Class information for:
Level 2: CYSTINOSIS//NEPHROPATHIC CYSTINOSIS//SALLA DISEASE

Basic class information

Class id #P Avg. number of
references
Database coverage
of references
3898 833 27.9 64%



Bar chart of Publication_year

Last years might be incomplete

Hierarchy of classes

The table includes all classes above and classes immediately below the current class.



Cluster id Level Cluster label #P
0 4 BIOCHEMISTRY & MOLECULAR BIOLOGY//CELL BIOLOGY//ONCOLOGY 4064930
551 3       FABRY DISEASE//GAUCHER DISEASE//LYSOSOMAL STORAGE DISEASE 18099
3898 2             CYSTINOSIS//NEPHROPATHIC CYSTINOSIS//SALLA DISEASE 833
13496 1                   CYSTINOSIS//NEPHROPATHIC CYSTINOSIS//SALLA DISEASE 833

Terms with highest relevance score



rank Term termType Chi square Shr. of publ. in
class containing
term
Class's shr. of
term's tot. occurrences
#P with
term in
class
1 CYSTINOSIS authKW 5894044 22% 88% 182
2 NEPHROPATHIC CYSTINOSIS authKW 927028 3% 90% 28
3 SALLA DISEASE authKW 789986 3% 86% 25
4 CYSTEAMINE authKW 487163 9% 17% 77
5 CYSTINOSIN authKW 485145 2% 88% 15
6 SECT HUMAN BIOCHEM GENET address 368009 4% 29% 35
7 INFANTILE SIALIC ACID STORAGE DISEASE authKW 366557 1% 100% 10
8 CYSTINE DIMETHYL ESTER authKW 333231 1% 91% 10
9 CTNS authKW 316805 1% 79% 11
10 CTNS GENE authKW 256590 1% 100% 7

Web of Science journal categories



Rank Term Chi square Shr. of publ. in
class containing
term
Class's shr. of
term's tot. occurrences
#P with
term in
class
1 Pediatrics 3894 24% 0% 200
2 Urology & Nephrology 2494 16% 0% 137
3 Genetics & Heredity 951 15% 0% 126
4 Medicine, Research & Experimental 453 10% 0% 81
5 Ophthalmology 382 6% 0% 53
6 Biochemistry & Molecular Biology 352 21% 0% 173
7 Endocrinology & Metabolism 229 8% 0% 63
8 Medical Laboratory Technology 110 2% 0% 18
9 Biophysics 99 5% 0% 42
10 Transplantation 52 2% 0% 17

Address terms



Rank Term Chi square Shr. of publ. in
class containing
term
Class's shr. of
term's tot. occurrences
#P with
term in
class
1 SECT HUMAN BIOCHEM GENET 368009 4% 29% 35
2 PEDIAT NEPHROL GROWTH REGENERAT 76360 1% 42% 5
3 HERITABLE DISORDERS BRANCH 73775 3% 10% 21
4 HUMAN GENET BRANCH HUMAN BIOCHEM GENET SECT 73311 0% 100% 2
5 UMRS574 73311 0% 100% 2
6 CPNT 48873 0% 67% 2
7 REFERENCE SUD OUEST MALAD RENALES RA SORAR 48873 0% 67% 2
8 MED GENET BRANCH 43956 3% 4% 28
9 U423 41222 1% 13% 9
10 U574 38978 1% 11% 10

Journals



Rank Term Chi square Shr. of publ. in
class containing
term
Class's shr. of
term's tot. occurrences
#P with
term in
class
1 PEDIATRIC NEPHROLOGY 20345 7% 1% 58
2 JOURNAL OF INHERITED METABOLIC DISEASE 6314 3% 1% 28
3 MOLECULAR GENETICS AND METABOLISM 4213 2% 1% 19
4 PEDIATRIC RESEARCH 3113 3% 0% 28
5 JOURNAL OF PEDIATRICS 2095 3% 0% 28
6 ACTA PAEDIATRICA BELGICA 914 0% 3% 1
7 BIOCHEMICAL MEDICINE AND METABOLIC BIOLOGY 845 0% 1% 4
8 JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY 832 2% 0% 13
9 ORPHANET JOURNAL OF RARE DISEASES 816 1% 0% 5
10 EUROPEAN JOURNAL OF PEDIATRICS 691 1% 0% 12

Author Key Words



Rank Term Chi square Shr. of publ. in
class containing
term
Class's shr. of
term's tot. occurrences
#P with
term in
class
LCSH search Wikipedia search
1 CYSTINOSIS 5894044 22% 88% 182 Search CYSTINOSIS Search CYSTINOSIS
2 NEPHROPATHIC CYSTINOSIS 927028 3% 90% 28 Search NEPHROPATHIC+CYSTINOSIS Search NEPHROPATHIC+CYSTINOSIS
3 SALLA DISEASE 789986 3% 86% 25 Search SALLA+DISEASE Search SALLA+DISEASE
4 CYSTEAMINE 487163 9% 17% 77 Search CYSTEAMINE Search CYSTEAMINE
5 CYSTINOSIN 485145 2% 88% 15 Search CYSTINOSIN Search CYSTINOSIN
6 INFANTILE SIALIC ACID STORAGE DISEASE 366557 1% 100% 10 Search INFANTILE+SIALIC+ACID+STORAGE+DISEASE Search INFANTILE+SIALIC+ACID+STORAGE+DISEASE
7 CYSTINE DIMETHYL ESTER 333231 1% 91% 10 Search CYSTINE+DIMETHYL+ESTER Search CYSTINE+DIMETHYL+ESTER
8 CTNS 316805 1% 79% 11 Search CTNS Search CTNS
9 CTNS GENE 256590 1% 100% 7 Search CTNS+GENE Search CTNS+GENE
10 SIALIC ACID STORAGE DISEASE 188513 1% 86% 6 Search SIALIC+ACID+STORAGE+DISEASE Search SIALIC+ACID+STORAGE+DISEASE

Core articles

The table includes core articles in the class. The following variables is taken into account for the relevance score of an article in a cluster c:
(1) Number of references referring to publications in the class.
(2) Share of total number of active references referring to publications in the class.
(3) Age of the article. New articles get higher score than old articles.
(4) Citation rate, normalized to year.



Rank Reference # ref. in
cl.
Shr. of ref. in
cl.
Citations
1 ELMONEM, MA , VEYS, KR , SOLIMAN, NA , VAN DYCK, M , VAN DEN HEUVEL, LP , LEVTCHENKO, E , (2016) CYSTINOSIS: A REVIEW.ORPHANET JOURNAL OF RARE DISEASES. VOL. 11. ISSUE . P. - 91 90% 3
2 NESTEROVA, G , GAHL, W , (2008) NEPHROPATHIC CYSTINOSIS: LATE COMPLICATIONS OF A MULTISYSTEMIC DISEASE.PEDIATRIC NEPHROLOGY. VOL. 23. ISSUE 6. P. 863 -878 104 96% 54
3 BARSHOP, BA , LANGMAN, CB , DESCHENES, G , EMMA, F , GOODYER, P , LIPKIN, G , MIDGLEY, JP , OTTOLENGHI, C , SERVAIS, A , SOLIMAN, NA , ET AL (2016) CONTROVERSIES AND RESEARCH AGENDA IN NEPHROPATHIC CYSTINOSIS: CONCLUSIONS FROM A "KIDNEY DISEASE: IMPROVING GLOBAL OUTCOMES" (KDIGO) CONTROVERSIES CONFERENCE.KIDNEY INTERNATIONAL. VOL. 89. ISSUE 6. P. 1192 -1203 83 87% 2
4 CHERQUI, S , COURTOY, PJ , (2017) THE RENAL FANCONI SYNDROME IN CYSTINOSIS: PATHOGENIC INSIGHTS AND THERAPEUTIC PERSPECTIVES.NATURE REVIEWS NEPHROLOGY. VOL. 13. ISSUE 2. P. 115 -131 103 64% 0
5 ARICETA, G , CAMACHO, JA , FERNANDEZ-OBISPO, M , FERNANDEZ-POLO, A , GAMEZ, J , GARCIA-VILLORIA, J , MONTECZUMA, EL , LEYES, P , MARTIN-BEGUE, N , OPPENHEIMER, F , ET AL (2015) CYSTINOSIS IN ADULT AND ADOLESCENT PATIENTS: RECOMMENDATIONS FOR THE COMPREHENSIVE CARE OF CYSTINOSIS.NEFROLOGIA. VOL. 35. ISSUE 3. P. 304 -321 72 81% 1
6 EMMA, F , NESTEROVA, G , LANGMAN, C , LABBE, A , CHERQUI, S , GOODYER, P , JANSSEN, MC , GRECO, M , TOPALOGLU, R , ELENBERG, E , ET AL (2014) NEPHROPATHIC CYSTINOSIS: AN INTERNATIONAL CONSENSUS DOCUMENT.NEPHROLOGY DIALYSIS TRANSPLANTATION. VOL. 29. ISSUE . P. 87 -94 57 95% 13
7 NESTEROVA, G , GAHL, WA , (2013) CYSTINOSIS: THE EVOLUTION OF A TREATABLE DISEASE.PEDIATRIC NEPHROLOGY. VOL. 28. ISSUE 1. P. 51-59 52 91% 26
8 WILMER, MJ , SCHOEBER, JP , VAN DEN HEUVEL, LP , LEVTCHENKO, EN , (2011) CYSTINOSIS: PRACTICAL TOOLS FOR DIAGNOSIS AND TREATMENT.PEDIATRIC NEPHROLOGY. VOL. 26. ISSUE 2. P. 205 -215 56 85% 19
9 BESOUW, MTP , EMMA, F , LEVTCHENKO, EN , (2013) MANAGEMENT OF NEPHROPATHIC CYSTINOSIS.EXPERT OPINION ON ORPHAN DRUGS. VOL. 1. ISSUE 12. P. 1031 -1039 51 93% 0
10 GAHL, WA , THOENE, JG , SCHNEIDER, JA , (2002) CYSTINOSIS.NEW ENGLAND JOURNAL OF MEDICINE. VOL. 347. ISSUE 2. P. 111 -121 46 96% 247

Classes with closest relation at Level 2



Rank Class id link
1 491 GAUCHER DISEASE//LYSOSOMAL STORAGE DISEASE//MUCOPOLYSACCHARIDOSIS
2 3395 PKAN//NBIA//PANTOTHENATE KINASE
3 1128 AUTOPHAGY//BECLIN 1//LC3
4 3543 ALKAPTONURIA//OCHRONOSIS//HOMOGENTISIC ACID
5 1273 GANGLIOSIDES//SIALIC ACID//GLYCOSPHINGOLIPIDS
6 2940 CHYLOTHORAX//CHYLOUS ASCITES//THORACIC DUCT
7 2899 OCULAR DRUG DELIVERY//OPHTHALMIC DRUG DELIVERY//OCULAR DELIVERY
8 3320 FABRY DISEASE//ALPHA GALACTOSIDASE A//ANDERSON FABRY DISEASE
9 1894 CYSTINURIA//REGULATORY VOLUME DECREASE//CELL VOLUME REGULATION
10 3980 SAND DUNE AGR EXPT STN//ZIRCONIUM ASCORBATE//PLASTID PROLIFERATION

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