Class information for:
Level 1: MUCOPOLYSACCHARIDOSIS//MUCOPOLYSACCHARIDOSES//HUNTER SYNDROME

Basic class information
Class id #P Avg. number of
references		 Database coverage
of references
1052 2880 32.2 70%


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
491 2             GAUCHER DISEASE//LYSOSOMAL STORAGE DISEASE//MUCOPOLYSACCHARIDOSIS 15457
1052 1                   MUCOPOLYSACCHARIDOSIS//MUCOPOLYSACCHARIDOSES//HUNTER SYNDROME 2880

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 MUCOPOLYSACCHARIDOSIS authKW 2813199 11% 82% 325
2 MUCOPOLYSACCHARIDOSES authKW 1403001 5% 88% 150
3 HUNTER SYNDROME authKW 1210746 4% 91% 126
4 HURLER SYNDROME authKW 944504 3% 90% 99
5 LYSOSOMAL DIS UNIT address 830886 4% 64% 123
6 LYSOSOMAL STORAGE DISEASE authKW 749496 9% 27% 261
7 MUCOPOLYSACCHARIDOSIS TYPE II authKW 744217 3% 95% 74
8 ENZYME REPLACEMENT THERAPY authKW 638215 8% 26% 234
9 MAROTEAUX LAMY SYNDROME authKW 625442 2% 100% 59
10 ALPHA L IDURONIDASE authKW 585724 2% 94% 59

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 Genetics & Heredity 23761 38% 0% 1093
2 Medicine, Research & Experimental 12507 25% 0% 723
3 Endocrinology & Metabolism 4389 16% 0% 459
4 Pediatrics 3921 13% 0% 388
5 Medical Laboratory Technology 2282 5% 0% 141
6 Biotechnology & Applied Microbiology 467 7% 0% 197
7 Biochemistry & Molecular Biology 322 13% 0% 385
8 Ophthalmology 309 3% 0% 99
9 Clinical Neurology 197 5% 0% 150
10 Pathology 192 3% 0% 87

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 LYSOSOMAL DIS UNIT 830886 4% 64% 123
2 WILLINK BIOCHEM GENET UNIT 224912 2% 34% 63
3 AMSTERDAM LYSOSOME SPHINX 193362 1% 79% 23
4 MED GENET SERV 136560 3% 16% 79
5 ERRO INNATOS METAB 66045 0% 69% 9
6 STUDY INBORN ERRORS METAB 62946 0% 42% 14
7 ANTHROPOMETRY 54516 0% 86% 6
8 METAB DIS H7 270 53004 0% 100% 5
9 STEM CELL NEUROTHER IES 47700 0% 75% 6
10 LYSOSOMAL STORAGE DIS 47494 0% 41% 11

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 MOLECULAR GENETICS AND METABOLISM 173511 8% 7% 226
2 JOURNAL OF INHERITED METABOLIC DISEASE 81800 6% 4% 187
3 ORPHANET JOURNAL OF RARE DISEASES 14451 1% 4% 39
4 HUMAN MUTATION 10509 2% 2% 63
5 MOLECULAR THERAPY 8961 2% 2% 55
6 PEDIATRIC ENDOCRINOLOGY REVIEWS PER 7052 0% 8% 8
7 CLINICA CHIMICA ACTA 4217 2% 1% 67
8 EXPERT OPINION ON ORPHAN DRUGS 3454 0% 3% 12
9 CLINICAL GENETICS 3267 1% 1% 40
10 HUMAN GENE THERAPY 3200 1% 1% 32

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 MUCOPOLYSACCHARIDOSIS 2813199 11% 82% 325 Search MUCOPOLYSACCHARIDOSIS Search MUCOPOLYSACCHARIDOSIS
2 MUCOPOLYSACCHARIDOSES 1403001 5% 88% 150 Search MUCOPOLYSACCHARIDOSES Search MUCOPOLYSACCHARIDOSES
3 HUNTER SYNDROME 1210746 4% 91% 126 Search HUNTER+SYNDROME Search HUNTER+SYNDROME
4 HURLER SYNDROME 944504 3% 90% 99 Search HURLER+SYNDROME Search HURLER+SYNDROME
5 LYSOSOMAL STORAGE DISEASE 749496 9% 27% 261 Search LYSOSOMAL+STORAGE+DISEASE Search LYSOSOMAL+STORAGE+DISEASE
6 MUCOPOLYSACCHARIDOSIS TYPE II 744217 3% 95% 74 Search MUCOPOLYSACCHARIDOSIS+TYPE+II Search MUCOPOLYSACCHARIDOSIS+TYPE+II
7 ENZYME REPLACEMENT THERAPY 638215 8% 26% 234 Search ENZYME+REPLACEMENT+THERAPY Search ENZYME+REPLACEMENT+THERAPY
8 MAROTEAUX LAMY SYNDROME 625442 2% 100% 59 Search MAROTEAUX+LAMY+SYNDROME Search MAROTEAUX+LAMY+SYNDROME
9 ALPHA L IDURONIDASE 585724 2% 94% 59 Search ALPHA+L+IDURONIDASE Search ALPHA+L+IDURONIDASE
10 SANFILIPPO SYNDROME 548615 2% 89% 58 Search SANFILIPPO+SYNDROME Search SANFILIPPO+SYNDROME

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 KHAN, S , ALMECIGA-DIAZ, CJ , SAWAMOTO, K , MACKENZIE, WG , THEROUX, MC , PIZARRO, C , MASON, RW , ORII, T , TOMATSU, S , (2017) MUCOPOLYSACCHARIDOSIS IVA AND GLYCOSAMINOGLYCANS.MOLECULAR GENETICS AND METABOLISM. VOL. 120. ISSUE 1-2. P. 78 -95 113 94% 0
2 LANGEREIS, EJ , VAN VLIES, N , WIJBURG, FA , (2015) DIAGNOSIS, CLASSIFICATION AND TREATMENT OF MUCOPOLYSACCHARIDOSIS TYPE I.EXPERT OPINION ON ORPHAN DRUGS. VOL. 3. ISSUE 3. P. 307 -320 125 83% 0
3 PECK, SH , CASAL, ML , MALHOTRA, NR , FICICIOGLU, C , SMITH, LJ , (2016) PATHOGENESIS AND TREATMENT OF SPINE DISEASE IN THE MUCOPOLYSACCHARIDOSES.MOLECULAR GENETICS AND METABOLISM. VOL. 118. ISSUE 4. P. 232 -243 128 74% 0
4 JAKOBKIEWICZ-BANECKA, J , GABIG-CIMINSKA, M , KLOSKA, A , MALINOWSKA, M , PIOTROWSKA, E , BANECKA-MAJKUTEWICZ, Z , BANECKI, B , WEGRZYN, A , WEGRZYN, G , (2016) GLYCOSAMINOGLYCANS AND MUCOPOLYSACCHARIDOSIS TYPE III.FRONTIERS IN BIOSCIENCE-LANDMARK. VOL. 21. ISSUE . P. 1393 -1409 90 95% 1
5 VALSTAR, MJ , RUIJTER, GJG , VAN DIGGELEN, OP , POORTHUIS, BJ , WIJBURG, FA , (2008) SANFILIPPO SYNDROME: A MINI-REVIEW.JOURNAL OF INHERITED METABOLIC DISEASE. VOL. 31. ISSUE 2. P. 240 -252 94 92% 109
6 NOH, H , LEE, JI , (2014) CURRENT AND POTENTIAL THERAPEUTIC STRATEGIES FOR MUCOPOLYSACCHARIDOSES.JOURNAL OF CLINICAL PHARMACY AND THERAPEUTICS. VOL. 39. ISSUE 3. P. 215-224 93 89% 13
7 TOMATSU, S , ALMECIGA-DIAZ, CJ , MONTANO, AM , YABE, H , TANAKA, A , DUNG, VC , GIUGLIANI, R , KUBASKI, F , MASON, RW , YASUDA, E , ET AL (2015) THERAPIES FOR THE BONE IN MUCOPOLYSACCHARIDOSES.MOLECULAR GENETICS AND METABOLISM. VOL. 114. ISSUE 2. P. 94 -109 96 83% 6
8 BRAUNLIN, EA , HARMATZ, PR , SCARPA, M , FURLANETTO, B , KAMPMANN, C , LOEHR, JP , PONDER, KP , ROBERTS, WC , ROSENFELD, HM , GIUGLIANI, R , (2011) CARDIAC DISEASE IN PATIENTS WITH MUCOPOLYSACCHARIDOSIS: PRESENTATION, DIAGNOSIS AND MANAGEMENT.JOURNAL OF INHERITED METABOLIC DISEASE. VOL. 34. ISSUE 6. P. 1183 -1197 96 86% 32
9 MITCHELL, J , BERGER, KI , BORGO, A , BRAUNLIN, EA , BURTON, BK , GHOTME, KA , KIRCHER, SG , MOLTER, D , ORCHARD, PJ , PALMER, J , ET AL (2016) UNIQUE MEDICAL ISSUES IN ADULT PATIENTS WITH MUCOPOLYSACCHARIDOSES.EUROPEAN JOURNAL OF INTERNAL MEDICINE. VOL. 34. ISSUE . P. 2 -10 87 94% 0
10 VALAYANNOPOULOS, V , NICELY, H , HARMATZ, P , TURBEVILLE, S , (2010) MUCOPOLYSACCHARIDOSIS VI.ORPHANET JOURNAL OF RARE DISEASES. VOL. 5. ISSUE . P. - 81 92% 87

Classes with closest relation at Level 1


Rank Class id link
1 11213 METACHROMATIC LEUKODYSTROPHY//ARYLSULFATASE A//MULTIPLE SULFATASE DEFICIENCY
2 21054 ALPHA MANNOSIDOSIS//BETA MANNOSIDOSIS//MANNOSIDOSIS
3 31161 CARPAL COALITION//FAMILIAL CARPAL TUNNEL SYNDROME//MELBOURNE PEDIAT HAND
4 10597 POMPE DISEASE//GLYCOGEN STORAGE DISEASE TYPE II//ACID ALPHA GLUCOSIDASE
5 12163 SANDHOFF DISEASE//GM2 GANGLIOSIDOSIS//TAY SACHS DISEASE
6 16795 KRABBE DISEASE//GLOBOID CELL LEUKODYSTROPHY//PSYCHOSINE
7 4302 FABRY DISEASE//ALPHA GALACTOSIDASE A//ANDERSON FABRY DISEASE
8 1959 GAUCHER DISEASE//GLUCOCEREBROSIDASE//GAUCHER CLIN
9 24263 FUCOSIDOSIS//ALPHA L FUCOSIDASE//CANINE FUCOSIDOSIS
10 27062 ASPARTYLGLUCOSAMINURIA//GLYCOSYLASPARAGINASE//ASPARTYLGLUCOSAMINIDASE

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