Hyperlipoproteinemia Type II (Familial Hypercholesterolemia)

2116  relevant articles (84 outcomes, 225 trials/studies) found for this Disease

Familial Hypercholesterolemia; Hypercholesterolemia, Familial; Apolipoprotein B-100, Familial Ligand-Defective; Familial Combined Hyperlipoproteinemia; Hyper-Low Density Lipoproteinemia; Hypercholesterolemia, Autosomal Dominant; Hyperlipoproteinemia Type IIa; Hyperlipoproteinemia Type IIb; LDL Receptor Disorder; Apolipoprotein B 100, Familial Ligand Defective; Autosomal Dominant Hypercholesterolemia; Combined Hyperlipoproteinemia, Familial; Essential Hypercholesterolemia; Essential Hypercholesterolemias; Familial Hypercholesterolemias; Hyper Low Density Lipoproteinemia; Hyper-Low Density Lipoproteinemias; Hyperbetalipoproteinemias; Hypercholesterolemias, Essential; Hypercholesterolemias, Familial; Hyperlipoproteinemia, Familial Combined; Lipoproteinemia, Hyper-Low Density; Lipoproteinemias, Hyper-Low Density; Hyperbetalipoproteinemia; Hypercholesterolemia, Essential

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Disease Context: Research Results

Related Diseases

1.	Atherosclerosis (Atheroma)
2.	Hypercholesterolemia
3.	Coronary Artery Disease (Coronary Atherosclerosis)
4.	Coronary Disease (Coronary Heart Disease)
5.	Hyperlipidemias (Hyperlipidemia)

Experts

1.	Kastelein, John J P: 24 articles (06/2008 - 07/2002)
2.	Stalenhoef, Anton F H: 9 articles (04/2008 - 01/2003)
3.	Mabuchi, Hiroshi: 8 articles (12/2006 - 05/2002)
4.	Gagné, Claude: 8 articles (09/2006 - 02/2002)
5.	Trip, Mieke D: 7 articles (06/2008 - 03/2003)
6.	Couture, Patrick: 7 articles (09/2006 - 02/2002)
7.	Hutten, Barbara A: 6 articles (06/2008 - 07/2002)
8.	Wiegman, Albert: 6 articles (06/2008 - 06/2003)
9.	Rodenburg, Jessica: 6 articles (06/2008 - 06/2003)
10.	Mata, Pedro: 6 articles (04/2008 - 11/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hyperlipoproteinemia Type II:

1.	CholesterolIBA 01/01/1998 - "This method also helped us to diagnose familial hypercholesterolemia, or to exclude from carrier status, 11 children with borderline high cholesterol levels." 08/01/1986 - "Safety and efficacy of long-term diet and diet plus bile acid-binding resin cholesterol-lowering therapy in 73 children heterozygous for familial hypercholesterolemia." 01/01/1979 - "These composite data indicated that the definable metabolic defects of these two sisters with homozygous familial hypercholesterolemia were the sluggish clearance of cholesterol from the body coupled with low total body synthesis of cholesterol." 08/01/1977 - "The efficacy of a fat-modified, low-cholesterol diet was studied in 38 children and adolescents with familial hypercholesterolemia on an out-patient basis, and the results compared with those achieved with dietary treatment in hospital. " 04/01/2005 - "A role for PCSK9 in cholesterol metabolism was proposed from the expression studies and confirmed by the discovery that PCSK9 missense mutations were associated with a form of autosomal dominant hypercholesterolemia, Hchola3. " Order ALL the reference details at left...
2.	Lipoproteins (Lipoprotein)IBA 04/01/1990 - "Thus, we have identified a primate model for human familial hypercholesterolemia which will be useful for studying the relationship between the LDLR and lipoprotein metabolism and for assessing the efficacy of diets and drugs in the treatment of human familial hypercholesterolemia." 06/01/2005 - "Recent structural and functional studies of the receptor, combined with the plethora of prior knowledge about normal receptor function and the effects of disease-associated mutations that cause familial hypercholesterolemia, reveal a detailed molecular model for how the acidic environment of the endosome triggers the release of bound lipoprotein particles. " 10/09/2004 - "BACKGROUND AND OBJECTIVE: To compare the lipoprotein phenotype between FDB and heterozygous familial hypercholesterolemia (FH); to study the prevalence and possible founder effect of familial ligand-defective apo B100 (FDB) in a Mediterranean population, and to analyze the clinical and biochemical characteristics of FDB patients. " 10/01/2002 - "This study evaluated the frequency of abnormal TG and HDL-C levels in patients with classical familial hypercholesterolemia (FH) and assessed therapeutic response at different baseline levels of these lipoproteins after 1 year of statin therapy. " 12/01/2001 - "BACKGROUND: The aim of this study was to determine the effects of low-density lipoprotein (LDL) particle size and composition on the susceptibility of LDL to oxidation in subjects with Familial Hypercholesterolemia (FH). " Order ALL the reference details at left...
3.	atorvastatin (Lipitor)FDA Link 01/15/2005 - "Long-term safety and efficacy of high-dose atorvastatin treatment in patients with familial hypercholesterolemia." 01/01/2003 - "Efficacy and tolerability of atorvastatin (20 mg/day) were assessed in a 3 month study on 19 patients (5 men, 14 women, mean age 52.3 years) with familial hypercholesterolemia. " 04/01/2008 - "[Cost-effectiveness of managing familial hypercholesterolemia using atorvastatin-based preventive therapy]" 09/01/2004 - "Participants were adults with heterozygous familial hypercholesterolemia (HeFH), CHD, or multiple (> or =2) cardiovascular risk factors, and a LDL-C level > or =130 mg/dL after a 6- to 10-week dietary stabilization and atorvastatin (10 mg/day) open-label run-in period. " 08/01/2003 - "Fifty-five patients with non-familial hypercholesterolemia were randomized to treatment with atorvastatin 10 mg/day or placebo for 3 months. " Order ALL the reference details at left...
4.	LDL CholesterolIBA 02/01/1977 - "Dietary therapy in the 2- to 7-year-old children heterozygous for familial hypercholesterolemia provided mean overall reduction of total and Ldl cholesterol levels between 6% and 15%, and appeared to be the most effective when instituted in children at ages 1 or 2." 11/01/2004 - "Statins are not only effective in reducing low-density lipoprotein cholesterol levels in children with familial hypercholesterolemia but also improve endothelial function and reduce the progressive thickening of the intima media complex of the carotid arteries. " 02/01/2004 - "In a second study, GH dose-dependently reduced LDL cholesterol and increased Lp(a) levels in 3 groups of males: younger and elderly healthy subjects and heterozygous familial hypercholesterolemia (FH). " 07/01/2009 - "Thus, by studying for the first time the impact of PCSK9 polymorphism on LDL-cholesterol levels of FH patients carrying a same LDLR mutation, we show that PCSK9 might constitute a modifier gene in familial hypercholesterolemia." 04/01/2009 - "INTRODUCTION: Familial hypercholesterolemia (FH) is characterized by exposure to severely elevated LDL-cholesterol from birth, which produces lipid deposits, which can be measured by means of intima-media thickness (IMT). " Order ALL the reference details at left...
5.	Lovastatin (Mevacor)FDA LinkGeneric 05/01/1989 - "In this study, lovastatin was a well tolerated and effective agent for the treatment of non familial and heterozygous familial hypercholesterolemia." 11/01/1987 - "CONCLUSION: Lovastatin was well tolerated and effective in the treatment of familial hypercholesterolemia." 09/01/2005 - "CONCLUSIONS: Lovastatin offers an efficacious and well-tolerated treatment option for improving lipid profiles in adolescent girls with familial hypercholesterolemia." 09/01/1986 - "Comparative efficacy of once versus twice daily mevinolin in the therapy of familial hypercholesterolemia." 09/01/2005 - "OBJECTIVE: The present study was designed to evaluate the lipid-altering efficacy, safety, and tolerability of lovastatin treatment in adolescent girls with heterozygous familial hypercholesterolemia. " Order ALL the reference details at left...
6.	Simvastatin (Zocor)FDA LinkGeneric 02/01/1989 - "Simvastatin seems to be an effective and safe drug as monotherapy in the treatment of heterozygous familial hypercholesterolemia." 12/18/2002 - "OBJECTIVES: This study was designed to determine whether simvastatin improves endothelial function in children with familial hypercholesterolemia (FH). " 10/22/2002 - "Efficacy and safety of statin therapy in children with familial hypercholesterolemia: a randomized, double-blind, placebo-controlled trial with simvastatin." 07/15/2002 - "Two-year efficacy and safety of simvastatin 80 mg in familial hypercholesterolemia (the Examination of Probands and Relatives in Statin Studies With Familial Hypercholesterolemia [ExPRESS FH])." 01/01/1999 - "This study was a 1-year clinical study on 16 (7 males and 9 females) pediatric patients with heterozygous familial hypercholesterolemia treated with hypocholesterolemic diet only, or with diet plus drug (simvastatin 10 mg/day). " Order ALL the reference details at left...
7.	LDL Receptors (LDL Receptor)IBA 03/01/2007 - "BACKGROUND: Phenocopies of homozygous familial hypercholesterolemia (hoFH) having autosomal recessive inheritance, were recently found to arise from defects in the LDL receptor (LDLR) adapter protein, called ARH, which facilitates the clearance of circulating LDL. " 12/01/2002 - "Familial hypercholesterolemia is an autosomal dominant disorder with a gene-dosage effect that is usually caused by mutations in the LDL receptor gene that disrupt normal clearance of LDL. " 11/01/1998 - "In view of the presence of some 190 mutations in the low density lipoprotein receptor (LDL-R) gene and a lack of simple detection methods, we have developed an improved assay system for detecting familial hypercholesterolemia (FH) using mitogen-induced proliferating lymphocytes. " 11/01/1998 - "Improved detection of familial hypercholesterolemia by determining low density lipoprotein receptor expression in mitogen-induced proliferating lymphocytes." 02/01/2009 - "BACKGROUND: The objective of this study was to develop a new and simple method for measuring low-density lipoprotein receptor (LDLR) activity using peripheral lymphocytes enabling us to clinically diagnose familial hypercholesterolemia (FH) and ascertain the involved mutations (such as K790X mutation), that might not be clearly detected in the conventional method. " Order ALL the reference details at left...
8.	Pravastatin (Pravachol)FDA LinkGeneric 05/01/1996 - "The safety, tolerability, and efficacy of a 12-wk treatment with pravastatin, 5, 10, and 20 mg/d, was evaluated in 72 children with heterozygous familial hypercholesterolemia (FH) in a double-blind, randomized and placebo-controlled study. " 06/01/2008 - "METHOD: Trials have shown that treatment with pravastatin is safe in older patients as well as in children with familial hypercholesterolemia. " 08/07/2007 - "BACKGROUND: We previously demonstrated in a randomized placebo-controlled trial that 2-year pravastatin treatment induced a significant regression of carotid intima-media thickness (IMT) in 8- to 18-year-old children with familial hypercholesterolemia. " 04/01/1993 - "We conducted a double-blind, placebo controlled study of pravastatin in 51 patients with familial hypercholesterolemia (FH) (n = 43) or probable FH (n = 8). " 04/01/2005 - "Efficacy and safety of pravastatin in children and adolescents with heterozygous familial hypercholesterolemia: a prospective clinical follow-up study." Order ALL the reference details at left...
9.	Colestipol (Colestid)FDA LinkGeneric 12/01/1992 - "We examined the growth of 30 children with familial hypercholesterolemia, some of whom were also treated with colestipol, a bile acid-binding resin. " 03/04/1992 - "[Colestipol in the treatment of heterozygous familial hypercholesterolemia]" 01/01/1982 - ""Low dose" colestipol in children, adolescents and young adults with familial hypercholesterolemia." 01/01/1975 - "[Long term treatment of familial hypercholesterolemia with Colestipol, a new anionic exchange resin (author's transl)]" 09/01/1980 - "[Results of 6 months, therapy of familial hypercholesterolemia (hyperlipoproteinemia type II) with colestipol]" Order ALL the reference details at left...
10.	HDL CholesterolIBA 10/01/2005 - "The objective of this study was to determine the extent to which common genetic variants can explain the variation of high-density lipoprotein cholesterol (HDL-C) plasma levels in familial hypercholesterolemia (FH). " 01/01/1992 - "Patients with familial hypercholesterolemia have a progression/regression ratio of between 3 and 7. There is a consistent relationship between the progression/regression ratio and the LDL-/HDL-cholesterol ratio in both control and intervention groups in these trials. " 01/01/2006 - "Different genes and polymorphisms affecting high-density lipoprotein cholesterol levels in Greek familial hypercholesterolemia patients." 10/01/2005 - "Genetic determinants of plasma HDL-cholesterol levels in familial hypercholesterolemia." 01/01/2002 - "HDL cholesterol levels in patients with molecularly defined familial hypercholesterolemia." Order ALL the reference details at left...

