Acute digestive system disorder	Acute metabolic disorder	Autosomal recessive hereditary disorder	Digestive system hereditary disorder	Familial hypertriglyceridemia	Hereditary metabolic disease	Steatosis of liver
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Transient infantile hypertriglyceridemia and hepatosteatosis (disorder)
	Transient infantile hypertriglyceridemia and hepatosteatosis
	Transient infantile hypertriglyceridemia and fatty liver
	A rare genetic hepatic disease characterized by massive hepatomegaly, moderate to severe transient hypertriglyceridemia and hepatic steatosis (followed by fibrosis) manifesting in infancy with failure to thrive, vomiting, an enlarged abdomen and a fatty liver. Reduction or normalization of triglyceride serum levels occurs with advancing age. Caused by homozygous or compound heterozygous mutation in the GPD1 gene on chromosome 12q13.
	passagère infantiele hypertriglyceridemie en hepatosteatose
	transiënte infantiele hypertriglyceridemie en hepatosteatose voorbijgaande infantiele hypertriglyceridemie en hepatosteatose

Id	773649005
Status	Primitive

Associated morphology	Fatty degeneration
Finding site	Liver structure
Occurrence	Infancy

Has interpretation	Above reference range
Interprets	Lipids measurement

Clinical course

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	P74.8
Rule	TRUE
Advice	ALWAYS P74.8
Correlation	SNOMED CT source code to target map code correlation not specified

Target	K76.0
Rule	TRUE
Advice	ALWAYS K76.0
Correlation	SNOMED CT source code to target map code correlation not specified