Table 3

Points scoring system for familial chylomicronaemia syndrome79 80

ParameterPoints
Fasting TG >10 mmol/L (at least 3 times; measured 1 month apart)+5
Fasting TG >20 mmol/L (at least once)+1
Previous TG <2 mmol/L−5
No secondary factors (except pregnancy or oestrogen therapy)
Exclude alcohol, diabetes, metabolic syndrome, hypothyroidism, corticosteroids and additional drugs
+2
History of pancreatitis+1
Unexplained recurrent abdominal pain+1
No history of FCHL+1
TG response to drug therapy <20%+1
Age of onset
<10 years+3
<20 years+2
<40 years+1
  • FCS likely >10 pts; FCS unlikely <9 pts; FCS very unlikely <8 pts.

  • AUROC 0.91; Sensitivity 88%; Specificity 85%.

  • FCHL, familial combined hyperlipidaemia; FCS, familial chylomicronaemia syndrome; TG, triglycerides.