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Hyperlipidemia: Practice Gaps
Hyperlipidemia: Practice Gaps
Jeremy Stewart, MD,* Tracy McCallin, MD,* Julian Martinez,† Sheebu Chacko, MD,‡ Shabana Yusuf, MD, MEd‡
*Section of Emergency Medicine, Department of Pediatrics, Baylor College of Medicine, The Children’s Hospital of San Antonio, San Antonio, TX
†
Office of Student Affairs, Baylor College of Medicine, Houston, TX
‡
Section of Emergency Medicine, Department of Pediatrics, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX
Practice Gaps
1. The US Preventive Services Task Force recently gave screening an “I,”
which is insufficient evidence for recommending universal screening
for hyperlipidemia in children.
2. Pediatricians need guidance on causes, screening, and treatment
options in the era of a childhood obesity epidemic.
3. Pediatricians need to be aware of the latest recommendation on
screening tests, treatment, prevention, and dietary counseling.
LDL
hypercholesterolemia
low-density lipoprotein
Abstract
LDL-C low-density lipoprotein
Cardiovascular disease remains the top cause of morbidity and mortality
cholesterol
LPL lipoprotein lipase
in the United States. Atherosclerotic plaques are known to start in
NHLBI National Heart, Lung, and Blood adolescence, and, therefore, young adults can be affected by coronary
Institute artery disease. Children with known risk factors, such as genetic
TG triglyceride
predisposition, including familial hyperlipidemias, diabetes, and renal
USPSTF United States Preventive Services
Task Force diseases, are at higher risk. With childhood obesity becoming an epidemic
VLDL very low-density lipoprotein in certain parts of the United States, this problem is further highlighted as
VLDL-C very low-density lipoprotein an important issue affecting children’s health. There are unclear
cholesterol
TABLE 1. Lipid Cutoff Levels for Pediatric Patients Established by the 2011
Expert Panel Integrated Guidelines for Cardiovascular Health
and Risk Reduction in Children and Adolescents
CUTOFF LEVEL
CATEGORY, mg/dL (mmol/L)
LIPID ACCEPTABLE BORDERLINE HIGH/LOW
Heterozygous familial 1 in 200 to 1 in 500 Asymptomatic Diet and exercise for 6 mo, add statins
hypercholesterolemia
Homozygous familial 1 in 160,000 Xanthomas Diet, exercise and statins, bile acids,
hypercholesterolemia cholesterol absorption inhibitors
Primary hypertriglyceridemia <0.2% Identified by pancreatitis Fibrates
Nicotinic acid
Plasmapheresis
Secondary hypertriglyceridemia 10.7% Other secondary diagnosis, Fibrates
such as diabetes, renal dysfunction Nicotinic acid
Omega fatty acids
Familial combined hyperlipidemia 0.5%–1% May be obese Diet, exercise, statins
Finally, dysbetalipoproteinemia is a disorder with a has previously been recommended by the American Academy
homozygous mutation in apoE that binds chylomicrons, of Pediatrics (AAP) based on recommendations from the
intermediate-density lipoprotein, and VLDL to the surface of National Heart, Lung, and Blood Institute (NHLBI). Some
hepatocytes. This leads to increased levels of chylomicron would recommend performing these screenings during health
and VLDL remnants as well as an increase in TG and total supervision visits when children receive their immunizations
cholesterol levels. Palmar crease xanthomas have been at regular intervals. (23) However, the US Preventive Services
shown to be pathognomonic in the pediatric population. Task Force (USPSTF) in 2016 reported an “I” recommendation
Although LDL-C levels and apoB levels are low, patients with (not for or against universal screening) and concluded that
dysbetalipoproteinemia have an elevated risk of CVD; ele- there was insufficient evidence to make recommendations
vated levels of b-VLDL have a powerful action on monocyte- regarding benefits and harms of cholesterol screening in
macrophage cells and rapidly transform these cells into childhood. (20)(21) The USPSTF does recommend screening
foam cells activating the atherosclerosis pathway. (17) Com- for obesity at 6 years of age (B recommendation), which would
pared with FH, dyspbetalipoproteinemia can lead to earlier include evaluation of lipid panels and other laboratory values as
development of hypercholesterolemia, with resulting CAD, discussed later herein. (20) A study found that 0.3% of 10,095
peripheral artery disease, and a great risk of developing children tested had positive screening results. (5)
glomerular nephritis and pancreatitis. (17)
Universal Screening
MANAGEMENT
The USPSTF has assigned an “I” (insufficient evidence) to
Management of hyperlipidemia should be centered around recommend cholesterol screening, whereas the NHLBI and
the underlying cause of elevated cholesterol levels. It should the AAP recommend screening patients at 9 to 11 years of age
be determined whether patients have a primary cause for (B rating) (7)(22)(24)(25) and again at 17 to 21 years, with
hyperlipidemia, such as FH, or whether the cause is inci- testing at other ages indicated for positive family history, high-
dental or secondary. (18)(19) It is important to monitor for level risk factors, or new high-risk medical conditions. (21)(26)
the secondary causes of hyperlipidemia, namely, nephrotic Concerns exist that targeted approaches to cholesterol screen-
syndrome, hypothyroidism, diabetes, cholangiolithic hepa- ing for those with CVD risk factors may miss some children
titis, obesity, anorexia nervosa, diet-related (excessive intake and adolescents, but the USPSTF argues that the abnormal
of dairy products), or drug-induced (oral contraceptive pills, lipid levels used for screening are based on population distri-
corticosteroids, cyclosporine, etc). (4)(7)(8) butions rather than on health outcomes. (8)(24) The USPSTF
also recognizes that the long-term outcome of cholesterol-
Hyperlipidemia Screening lowering medication has not been fully evaluated. (24)
Routine screening of pediatric patients for elevated cholesterol Regarding targeted versus universal screening, a cost analysis
levels remains controversial because it involves testing for a using a simulated cohort of 4.1 million children from the
risk factor of atherosclerosis and not for the disease itself. National Health and Nutrition Examination Survey found that
(5)(20)(21)(22) Universal screening of the pediatric population universal screening, rather than targeted screening based on
1. A 17-year-old boy is seen in clinic for a sports preparticipation evaluation. REQUIREMENTS: Learners
You learn that many of his family members have “high cholesterol,” but he can take Pediatrics in Review
does not know anything more specific. Several of his family members have quizzes and claim credit
had recurrent episodes of pancreatitis. You perform targeted screening and online only at: http://
pedsinreview.org.
