Familial Hypercholesterolemia

NLA Familial Hypercholesterolemia

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Treatment Options During Pregnancy ÎStatins, ezetimibe, and niacin should NOT be used during pregnancy. Use of other lipid lowering medications (e.g., colesevelam) may be considered under the guidance of the healthcare practitioner. ÎConsider LDL apheresis during pregnancy if there is significant atherosclerotic disease or if the patient has homozygous FH. Hard to Manage Patients ÎIf other treatment options are inadequate or the FH patient cannot tolerate pharmacotherapy or LDL apheresis, other treatment options include ileal bypass and liver transplantation (both are used rarely), and, potentially, new drugs in development. Children ÎPrimary care clinicians should be responsible for screening and diagnosis. ÎHomozygous FH should always be managed by a lipid specialist. Screening ÎUniversal screening at age 9 to 11 years with a fasting lipid profile or nonfasting non–HDL-C measurement is recommended to identify all children with FH. Comment: This age identifies individuals at the potential onset of advanced atherosclerosis, and provides the best discrimination between those with and without inherited dyslipidemias by avoiding confounding due to changes in lipid levels associated with puberty. ÎIf a nonfasting non–HDL-C concentration of ≥ 145 mg/dL is detected, then a fasting lipid profile should be performed. ÎScreening should occur earlier (≥ 2 years of age) in the presence of a positive family history for hypercholesterolemia or premature CHD or the presence of other major CHD risk factors. ÎEvaluation (history, physical examination, selected laboratory tests) of possible secondary causes of dyslipidemia should be performed. • Secondary causes include hypothyroidism, nephrotic syndrome, and liver disease.

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