Low HDL Cholesterol (Hypoalphalipoproteinemia) Follow-up

  • Author: Vibhuti N Singh, MD, MPH, FACC, FSCAI; Chief Editor: George T Griffing, MD   more...
 
Updated: Dec 29, 2011
 

Further Inpatient Care

  • Generally, patients with hypoalphalipoproteinemia (HA) are discovered during routine lipid profile testing. Such patients are ambulatory and do not usually require hospitalization or inpatient care.
  • Inpatient care is usually required for complications arising from accelerated atherosclerosis.
  • In patients with secondary causes of HA, inpatient care may become necessary based on the primary pathology.
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Further Outpatient Care

  • Provide outpatient care at regular intervals, especially clinical evaluation, lipid profile assessment, and follow-up evaluations for complications (such as coronary heart disease).
  • Monitor patients frequently to ascertain the effectiveness of medical treatment and to determine whether complications are arising from drug therapy. For example, monitor liver function tests and eye function when treating patients with lipid-lowering agents.
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Inpatient & Outpatient Medications

  • Niacin or nicotinic acid
  • Gemfibrozil (Lopid) or fenofibrate (Tricor)
  • Statins
    • Atorvastatin (Lipitor)
    • Simvastatin (Zocor)
    • Pravastatin (Pravachol)
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Transfer

  • Patients with hypoalphalipoproteinemia (HA) rarely require transfer, and no specific recommendations are available for this purpose. In patients who are admitted to a health care facility, transfer depends mainly on the underlying condition or on complications of HA or premature atherosclerosis, such as myocardial infarction, arrhythmia, or hypotension.
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Deterrence/Prevention

  • Familial or genetically inheritable forms of HA are not amenable to prevention. Genetic counseling and screening may be applicable in rare cases.
  • Hypoalphalipoproteinemia resulting from secondary causes can frequently be managed by treating those causes. Examples include quitting smoking and initiating regular physical activity.
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Complications

  • Premature atherosclerosis
  • Corneal opacification
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Prognosis

  • If hypoalphalipoproteinemia (HA) is diagnosed early and monitored closely, the prognosis for patients with HA is generally reasonably good. The risk derives from the development of complications.
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Patient Education

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Contributor Information and Disclosures
Author

Vibhuti N Singh, MD, MPH, FACC, FSCAI  Director, Suncoast Cardiovascular Center; Chair, Cardiology Division and Cath Labs, Department of Medicine, Bayfront Medical Center; Clinical Assistant Professor, Division of Cardiology, University of South Florida College of Medicine

Vibhuti N Singh, MD, MPH, FACC, FSCAI is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, and Florida Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Elena Citkowitz, MD, PhD, FACP  Clinical Professor of Medicine, Yale University School of Medicine; Director, Cholesterol Management Center, Director, Cardiac Rehabilitation, Department of Medicine, Hospital of St Raphael

Elena Citkowitz, MD, PhD, FACP is a member of the following medical societies: American College of Physicians, American Heart Association, National Lipid Association, and Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

Ghassem Pourmotabbed, MD†  Former Associate Professor, Department of Internal Medicine, Division of Endocrinology and Metabolism, University of Tennessee School of Medicine and Health Science Center

Ghassem Pourmotabbed, MD† is a member of the following medical societies: American Diabetes Association, American Federation for Medical Research, and Endocrine Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Yoram Shenker, MD  Chief of Endocrinology Section, Veterans Affairs Medical Center of Madison; Interim Chief, Associate Professor, Department of Internal Medicine, Section of Endocrinology, Diabetes and Metabolism, University of Wisconsin at Madison

Yoram Shenker, MD is a member of the following medical societies: American Heart Association, Central Society for Clinical Research, and Endocrine Society

Disclosure: Nothing to disclose.

Mark Cooper, MBBS, PhD, FRACP  Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University

Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD  Professor of Medicine, St Louis University School of Medicine

George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

References

  1. Yamakawa-Kobayashi K, Yanagi H, Fukayama H, et al. Frequent occurrence of hypoalphalipoproteinemia due to mutant apolipoprotein A-I gene in the population: a population-based survey. Hum Mol Genet. Feb 1999;8(2):331-6. [Medline]. [Full Text].

  2. Executive Summary of the Third Report of the National Cholesterol Education Program (NCEP) Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults (Adult Treatment Panel III). JAMA. May 16 2001;285(19):2486-97. [Medline].

  3. Third Report of the Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults (ATP III Final Report). US Department of Health and Human Services; May 2001. [Full Text].

  4. Grundy SM, Cleeman JI, Merz CN, et al. Implications of recent clinical trials for the National Cholesterol Education Program Adult Treatment Panel III guidelines. Circulation. Jul 13 2004;110(2):227-39. [Medline]. [Full Text].

  5. Singh VN. New ATP III lipid guidelines update for patients at high risk for cardiovascular events. eMedicine Feature Series - Lipid Newsletter [serial online]. Jul 21, 2005;series 1(9):Available at http://web.archive.org/web/20080211223356/http://www.emedicine.com/email/lipid/issue9.htm.

  6. Dioguardi N, Vergani C. [Familial alpha lipoprotein deficiency. Tangier disease, familial hypoalphalipoproteinemia and familial deficiency of lecithin cholesterol acyltransferase deficiency]. Minerva Med. Mar 24 1983;74(12):585-94. [Medline].

  7. Sorrenson B, Suetani RJ, Bickley VM, George PM, Williams MJ, Scott RS, et al. An ABCA1 truncation shows no dominant negative effect in a familial hypoalphalipoproteinemia pedigree with three ABCA1 mutations. Biochem Biophys Res Commun. Jun 10 2011;409(3):400-5. [Medline].

  8. Chien KL, Chen MF, Hsu HC, et al. Genetic association study of APOA1/C3/A4/A5 gene cluster and haplotypes on triglyceride and HDL cholesterol in a community-based population. Clin Chim Acta. Feb 2008;388(1-2):78-83. [Medline].

  9. Asztalos BF, Schaefer EJ, Horvath KV, et al. Role of LCAT in HDL remodeling: investigation of LCAT deficiency states. J Lipid Res. Mar 2007;48(3):592-9. [Medline]. [Full Text].

  10. Tietjen I, Hovingh GK, Singaraja R, Radomski C, McEwen J, Chan E, et al. Increased self-reported risk of coronary artery disease in Caucasians with extremely low HDL cholesterol due to mutations in ABCA1, APOA1, and LCAT. Biochim Biophys Acta. Aug 19 2011;[Medline].

  11. Santamarina-Fojo S, Hoeg JM, Assmann G. Lecithin cholesterol acyltransferase deficiency and fish eye disease. In: Scriver CR, Sly WS, Childs B, et al, eds. Metabolic and Molecular Bases of Inherited Disease. vol 2. 8th ed. New York, NY: McGraw-Hill; 2001:2817-33.

  12. Asada S, Kuroda M, Aoyagi Y, Bujo H, Tanaka S, Konno S, et al. Disturbed apolipoprotein A-I-containing lipoproteins in fish-eye disease are improved by the lecithin:cholesterol acyltransferase produced by gene-transduced adipocytes in vitro. Mol Genet Metab. Feb 2011;102(2):229-31. [Medline].

  13. Uehara Y, Tsuboi Y, Zhang B, et al. POPC/apoA-I discs as a potent lipoprotein modulator in Tangier disease. Atherosclerosis. Mar 2008;197(1):283-9. [Medline].

  14. Assmann G, von Eckardstein A, Brewer HB Jr. Familial analphalipoproteinemia: Tangier disease. In: Scriver CR, Sly WS, Childs B, et al, eds. Metabolic and Molecular Bases of Inherited Disease. Vol 2. 8th ed. New York, NY: McGraw-Hill; 2001:2937-60.

  15. Herbert PN, Assmann G, Gotto AM Jr, et al. Familial lipoprotein deficiency (abetalipoproteinemia and Tangier disease). In: Stanbury JB, Wyngaarden JB, Fredrickson DS, et al, eds. The Metabolic Basis of Inherited Disease. New York, NY: McGraw-Hill; 1982.

  16. Brites FD, Bonavita CD, De Geitere C, et al. Alterations in the main steps of reverse cholesterol transport in male patients with primary hypertriglyceridemia and low HDL-cholesterol levels. Atherosclerosis. Sep 2000;152(1):181-92. [Medline].

  17. Satta MA, Scoppola A, Melina D, et al. [The relationship between high-density lipoproteins, thromboxane B2 and arteriosclerosis in a case of primary hypoalphalipoproteinemia]. Minerva Med. Dec 1989;80(12):1345-9. [Medline].

  18. DeLong DM, DeLong ER, Wood PD, et al. A comparison of methods for the estimation of plasma low- and very low-density lipoprotein cholesterol. The Lipid Research Clinics Prevalence Study. JAMA. Nov 7 1986;256(17):2372-7. [Medline].

  19. Ordovas JM, Schaefer EJ, Salem D, et al. Apolipoprotein A-I gene polymorphism associated with premature coronary artery disease and familial hypoalphalipoproteinemia. N Engl J Med. Mar 13 1986;314(11):671-7. [Medline].

  20. Frohlich J, Westerlund J, Sparks D, et al. Familial hypoalphalipoproteinemias. Clin Invest Med. Aug 1990;13(4):202-10. [Medline].

  21. Godin DV, Garnett ME, Hoag G, et al. Erythrocyte abnormalities in a hypoalphalipoproteinemia syndrome resembling fish eye disease. Eur J Haematol. Aug 1988;41(2):176-81. [Medline].

  22. Barkowski RS, Frishman WH. HDL metabolism and CETP inhibition. Cardiol Rev. May-Jun 2008;16(3):154-62. [Medline].

  23. Barter PJ, Brewer HB Jr, Chapman MJ, et al. Cholesteryl ester transfer protein: a novel target for raising HDL and inhibiting atherosclerosis. Arterioscler Thromb Vasc Biol. Feb 1 2003;23(2):160-7. [Medline]. [Full Text].

  24. de Grooth GJ, Kuivenhoven JA, Stalenhoef AF, et al. Efficacy and safety of a novel cholesteryl ester transfer protein inhibitor, JTT-705, in humans: a randomized phase II dose-response study. Circulation. May 7 2002;105(18):2159-65. [Medline]. [Full Text].

  25. Nissen SE, Tsunoda T, Tuzcu EM, et al. Effect of recombinant ApoA-I Milano on coronary atherosclerosis in patients with acute coronary syndromes: a randomized controlled trial. JAMA. Nov 5 2003;290(17):2292-300. [Medline]. [Full Text].

  26. Hulley S, Grady D, Bush T, et al. Randomized trial of estrogen plus progestin for secondary prevention of coronary heart disease in postmenopausal women. Heart and Estrogen/progestin Replacement Study (HERS) Research Group. JAMA. Aug 19 1998;280(7):605-13. [Medline]. [Full Text].

  27. Lamon-Fava S, Postfai B, Diffenderfer M, et al. Role of the estrogen and progestin in hormonal replacement therapy on apolipoprotein A-I kinetics in postmenopausal women. Arterioscler Thromb Vasc Biol. Feb 2006;26(2):385-91. [Medline]. [Full Text].

  28. Eslick GD, Howe PR, Smith C, et al. Benefits of fish oil supplementation in hyperlipidemia: a systematic review and meta-analysis. Int J Cardiol. Sep 5 2008;[Medline].

  29. Vega GL, Grundy SM. Comparison of lovastatin and gemfibrozil in normolipidemic patients with hypoalphalipoproteinemia. JAMA. Dec 8 1989;262(22):3148-53. [Medline].

