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SNOMED CT
Id | http://purl.bioontology.org/ontology/SNOMEDCT/722763000
http://purl.bioontology.org/ontology/SNOMEDCT/722763000
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Preferred Name | Dopamine transporter deficiency syndrome |
Definitions |
An extremely rare inherited neurological syndrome that presents in early infancy with hypokinetic parkinsonism and dystonia and that can be fatal. The prevalence is unknown. The disease presents soon after birth with irritability and feeding difficulties, followed by progressive parkinsonism, dystonia, axial hypotonia, limb hypertonicity and pyramidal tract signs. Clinically it can resemble cerebral palsy. Caused by mutations in the SLC6A3 gene (5p15.33), which encodes a human dopamine transporter mediating the active reuptake of extracelluar dopamine. Mutations in this gene lead to a reduction in the level of mature dopamine transporter and therefore an impairment in dopaminergic neurotransmission. Inherited in an autosomal recessive manner.
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Synonyms |
Infantile dystonia parkinsonism (disorder)
Parkinsonism-dystonia infantile
Infantile dystonia parkinsonism
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Type | http://www.w3.org/2002/07/owl#Class |
All Properties
definition | An extremely rare inherited neurological syndrome that presents in early infancy with hypokinetic parkinsonism and dystonia and that can be fatal. The prevalence is unknown. The disease presents soon after birth with irritability and feeding difficulties, followed by progressive parkinsonism, dystonia, axial hypotonia, limb hypertonicity and pyramidal tract signs. Clinically it can resemble cerebral palsy. Caused by mutations in the SLC6A3 gene (5p15.33), which encodes a human dopamine transporter mediating the active reuptake of extracelluar dopamine. Mutations in this gene lead to a reduction in the level of mature dopamine transporter and therefore an impairment in dopaminergic neurotransmission. Inherited in an autosomal recessive manner. |
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altLabel |
Infantile dystonia parkinsonism (disorder)
Parkinsonism-dystonia infantile
Infantile dystonia parkinsonism
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prefLabel | Dopamine transporter deficiency syndrome
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Type ID |
900000000000003001
900000000000013009
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CASE SIGNIFICANCE ID | 900000000000448009
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notation | 722763000
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Effective time | 20170131
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Active | 1
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Has finding site | |
interprets | |
subClassOf | |
Semantic type UMLS property | |
Has interpretation | |
type | |
Subset member | 447562003~MAPRULE~TRUE
900000000000497000~MAPTARGET~XUuri
6011000124106~MAPADVICE~ALWAYS G20 | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE
6011000124106~MAPTARGET~G20.C
6011000124106~MAPGROUP~1
6011000124106~MAPGROUP~2
6011000124106~MAPTARGET~G24.9
900000000000508004~ACCEPTABILITYID~900000000000549004
447562003~MAPGROUP~1
447562003~CORRELATIONID~447561005
6011000124106~MAPADVICE~ALWAYS G20.C | CONSIDER ADDITIONAL CODE TO IDENTIFY SPECIFIC CONDITION OR DISEASE
6011000124106~MAPCATEGORYID~447637006
900000000000509007~ACCEPTABILITYID~900000000000548007
900000000000508004~ACCEPTABILITYID~900000000000548007
447562003~MAPPRIORITY~1
447562003~MAPCATEGORYID~447637006
6011000124106~MAPADVICE~ALWAYS G24.9
447562003~MAPTARGET~G24.8
6011000124106~MAPPRIORITY~1
6011000124106~MAPTARGET~G20
6011000124106~CORRELATIONID~447561005
6011000124106~MAPRULE~TRUE
900000000000509007~ACCEPTABILITYID~900000000000549004
447562003~MAPADVICE~ALWAYS G24.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
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DEFINITION STATUS ID | 900000000000074008
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tui | T047
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CTV3ID | XUuri
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Occurs in | |
cui | C2751067
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