Class information for:
Level 1: HERMANSKY PUDLAK SYNDROME//CHEDIAK HIGASHI SYNDROME//LYST

Basic class information
ID Publications Average number
of references		 Avg. shr. active
ref. in WoS
12579 833 37.9 67%


Bar chart of Publication_year

Last years might be incomplete

Classes in level above (level 2)


ID, lev.
above		 Publications Label for level above
2927 2251 HERMANSKY PUDLAK SYNDROME//CHEDIAK HIGASHI SYNDROME//GRISCELLI SYNDROME

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 HERMANSKY PUDLAK SYNDROME Author keyword 104 73% 9% 79
2 CHEDIAK HIGASHI SYNDROME Author keyword 91 69% 9% 77
3 LYST Author keyword 23 79% 2% 15
4 SECT HUMAN BIOCHEM GENET Address 21 37% 5% 45
5 PLATELET DENSE GRANULE Author keyword 12 86% 1% 6
6 HERMANSKY PUDLAK Author keyword 11 78% 1% 7
7 CHEDIAK HIGASHI Author keyword 9 64% 1% 9
8 PLATELET DENSE GRANULES Author keyword 9 83% 1% 5
9 BEIGE Author keyword 9 48% 2% 13
10 HPS1 Author keyword 8 100% 1% 5

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 HERMANSKY PUDLAK SYNDROME 104 73% 9% 79 Search HERMANSKY+PUDLAK+SYNDROME Search HERMANSKY+PUDLAK+SYNDROME
2 CHEDIAK HIGASHI SYNDROME 91 69% 9% 77 Search CHEDIAK+HIGASHI+SYNDROME Search CHEDIAK+HIGASHI+SYNDROME
3 LYST 23 79% 2% 15 Search LYST Search LYST
4 PLATELET DENSE GRANULE 12 86% 1% 6 Search PLATELET+DENSE+GRANULE Search PLATELET+DENSE+GRANULE
5 HERMANSKY PUDLAK 11 78% 1% 7 Search HERMANSKY+PUDLAK Search HERMANSKY+PUDLAK
6 CHEDIAK HIGASHI 9 64% 1% 9 Search CHEDIAK+HIGASHI Search CHEDIAK+HIGASHI
7 PLATELET DENSE GRANULES 9 83% 1% 5 Search PLATELET+DENSE+GRANULES Search PLATELET+DENSE+GRANULES
8 BEIGE 9 48% 2% 13 Search BEIGE Search BEIGE
9 HPS1 8 100% 1% 5 Search HPS1 Search HPS1
10 LYSOSOME RELATED ORGANELLE 7 53% 1% 9 Search LYSOSOME+RELATED+ORGANELLE Search LYSOSOME+RELATED+ORGANELLE

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 PALE EAR EP 68 82% 5% 40
2 STORAGE POOL DEFICIENCY 54 46% 10% 87
3 BETA 3A SUBUNIT 52 68% 5% 45
4 HERMANSKY PUDLAK SYNDROME 50 29% 18% 148
5 LYSOSOME RELATED ORGANELLES 40 40% 9% 78
6 HPS GENE 33 100% 2% 13
7 CHEDIAK HIGASHI SYNDROME 29 23% 13% 109
8 INHERITED ABNORMALITIES 27 92% 1% 11
9 SYNDROME BEIGE MOUSE 26 100% 1% 11
10 BEIGE 26 56% 4% 31

Journals

Reviews


Title Publ. year Cit. Active references % act. ref.
to same field
Hermansky-Pudlak syndrome: a disease of protein trafficking and organelle function 2006 185 131 69%
Disorders of lysosome-related organelle biogenesis: Clinical and molecular genetics 2008 110 120 45%
Molecular determinants of platelet delta storage pool deficiencies: an update 2013 16 36 58%
HermanskyPudlak syndrome: pigmentary and non-pigmentary defects and their pathogenesis 2013 15 174 57%
Hermansky-Pudlak syndrome Overview of clinical and molecular features and case report of a new HPS-1 variant 2014 2 53 79%
Neonatal and maternal risk in Hermansky-Pudlak syndrome: peripartum management-brief report and review of literature 2014 1 9 100%
Murine Hermansky-Pudiak syndrome genes: regulators of lysosome-related organelles 2004 108 78 65%
Hermansky-Pudlak Syndrome: Health Care Throughout Life 2013 8 22 64%
The cell biology of Hermansky-Pudiak syndrome: Recent advances 2005 108 58 50%
Towards the targeted management of Chediak-Higashi syndrome 2014 2 66 56%

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 21 37% 5.4% 45
2 LEUVEN AUTISM CONSORTIUM LAU 6 80% 0.5% 4
3 HUMAN MED GENET PROGRAM 2 13% 2.2% 18
4 HERITABLE DISORDERS BRANCH 2 10% 2.6% 22
5 HOSP JM MORALES MESEGUER 2 67% 0.2% 2
6 OPHTHALM GENET CLIN SERV BRANCH 2 26% 0.6% 5
7 OFF RARE DIS 1 14% 1.2% 10
8 COMPARAT GENET PROGRAM 1 50% 0.2% 2
9 GENOM TARGETS CANC 1 50% 0.2% 2
10 INTERNAL MED ENDOCRINOL METAB HEMATOL 1 100% 0.2% 2

Related classes at same level (level 1)


Rank Relatedness score Related classes
1 0.0000178357 ALBINISM//OCULOCUTANEOUS ALBINISM//OCULAR ALBINISM
2 0.0000170611 MYOSIN VA//RAB27A//GRISCELLI SYNDROME
3 0.0000110574 BERNARD SOULIER SYNDROME//MAY HEGGLIN ANOMALY//EPSTEIN SYNDROME
4 0.0000089083 CLATHRIN//AP180//CLATHRIN ASSEMBLY PROTEIN
5 0.0000072529 DANON DISEASE//LAMP 2 DEFICIENCY//LAMP 2
6 0.0000070682 MUCOLIPIDOSIS TYPE IV//MUCOLIPIN//MUCOLIPIDOSIS IV
7 0.0000056209 MELANOGENESIS//MITF//TYROSINASE
8 0.0000055303 HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS//HEMOPHAGOCYTIC SYNDROME//HAEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS
9 0.0000054866 PIRFENIDONE//FLUOROFENIDONE//AKF PD
10 0.0000054577 DISC1//DYSBINDIN//NEUREGULIN 1