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Clinical Practice Guidelines

Hypercholesterolaemia

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  • Key points

    1. Hypercholesterolaemia in children may be inherited, secondary, or lifestyle-related
    2. Familial hypercholesterolaemia (FH) is the most common inherited cause and should be suspected when low density lipoprotein cholesterol (LDL-C) is >4 mmol/L, or in the presence of a strong family history
    3. Early identification and treatment, including with statins, improves long-term cardiovascular outcomes
    4. Very high LDL-C (>13 mmol/L) or early xanthomas suggest homozygous FH and requires urgent lipid specialist consultation

    Background

    • Hypercholesterolaemia refers to elevated LDL-C above age and sex specific norms
    • For practical purposes, LDL-C ≥3.5 mmol/L in childhood is considered elevated
    • Secondary causes include thyroid, renal and liver disease, diabetes, medications and lifestyle-related
    • Primary/inherited high LDL-cholesterol is most commonly due to familial hypercholesterolaemia (FH)
    • FH is an autosomal dominant condition leading to lifelong elevation of LDL-C with a prevalence of approx. 1 in 250 children
    • Early detection is essential, as atherosclerosis begins in childhood
    • There is currently no universal screening for FH, but if other blood tests are being done in adolescence, a one-off lipid profile is a reasonable addition

    Assessment

    History

    • Raised LDL-C on screening or incidental finding on routine testing
    • Family history of premature coronary artery disease (males <55 years, females <65 years), hypercholesterolaemia or known FH
    • Symptoms or signs of secondary causes
      • Hypothyroidism, diabetes, liver disease, nephrotic syndrome
    • Medication use, eg steroids, atypical antipsychotics, some antiepileptics
    • Obesity or metabolic syndrome
    • Diet high in saturated fats

    Examination

    • Typically normal
    • Co-morbid obesity and hypertension can occur. Plot BMI and blood pressure in all children
    • Look specifically for tendon xanthomas, xanthelasma, corneal arcus (rare in children)

    Xanthelasma and xanthoma_crop

    Red flags - consider homozygous FH

    • LDL-C >13 mmol/L
    • Parental consanguinity with elevated LDL-C
    • Tendon xanthomas

    Management

    CPG hypercholesterolaemia flowchart

    Specialist services vary in each state/territory. This may be a specialist lipid service, cardiology, endocrinology, metabolic or general paediatric service. Local specialist services may be found here FH Australia find a clinician

    Investigations

    • Confirm fasting lipid profile. Repeat if initial sample non-fasting or unexpected
    • Screen for secondary causes
      • FBE, UEC, LFTs, TFTs, HbA1C
      • Urinalysis +/- Alb:Cr ratio
    • If FH is suspected
      • Consider genetic testing if criteria met (see MBS Item 73352)
      • Perform extended biochemical profile: baseline CK, Lp(a)
      • Advise screening of all first-degree relatives

    Treatment

    General management for all children with elevated cholesterol

    • Heart-healthy diet (reduced saturated fats, include plant sterols)
    • Regular physical activity
    • Avoid smoking, vaping and alcohol
    • Optimise control of comorbidities (diabetes, obesity, thyroid, renal, hepatic)

    Statin therapy

    • Recommended from age 8 years (or earlier, if high risk) if LDL-C remains above age-specific target despite lifestyle changes for up to 6 months
    • Target LDL-C
      • <4.0 mmol/L (8-10 years)
      • <3.5 mmol/L (>10 years)
    • Common agents in table below.
    Statin Usual starting dose Considerations
    Rosuvastatin 5 mg daily
    Titrate to max 20 mg    		 Start low dose
    Dose adjust in renal impairment
    Usually first choice
    Atorvastatin 10 mg daily
    Titrate to max 40 mg    		 Start low dose
    Increased side effects at higher doses
    Pravastatin 10-20 mg nocte (improved efficacy if taken nocte)
    Titrate to max 40mg    		 Less potent LDL-C reduction
    Myalgia less common; may be useful if myalgia occurred with other statin
    • Repeat lipids, LFTs, CK at 6-12 weeks after initiation or dose change
    • Side effects (generally rare): include myalgia, headache and gastrointestinal upset which are usually self-limiting
    • Rarer side effects include myopathy, rhabdomyolysis and severe hepatotoxicity, at which time prompt cessation is advised
    • Consider ezetimibe if statin alone inadequate
    • Effective contraception should be advised for adolescents starting a statin

    Consider consultation with local paediatric team when

    • Diagnosis of FH suspected
    • Uncertainty around initiation of statin therapy

    Consultation with specialist lipid team when

    Urgent

    • Features suggestive of homozygous FH. See Red flags
    • Multidisciplinary management required, eg genetics, cardiology, apheresis

    Routine

    • Further specialist input required
    • LDL-C remains above target despite treatment
    • Further input required regarding genetic testing or cascade screening

    For emergency advice and paediatric or neonatal ICU transfers, see Retrieval Services

    Parent information

    Additional notes

    Image sources*

    Last updated June 2026