DiseaseID 2654
壳状牙
disease
Amelogenesis imperfecta-nephrocalcinosis, also called enamel-renal syndrome, is an extremely rare syndrome which is characterized by hypoplastic amelogenesis imperfecta (hypoplastic dental enamel) and
Relationship Network
Interactive first-hop connections across herbs, ingredients, formulas, targets, diseases, symptoms, syndromes, evidence, and monographs.
Click a node to open it in a new tab
Disease: 1Formula: 24Herb: 12Symptom: 6Target: 24Links: 66
Arranging relationship network...
Record Fields
Scalar fields from the final disease record.
- Disease Id
- 2654
- Core Entity Id
- 3010
- Source Entity Count
- 1
- Preferred Name
- Shell Teeth
- Name Cn
- 壳状牙
- Name Pinyin
- Ke Zhuang Ya
- Name En
- Shell Teeth
- Name Latin
- Bilingual Status
- complete
- Disease Type
- disease
- Umls Disease Type
- Anatomical Abnormality
- Disgenet Type
- disease
- Mesh Class
- Female Urogenital Diseases and Pregnancy Complications; Stomatognathic Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Male Urogenital DiseasesStomatognathic DiseasesStomatognathic Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities
- Do Class
- disease of anatomical entitydisease of anatomical entity; genetic disease
- Hpo Class
- Abnormality of head or neckAbnormality of head or neck; Abnormality of connective tissue; Abnormality of the skeletal system
- Mesh Class Name
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Stomatognathic DiseasesCongenital, Hereditary, and Neonatal Diseases and Abnormalities; Stomatognathic DiseasesStomatognathic Diseases
- Hpo Class Name
- Abnormality of head or neckAbnormality of head or neck; Abnormality of the skeletal system; Abnormality of connective tissue
- Do Class Name
- disease of anatomical entitygenetic disease; disease of anatomical entity
- Disease Definition
- Amelogenesis imperfecta-nephrocalcinosis, also called enamel-renal syndrome, is an extremely rare syndrome which is characterized by hypoplastic amelogenesis imperfecta (hypoplastic dental enamel) and
- Version
- v1
- Suppressed
- No
Names
Preferred names, aliases, and source labels retained in the final schema.
Name
Shell Teeth
Role
preferred
Name
Amelogenesis Imperfecta, Hypomaturation Type, Iia1
Role
preferred
Name
Amelogenesis Imperfecta, Hypomaturation Type, Iia2
Role
preferred
Name
Amelogenesis Imperfecta, Hypomaturation Type, Iia3
Role
preferred
Name
Amelogenesis Imperfecta, Hypomaturation Type, Iia4
Role
preferred
Name
Amelogenesis Imperfecta, Hypomaturation Type, Iia5
Role
preferred
Name
Amelogenesis Imperfecta, Type Ia
Role
preferred
Name
Amelogenesis Imperfecta, Type Ib
Role
preferred
Name
Amelogenesis Imperfecta, Type Ie
Role
preferred
Name
Amelogenesis Imperfecta, Type If
Role
preferred
Name
Amelogenesis Imperfecta, Type Ih
Role
preferred
Name
Amelogenesis Imperfecta, Type Iv
Role
preferred
Name
Amelogenesis Imperfecta-Gingival Hyperplasia Syndrome
Role
preferred
Name
Dentin Dysplasia
Role
preferred
Name
Dentin Dysplasia, Type I
Role
preferred
Name
Dentin Dysplasia, Type Ii
Role
preferred
Name
AMELOGENESIS IMPERFECTA, HYPOPLASTIC/HYPOMATURATION, X-LINKED 1
Role
preferred
Name
Amelogenesis Imperfecta
Role
preferred
Name
Amelogenesis Imperfecta Nephrocalcinosis
Role
preferred
Name
Amelogenesis Imperfecta, Hypomaturation Type, With Snow-Capped Teeth
Role
preferred
Name
Amelogenesis Imperfecta, Type III
Role
preferred
Name
Amelogenesis Imperfecta-Nephrocalcinosis Syndrome
Role
preferred
Name
DENTIN DYSPLASIA, TYPE I, WITH EXTREME MICRODONTIA AND MISSHAPEN TEETH
Role
preferred
Name
Dental Pulp Stone
Role
preferred
Name
Dentin Dyspalsia, Shields Type 2
Role
preferred
Name
Dentin Dysplasia, Type 1
Role
preferred
Name
Dentinogenesis Imperfecta
Role
preferred
Name
Dentinogenesis Imperfecta - Shield'S Type Iii (Disorder)
Role
preferred
Name
Dentinogenesis Imperfecta Limited To Primary Teeth
Role
preferred
Name
Dentinogenesis Imperfecta Without Osteogenesis Imperfecta
Role
preferred
Name
Hereditary Disturbances In Tooth Structure, Not Elsewhere Classified In Icd10Cm
Role
preferred
Name
Hereditary Opalescent Dentin (Disorder)
Role
preferred
Name
Odontodysplasia
Role
preferred
Name
Odontogenesis Imperfecta
Role
preferred
Name
Opalescent Dentin
Role
preferred
Name
AI1A
Role
alias
Name
AI1B
Role
alias
Name
AI1E
Role
alias
Name
AI1F
Role
alias
Name
AI1H
Role
alias
Name
AI2A1
Role
alias
Name
AI2A2
Role
alias
Name
AI2A3
Role
alias
Name
AI2A4
Role
alias
Name
AI2A5
Role
alias
Name
AI4
Role
alias
Name
AIH1
Role
alias
Name
AIH2
Role
alias
Name
AIHHT
Role
alias
Name
AMELOGENESIS IMPERFECTA, HYPOMATURATION-HYPOPLASTIC TYPE, WITH TAURODONTISM
Role
alias
Name
AMELOGENESIS IMPERFECTA, HYPOPLASTIC LOCAL, AUTOSOMAL DOMINANT
Role
alias
Name
AMELOGENESIS IMPERFECTA, HYPOPLASTIC TYPE IA
Role
alias
Name
AMELOGENESIS IMPERFECTA, HYPOPLASTIC TYPE IF
Role
alias
Name
AMELOGENESIS IMPERFECTA, PIGMENTED HYPOMATURATION TYPE, 1
Role
alias
Name
AMELOGENESIS IMPERFECTA, PIGMENTED HYPOMATURATION TYPE, 2
Role
alias
Name
AMELOGENESIS IMPERFECTA, X-LINKED 1
Role
alias
Name
ANOMALOUS DYSPLASIA OF DENTIN
Role
alias
Name
Amelogenesis Imperfecta Hypomaturation Type 2A2
Role
alias
Name
Amelogenesis Imperfecta Hypomaturation Type 2A3
Role
alias
Name
Amelogenesis Imperfecta Hypomaturation Type 2A4
Role
alias
Name
Amelogenesis Imperfecta Hypomaturation Type 2A5
Role
alias
Name
Amelogenesis Imperfecta Type 1A
Role
alias
Name
Amelogenesis Imperfecta Type 1B
Role
alias
Name
Amelogenesis Imperfecta Type 1C
Role
alias
Name
Amelogenesis Imperfecta Type 1E
Role
alias
Name
Amelogenesis Imperfecta Type 1F
Role
alias
Name
Amelogenesis Imperfecta Type 1G
Role
alias
Name
Amelogenesis Imperfecta Type 1H
Role
alias
Name
Amelogenesis Imperfecta Type 2A1
Role
alias
Name
Amelogenesis Imperfecta Type 3A
Role
alias
Name
Amelogenesis Imperfecta Type 4
Role
alias
Name
Amelogenesis Imperfecta, Hypoplastic-Hypomaturation, X-Linked 1
Role
alias
Name
Amelogenesis Imperfecta, Type Ig
Role
alias
Name
Amelogenesis Imperfecta, Type Iiia
Role
alias
Name
Brandywine Type Dentinogenesis Imperfecta
Role
alias
Name
CORONAL DENTIN DYSPLASIA
Role
alias
Name
DENTIN DYSPLASIA, SHIELDS TYPE I
Role
alias
Name
DENTIN DYSPLASIA, SHIELDS TYPE II
Role
alias
Name
DTDP1
Role
alias
Name
DTDP2
Role
alias
Name
Dental Pulp Calcification
Role
alias
Name
Dentin, Opalescent
Role
alias
Name
Dentinogenesis Imperfecta 1
Role
alias
Name
Dentinogenesis Imperfecta of Baby Teeth
Role
alias
Name
Dentinogenesis Imperfecta, Shields Type 3
Role
alias
Name
Dentinogenesis Imperfecta, Shields Type Iii
Role
alias
Name
ENAMEL HYPOPLASIA, HEREDITARY LOCALIZED
Role
alias
Name
ENAMEL HYPOPLASIA, X-LINKED
Role
alias
Name
Enamel-Renal Syndrome
Role
alias
Name
Hereditary Disturbances In Tooth Structure, Not Elsewhere Classified
Role
alias
Name
Hereditary Opalescent Dentin
Role
alias
Name
PULP STONES
Role
alias
Name
PULPAL DYSPLASIA
Role
alias
Name
Pulp Calcifications
Role
alias
Name
Pulp Degeneration
Role
alias
Name
Pulp Denticles
Role
alias
Name
RADICULAR DENTIN DYSPLASIA
Role
alias
Name
ROOTLESS TEETH
Role
alias
Name
Teeth With Dentinal Dysplasia
Role
alias
Name
Teeth With Thin Dentin And Large Pulp Chambers
Role
alias
Name
Teeth With Type Iii Dentinogenesis Imperfecta
Role
alias
Name
Tooth With Dentin Dysplasia Type I
Role
alias
Name
X-Linked Amelogenesis Imperfecta Hypoplastic/Hypomaturation 2
Role
alias
Cross References
Trusted external identifiers retained for this final record.
Hpo
HP:0000694HP:0000703HP:0000705HP:0003771HP:0011060HP:0011072
Herb
HBDIS000122HBDIS000746HBDIS000749HBDIS002162HBDIS004292HBDIS008830HBDIS008832HBDIS008833HBDIS008834HBDIS008835HBDIS012248HBDIS014442HBDIS016171HBDIS019299HBDIS019413HBDIS020351HBDIS020510HBDIS020515HBDIS021119HBDIS022972HBDIS023132HBDIS024429HBDIS025323HBDIS028299HBDIS028862HBDIS029010HBDIS029186HBDIS029406HBDIS029566HBDIS029871
Me Sh
D000567D003784D003805D003811D018126
Omim
104500104510104530125400125420125490125500130900204690204700301200612529613211614253614832615887616221616270
Umls
C0011430C0399368C0399376C0399379C0399380C0403549C1527284C1845052C1845053C1863012C2673922C2675858C2750771C3276551C3553830C4011403C4014578C4015557C4225394
Icd10
K00.5
Sym Map
SMDE00095SMDE00373SMDE01224SMDE02738SMDE02823SMDE02928SMDE03533SMDE03602SMDE03986SMDE04070SMDE04174SMDE04558SMDE04643SMDE04818SMDE04896SMDE04960SMDE07797
Do Class
DOID:630DOID:7
Dis Ge Net
C0002452C0011430C0011436C0028878C0205730C0206554C0399368C0399376C0399378C0399379C0399380C0868847C1527284C1845052C1845053C1863012C2673922C2675858C2750771C2931783C2973527C2981132C3276551C3553830C4011403C4014578C4015557C4023558C4049050C4225394
Orphanet
1031171836
Umls Sty
T019T047T190
Hpo Class
HP:0000152HP:0000924HP:0003549
Me Sh Class
C07C12C13C16C18
Etcm Disease
Amelogenesis ImperfectaAmelogenesis Imperfecta, Hypomaturation Type, Iia2Amelogenesis Imperfecta, Hypomaturation Type, Iia3Amelogenesis Imperfecta, Hypomaturation Type, Iia4Amelogenesis Imperfecta, Hypomaturation Type, Iia5Amelogenesis Imperfecta, Type IaAmelogenesis Imperfecta, Type IbAmelogenesis Imperfecta, Type IeAmelogenesis Imperfecta, Type IhAmelogenesis Imperfecta, Type IvAmelogenesis Imperfecta, Type ifDentin DysplasiaDentin Dysplasia, Type IDentin Dysplasia, Type IiDentinogenesis Imperfecta
