RM IDTitleAuthor(s)YearJournalQuestionReason(s) for exclusion
2380Diagnosis of familial hypercholesterolaemia using DNA probes for the low-density lipoprotein (LDL) receptor gene.Armston AE; Iversen SA; Burke JF;1988Annals of Clinical Biochemistry1This is an old study which reflects early development of DNA and is not relevant to current practice.
270The relationship among apolipoprotein(a) polymorphisms, the low-density lipoprotein receptor-related protein, and the very low density lipoprotein.Benes P; Muzik J; Benedik J; Znojil V; Vacha J;2002Human Biology1Does not answer question
1169Linkage Between Familial Hypercholesterolemia with Xanthomatosis and the C3- Polymorphism Confirmed.Berg K; Heiberg A;1978Cytogenetics and Cell Genetics1No population data
5221Genetic polymorphisms affecting the phenotypic expression of familial hypercholesterolemia.Bertolini S; Pisciotta L; Di S; Langheim S; Bellocchio A; Masturzo P; Cantafora A; Martini S; Averna M; Pes G; Stefanutti C; Calandra S;2004Atherosclerosis1Within DNA positive group comparison
1550Diagnosis and screening for familial hypercholesterolaemia, Finding the patients, finding the genes.Bhatnagar D;2006Annals of Clinical Biochemistry1This is a narrative review
103LDL receptor mutation genotype and vascular disease phenotype in heterozygous familial hypercholesterolaemia.Brorholt-Petersen JU; Jensen HK; Jensen JM; Refsgaard J; Christiansen T; Hansen LB; Gregersen N; Faergeman O;2002Clinical Genetics1Did not answer question, wrong outcome measure
2602Familial hypercholesterolemia, genetic, biochemical and pathophysiologic considerations.Brown MS; Goldstein JL;1975Advances in Internal Medicine1Narrative review
1548Clinical features of familial hypercholesterolemia in Japan in a database from 1996–1998 by the research committee of the ministry of health, labour and welfare of Japan.Bujo H; Takahashi K; Saito Y; Maruyama T; Yamashita S; Matsuzawa Y; Ishibashi S; Shionoiri F; Yamada N; Kita T; Research C;2004Journal of Atherosclerosis & Thrombosis1Not a direct comparison of DNA positive and negative participants
1553Detection of familial hypercholesterolemia in a cohort of children with hypercholesterolemia, Results of a family and DNA-based screening.Campagna F; Martino F; Bifolco M; Montali A; Martino E; Morrone F; Antonini R; Cantafora A; Verna R; Arca M;2006Atherosclerosis1Did not answer question 1 - this is a study about screening
1126Coexisting dysbetalipoproteinemia and familial hypercholesterolemia, Clinical and laboratory observations.Carmena R; Roy M; Roederer G; Minnich A; Davignon J;2000Atherosclerosis1Within DNA positive group comparison
316Association between the TaqIB polymorphism in the cholesteryl ester transfer protein gene locus and plasma lipoprotein levels in familial hypercholesterolemia.Carmena-Ramon R; Ascaso JF; Real JT; Najera G; Ordovas JM; Carmena R;2001Metabolism, Clinical & Experimental1Within DNA positive group comparison
214A common variant in the ABCA1 gene is associated with a lower risk for premature coronary heart disease in familial hypercholesterolaemia.Cenarro A; Artieda M; Castillo S; Mozas P; Reyes G; Tejedor D; Alonso R; Mata P; Pocovi M; Civeira F; Spanish FH;2003Journal of Medical Genetics1Within DAN positive group study
1783Improved detection of familial hypercholesterolemia by determining low density lipoprotein receptor expression in mitogen-induced proliferating lymphocytes.Chan PC; Edwards A; Lafreniere R; Parsons HG;1998Journal of Lipid Research1This is a review of an assay which is expensive and time consuming and will not be considered in this review
62Guidelines for the diagnosis and management of heterozygous familial hypercholesterolemia.Civeira F; International P;2004Atherosclerosis1These guidelines do not provide the method of review but offer a good summary and background
62Guidelines for the diagnosis and management of heterozygous familial hypercholesterolemia.Civeira F; International P;2004Atherosclerosis1Method of review not provided
1549FH clinical phenotype in Greek patients with LDL-R defective vs. negative mutations.Dedoussis GV; Skoumas J; Pitsavos C; Choumerianou DM; Genschel J; Schmidt H; Stefanadis C;2004European Journal of Clinical Investigation1Not a direct comparison of DNA positive and negative participants
778Different genes and polymorphisms affecting high- density lipoprotein cholesterol levels in Greek familial hypercholesterolemia patients.Dedoussis GVZ; Maumus S; Choumerianou DM; Skoumas J; Pitsavos C; Stefanadis C; Visvikis-Siest S;2002Genetic Testing1Not a direct comparison of DNA positive and negative participants. Did not answer question.
5222DNA polymorphisms of the apolipoprotein B gene (XbaI, EcoRI, and MspI RFLPs) in Norwegians at risk of atherosclerosis and healthy controls.Delghandi M; Thangarajah R; Nilsen M; Grimsgaard S; Bonaa KH; Tonstad S; Jorgensen L;1999Acta Cardiologica1FH population not analysed separately
53Update of the molecular basis of familial hypercholesterolemia in The Netherlands.Fouchier SW; Kastelein JJ; Defesche JC;2005Human Mutation1Within DNA positive group comparison
2438The diagnosis and management of hyperlipidemia.Gotto AMJ; Jones PH; Scott LW;1986Disease-A-Month1Narrative review
1544Genotype of the mutant LDL receptor allele is associated with LDL particle size heterogeneity in familial hypercholesterolemia.Hogue JC; Lamarche B; Gaudet D; Tremblay AJ; Despres JP; Gagne C; Couture P;2006Atherosclerosis1Not a direct comparison of DNA positive and negative participants
2630Studies in essential hypercholesterolemia and xanthomatosis, relationships between age, sex, cholesterol concentrations in plasma fractions, and size of tendinous deposits.Hood B; Angervall G;1959American Journal of Medicine1This is not an FH specific population
1702Type-Iii Dyslipoproteinemia in Patients Heterozygous for Familial Hypercholesterolemia and Apolipoprotein-E2 - Evidence for A Gene Gene Interaction.Hopkins PN; Wu LL; Schumacher MC; Emi M; Hegele RM; Hunt SC; Lalouel JM; Williams RR;1991Arteriosclerosis & Thrombosis1DNA testing not done in all participants
1928Genetic diagnosis of familial hypercholesterolemia in affected relatives using pedigree tracing.Hsia SH; Connelly PW; Hegele RA;1996Clinical Biochemistry1Case study
2428Familial hypercholesterolaemia as an example of early diagnosis of coronary artery disease risk by DNA techniques.Humphries SE;1986British Heart Journal1This is an editorial
1710The Application of Molecular- Biology Techniques to the Diagnosis of Hyperlipidaemia and Other Risk-Factors for Cardiovascular-Disease.Humphries SE;1993Clinica Chimica Acta1Narrative for background use only
1883Genetic testing for familial hypercholesterolaemia, practical and ethical issues.Humphries SE; Galton D; Nicholls P;1997Quarterly Journal of Medicine1Narrative review
1542Genetic causes of familial hypercholesterolaemia in patients in the UK, relation to plasma lipid levels and coronary heart disease risk.Humphries SE; Whittall RA; Hubbart CS; Maplebeck S; Cooper JA; Soutar AK; Naoumova R; Thompson GR; Seed M; Durrington PN; Miller JP; Betteridge DJ; Neil HA; Simon B;2006Journal of Medical Genetics1
228The molecular genetic basis and diagnosis of familial hypercholesterolemia in Denmark.Jensen HK;2002Danish Medical Bulletin1This is a Ph.D. thesis which provides a narrative review of the genotype-phenotype relationship in Danish FH patients and of the clinical versus the DNA diagnosis in Danish FH patient
3315Screening for familial hypercholesterolaemia, effective, safe treatments and DNA testing make screening attractive.Kastelein JJP;2000British Medical Journal1Comment only - not study or trial
24Low-density lipoprotein receptor genotype and response to pravastatin in children with familial hypercholesterolemia, substudy of an intima-media thickness trial.Koeijvoets KC; