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primaire cardiomyovasculopathie met depositie van triglyceriden (aandoening)
primaire cardiomyovasculopathie met depositie van triglyceriden
primaire cardiomyovasculopathie met afzetting van triacylglycerol
Primary triglyceride deposit cardiomyovasculopathy
Neutral lipid storage disease with severe cardiovascular involvement
A rare inborn error of metabolism with characteristics of massive accumulation of triglycerides in the myocardium and coronary arteries while plasma triglyceride levels are normal. Patients present in adulthood with signs and symptoms of coronary artery disease and severe heart failure. Concomitant skeletal myopathy is common. Vacuole formation in polymorphonuclear leukocytes is typically observed.
Id1279844009
StatusPrimitive
Occurrencecongenitaal
Associated morphologyvetafzetting
Finding sitestructuur van coronairarterie
Associated morphologyvetafzetting
Finding sitestructuur van myocardium
SNOMED CT to ICD-10 extended map
TargetE75.5
RuleTRUE
AdviceALWAYS E75.5 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified