25096_Keeping Hearts Healthy - Preventive Treatment in Familial Hypercholesterolemia Evaluation- Self Study

Question 1

The inheritance pattern of familial hypercholesterolemia is:

Autosomal recessive with only those that are homozygous exhibiting this metabolic disease

Autosomal dominant, with all equally affected

Autosomal dominant, with those who are homozygous having the most severe disease

X-linked dominant, passed on to male and female offspring.

Question 2

The major lines of evidence for childhood risk factors and adult cardiovascular disease include:

There is evidence of atherosclerosis in children and young adults.

This arteriosclerosis is related to identifiable risk factors

there's evidence of end organ damage in children with identifiable cardiovascular disease risk factors including children with familial hypercholesterolemia

Risk factors track into adulthood

Untreated patients with familial hypercholesterolemia have marked increase in cardiovascular disease early in life and treatment with statins and childhood appears to lower risk of adult cardiovascular disease

All of the above

Question 3

A 10-year-old patient you have been following for several years has had severely elevated LDL-C levels despite lifestyle changes. He has had the following LDL-C levels over the past several months and serial draws: 195 mg/dL, 200 mg/dL, and 192 mg/dL. His father had similar cholesterol levels in childhood and is on statin medications. According to guidelines, what is an appropriate next diagnostic step?

Obtain an echocardiogram

Continue with lifestyle changes and re-check cholesterol in 1 year

Refer to genetics for testing.

Obtain CXR

Initiate high-dose statin treatment.

Question 4

Statins are one particular treatment for dyslipidemias. Their primary action is to: (select all that apply):

Act as a cholesterol absorption inhibitor in the intestine.

Decrease intrahepatic cholesterol by increasing uptake of circulating LDL

Work by binding to bile acids in the intestine, preventing their reabsorption and leading to their excretion in the feces

Increase uptake of circulating VLDL by LDL-R

Decrease intrahepatic VLDL synthesis

Question 5

Which statin can be given as young as 7 years of age if there is a history of homozygous familial hypercholesterolemia?

Rosuvastin

Lovastatin

Atorvastatin

Sivastatin

Question 6

Following statin initiation, when should you check fasting lipid panel, ALT and AST and what is the appropriate plan?

4 weeks and if target LDL not achieved, discontinue

8 weeks and continue therapy as started as long as AST and ALT are stable

4 weeks and if target LDL not achieved, can increase statin dose by one increment.

12 weeks and if target LDL not achieved, can increase statin dose by one increment.

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25096_Keeping Hearts Healthy - Preventive Treatment in Familial Hypercholesterolemia Evaluation- Self Study

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25096_Keeping Hearts Healthy - Preventive Treatment in Familial Hypercholesterolemia Evaluation- Self Study