  30. HDL-Atherosclerosis Treatment Study (HATS). Cardiosource: American College of Cardiology. Available at http://www.cardiosource.com/clinicaltrials/trial.asp?trialID=486. Accessed 2/10/09.

  31. Arterial Biology for the Investigation of the Treatment Effects of Reducing Cholesterol 2 (ARBITER 2). Cardiosource: American College of Cardiology. Available at http://www.cardiosource.com/clinicaltrials/trial.asp?trialID=1144. Accessed 2/10/09.

  32. [Best Evidence] Barter PJ, Caulfield M, Eriksson M, et al. Effects of torcetrapib in patients at high risk for coronary events. N Engl J Med. Nov 22 2007;357(21):2109-22. [Medline]. [Full Text].

  33. Nissen SE, Tsunoda T, Tuzcu EM, et al. Effect of recombinant ApoA-I Milano on coronary atherosclerosis inpatients with acute coronary syndromes: a randomized controlled trial. JAMA. Nov 5 2003;290(17):2292-300. [Medline]. [Full Text].

  34. Parolini C, Marchesi M, Lorenzon P, et al. Dose-related effects of repeated ETC-216 (recombinant apolipoprotein A-I Milano/1-palmitoyl-2-oleoyl phosphatidylcholine complexes) administrations on rabbit lipid-rich soft plaques: in vivo assessment by intravascular ultrasound and magnetic resonance imaging. J Am Coll Cardiol. Mar 18 2008;51(11):1098-103. [Medline].

  35. Ibanez B, Vilahur G, Cimmino G, et al. Rapid change in plaque size, composition, and molecular footprint after recombinant apolipoprotein A-I Milano (ETC-216) administration: magnetic resonance imaging study in an experimental model of atherosclerosis. J Am Coll Cardiol. Mar 18 2008;51(11):1104-9. [Medline].

  36. Ahumada Ayala M, Jimenez Villanueva C, Cardoso Saldana G, et al. [Hypoalphalipoproteinemia and atherosclerosis. Genetic and biochemical profile of 10 families]. Arch Inst Cardiol Mex. Jan-Feb 1989;59(1):9-18. [Medline].

  37. Boden WE, Probstfield JL, Anderson T, Chaitman BR, Desvignes-Nickens P, Koprowicz K, et al. Niacin in patients with low HDL cholesterol levels receiving intensive statin therapy. N Engl J Med. Dec 15 2011;365(24):2255-67. [Medline].

  38. Daum U, Leren TP, Langer C, et al. Multiple dysfunctions of two apolipoprotein A-I variants, apoA- I(R160L)Oslo and apoA-I(P165R), that are associated with hypoalphalipoproteinemia in heterozygous carriers. J Lipid Res. Mar 1999;40(3):486-94. [Medline]. [Full Text].

  39. Dioguardi N. [Familial hypoalphalipoproteinemia. Vergani's disease]. Minerva Med. Nov 16 1983;74(44):2659-64. [Medline].

  40. Hersberger M, von Eckardstein A. Low high-density lipoprotein cholesterol: physiological background, clinical importance and drug treatment. Drugs. 2003;63(18):1907-45. [Medline].

  41. Jones PJ, Ntanios FY, Raeini-Sarjaz M, et al. Cholesterol-lowering efficacy of a sitostanol-containing phytosterol mixture with a prudent diet in hyperlipidemic men. Am J Clin Nutr. Jun 1999;69(6):1144-50. [Medline].

  42. Kort EN, Ballinger DG, Ding W, et al. Evidence of linkage of familial hypoalphalipoproteinemia to a novel locus on chromosome 11q23. Am J Hum Genet. Jun 2000;66(6):1845-56. [Medline]. [Full Text].

  43. Meco JF, Pinto X, Quintana E, et al. [Efficacy of hygienic and dietary therapy in coronary patients with isolated hypoalphalipoproteinemia]. An Med Interna. Dec 1999;16(12):620-5. [Medline].

  44. Mingpeng S, Zongli W. The protective role of high-density lipoproteins in atherosclerosis. Exp Gerontol. 1999;34(4):539-48. [Medline].

  45. Mora S, Buring JE, Ridker PM, Cui Y. Association of high-density lipoprotein cholesterol with incident cardiovascular events in women, by low-density lipoprotein cholesterol and apolipoprotein b100 levels: a cohort study. Ann Intern Med. Dec 6 2011;155(11):742-50. [Medline]. [Full Text].

  46. Mott S, Yu L, Marcil M, et al. Decreased cellular cholesterol efflux is a common cause of familial hypoalphalipoproteinemia: role of the ABCA1 gene mutations. Atherosclerosis. Oct 2000;152(2):457-68. [Medline].

  47. Rader DJ. High-density lipoproteins as an emerging therapeutic target for atherosclerosis. JAMA. Nov 5 2003;290(17):2322-4. [Medline].

  48. Saku K, Zhang B, Shirai K, et al. Hyperinsulinemic hypoalphalipoproteinemia as a new indicator for coronary heart disease. J Am Coll Cardiol. Nov 1 1999;34(5):1443-51. [Medline].

  49. Schaefer EJ. Clinical, biochemical, and genetic features in familial disorders of high density lipoprotein deficiency. Arteriosclerosis. Jul-Aug 1984;4(4):303-22. [Medline].

  50. Singh VN. Need for more aggressive statin use in various ethnic groups: Latino, Asian, and African American populations. eMedicine Feature Series - Lipid Newsletter [serial online]. Oct 20, 2005;series 1(12):Available at http://web.archive.org/web/20080211223351/http://www.emedicine.com/email/lipid/issue12.htm.

  51. Singh VN. The USDA "Food Pyramid" needs to go on a diet. Pinellas County Medical Society (PICOMESO) Journal. 2004;43(4):3, 18-19.

  52. Tall AR. Plasma high density lipoproteins. Metabolism and relationship to atherogenesis. J Clin Invest. Aug 1990;86(2):379-84. [Medline]. [Full Text].

  53. Third JL, Montag J, Flynn M, et al. Primary and familial hypoalphalipoproteinemia. Metabolism. Feb 1984;33(2):136-46. [Medline].

  54. Zema MJ. Gemfibrozil, nicotinic acid and combination therapy in patients with isolated hypoalphalipoproteinemia: a randomized, open-label, crossover study. J Am Coll Cardiol. 2000;35(3):640-6. [Medline].

 
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Table. Hypoalphalipoproteinemia
VariantMolecular DefectInheritanceMetabolic DefectLipoprotein AbnormalityClinical FeaturesPremature Atherosclerosis
Familial apo A-IApo deficiencyAutosomal codominantAbsent apo A-1 biosynthesisHDL < 5 mg/dL; TGs normalPlanar xanthomas, corneal opacitiesYes
Familial apo A-I structural mutationsAbnormal apo A-IAutosomal dominantRapid apo A-1 catabolismHDL 15-30 mg/dL; TGs increasedOften none; sometimes corneal opacitiesNo
Familial LCATLCAT deficiency (complete)Autosomal



recessive



Rapid HDL catabolismHDL < 10 mg/dL; TGs increasedCorneal opacities, anemia, proteinuria, renal insufficiencyNo
Fish-eye diseaseLCAT deficiency (partial)Autosomal recessiveRapid HDL catabolismHDL < 10 mg/dL; TGs increasedCorneal opacitiesNo
Tangier diseaseUnknownAutosomal codominantVery rapid HDL catabolismHDL < 5 mg/dL; TGs usually increasedCorneal opacities, enlarged orange tonsils, hepatosplenomegaly, peripheral neuropathyNo to yes
Familial HAUnknownAutosomal dominantUsually rapid HDL catabolismHDL 15-35 mg/dL; TGs normalOften none; sometimes corneal opacitiesNo to yes
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