Class information for:
Level 2: LIPOID PROTEINOSIS//JUVENILE HYALINE FIBROMATOSIS//HAJDU CHENEY SYNDROME

Basic class information

Class id #P Avg. number of
references
Database coverage
of references
3791 968 24.2 57%



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
382 3       DUPUYTRENS DISEASE//PRESSURE ULCER//VARICOSE VEINS 31698
3791 2             LIPOID PROTEINOSIS//JUVENILE HYALINE FIBROMATOSIS//HAJDU CHENEY SYNDROME 968
26933 1                   HAJDU CHENEY SYNDROME//ACROOSTEOLYSIS//SERPENTINE FIBULA POLYCYSTIC KIDNEY SYNDROME 241
27465 1                   JUVENILE HYALINE FIBROMATOSIS//INFANTILE SYSTEMIC HYALINOSIS//HYALINE FIBROMATOSIS SYNDROME 229
27558 1                   LIPOID PROTEINOSIS//EXTRACELLULAR MATRIX PROTEIN 1//HYALINOSIS CUTIS ET MUCOSAE 226
30234 1                   MEGALOCORNEA//NEUHAUSER SYNDROME//ANTERIOR MEGALOPHTHALMOS 172
35239 1                   BONES METABOLIC ABNORMALITIES//PARATHYROID SECONDARY HYPERPARATHYROIDISM//REUMATOL E BRUZZONE 100

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 LIPOID PROTEINOSIS authKW 1892596 6% 100% 60
2 JUVENILE HYALINE FIBROMATOSIS authKW 1230188 4% 100% 39
3 HAJDU CHENEY SYNDROME authKW 1147021 4% 91% 40
4 INFANTILE SYSTEMIC HYALINOSIS authKW 567779 2% 100% 18
5 HYALINOSIS CUTIS ET MUCOSAE authKW 536236 2% 100% 17
6 EXTRACELLULAR MATRIX PROTEIN 1 authKW 486663 2% 86% 18
7 URBACH WIETHE DISEASE authKW 467297 2% 74% 20
8 ACROOSTEOLYSIS authKW 439286 3% 45% 31
9 MEGALOCORNEA authKW 337617 2% 63% 17
10 SERPENTINE FIBULA POLYCYSTIC KIDNEY SYNDROME authKW 252346 1% 100% 8

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 Dermatology 4240 16% 0% 157
2 Ophthalmology 2586 14% 0% 139
3 Pediatrics 1039 12% 0% 117
4 Radiology, Nuclear Medicine & Medical Imaging 713 12% 0% 112
5 Genetics & Heredity 626 12% 0% 114
6 Rheumatology 615 5% 0% 47
7 Orthopedics 155 4% 0% 39
8 Dentistry, Oral Surgery & Medicine 136 4% 0% 34
9 Endocrinology & Metabolism 105 5% 0% 51
10 Pathology 103 4% 0% 35

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 DUKE ORTHOPAED CELLULAR DEV GENOME S 84113 0% 67% 4
2 UCONN MUSCULOSKELETAL 35482 0% 38% 3
3 ABT NEPHROL STOFFWECHSELERKRANKUNGEN 31543 0% 100% 1
4 ADRIANO JORGE HOSP FDN FHAJ 31543 0% 100% 1
5 AMBULANZ IELEN KINDEROPHTHALMOL REHABIL SE 31543 0% 100% 1
6 ARRD 31543 0% 100% 1
7 ARTHRIT BONE METAB GASTROINTESTINAL DIS AREA 31543 0% 100% 1
8 CELL GENET SKIN DIS GRP 31543 0% 100% 1
9 CHILDRENS HOSP SHANXI PROVINCE 31543 0% 100% 1
10 COCUK SAGLILIGI HASTALIKLARI 31543 0% 100% 1

