Table 1 DLCN Diagnostic Criteria for FH

Group 1: Family History

Points

     i. First-degree relative with premature CHD^a

1

     ii. First-degree relative with LDL-C > 95th percentile by age, gender for country

1

     iii. First-degree relative with tendinous xanthomata and/or arcus cornealis

2

     iv. Children under 18 years with LDL-C > 95^th percentile by age, gender for country

2

Group 2: Clinical History

Points

     i. Premature CHD

2

     ii. Premature cerebrovascular or peripheral vascular disease

1

Group 3: Physical Examination Points

     i. Tendinous xanthomata

6

     ii. Arcus cornealis prior to 45 years

4

Group 4: LDL-C Levels

Points

     i. LDL-C > 8.5 mmol/l (~330 mg/dl)

8

     ii. LDL-C 6.5-8.4 mmol/l (~250-329 mg/dl)

5

     iii. LDL-C 5.0-6.4 mmol/l (~190-249 mg/dl)

3

     iv. LDL-C 4.0-4.9 mmol/l (~155-189 mg/dl)

1

Group 5: DNA Analysis Points

     i. Causative mutation in the LDLR, ApoB or PCSK9 gene

8