- This is the untreated LDL-cholesterol concentration. See supporting documentation for method of calculation.
The DLCNS is a validated set of criteria based on the patients family history of premature cardiovascular disease (CVD) in their first degree relatives, their own CVD history, their untreated lipid levels and physical signs such as the presence of tendon xanthomata or arcus cornealis prior to the age of 45. The subsequent score categorizes patients by the likelihood of Familial Hypercholesterolaemia (FH) diagnosis.
| Patient Name ___________________________ DOB __________________ Date __________________ Criteria | Score | Patient Score | |
| Family history | |||
| First degree relative with known premature coronary and/or vascular disease (men aged <55 years, women aged <60 years)
OR First degree relative with known LDL-cholesterol above the 95th percentile for age and gender |
1 | ||
| First degree relative with tendinous xanthomata and/or arcus cornealis
OR Children aged <18 years with LDL-cholesterol above the 95th percentile for age and gender |
2 | ||
| Clinical history | |||
| Patients with premature coronary artery disease (men aged <55 years, women aged <60 years) | 2 | ||
| Patients with premature cerebral or peripheral vascular disease (men aged <55 years, women aged <60 years) | 1 | ||
| Physical examination | |||
| Tendinous xanthomata | 6 | ||
| Arcus cornealis before 45 years of age | 4 | ||
| Investigation | |||
| LDL-cholesterol (mmol/L) | LDL-C ≥8.5 | 8 | |
| LDL-C 6.5–8.4 | 5 | ||
| LDL-C 5.0–6.4 | 3 | ||
| LDL-C 4.0–4.9 | 1 | ||
| Definite FH | >8 | ||
| Probable FH | 6-8 | ||
| Possible FH | 3-5 | ||
| Unlikely FH | <3 | ||
Table 4. Dutch Lipid Clinic Network diagnostic criteria for Familial Hypercholesterolemia1-3
| Points | |
| CriteriaFamily history | |
| First-degree relative with known premature* coronary and vascular disease, OR First-degree relative with known LDL-C level above the 95th percentile | 1 |
| First-degree relative with tendinous xanthomata and/or arcus cornealis, OR Children aged less than 18 years with LDL-C level above the 95th percentile | 2 |
| Clinical history | |
| Patient with premature* coronary artery disease | 2 |
| Patient with premature* cerebral or peripheral vascular disease | 1 |
| Physical examination | |
| Tendinous xanthomata | 6 |
| Arcus cornealis prior to age 45 years | 4 |
| Cholesterol levels mg/dl (mmol/liter) | |
| LDL-C >= 330 mg/dL ( ≥8.5) | 8 |
| LDL-C 250 – 329 mg/dL (6.5–8.4) | 5 |
| LDL-C 190 – 249 mg/dL (5.0–6.4) | 3 |
| LDL-C 155 – 189 mg/dL (4.0–4.9) | 1 |
| DNA analysis | |
| Functional mutation in the LDLR, apo B or PCSK9 gene | 8 |
| Diagnosis (diagnosis is based on the total number of points obtained) | |
| Definite Familial Hypercholesterolemia | >8 |
| Probable Familial Hypercholesterolemia | 6 – 8 |
| Possible Familial Hypercholesterolemia | 3 – 5 |
| Unlikely Familial Hypercholesterolemia | <3 |
* Premature = < 55 years in men; < 60 years in women LDL-C = low density lipoprotein cholesterol; FH, familial hypercholesterolemia. LDLR = low density lipoprotein receptor Apo B = apolipoprotein B PCSK9 = Proprotein convertase subtilisin/kexin type 9
1Austin MA, Hutter CM, Zimmern RL, Humphries SE. Genetic causes of monogenic heterozygous familial hypercholesterolemia: a HuGE prevalence review. American journal of epidemiology. 2004;160:407-420.
2Haase A, Goldberg AC. Identification of people with heterozygous familial hypercholesterolemia. Current opinion in lipidology. 2012;23:282-289.
3Nordestgaard BG, Chapman MJ, Humphries SE, et al. Familial hypercholesterolaemia is underdiagnosed and undertreated in the general population: guidance for clinicians to prevent coronary heart disease: consensus statement of the European Atherosclerosis Society. European heart journal. 2013;34:3478-3490a.