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 RADIOLOGE 5021 3% 1% 25
2 FORTSCHRITTE AUF DEM GEBIETE DER RONTGENSTRAHLEN UND DER NUKLEARMEDIZIN 4914 2% 1% 20
3 JOURNAL OF PEDIATRIC OPHTHALMOLOGY & STRABISMUS 3182 1% 1% 13
4 OPHTHALMIC PAEDIATRICS AND GENETICS 2977 1% 2% 6
5 CLINICAL DYSMORPHOLOGY 2812 1% 1% 10
6 AMERICAN JOURNAL OF MEDICAL GENETICS 2588 3% 0% 27
7 PEDIATRIC DERMATOLOGY 1652 1% 0% 14
8 INTERNATIONAL JOURNAL OF DERMATOLOGY 1534 2% 0% 19
9 AKTUELLE RHEUMATOLOGIE 1290 1% 1% 7
10 HAUTARZT 1270 1% 0% 14

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 LIPOID PROTEINOSIS 1892596 6% 100% 60 Search LIPOID+PROTEINOSIS Search LIPOID+PROTEINOSIS
2 JUVENILE HYALINE FIBROMATOSIS 1230188 4% 100% 39 Search JUVENILE+HYALINE+FIBROMATOSIS Search JUVENILE+HYALINE+FIBROMATOSIS
3 HAJDU CHENEY SYNDROME 1147021 4% 91% 40 Search HAJDU+CHENEY+SYNDROME Search HAJDU+CHENEY+SYNDROME
4 INFANTILE SYSTEMIC HYALINOSIS 567779 2% 100% 18 Search INFANTILE+SYSTEMIC+HYALINOSIS Search INFANTILE+SYSTEMIC+HYALINOSIS
5 HYALINOSIS CUTIS ET MUCOSAE 536236 2% 100% 17 Search HYALINOSIS+CUTIS+ET+MUCOSAE Search HYALINOSIS+CUTIS+ET+MUCOSAE
6 EXTRACELLULAR MATRIX PROTEIN 1 486663 2% 86% 18 Search EXTRACELLULAR+MATRIX+PROTEIN+1 Search EXTRACELLULAR+MATRIX+PROTEIN+1
7 URBACH WIETHE DISEASE 467297 2% 74% 20 Search URBACH+WIETHE+DISEASE Search URBACH+WIETHE+DISEASE
8 ACROOSTEOLYSIS 439286 3% 45% 31 Search ACROOSTEOLYSIS Search ACROOSTEOLYSIS
9 MEGALOCORNEA 337617 2% 63% 17 Search MEGALOCORNEA Search MEGALOCORNEA
10 SERPENTINE FIBULA POLYCYSTIC KIDNEY SYNDROME 252346 1% 100% 8 Search SERPENTINE+FIBULA+POLYCYSTIC+KIDNEY+SYNDROME Search SERPENTINE+FIBULA+POLYCYSTIC+KIDNEY+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 HAIDAR, Z , TEMANNI, R , CHOUERY, E , JITHESH, P , LIU, W , AL-ALI, R , WANG, E , MARINCOLA, FM , JALKH, N , HADDAD, S , ET AL (2017) DIAGNOSIS IMPLICATIONS OF THE WHOLE GENOME SEQUENCING IN A LARGE LEBANESE FAMILY WITH HYALINE FIBROMATOSIS SYNDROME.BMC GENETICS. VOL. 18. ISSUE . P. - 48 80% 0
2 EL-MAAYTAH, M , JERJES, W , SHAH, P , UPILE, T , MURPHY, C , AYLIFFE, P , (2010) GINGIVAL HYPERPLASIA ASSOCIATED WITH JUVENILE HYALINE FIBROMATOSIS: A CASE REPORT AND REVIEW OF THE LITERATURE.JOURNAL OF ORAL AND MAXILLOFACIAL SURGERY. VOL. 68. ISSUE 10. P. 2604 -2608 33 100% 4
3 HALEEM, A , AL-HINDI, HN , AL JUBOURY, M , AL HUSSEINI, H , AL AJLAN, A , (2002) JUVENILE HYALINE FIBROMATOSIS - MORPHOLOGIC, IMMUNOHISTOCHEMICAL, AND ULTRASTRUCTURAL STUDY OF THREE SIBLINGS.AMERICAN JOURNAL OF DERMATOPATHOLOGY. VOL. 24. ISSUE 3. P. 218-224 37 100% 15
