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Pediatric Lipid Disorders in Clinical Practice Medication

  • Author: Henry J Rohrs, III, MD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Jan 29, 2015
 
 

Medication Summary

See Medical Care for specific guidelines.

 
 
Contributor Information and Disclosures
Author

Henry J Rohrs, III, MD Assistant Professor, Department of Pediatrics, Division of Pediatric Endocrinology, University of Florida College of Medicine

Henry J Rohrs, III, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Coauthor(s)

William E Winter, MD Professor, Departments of Pathology and Laboratory Medicine and Pediatrics, University of Florida College of Medicine

William E Winter, MD is a member of the following medical societies: American Diabetes Association, American Association for Clinical Chemistry

Disclosure: Nothing to disclose.

Desmond Schatz, MBBCh, MD Professor, Medical Director of Diabetes Center, Department of Pediatrics, Division of Endocrinology, University of Florida College of Medicine

Desmond Schatz, MBBCh, MD is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, Endocrine Society, Florida Medical Association, Pediatric Endocrine Society, Society for Pediatric Research

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Sanofi Aventis<br/>Received income in an amount equal to or greater than $250 from: Sanofi Aventis.

Vanessa Davis, MD Fellow, Department of Pediatrics, Division of Endocrinology, University of Florida

Vanessa Davis, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Diabetes Association, American Medical Association, Endocrine Society, Pediatric Endocrine Society, National Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Christopher Johnsrude, MD, MS Chief, Division of Pediatric Cardiology, University of Louisville School of Medicine; Director, Congenital Heart Center, Kosair Children's Hospital

Christopher Johnsrude, MD, MS is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology

Disclosure: Nothing to disclose.

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Indications for lipid testing, type of testing, and follow-up testing.
Pharmacologic approach to the treatment of type IIA hyperlipoproteinemia (HLP).
Pharmacologic approach to the treatment of type IIB hyperlipoproteinemia (HLP).
Pharmacologic approach to the treatment of type IV hyperlipoproteinemia (HLP).
Table 1. Biology of Lipoproteins
LipoproteinMajor Lipid CompositionRole in Normal Fasting PlasmaMeasured Substance
High-density lipoprotein cholesterol (HDL-C)CholesterolAntiatherogenic (involved in reverse cholesterol transport from the tissues to the liver)HDL-C
LDL-CCholesterolMajor cholesterol carrierCan be measured directly (direct LDL-C) or can be calculated*
Intermediate-density lipoprotein cholesterol (IDL-C)TG and cholesterolIntermediate between very–low density lipoprotein (VLDL) and low-density lipoprotein (LDL)Not routinely measured; can be assessed by LPE† or measured by ultracentrifugation
VLDLTGMajor TG carrierTG‡
ChylomicronTGAbsentNot routinely measured; can be assessed by LPE or measured by ultracentrifugation
* Calculated using the Friedewald equation: LDL-C = Total cholesterol (TC) - HDL-C - TG/5



† LPE = Lipoprotein electrophoresis



‡ TG/5 is the estimate of the VLDL-C.



Table 2. Frederickson Classification of Dyslipidemias
PhenotypeElevated ParticlesMajor Lipid IncreasedFrequency
IChylomicronTGVery rare
IIALDLLDL-CCommon
IIBLDL and VLDLLDL-C, TGCommon
IIIIDL and remnantsTC, TGRare
IVVLDLTGCommon
VChylomicron and VLDLTGUncommon
Table 3. NCEP Lipid Assessments for Children and Adults
Children (< 20 y)Desirable level (mg/dL)Borderline level (mg/dL)Undesirable level (mg/dL)
TC< 170170-199≥200
LDL-C< 110110-129≥130
HDL-C*>4535-45< 35
TG< 125...≥125
Adults (≥20 y)Desirable level (mg/dL)Borderline level (mg/dL)Undesirable level (mg/dL)
TC< 200200-239≥240
LDL-C§< 130130-159≥160
HDL-C||≥40...< 40
TGs< 150150-199≥200
* This was not established by NCEP; these values were the adult cutpoints used at the time that the pediatric NCEP guidelines were established.



This was not established by NCEP; a TG level of 125 mg/dL approximates the mean 95th percentile for TGs in boys and girls during childhood and adolescence.



In March of 2001, cutoff points for desirable and undesirable cholesterol, HDL-C, and other levels were revised in the Adult Treatment Panel III (ATPIII).[12]



§ The optimal LDL-C concentration is less than 100 mg/dL; in patients with cardiovascular disease or diabetes, the optimal LDL-C level is less than 70 mg/dL.



|| If the HDL-C level is 60 mg/dL or higher, one risk factor for coronary heart disease can be subtracted in adults.