Therapies and Procedures

1.	Blood Component Removal (Apheresis) 01/01/2001 - "CONCLUSIONS: At present, LDL-apheresis is the most effective and safe therapeutic approach to the treatment of homozygous, heterozygous and double heterozygous familial hypercholesterolemia." 08/01/2003 - "The present data demonstrate that treatment with LDL apheresis of patients suffering from familial hypercholesterolemia resistant to maximum conservative therapy is very effective and safe even in long-term application." 02/01/2002 - "The present data demonstrate that treatment with LDL apheresis of patients suffering from familial hypercholesterolemia resistant to maximum conservative therapy is very effective and safe even in long-term application." 09/01/1999 - "The present data demonstrate that treatment with LDL-apheresis of patients suffering from familial hypercholesterolemia resistant to maximum conservative therapy is very effective and safe, even in long-term application." 02/01/1997 - "The present data clearly demonstrate that treatment with LDL apheresis of patients suffering from familial hypercholesterolemia, resistant to maximum conservative therapy, is very effective and safe, even in long-term application." Order ALL the reference details at left...
2.	Plasma Exchange 01/01/1985 - "Several non-pharmacological treatments have been tested for homozygous familial hypercholesterolemia and, among these, plasma-exchange has been the most safe and effective. " 11/01/1985 - "Repeated plasma exchange is an effective treatment for young patients with familial hypercholesterolemia. " 07/01/1998 - "BACKGROUND: The use of plasma-exchange therapy has increased the longevity of patients with homozygous familial hypercholesterolemia (HFH), and pregnancy in affected women is expected to become more common. " 02/01/1993 - "Familial hypercholesterolemia treated by plasma exchange and immunoadsorption." 01/01/1990 - "Two patients with familial hypercholesterolemia were treated by long-term plasma exchange via an arteriovenous Cimino shunt using unselective membrane separation. " Order ALL the reference details at left...
3.	Drug Therapy (Chemotherapy) 01/01/1994 - "This system was evaluated in a nine-center controlled study of patients with familial hypercholesterolemia (FH) who had not responded adequately to diet and drug therapy. " 08/01/1997 - "We studied 11 patients with familial hypercholesterolemia (FH), 2 with homozygous FH and 9 with severe heterozygous FH who received combined LA and drug therapy for a mean of 7.7 years. " 01/01/1997 - "There are now several efficient and safe extracorporeal LDL eliminating procedures for the treatment of patients with familial hypercholesterolemia (FH) resistant to diet and combined drug therapy. " 09/30/1993 - "How effective is drug therapy in heterozygous familial hypercholesterolemia?" 01/01/1988 - "Drug therapy in children and adolescents with familial hypercholesterolemia." Order ALL the reference details at left...
4.	Diet Therapy (Therapy, Diet) 05/01/1987 - "[Changes in the fatty acid composition of erythrocyte and platelet membranes after diet therapy in patients with hyperlipoproteinemia type II]" 08/30/1992 - "[Familial hypercholesterolemia--intensive diet therapy combined with drug therapy]" 04/01/1990 - "These results indicate that diet therapy with free-cholesterol and a high ratio of P/S is highly effective in controlling plasma cholesterol levels in heterozygous familial hypercholesterolemia." 04/01/1990 - "We have studied the effect of diet therapy on plasma lipoprotein metabolism in heterozygous familial hypercholesterolemia. " 07/01/1984 - "For individuals with severe lipoprotein disorders such as familial hypercholesterolemia where diet therapy is helpful but not adequate, the use of medications is now indicated (bile acid binding resins and nicotinic acid). " Order ALL the reference details at left...
5.	Surgical Portacaval Shunt (Portacaval Anastomosis) 05/01/1996 - "BACKGROUND: Portacaval anastomosis has an hypolipemic effect in familial hypercholesterolemia and in healthy animals. " 09/01/1986 - "We report the cases of two siblings, both suffering from familial hypercholesterolemia in homozygotic form, followed up before and after portacaval shunt. " 09/01/1986 - "[Portacaval anastomosis in 2 cases of homozygote familial hypercholesterolemia]" 12/01/1985 - "Since 1973 the portacaval shunt has been used as a treatment for homozygous familial hypercholesterolemia. " 02/15/1985 - "In addition, hepatic membranes from a patient with familial hypercholesterolemia were studied and compared before and after portacaval shunt surgery. " Order ALL the reference details at left...