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MS

Enzyme or protein defect

LDL receptor (or apoB-100, PCSK9 gain-of-function)

Accumulates or becomes deficient

LDL cholesterol

Key features

Tendon xanthomas, xanthelasma, corneal arcus, premature coronary disease

Management

High-intensity statin, ezetimibe, PCSK9 inhibitor

Not clinical guidance. Written from standard clinical biochemistry, not from the uploaded documents, and marked unreviewed. Teaching-level reference for study and database seeding only. It must not be used to manage a patient.

Recent literature

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