Table 4. Summary of Evidence Based Recommendations for the CHILD-1
AgeDietary Recommendations
Birth to 6 months
  • Infants should be exclusively breastfed until age 6 months
6-12 months
  • Continue breastfeeding until at least 12 months of age (or feed iron-fortified formula if unable to breastfeed), gradually adding solid foods
  • No restriction in fat intake without medical recommendation
  • Water should be encouraged
  • Limit other types of drinks to 100% fruit juice, intake of which should be limited to 4 ounces/day or less
  • No sweetened beverages
12-24 months
  • Switch to reduced fat milk (2% to fat free)
  • Limit or avoid sugar-sweetened drinks
  • Water should be encouraged
  • Transition to table food with total fat content of 30% of daily kcal/estimated energy requirement (EER), saturated fat content of 8-10% of daily kcal/EER, and monounsaturated and polyunsaturated fat content of up to 20% of daily kcal/EER
  • Avoid trans fat as much as possible
  • Total daily cholesterol less than 300 mg
2-10 years
  • Fat-free milk
  • Limit or avoid sugar-sweetened drinks
  • Water should be encouraged
  • Limit total fat to 25-30% of daily kcal/EER, saturated fat to 8-10% of daily kcal/EER, and monounsaturated and polyunsaturated fat to up to 20% of daily kcal/EER
  • Avoid trans fat as much as possible
  • Total daily cholesterol less than 300 mg
  • Encourage high dietary fiber intake from foods
11-21 years
  • Fat-free milk
  • Limit or avoid sugar-sweetened drinks
  • Water should be encouraged
  • Limit total fat to 25-30% of daily kcal/EER, saturated fat to 8-10% of daily kcal/EER, and monounsaturated and polyunsaturated fat to up to 20% of daily kcal/EER
  • Avoid trans fat as much as possible
  • Total daily cholesterol less than 300 mg
  • Encourage high dietary fiber intake from foods
Table 5. Dosing of HMG-CoA–Reductase Inhibitors
Generic NameAdult DosePediatric DoseDose Adjustment for Renal Insufficiency or Coadministration with Food or Drugs That Decrease Clearance*
Lovastatin (Mevacor)Initial: 20 mg/d orally every bedtime



Followed by: 10-80 mg/d orally every bedtime or divided twice daily



10-17 years: 10-20 mg/d orally every bedtime initially; maintenance dosage ranges from 10-40 mg/dNot to exceed 20 mg/d
Simvastatin (Zocor)Initial: 5-10 mg/d orally every bedtime



Followed by 5-80 mg/d orally every bedtime or divided twice daily



10-17 years: 10 mg/d orally every bedtime initially; maintenance dosage ranges from 10-40 mg/d5 mg/d initially; not to exceed 20 mg/d
Pravastatin (Pravachol)Initial: 10-20 mg/d orally every bedtime



Followed by 5-40 mg/d orally every bedtime



8-13 years: 20 mg orally every day



14-18 years: 40 mg orally every day



Initiate at 5-10 mg/d; not to exceed 20 mg/d (also decrease with hepatic impairment)
Fluvastatin (Lescol)Initial: 20-30 mg/d orally every bedtime



Followed by 20-80 mg/d orally every bedtime; for 80 mg/d, divide twice daily



10-16 years: 20 mg orally every day initially; maintenance dosage ranges from 20-80 mg/dNo adjustment
Atorvastatin (Lipitor)Initial: 10 mg/d PO orally every bedtime



Followed by 10-80 mg/d orally every bedtime



10-17 years: 10 mg orally every day initially; maintenance dosages do not exceed 20 mg/dNo adjustment for renal insufficiency; decrease dose or avoid with drugs that decrease clearance
Rosuvastatin (Crestor)10-20 mg orally every day initially; maintenance dosage range is 5-40 mg/dNot established5 mg orally every day initially; not to exceed 10 mg/d
* Renal insufficiency is indicated by a creatinine clearance of less than 30 mL/min; agents known to decrease HMG-CoA–reductase inhibitor clearance include grapefruit juice, gemfibrozil, ritonavir, cyclosporine, danazol, amiodarone, azole antifungals, macrolide antibiotics, and verapamil.
Table 6. FDA-Approved Uses and Doses of Fibric Acid Derivatives
Drug NameApproved IndicationsAdult Dose
Gemfibrozil (Lopid)HLP types IIB, IV, and V600 mg orally twice daily (ie, 1200 mg total daily dose) 30 min before meals (ie, before breakfast and dinner)
Fenofibrate (Tricor)HLP types IIA, IIB, IV and VInitial: 67 mg/d orally; not to exceed 67 mg orally twice daily
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