Class information for:
Level 1: AICARDI SYNDROME//GOLTZ SYNDROME//FOCAL DERMAL HYPOPLASIA

Basic class information
ID Publications Average number
of references		 Avg. shr. active
ref. in WoS
19675 430 20.6 60%


Bar chart of Publication_year

Last years might be incomplete

Classes in level above (level 2)


ID, lev.
above		 Publications Label for level above
3008 2083 SMITH LEMLI OPITZ SYNDROME//INCONTINENTIA PIGMENTI//CHONDRODYSPLASIA PUNCTATA

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 AICARDI SYNDROME Author keyword 81 79% 12% 52
2 GOLTZ SYNDROME Author keyword 62 92% 6% 24
3 FOCAL DERMAL HYPOPLASIA Author keyword 53 79% 8% 34
4 INFANTILE DIGITAL FIBROMATOSIS Author keyword 21 90% 2% 9
5 CHORIORETINAL LACUNAE Author keyword 18 89% 2% 8
6 GOLTZ GORLIN SYNDROME Author keyword 9 67% 2% 8
7 PORCN Author keyword 9 67% 2% 8
8 AICARDIS SYNDROME Author keyword 8 100% 1% 5
9 PORCN GENE Author keyword 8 100% 1% 5
10 ASTROCYTIC INCLUSIONS Author keyword 6 80% 1% 4

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 AICARDI SYNDROME 81 79% 12% 52 Search AICARDI+SYNDROME Search AICARDI+SYNDROME
2 GOLTZ SYNDROME 62 92% 6% 24 Search GOLTZ+SYNDROME Search GOLTZ+SYNDROME
3 FOCAL DERMAL HYPOPLASIA 53 79% 8% 34 Search FOCAL+DERMAL+HYPOPLASIA Search FOCAL+DERMAL+HYPOPLASIA
4 INFANTILE DIGITAL FIBROMATOSIS 21 90% 2% 9 Search INFANTILE+DIGITAL+FIBROMATOSIS Search INFANTILE+DIGITAL+FIBROMATOSIS
5 CHORIORETINAL LACUNAE 18 89% 2% 8 Search CHORIORETINAL+LACUNAE Search CHORIORETINAL+LACUNAE
6 GOLTZ GORLIN SYNDROME 9 67% 2% 8 Search GOLTZ+GORLIN+SYNDROME Search GOLTZ+GORLIN+SYNDROME
7 PORCN 9 67% 2% 8 Search PORCN Search PORCN
8 AICARDIS SYNDROME 8 100% 1% 5 Search AICARDIS+SYNDROME Search AICARDIS+SYNDROME
9 PORCN GENE 8 100% 1% 5 Search PORCN+GENE Search PORCN+GENE
10 ASTROCYTIC INCLUSIONS 6 80% 1% 4 Search ASTROCYTIC+INCLUSIONS Search ASTROCYTIC+INCLUSIONS

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 GOLTZ SYNDROME 33 67% 7% 29
2 DERMAL APLASIA 31 92% 3% 12
3 CONGENITAL MICROPHTHALMIA 28 81% 4% 17
4 LINEAR SKIN DEFECTS 25 62% 6% 26
5 TO DAUGHTER TRANSMISSION 17 100% 2% 8
6 MIDAS SYNDROME MICROPHTHALMIA 15 77% 2% 10
7 PORCN GENE 15 88% 2% 7
8 SCLEROCORNEA 14 59% 4% 16
9 MLS SYNDROME 13 61% 3% 14
10 XP222 PTER 13 80% 2% 8

Journals

Reviews


Title Publ. year Cit. Active
references		 % act. ref.
to same field
Focal dermal hypoplasia: updates 2014 2 41 88%
Aicardi syndrome 2005 78 25 84%
Palliative epilepsy surgery in Aicardi syndrome: a case series and review of literature 2014 1 13 77%
Focal dermal hypoplasia: a case report and literature review 2011 4 13 92%
AICARDI SYNDROME WITH MULTIPLE TUMORS - A CASE-REPORT WITH LITERATURE-REVIEW 1995 16 7 86%
Dosage compensation of the mammalian X chromosome influences the phenotypic variability of X-linked dominant male-lethal disorders 2008 31 47 36%
Infantile digital fibromatosis 2008 5 36 83%
Focal dermal hypoplasia resulting from a new nonsense mutation, p.E300X, in the PORCN gene 2008 15 8 50%
Microphthalmia with linear skin defects (MLS), Aicardi, and Goltz syndromes: are they related X-linked dominant male-lethal disorders? 2002 21 86 60%
Aicardi syndrome with favorable outcome: Case report and review 2007 4 9 89%

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 ANAESTHESIA INTENS CARE DERMATOL SCI 1 50% 0.2% 1
2 CHIRURG PLAST INFANTILE 1 50% 0.2% 1
3 HLTH SCI COMMUNITY 1 50% 0.2% 1
4 HOSP DI VENERE 1 50% 0.2% 1
5 NEUROSURG HANNOVER 1 50% 0.2% 1
6 UNITE MIXTE RECH SANTE 910 1 50% 0.2% 1
7 BOSTON CITY HOSP MED PEDIAT 0 33% 0.2% 1
8 CHARLES S KUBIK NEUROPATHOL 0 33% 0.2% 1
9 SERV CHIRURG GEN PEDIAT 0 33% 0.2% 1
10 SERV NEONATOL REANIMAT INFANTILE 0 33% 0.2% 1

Related classes at same level (level 1)


Rank Relatedness score Related classes
1 0.0000167536 VICI SYNDROME//AGENESIS OF THE CORPUS CALLOSUM//CALLOSAL AGENESIS
2 0.0000104252 INCONTINENTIA PIGMENTI//HYPOMELANOSIS OF ITO//BLOCH SULZBERGER SYNDROME
3 0.0000102675 RETROMER//SORTING NEXIN//VPS35
4 0.0000081872 NANOPHTHALMOS//ANOPHTHALMIA//MICROPHTHALMIA
5 0.0000067055 OPTIC DISC PIT//OPTIC PIT//MORNING GLORY SYNDROME
6 0.0000062998 WEST SYNDROME//INFANTILE SPASMS//HYPSARRHYTHMIA
7 0.0000056625 CHOROID PLEXUS PAPILLOMA//CHOROID PLEXUS CARCINOMA//CHOROID PLEXUS TUMORS
8 0.0000054991 INFANTILE MYOFIBROMATOSIS//MYOFIBROMATOSIS//MYOFIBROMA
9 0.0000054295 ELLIS VAN CREVELD SYNDROME//SHORT RIB POLYDACTYLY SYNDROME//JEUNE SYNDROME
10 0.0000052004 ADAMS OLIVER SYNDROME//APLASIA CUTIS CONGENITA//SETLEIS SYNDROME