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Non-Wilsonian hepatic copper toxicosis of infancy and childhood (disorder)
Idiopathic copper associated cirrhosis of liver
Non-Wilsonian hepatic copper toxicosis of infancy and childhood
A rare copper-overload liver disease with rapidly progressive liver cirrhosis from the first few years of life leading to hepatic insufficiency. Specific pathological aspects; pericellular fibrosis, inflammatory infiltration, hepatocyte necrosis, absence of steatosis, poor regeneration and histochemical copper staining.
niet-Wilsoniaanse hepatische kopertoxicose op zuigelingenleeftijd en kinderleeftijd
idiopathische kopergeassocieerde levercirrose
niet-Wilsoniaanse hepatische kopertoxicose bij zuigeling en kind
Id715864007
StatusPrimitive
Associated morphologyChronic fibrosis
Finding siteLiver structure
OccurrenceInfancy
OccurrenceChildhood
Clinical courseProgressive
Associated morphologyNodular regeneration
Finding siteLiver structure
DHD Diagnosis thesaurus reference set
ICD-10 complex map reference set
TargetK74.6
RuleTRUE
AdviceALWAYS K74.6
CorrelationSNOMED CT source code to target map code correlation not specified
TargetE83.0
RuleTRUE
AdviceALWAYS E83.0
CorrelationSNOMED CT source code to target map code correlation not specified