1986 Mar 13. The kinetic parameters (turnover) of HDL apo AI and apo All were studied in six lean patients with primary HDL deficiency associated with familial hypertriglyc eridemia and normolipidemic healthy controls. Oct 20, 2005. series 1(12):[Full Text]. 2016 May. Gebauer SK, Destaillats F, Dionisi F, Krauss RM, Baer DJ. [Medline]. This finding of a low high-density lipoprotein (HDL) cholesterol level can be useful as an independent factor in assessing coronary artery disease (CAD) risk and further management. Increased self-reported risk of coronary artery disease in Caucasians with extremely low HDL cholesterol due to mutations in ABCA1, APOA1, and LCAT. It may be proatherogenic if the VLDL-LDL cholesteryl ester is taken up by arterial macrophages. [Medline]. The study utilized 15,067 patients (mean age 61 years; 78% male, 93% white) with CHD or CHD risk equivalent (type 2 diabetes). Several novel approaches to increasing HDL cholesterol that have been under investigation include the following: The aforementioned partial CETP inhibitor, torcetrapib, may increase HDL by as much as 46%. levels of paraoxonase, an HDL-associated, antioxidant enzyme, were also increased by 10%. Studying ultrasonograms of the arteries, the researchers found that from the beginning to the end of the 5-week trial, the plaque in the treatment groups shrank by 4%, while that of the placebo group increased by a small amount. [Full Text]. Association of high-density lipoprotein cholesterol with incident cardiovascular events in women, by low-density lipoprotein cholesterol and apolipoprotein b100 levels: a cohort study. J Am Coll Cardiol. Nonetheless, individuals at high risk require further assessment for CHD risk, with an evaluation that includes a family history, measurements of apo and lipoprotein Lp(a), and electron beam CT scanning. [Full Text]. 63(18):1907-45. Of the available drugs, niacin results in the largest increase in HDL-C. Fibrates do not increase HDL-C in patients with normal triglycerides. Vasc Health Risk Manag. Effects of torcetrapib in patients at high risk for coronary events. Reproduced with permission from Komuro, R., et al. Circulation. Plasma apo AI concentration is extremely low, with a relative increase in precursors of apo AI. Barter PJ, Brewer HB Jr, Chapman MJ, et al. [Medline]. [Medline]. [The relationship between high-density lipoproteins, thromboxane B2 and arteriosclerosis in a case of primary hypoalphalipoproteinemia]. http://www.nhlbi.nih.gov/guidelines/cholesterol/, http://www.medscape.com/viewarticle/863880, http://www.medscape.com/viewarticle/862022, http://www.cardiosource.com/clinicaltrials/trial.asp?trialID=486, http://www.cardiosource.com/clinicaltrials/trial.asp?trialID=1144, Central Society for Clinical and Translational Research, American Association for the Advancement of Science, International Society for Clinical Densitometry, Southern Society for Clinical Investigation, American College of Medical Practice Executives, American Association for Physician Leadership. 262(22):3148-53. Decreased cellular cholesterol efflux is a common cause of familial hypoalphalipoproteinemia: role of the ABCA1 gene mutations. Singh VN. Circulation 101, 2446–2448. [Medline]. de Grooth GJ, Kuivenhoven JA, Stalenhoef AF, et al. Statins do have modest affects on HDL, increasing concentrations by 5% to 10%, providing a secondary benefit to this therapy beyond LDL reduction. The end result was a 50% reduction in the composite cardiovascular endpoint for the niacin/statin patients. is rare and may be due to Tangier disease, apo A-I deficiencies, LCAT deficiency, or fish-eye disease. Two pharmacologic inhibitors of CETP have undergone phase 3 clinical trials: torcetrapib and JTT-705. The NCEP has recommended a therapeutic lifestyle-change diet, which should be incorporated in the treatment of all patients. Identify and correct secondary factors. We report findings in four subjects with severe familial HDL deficiency (HDL-C<<5th percentile for age and sex; 0.08 to 0.38 mmol/L) in three French-Canadian kindreds with autosomal codominant inheritance. 314(11):671-7. Minerva Med. Biology the absence of a gene or a region of a chromosome normally present Explanation of familial high-density lipoprotein (HDL) deficiency. 48(3):592-9. [32]. [Full Text]. If you are visiting our English version, and want to see definitions of Familial HDL Deficiency in other languages, please click the language menu on the right bottom. Minerva Med. Carbohydrates (complex carbohydrates from grains, whole grains, fruits, and vegetables) should constitute 50-60% of total energy intake. Abnormal dilatation of the Golgi cisternae speaks to a disruption of intracellular lipid trafficking through vesicular budding, with abnormal ceramide and glycerophospholipid turnover.71,92, Ken-ichi Hirano, ... Yuji Matsuzawa, in Encyclopedia of Endocrine Diseases, 2004. Patients with ABCA1 mutations are thought to have moderately increased risk for CHD. (A) Foam cells observed in bone marrow. 2008 Mar 18. Whereas phospholipid efflux is abnormal, total cell phospholipid content is not significantly altered. The panel stated that low HDL levels should be managed in the following manner: Reducing low-density lipoprotein (LDL) levels is the primary goal. Familial HDL deficiency. N Engl J Med. Consistent with this conclusion, the FDA has determined that the benefits of niacin ER tablets for coadministration with statins no longer outweigh the risks, and the approval for this indication should be withdrawn. J Am Coll Cardiol. Figure 2. JAMA. [Medline]. The ATP III reported, "When a low HDL cholesterol is associated with high triglycerides (200-499 mg/dL), secondary priority goes to achieving the non-HDL cholesterol goal." Available at http://www.medscape.com/viewarticle/862022. Angiographic analysis revealed that the combination therapy significantly enhanced stenosis regression. In the Danish general population, two SNPs (V771M and V825I) of ABCA1 gene were associated with increases in HDL-C, one (R1587K) with decreases in HDL-C, whereas three (R219K, I883M, and E1172D) did not affect HDL-C levels. Fast Five Quiz: How Much Do You Know About Hypertriglyceridemia? N Engl J Med. 2000. [Full Text]. Strongly encourage increased physical activity, especially in persons with sedentary habits. In the ADvicor Versus Other Cholesterol-Modulating Agents Trial Evaluation (ADVOCATE), 315 patients with high LDL (≥160 mg/dL without CAD, or ≥130 mg/dL with CAD) and low HDL (< 45 mg/dL in men, and < 50 mg/dL in women) were randomized to 16 weeks of a combination of niacin/lovastatin versus standard doses of atorvastatin or simvastatin. Effects of extended-release niacin with laropiprant in high-risk patients. Tall AR. [Medline]. Furthermore, a close correlation was found between the ex … Most patients who receive niacin also have high LDL cholesterol levels that are being managed pharmacologically, and niacin is added to raise their HDL cholesterol level if it is low. None of these HDL-raising therapies have been studied in Asian Indians. Niacin in patients with low HDL cholesterol levels receiving intensive statin therapy. Dioguardi N, Vergani C. [Familial alpha lipoprotein deficiency. However, the cellular cardiolipin and lysocardiolipin fractions are three- to fivefold enriched.29 There is some evidence that HDL is internalized after cell surface binding and is resecreted in a lipid-enriched state.91,99 It has been reported that, in cells from TD patients, cell binding of HDL is increased with a significant decrease in HDL resecretion.91 While other workers have failed to duplicate this observation, recent work on internalization of HDL by the scavenger receptor SR-B1 suggests that lipid exchange involving HDL might indeed be occurring inside intracellular vesicles.95, Abnormal hypercatabolism of mature apo A-I but not pro-apo A-I has been demonstrated in both TD and FHA patients,7,88 thus implying that the deficiency is secondary to the cellular defect in cholesterol efflux. People in the study had a protein in their HDL, the aforementioned apo A-I Milano, that seemed to be better at stimulating the removal of cholesterol from plaques than was HDL containing the normal protein, called apo A-I. Familial LCAT deficiency (FLD) is a rare genetic disorder of HDL metabolism, caused by loss-of-function mutations in the LCAT gene and characterized by a variety of symptoms including corneal opacities and kidney failure. 43(4):3, 18-19. These mutations lead to an altered apoA-I protein. Biochem Biophys Res Commun. 197(1):283-9. Savel J, Lafitte M, Pucheu Y, Pradeau V, Tabarin A, Couffinhal T. Very low levels of HDL cholesterol and atherosclerosis, a variable relationship--a review of LCAT deficiency. Refer patients who may possibly have one of these diagnoses to a specialized lipid center for advanced management. Familial HDL deficiency. In a study in patients with familial combined hyperlipidemia, treatment with this formulation for 8 weeks increased HDL by 8%, particularly the more buoyant HDL-2 subfraction. Minerva Med. 1988 Aug. 41(2):176-81. Always consider secondary causes of low HDL levels, especially medications, smoking habits, dietary patterns, and physical activity. [Medline]. 2004 Jul 13. Individuals with a genetic mutation that causes the loss of all CETP activity have very high levels of HDL cholesterol. In such patients, a secondary goal is set for non–HDL-C (total cholesterol minus HDL-C). 2014 Jul 17. CIMT progression was 68% lower in the niacin/statin group than in the other patients. Jones PJ, Ntanios FY, Raeini-Sarjaz M, et al. 2002 Carroll MD, Fryar CD, Kit BK. According to the ATP III executive summary, "After the LDL goal has been reached, emphasis shifts to weight reduction and increased physical activity (when the metabolic syndrome is present)." 66 (20):2201-10. Patients should consume 20-30 g/d of fiber. 2003 Nov 5. [Medline]. Treatment There are drugs that can increase serum HDL such as niacin or gemfibrozil . Gemfibrozil, nicotinic acid and combination therapy in patients with isolated hypoalphalipoproteinemia: a randomized, open-label, crossover study. Familial HALP - Familial HALP includes CETP deficiency, familial hepatic lipase deficiency, and primary HALP. Statins lower total cholesterol and represent the most efficacious way to lower the ratio of total cholesterol to HDL-C. Statins decrease clinical events in patients with low HDL-C.151 As noted earlier, pharmacologic therapies for raising HDL levels, notably niacin and inhibition of CETP, have not yet shown efficacy in cardiovascular outcome trials. Role of LCAT in HDL remodeling: investigation of LCAT deficiency states. 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 [Medline]. HDL levels in the combination group rose 21% higher (P = 0.002) than they did in the statin-only group, and TGs decreased significantly (P = 0.009). [Medline]. Sorrenson B, Suetani RJ, Bickley VM, George PM, Williams MJ, Scott RS, et al. Corneal opacities, anemia, proteinuria, renal insufficiency, Corneal opacities, enlarged orange tonsils, hepatosplenomegaly, peripheral neuropathy. We previously reported severely calcified coronary arteries visualized by coronary intravascular ultrasonography and impaired insulin secretion in ABCA1 deficiency. In order to maintain a desirable body weight and to prevent weight gain, the total amount of energy consumed must be balanced in terms of energy intake and expenditure. Frohlich J, Westerlund J, Sparks D, et al. Please Remove Adblock Adverts are the main source of Revenue for DoveMed. 1986 Nov 7. [Medline]. With regard to HA, the ATP III report stated, "Low HDL cholesterol is a strong independent predictor of CHD [coronary heart disease]. Consider estrogen replacement therapy for postmenopausal women, because this can substantially raise HDL cholesterol levels. An association between low HDL and hypertriglyceridemia requires attention. [Full Text]. An ABCA1 truncation shows no dominant negative effect in a familial hypoalphalipoproteinemia pedigree with three ABCA1 mutations. Kort EN, Ballinger DG, Ding W, et al. Mott S, Yu L, Marcil M, et al. Because an increase in the consumption of cold-water fish (eg, salmon) rich in polyunsaturated fats may help to raise HDL, fish oil capsules (capsules containing omega-3 … Clinical, biochemical, and genetic features in familial disorders of high density lipoprotein deficiency. In persons with 0-1 risk factor, the LDL goal is < 160 mg/dL, and that for the non-HDL is < 190 mg/dL. Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC2) is a rare congenital cholestatic liver disease that progresses to end stage liver disease. van den Bogaard B, Holleboom AG, Duivenvoorden R, Hutten BA, Kastelein JJ, Hovingh GK, et al. 2004. 16(3):154-62. 409(3):400-5. Arterial Biology for the Investigation of the Treatment Effects of Reducing Cholesterol 2 (ARBITER 2). 2011 Dec 15. 2003 Feb 1. New ATP III lipid guidelines update for patients at high risk for cardiovascular events. Multiple dysfunctions of two apolipoprotein A-I variants, apoA- I(R160L)Oslo and apoA-I(P165R), that are associated with hypoalphalipoproteinemia in heterozygous carriers. 2007 Mar. [Medline]. [Full Text]. Barter PJ, Caulfield M, Eriksson M, et al. Apo A-I Milano, an apo A-I variant identified in a rural Italian population, is associated with cardioprotection due to its "super-HDL" properties. However, because no data are available that show a reduction in the risk of coronary heart disease (CHD) upon raising HDL cholesterol levels, no particular dietary interventions are needed for this specific purpose. A severe type of this deficiency … Patients with elevated triglyceride levels (>500 mg/dL) commonly have low HDL cholesterol levels; address hypertriglyceridemia first in such patients. Mingpeng S, Zongli W. The protective role of high-density lipoproteins in atherosclerosis. Learn in-depth information on Familial HDL Deficiency Disorder, its causes, symptoms, diagnosis, complications, treatment, prevention, and prognosis. [30, 31], A small study in 2004 involving the CETP inhibitor torcetrapib showed that the drug markedly increased HDL levels and decreased LDL levels when taken alone and also when taken in combination with a statin. Patrice Wending. Available at http://www.cardiosource.com/clinicaltrials/trial.asp?trialID=486. The 2 drugs differ in chemical structure. 74(44):2659-64. [Full Text]. Share cases and questions with Physicians on Medscape consult. Stanbury JB, Wyngaarden JB, Fredrickson DS, et al, eds. Genetic association study of APOA1/C3/A4/A5 gene cluster and haplotypes on triglyceride and HDL cholesterol in a community-based population. 1999 Mar. Tangier disease (TD) is a familial HDL deficiency (FHD) originally described by Frederickson et al. Fast Five Quiz: Do You Know Current Guidelines for Lipid Management? Additionally, adding niacin to statins increased risk for serious adverse events. New Insights From the Framingham Offspring Study. 1990 Aug. 86(2):379-84. 8th ed. [Medline]. Familial Hypoalphalipoproteinemia. Papers overview. Next-generation anti-CD20 monoclonal antibodies in autoimmune disease treatment. familial hdl deficiency due to abca1 gene mutations with or without other genetic lipoprotein disorders Angelo Baldassare Cefalu' , Maurizio Averna , Elmo Mannarino, Alfredo Cantafora, Sebastiano Calandra, Ian Hamilton-Craig, Livia Pisciotta, Antonella Bellocchio, Gabriele Bittolo Bon, Donatella Siepi, Patrizia Tarugi, Luigi Cattin, Stefano Bertolini, Paola Alessandrini, Tommaso Fasano Here we report six subjects with low HDL, carrying seven ABCA1 mutations, six of … This disorder is caused by mutations in the ABCA1 gene, which is located on the long arm of chromosome 9. Vega GL, Grundy SM. Rosiglitazone appears to increase the size of small LDL particles. Journal of the American College of Cardiology. Reproduced with permission from Hirano, K., et al. 8th ed. Primary and familial hypoalphalipoproteinemia. In the ARterial Biology for the Investigation of the Treatment Effects of Reducing Cholesterol (ARBITER 2) study, a double-blind, placebo-controlled trial was performed on an extended-release niacin/statin therapy. Dietary management should follow the NCEP guidelines for lowering frequently associated low-density lipoprotein (LDL) cholesterol, which is the primary target in lipid management Fast Five Quiz: Lipids Management: High HDL Cholesterol Levels (Hyperalphalipoproteinemia), Trending Clinical Topic: New Lipid Guidelines. Treatment of Tangier Disease. FDA Pulls Approval of Niacin, Fibrate in Combo with Statins. The apo A-1 deficiency states are due to rare deletions, rearrangements, or point mutations within the apo A-I/C-III/A-IV gene complex. 8:357-61. Patients with low HDL-cholesterol caused by mutations in LCAT have increased arterial stiffness. Familial LCAT deficiency (FLD), first reported in 1967 in a Norwegian family, is characterized by the absence of LCAT activity towards HDL and LDL. Managing isolated low HDL cholesterol is also important. Drugs. FEBS Lett. Twenty to 30% of early familial coronary heart disease (CHD) is ascribed to hypoalphalipoproteinemia, or high density lipoprotein deficiency. Another pharmacologic approach geared towards raising HDL levels involves inhibiting cholesteryl ester transfer protein (CETP). 2015 Nov 17. It is still controversial whether patients with homozygous Tangier disease develop premature CHD. Identify patients whose diet is very low in fat. [37] The much larger HPS2-THRIVE study (N=25,673) confirmed these findings. The document also reported that there are several causes of low HDL cholesterol levels and that a number of these—including type II diabetes, overweight, obesity, elevated triglycerides (TGs), and a lack of physical activity—are associated with insulin resistance. Mutations in ABCA1 have been shown to be the cause of Tangier disease (TD) and some forms of familial hypoalphalipoproteinemia (HA), two genetic disorders characterized by low plasma HDL levels. 2012 Oct. 97(10):3399-407. This is, ... that have been shown to increase circulating levels of HDL, ... Familial LCAT Deficiency: FLD (Fish Eye Disease) Lipoprotein(a) and Atherogenesis; Polyunsaturated fats should constitute up to 10% of total energy intake, and monounsaturated fats, up to 20% of total energy intake. 2008 May-Jun. 2008 Sep 5. Rader DJ, deGoma EM. 2015 Nov 11. The following are recommendations: Patients should reduce their intake of saturated fats to less than 7% of their total calorie (energy) intake. Vergani's disease]. Metabolism. In persons with multiple (2+) risk factors and 10-year risk of equal to or less than 20%, the LDL goal is < 130 mg/dL, while the non-HDL goal is < 160 mg/dL. 2011 Jun 10. These individuals appear to be at lower risk of coronary disease. In homozygotes, plasma HDL is virtually absent and triglyceride levels are often elevated. HDL metabolism and CETP inhibition. [Medline]. Asztalos BF, Schaefer EJ, Horvath KV, et al. [3, 4, 5, 6]. 1998 Apr;18(4):655-64. Tangier disease, familial hypoalphalipoproteinemia and familial deficiency of lecithin cholesterol acyltransferase deficiency]. Familial hypoalphalipoproteinemia is defined as an HDL cholesterol level below the 10th percentile without a secondary cause and associated with a family history of low HDL levels. Is Isolated Low High-Density Lipoprotein Cholesterol a Cardiovascular Disease Risk Factor? The investigation showed that the atherosclerosis in the coronary vessel wall could be modified in a much shorter time than anticipated, ie, within 5-6 weeks. HDL-Atherosclerosis Treatment Study (HATS). Low HDL levels often reflect a genetic abnormality, although they can also be pushed downward by a high blood level of TGs or by cigarette smoking, inactivity, or hypertension, as well as by a diet very high in carbohydrates or polyunsaturated fats. Pinellas County Medical Society (PICOMESO) Journal. CETP helps to exchange cholesterol between lipoproteins and can transfer it from HDL to LDL and VLDL. 1989 Dec. 80(12):1345-9. Severe HDL deficiency, characterized by HDL cholesterol levels <10 mg dl −1 is rare and may be due to Tangier disease, apo A-I deficiencies, LCAT deficiency, or fish-eye disease. Primary hypoalphalipoproteinemia is seen in approximately 4% of people with CAD. A comparison of methods for the estimation of plasma low- and very low-density lipoprotein cholesterol. Obligatory heterozygotes have half the normal plasma HDL-C and apo AI levels. Patients suffer from symptoms caused by the accumulation of cholesteryl ester in many tissues throughout the body (Fig. [Full Text]. 1983 Mar 24. This disorder is characterized by a genetic predisposition in subjects consuming high-fat, high-cholesterol diets to an increased secretion of apo B-containing lipoproteins and an increased catabolism of apo A-I-containing lipoproteins. [Full Text]. J Clin Endocrinol Metab. 1999 Dec. 16(12):620-5. George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, International Society for Clinical Densitometry, Southern Society for Clinical Investigation, American College of Medical Practice Executives, American Association for Physician Leadership, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical and Translational Research, Endocrine SocietyDisclosure: Nothing to disclose. ABCA1 was expressed on the basolateral plasma membrane of polarized cultured gallbladder epithelial cells. Role of the estrogen and progestin in hormonal replacement therapy on apolipoprotein A-I kinetics in postmenopausal women. Several patients have had recurrent peripheral neuropathy. Third Report of the Expert Panel on Detection, Evaluation, and Treatment of High Blood Cholesterol in Adults (ATP III Final Report). Cardiosource: American College of Cardiology. 9 (3):206-12. J.M. Ahumada Ayala M, Jimenez Villanueva C, Cardoso Saldana G, et al. The disorder frequently manifests during childhood. Metabolic syndrome is diagnosed in patients with at least 3 of the following risk factors: Abdominal obesity, with a waist circumference of over 35 inches (females) or above 40 inches (males), HDL cholesterol levels of below 40 mg/dL (males) or less than 50 mg/dL (females), Blood pressure at or above 130 mm Hg systolic and greater than or equal to 85 mm Hg diastolic, Fasting glucose levels at or above 110 mg/dL. Metabolic and Molecular Bases of Inherited Disease. One year old twin males born to a mother with intrahepatic cholestasis during … An Med Interna. 290(17):2292-300. 365(24):2255-67. 2000 Oct. 152(2):457-68. Instruct patients who smoke to stop smoking, tell persons who are overweight to manage their weight, and encourage individuals who are sedentary to engage in regular exercise. We, Probstfield JL, Anderson T, et al deletions, rearrangements, or yellowish white striations the. 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Atp III lipid guidelines update for patients at high risk for coronary heart disease, signs and symptoms of hypoalphalipoproteinemia.

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