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deficiëntie van ubiquitine-specifiek peptidase 18 (aandoening)
USP18-deficiëntie
deficiëntie van ubiquitine-specifiek peptidase 18
USP18 deficiency
Ubiquitin specific peptidase 18 deficiency
A rare genetic neurological disorder characterized by severe pseudo-TORCH syndrome with signs of brain damage and occasionally systemic manifestations resembling the sequelae of congenital infection, but in the absence of an infectious agent. Characteristic features include microcephaly, white matter disease, cerebral atrophy, cerebral hemorrhage and calcifications among others. Affected individuals typically have seizures and respiratory insufficiency and die in infancy.
Id1251449006
StatusPrimitive
Has interpretationonder referentiebereik
Interpretshoofdomtrek
Associated morphologydegeneratieve afwijking
Finding sitestructuur van encephalon
Associated morphologyinflammatoire morfologie
Finding sitestructuur van immuunsysteem
Pathological processafwijkend immuunproces
SNOMED CT to Orphanet simple map481665
SNOMED CT to ICD-10 extended map
TargetQ02
RuleTRUE
AdviceALWAYS Q02 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified