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multipele-mitochondriale-disfuncties-syndroom type 2 (aandoening)
multipele-mitochondriale-disfuncties-syndroom type 2
MMDS2
BOLA3-deficiƫntie
Multiple mitochondrial dysfunctions syndrome type 2
BOLA3 (bolA family member 3) deficiency
MMDS2 - multiple mitochondrial dysfunctions syndrome type 2
A rare mitochondrial disease characterized by infantile onset of severe regression after a period of normal development, epileptic encephalopathy, hypotonia, movement disorder, cardiomyopathy, hyperglycinemia, and lactic acidosis. Optic atrophy may also be present. Brain imaging findings are highly variable and include white matter abnormalities. The disease is typically fatal in infancy.
Id1208486005
StatusPrimitive
Occurrencecongenitaal
SNOMED CT to Orphanet simple map401874
SNOMED CT to ICD-10 extended map
TargetE88.8
RuleTRUE
AdviceALWAYS E88.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified