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C11ORF73-gerelateerde autosomaal recessieve hypomyeliniserende leukodystrofie (aandoening)
C11ORF73-gerelateerde autosomaal recessieve hypomyeliniserende leukodystrofie
hypomyeliniserende leukodystrofie door HIKESHI-deficiƫntie
C11ORF73-gerelateerde autosomaal recessieve hypomyeliniserende leuko-encefalopathie
C11ORF73-related autosomal recessive hypomyelinating leukodystrophy
Hypomyelinating leukodystrophy due to HIKESHI deficiency
C11ORF73-related autosomal recessive hypomyelinating leukoencephalopathy
A rare leukodystrophy characterized by infantile onset of lower limb spasticity and severe developmental delay associated with delayed myelination and periventricular white matter abnormalities. Other reported signs and symptoms include microcephaly, optic atrophy, nystagmus, ataxia, or seizures.
Id1172595004
StatusPrimitive
SNOMED CT to Orphanet simple map495844
SNOMED CT to ICD-10 extended map
TargetG93.8
RuleTRUE
AdviceALWAYS G93.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified