Table 1: Summary of Guidelines on the Management of Familial Hyperlipidaemia.

Guidelines	Recommendation	Therapy	Additional Comments
NLA [9,10]	Adults: pharmacologic therapy to reduce baseline LDL-C by > 50 % with maximum statin dose tolerated. Children/Young Adults: Consider initiation of medical therapy in FH pts >8 years old.	Lifestyle modification. Statin therapy should be initial drug of choice. If unable to tolerate initial statin, consider alternate statin or alternate day dosing. If statin contraindicated or poorly tolerated, consider addition of ezetimibe, bile acid sequestrants or niacin. Additional options: LDL apheresis.	Adults: Reduce LDL-C by >50 %. Higher risk patients may require more aggressive therapeutic goals (LDL<100 mg/dl). Children: Treatment goal is >50 % reduction in LDL-C or LDL-C <130 mg/dl.
NICE[11]	Children/Young Adults: Cholesterol lowering drugs should be considered in FH patients by age 10. Lipid lowering drugs may be considered before the age of 10 when there is a family history of CHD in early adulthood.	Lifestyle modification. Treatment with high-intensity statin to reduce LDL-C by 50 % should be first line medical therapy. Ezetimibe is recommended as monotherapy for patients intolerant of statins. Ezetimibe can be used as adjunctive therapy in heterozygous FH patients on statins with suboptimal TC or LDL-C or when the statin dose cannot be further titrated due to side effects. Additional options: LDL apheresis.	Adult FH patients at very high risk of coronary heart disease, or with intolerance or contraindication to statins or ezetimibe, should be referred to a FH specialist. Children and adolescents diagnosed with, or undergoing evaluation for, FH should be referred to a specialist in FH. No published studies to establish target LDL-C target levels in children with FH on lipid lowering therapy.
EAS[4,12]	Target LDL-C: Children: <135 mg/dl Adults: <100 mg/dl Adults with CHD or Diabetes: <70 mg/dl	Lifestyle modification. Lipid lowering therapy should be started between ages 8 and 10. - no safety data on the use of statins before age 8–10. Ezetimibe should be added as a second line agent if LDL is still above target. Bile acid resins are third-line agents.	Children: statins, ezetimibe, bile acid resins and LDL apheresis in homozygous FH.[4] Adults: maximal potency statins, ezetimibe, bile acid resins, fibrates (specifically fenofibrate), LDL apheresis in homozygous FH and treatment resistant heterozygous FH.[4]
ACC/AHA[13]	Recommendation to treat to a target LDL-C level was abandoned due to lack of randomised controlled trials demonstrating that treatment to a specific LDL level improved CVD outcomes. No specific recommendations for FH patients, but recent guidelines addresses patients with LDL-C >190	Lifestyle modification. High intensity statin is recommended Addition of nonstatin drugs may be considered.	A reasonable approach would be to reduce LDL-C by > 50 percent. High potency statins include atorvastatin 40 to 80 mg and rosuvastatin 20 to 40 mg.

NLA = National Lipid Association; NICE = National Institute for Health and Care Excellence; EAS = European Atherosclerosis Society; ACC/AHA = American College of Cardiology/American Heart Association; TC = Total cholesterol.