyword 26 87% 2% 13
5 INFANTILE SIALIC ACID STORAGE DISEASE Author keyword 23 100% 1% 10
6 CTNS Author keyword 15 77% 1% 10
7 SECT HUMAN BIOCHEM GENET Address 12 29% 4% 35
8 CYSTEAMINE Author keyword 12 16% 9% 67
9 CTNS GENE Author keyword 11 100% 1% 6
10 CYSTINE DIMETHYL ESTER Author keyword 11 100% 1% 6

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 CYSTINOSIS 330 86% 22% 171 Search CYSTINOSIS Search CYSTINOSIS
2 SALLA DISEASE 45 83% 3% 25 Search SALLA+DISEASE Search SALLA+DISEASE
3 NEPHROPATHIC CYSTINOSIS 32 80% 3% 20 Search NEPHROPATHIC+CYSTINOSIS Search NEPHROPATHIC+CYSTINOSIS
4 CYSTINOSIN 26 87% 2% 13 Search CYSTINOSIN Search CYSTINOSIN
5 INFANTILE SIALIC ACID STORAGE DISEASE 23 100% 1% 10 Search INFANTILE+SIALIC+ACID+STORAGE+DISEASE Search INFANTILE+SIALIC+ACID+STORAGE+DISEASE
6 CTNS 15 77% 1% 10 Search CTNS Search CTNS
7 CYSTEAMINE 12 16% 9% 67 Search CYSTEAMINE Search CYSTEAMINE
8 CTNS GENE 11 100% 1% 6 Search CTNS+GENE Search CTNS+GENE
9 CYSTINE DIMETHYL ESTER 11 100% 1% 6 Search CYSTINE+DIMETHYL+ESTER Search CYSTINE+DIMETHYL+ESTER
10 INFANTILE CYSTINOSIS 8 100% 1% 5 Search INFANTILE+CYSTINOSIS Search INFANTILE+CYSTINOSIS

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 NEPHROPATHIC CYSTINOSIS 233 75% 22% 170
2 CYSTEAMINE THERAPY 197 98% 6% 50
3 CTNS MUTATIONS 61 88% 4% 29
4 CORNEAL CRYSTALS 57 95% 2% 19
5 INFANTILE CYSTINOSIS 45 94% 2% 16
6 SALLA DISEASE 42 70% 4% 35
7 INFANTILE NEPHROPATHIC CYSTINOSIS 38 86% 2% 19
8 TOPICAL CYSTEAMINE 26 100% 1% 11
9 GENE CTNS 18 83% 1% 10
10 PHOSPHOCYSTEAMINE 18 89% 1% 8

Journals

Reviews


Title Publ. year Cit. Active references % act. ref.
to same field
Cystinosis: the evolution of a treatable disease 2013 16 57 91%
Cystinosis 2002 205 48 96%
Nephropathic cystinosis: late complications of a multisystemic disease 2008 44 108 96%
Cystinosis: practical tools for diagnosis and treatment 2011 16 66 86%
Nephropathic cystinosis: an international consensus document 2014 4 60 93%
Pharmacological treatment of nephropathic cystinosis with cysteamine 2004 37 47 100%
Cysteamine: an old drug with new potential 2013 10 54 43%
Corneal crystals in nephropathic cystinosis: Natural history and treatment with cysteamine eyedrops 2000 42 36 100%
Molecular and cellular basis of lysosomal transmembrane protein dysfunction 2009 45 163 27%
The pathogenesis of cystinosis: mechanisms beyond cystine accumulation 2010 19 115 53%

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 SECT HUMAN BIOCHEM GENET 12 29% 4.5% 35
2 HUMAN GENET BRANCH HUMAN BIOCHEM GENET SECT 1 100% 0.3% 2
3 NSB 1 50% 0.3% 2
4 PEDIAT NEPHROL GROWTH REGENERAT 1 50% 0.3% 2
5 REFERENCE SUD OUEST MALAD RENALES RA SORAR 1 100% 0.3% 2
6 UMRS574 1 100% 0.3% 2
7 U423 1 13% 1.2% 9
8 U574 1 11% 1.3% 10
9 HAYWARD GENET 1 11% 1.0% 8
10 NEMOURS PROGRAMS 1 25% 0.4% 3

Related classes at same level (level 1)


Rank Relatedness score Related classes
1 0.0000145546 TRANSALDOLASE DEFICIENCY//ESA 8080//NEONATAL HAEMORRHAGIC DIATHESIS
2 0.0000132223 PANTETHINE//ALLITHIAMINE//ALLITHIAMIN
3 0.0000066111 SERUM SIALIC ACID//LIPID BOUND SIALIC ACID//TOTAL SIALIC ACID
4 0.0000065792 DANON DISEASE//LAMP 2 DEFICIENCY//LAMP 2
5 0.0000065505 ASPARTYLGLUCOSAMINURIA//GLYCOSYLASPARAGINASE//ASPARTYLGLUCOSAMINIDASE
6 0.0000052322 CALPAIN CATHEPSIN HYPOTHESIS//OSMOTIC SENSITIVITY//LYSOSOME STABILITY
7 0.0000050282 DNPI//VESICULAR UPTAKE//BNPI
8 0.0000046079 POLYUNSATURATED FREE FATTY ACID//BRUSH BORDERS//ISF PHARM
9 0.0000045106 MUCOLIPIDOSIS TYPE IV//MUCOLIPIN//MUCOLIPIDOSIS IV
10 0.0000041709 GALACTOSIALIDOSIS//GM1 GANGLIOSIDOSIS//SIALIDASE