obtain a lipid panel, which showed the following values: low-density
lipoprotein cholesterol (LDL-C), 105 mg/dL (2.7 mmol/L) (acceptable: <110 To successfully complete
mg/dL [<2.9 mmol/L]); high-density lipoprotein cholesterol (HDL-C), 35 mg/ 2020 Pediatrics in Review
dL (0.91 mmol/L) (acceptable: >45 mg/dL [>1.17 mmol/L]); triglycerides, 450 articles for AMA PRA
mg/dL (5.1 mmol/L) (acceptable: <90 mg/dL [<1.0 mmol/L]). This clinical Category 1 CreditTM, learners
presentation and laboratory data are most consistent with which of the must demonstrate a minimum
performance level of 60% or
following diagnoses?
higher on this assessment.
A. Dysbetalipoproteinemia. If you score less than 60%
B. Familial combined hyperlipidemia. on the assessment, you
C. Familial hypertriglyceridemia. will be given additional
D. Heterozygous familial hypercholesterolemia. opportunities to answer
E. Homozygous familial hypercholesterolemia. questions until an overall 60%
or greater score is achieved.
2. You are evaluating a 9-year-old boy with abnormal lipids (LDL=550 mg/dl
This journal-based CME
[14.2 mmol/L]). His initial presentation included xanthomas around his knees
activity is available through
and elbows, a systolic heart murmur heard best at the right upper sternal
Dec. 31, 2022, however, credit
border, and intermittent chest pain. The patient has a strong family history will be recorded in the year in
of hypercholesterolemia. Genetic testing is ordered and is most likely to which the learner completes
show which of the following genetic patterns of inheritance in this patient? the quiz.
A. De novo mutation.
B. Heterozygous (autosomal recessive).
C. Homozygous (autosomal dominant).
D. X-linked recessive.
E. X-linked dominant. 2020 Pediatrics in Review is
3. An 11-year-old boy with familial hypercholesterolemia has failed lifestyle approved for a total of 30
modification management, and his LDL-C remains greater than 250mg/dl Maintenance of Certification
(>6.5 mmol/L). In consultation with the child and his parents, the decision is (MOC) Part 2 credits by the
American Board of Pediatrics
made to initiate drug therapy. You counsel the family regarding statins as
(ABP) through the AAP MOC
the preferred medication class you will prescribe and discuss statin side
Portfolio Program. Pediatrics in
effects. Which of the following side effects could potentially be attributable Review subscribers can claim
to this class of medication? up to 30 ABP MOC Part 2
A. Constipation. points upon passing 30
B. Diarrhea. quizzes (and claiming full
credit for each quiz) per year.
C. Flushing.
Subscribers can start claiming
D. Hypoglycemia.
MOC credits as early as
E. Myopathy. October 2020. To learn how
4. A 19-year-old woman is followed in a healthy lifestyles program for to claim MOC points, go to:
adolescents. At a follow-up visit, she has a BMI greater than the 95th%, https://www.aappublications.
hypertension (blood pressure >95th%), and acanthosis nigricans on physical org/content/moc-credit.
exam. She denies smoking but is sexually active, with no consistent method
of contraception. Her lipid profile continues to demonstrate elevated LDL
(200mg/dl [5.2 mmol/L]) and triglycerides (350 mg/dl [4.0 mmol/L]). You
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Hyperlipidemia
Jeremy Stewart, Tracy McCallin, Julian Martinez, Sheebu Chacko and Shabana Yusuf
Pediatrics in Review 2020;41;393
DOI: 10.1542/pir.2019-0053
The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://pedsinreview.aappublications.org/content/41/8/393
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