The Egyptian Heart Journal

Table 3 Different types of familial HTG [19,20,21,22]

From: Egyptian practical guidance in hypertriglyceridemia management 2021

	FCH (Type IIB)	FCS (Type I)	MFCS (Type IV)	FHTG (Type V)	FD (Type III)
Lipoprotein change	↑VLDL, LDL	↑Chylomicrons	↑VLDL	↑VLDL, chylomicrons	↑IDL
Lipid change	↑TG, TC	↑TG	↑TG	↑TG, TC	↑TG, TC
Incidence	1/100–200	1/500,000–1,000,000	1/600–1000	1/500	1/10,000
Genetics	Polygenic (TG, LDL raising alleles)	Monogenic homozygous (autosomal recessive)^a	Monogenic heterozygous (autosomal dominant)^a Polygenic	Monogenic heterozygous (autosomal dominant)^a Polygenic	Monogenic homozygous (defect in APOE gene)
Time of presentation	All in adulthood (earlier with secondary causes) except for FCS (type I) in childhood
Specific for diagnosis	Combination of: ApoB > 120 mg/dL TGs > 133 mg/dL FH of premature CVD	TG > 885 mg/dl Creamy appearance of the blood Failure to thrive, Recurrent abdominal pain, nausea, vomiting Acute pancreatitis (60–80% lifetime risk) Tuberous xanthoma Lipemia retinalis Hepatosplenomegaly	TG 150–885 mg/dl or > 885 mg/dl with secondary insult Responsive to standard therapy Require an aggravating effect	TG 150–885 mg/dl Require an aggravating effect Lower risk of pancreatitis	Palmer crease xanthomas are pathognomonic

FCH familial combined hyperlipidemia, FCS familial chylomicronemia syndrome, FD familial dysbetalipoproteinaemia, FHTG familial HTG, GPIHBP1 glycosylphosphatidylinositol-anchored high-density lipoprotein binding protein-1, MFCS multifactorial chylomicronemia syndrome, LDL low density lipoprotein, LMF1 Lipase maturation factor 1, LPL lipoprotein lipase, TC total cholesterol, TG TG, VLDL very low-density lipoprotein, FH family history
^aDefect in LPL, APOC2, APOA5, GPIHBP1 or LMF1 genes

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