DLCN_score v.1
The Dutch Lipid Clinic Network (DLCN) criteria are a set of 10 questions recommended by the European Atherosclerosis Society (EAS) for aiding the diagnosis of heterozygous Familial Hypercholesterolaemia (FH) in adults. The questions fall into 5 groups: family history (first degree relative with premature coronary heart disease, tendon xanthoma, corneal arcus, or LDL cholesterol >95th percentile); clinical history (premature coronary heart disease, or premature cerebral or peripheral vascular disease); physical examination (tendon xanthoma, or corneal arcus); biochemical results (LDL cholesterol); and molecular genetic testing (DNA analysis). Presence of each criterion carries a specific number of points, but only the highest scoring criterion present is selected from each group to contribute to the final DLCN score; mimimum score = 0 and maximum score = 26. DLCN score >8 is ‘definite FH’, score 6 - 8 is ‘probable FH', score 3 - 5 is ‘possible FH’ and score 0 - 2 is ‘unlikely FH’.
To support the diagnosis of heterozygous Familial Hypercholesterolaemia (FH) in adults.
To calculate DLCN score for diagnosing FH. The 10 DLCN criteria are point-based and fall into 5 groups: 1. family history - first-degree relative with known premature (<55 years, men; <60 years, women) coronary heart disease --> 1 point - first-degree relative with known LDL cholesterol >95th percentile by age and gender for country --> 1 point - first-degree relative with tendon xanthoma and/or corneal arcus --> 2 points - child(ren) <18 years with LDL cholesterol >95th percentile by age and gender for country --> 2 points 2. clinical history - individual has premature (<55 years, men; <60 years, women) coronary heart disease --> 2 points - individual has premature (<55 years, men; <60 years, women) cerebral or peripheral vascular disease --> 1 point 3. physical examination - finding of tendon xanthoma --> 6 points - finding of corneal arcus in a person <45 years --> 4 points 4. biochemical results ('untreated' LDL cholesterol) - >8.5 mmol/L (>325 mg/dL) --> 8 points - 6.5–8.4 mmol/L (251–325 mg/dL) --> 5 points - 5.0–6.4 mmol/L (191–250 mg/dL) --> 3 points - 4.0–4.9 mmol/L (155–190 mg/dL) --> 1 point 5. molecular genetic testing (DNA analysis) - causative mutation shown in the LDLR, APOB, or PCSK9 genes --> 8 points Only the highest scoring criterion present is selected from each group to contribute to the final DLCN score; mimimum score = 0 and maximum score = 26. DLCN score >8 is ‘definite FH’, score 6 - 8 is ‘probable FH', score 3 - 5 is ‘possible FH’ and score 0 - 2 is ‘unlikely FH’. DLCN score is assessed by a separate application: DLCN_score_Assessment.v1 * LDL cholesterol criteria are based on untreated or pre-treatment testing. If LDL cholesterol is taken after the individual has started using lipid-lowering therapy, a correction factor (see references) must be applied.
Nordestgaard BG, Chapman MJ, Humphries SE, Ginsberg HN, Masana L, Descamps OS, Wiklund O, Hegele RA, Raal FJ, Defesche JC, Wiegman A. Familial hypercholesterolaemia is underdiagnosed and undertreated in the general population: guidance for clinicians to prevent coronary heart disease. European heart journal. 2013 Dec 1;34(45):3478-90. Law MR, Wald NJ, Rudnicka AR. Quantifying effect of statins on low density lipoprotein cholesterol, ischaemic heart disease, and stroke: systematic review and meta-analysis. Bmj. 2003 Jun 26;326(7404):1423. https://www.fhscore.eu/#/about#section3
OBSERVATION.lab_test-lipids.v1, OBSERVATION.basic_demographic.v1, OBSERVATION.history_prior_medical_diagnosis.v1, OBSERVATION.dutch_lipid_clinic_network_score.v1, EVALUATION.dutch_lipid_clinic_network_score.v1