4 DENADAI, R , RAPOSO-AMARAL, CE , BERTOLA, D , KIM, C , ALONSO, N , HART, T , HAN, S , STELINI, RF , BUZZO, CL , RAPOSO-AMARAL, CA , ET AL (2012) IDENTIFICATION OF 2 NOVEL ANTXR2 MUTATIONS IN PATIENTS WITH HYALINE FIBROMATOSIS SYNDROME AND PROPOSAL OF A MODIFIED GRADING SYSTEM.AMERICAN JOURNAL OF MEDICAL GENETICS PART A. VOL. 158A. ISSUE 4. P. 732-742 26 100% 12
5 CHAN, I , LIU, L , HAMADA, T , SETHURAMAN, G , MCGRATH, JA , (2007) THE MOLECULAR BASIS OF LIPOID PROTEINOSIS: MUTATIONS IN EXTRACELLULAR MATRIX PROTEIN 1.EXPERIMENTAL DERMATOLOGY. VOL. 16. ISSUE 11. P. 881-890 40 69% 52
6 LI, Y , LI, YQ , ZHAO, JL , WANG, DY , MAO, QW , XIA, HB , (2016) PRODUCTION AND CHARACTERIZATION OF DOMAIN-SPECIFIC MONOCLONAL ANTIBODIES AGAINST HUMAN ECM1.PROTEIN EXPRESSION AND PURIFICATION. VOL. 121. ISSUE . P. 103 -111 28 88% 0
7 ALLEN, PW , (2001) SELECTED CASE FROM THE ARKADI M. RYWLIN INTERNATIONAL PATHOLOGY SLIDE SEMINAR: HYALINE FIBROMATOSIS.ADVANCES IN ANATOMIC PATHOLOGY. VOL. 8. ISSUE 3. P. 173-178 36 100% 5
8 MUNIZ, ML , LOBO, AZC , MACHADO, MCDR , VALENTE, NYS , KIM, CA , LOURENCO, SV , NICO, MMS , (2006) EXUBERANT JUVENILE HYALINE FIBROMATOSIS IN TWO PATIENTS.PEDIATRIC DERMATOLOGY. VOL. 23. ISSUE 5. P. 458-464 29 100% 15
9 NOFAL, A , SANAD, M , ASSAF, M , NOFAL, E , NASSAR, A , ALMOKADEM, S , ATTWA, E , ELMOSALAMY, K , (2009) JUVENILE HYALINE FIBROMATOSIS AND INFANTILE SYSTEMIC HYALINOSIS: A UNIFYING TERM AND A PROPOSED GRADING SYSTEM.JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY. VOL. 61. ISSUE 4. P. 695 -700 25 100% 25
10 RIBEIRO, SLE , GUEDES, EL , BOTAN, V , BARBOSA, A , DE FREITAS, EJG , (2009) JUVENILE HYALINE FIBROMATOSIS: A CASE REPORT AND REVIEW OF THE LITERATURE.ACTA REUMATOLOGICA PORTUGUESA. VOL. 34. ISSUE 1. P. 128-133 24 96% 7

Classes with closest relation at Level 2



Rank Class id link
1 2542 CADASIL//NOTCH3//NOTCH
2 2658 ACHONDROPLASIA//SKELETAL DYSPLASIA//HYPOCHONDROPLASIA
3 3356 DUPUYTRENS DISEASE//DUPUYTRENS CONTRACTURE//HYPERTROPHIC OSTEOARTHROPATHY
4 1524 OSTEOCLAST//OSTEOPETROSIS//RANKL
5 2793 SPINAL EPIDURAL ABSCESS//SPONDYLODISCITIS//DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS
6 2861 VULVODYNIA//FEMALE GENITAL MUTILATION//LICHEN SCLEROSUS
7 1686 ANTHRAX//BACILLUS ANTHRACIS//BACILLUS CEREUS
8 72 OSTEOARTHRITIS//OSTEOARTHRITIS AND CARTILAGE//CHONDROCYTE
9 1399 HEMANGIOMA//LYMPHANGIOMA//INFANTILE HEMANGIOMA
10 3833 CITED2//BRANCHIO OCULO FACIAL SYNDROME//AP 2 ALPHA

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