Tcmbank Disease
1026111398117361196912171275159631655516815190621907191620511211952237123043254282581526879311834203594270199647
Itcmdb Generated
ITX-DISEASE-099527487659ITX-DISEASE-0D9F3B3FC7A5ITX-DISEASE-1B43D7FA8417ITX-DISEASE-1F9B71705689ITX-DISEASE-35EF9D4604D9ITX-DISEASE-507FA1329ED3ITX-DISEASE-5087B2332117ITX-DISEASE-597216CB7C90ITX-DISEASE-60C10488342FITX-DISEASE-664137B93BE3ITX-DISEASE-793379971C7AITX-DISEASE-81111D17E739ITX-DISEASE-9C954C4F591FITX-DISEASE-9E0285FBC86BITX-DISEASE-ABA9F6AA07D8ITX-DISEASE-B058B98CF486ITX-DISEASE-B847F27C1BF3ITX-DISEASE-CFBA8A24D0C6ITX-DISEASE-D5C600B051E4ITX-DISEASE-F64E821E067A
Attributes
Merged source attributes and domain-specific metadata.
Version
v1v1,v2v2
Suppress
0
Page Title
Disease Amelogenesis Imperfecta Details pageDisease Amelogenesis Imperfecta, Hypomaturation Type, Iia2 Details pageDisease Amelogenesis Imperfecta, Hypomaturation Type, Iia3 Details pageDisease Amelogenesis Imperfecta, Hypomaturation Type, Iia4 Details pageDisease Amelogenesis Imperfecta, Hypomaturation Type, Iia5 Details pageDisease Amelogenesis Imperfecta, Type Ia Details pageDisease Amelogenesis Imperfecta, Type Ib Details pageDisease Amelogenesis Imperfecta, Type Ie Details pageDisease Amelogenesis Imperfecta, Type Ih Details pageDisease Amelogenesis Imperfecta, Type Iv Details pageDisease Amelogenesis Imperfecta, Type if Details pageDisease Dentin Dysplasia Details pageDisease Dentin Dysplasia, Type I Details pageDisease Dentin Dysplasia, Type Ii Details pageDisease Dentinogenesis Imperfecta Details page
Do Class Name
disease of anatomical entitygenetic disease; disease of anatomical entity
Disease Type
disease
Hpo Class Name
Abnormality of head or neckAbnormality of head or neck; Abnormality of the skeletal system; Abnormality of connective tissue
Do Disease Class
disease of anatomical entitydisease of anatomical entity; genetic disease
Hpo Disease Class
Abnormality of head or neckAbnormality of head or neck; Abnormality of connective tissue; Abnormality of the skeletal system
Umls Disease Type
Anatomical AbnormalityCongenital AbnormalityDisease or SyndromeDisease or Syndrome; Congenital Abnormality
Basic Information
Disease Name
Amelogenesis Imperfecta
Global Category
Fetal diseases;Genetic diseases;Rare diseases
Anatomical Category
Nephrological diseases;Oral diseases
Disease Name
Amelogenesis Imperfecta, Hypomaturation Type, Iia2
Global Category
Genetic diseases
Anatomical Category
Oral diseases
Disease Name
Amelogenesis Imperfecta, Hypomaturation Type, Iia3
Global Category
Genetic diseases
Anatomical Category
Oral diseases
Disease Name
Amelogenesis Imperfecta, Hypomaturation Type, Iia4
Global Category
Genetic diseases
Anatomical Category
Oral diseases
Disease Name
Amelogenesis Imperfecta, Hypomaturation Type, Iia5
Global Category
Genetic diseases
Anatomical Category
Oral diseases
Disease Name
Amelogenesis Imperfecta, Type Ia
Global Category
Fetal diseases;Genetic diseases;Rare diseases
Anatomical Category
Nephrological diseases;Oral diseases
Disease Name
Amelogenesis Imperfecta, Type Ib
Global Category
Fetal diseases;Genetic diseases;Rare diseases
Anatomical Category
Nephrological diseases;Oral diseases
Disease Name
Amelogenesis Imperfecta, Type Ie
Global Category
Fetal diseases;Genetic diseases;Rare diseases
Anatomical Category
Nephrological diseases;Oral diseases
Disease Name
Amelogenesis Imperfecta, Type Ih
Global Category
Fetal diseases;Genetic diseases;Rare diseases
Anatomical Category
Nephrological diseases;Oral diseases
Disease Name
Amelogenesis Imperfecta, Type Iv
Global Category
Fetal diseases;Genetic diseases;Rare diseases
Anatomical Category
Nephrological diseases;Oral diseases
Disease Name
Amelogenesis Imperfecta, Type if
Global Category
Fetal diseases;Genetic diseases;Rare diseases
Anatomical Category
Nephrological diseases;Oral diseases
Disease Name
Dentin Dysplasia
Global Category
Genetic diseases;Rare diseases
Anatomical Category
Oral diseases
Disease Name
Dentin Dysplasia, Type I
Global Category
Genetic diseases;Rare diseases
Anatomical Category
Oral diseases
Disease Name
Dentin Dysplasia, Type Ii
Global Category
Genetic diseases;Rare diseases
Anatomical Category
Oral diseases
Disease Name
Dentinogenesis Imperfecta
Global Category
Genetic diseases;Rare diseases
Anatomical Category
Bone diseases;Oral diseases
Disease Definition
Amelogenesis imperfecta-nephrocalcinosis, also called enamel-renal syndrome, is an extremely rare syndrome which is characterized by hypoplastic amelogenesis imperfecta (hypoplastic dental enamel) andHPO2016_07_04:Multiple punctate calcifications in the dental pulp. [HPO:ibailleulforestier]MSH2017_2016_08_12:An apparently hereditary disorder of dentin formation, marked by a normal appearance of coronal dentin associated with pulpal obliteration, faulty root formation, and a tendency for peripheral lesions without obvious cause. (From Dorland, 27th ed)This syndrome associates gingival fibromatosis with dental abnormalities including generalized thin hypoplastic amelogenesis imperfecta, intrapulpal calcifications, and delay of tooth eruption.
Me Sh Disease Class
Female Urogenital Diseases and Pregnancy Complications; Stomatognathic Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Male Urogenital DiseasesStomatognathic DiseasesStomatognathic Diseases; Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Dis Ge Net Disease Type
disease
Disease Class Name Me Sh
Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Female Urogenital Diseases and Pregnancy Complications; Male Urogenital Diseases; Stomatognathic DiseasesCongenital, Hereditary, and Neonatal Diseases and Abnormalities; Stomatognathic DiseasesStomatognathic Diseases
Umls Semantic Type Name
Anatomical AbnormalityCongenital AbnormalityDisease or SyndromeDisease or Syndrome; Congenital Abnormality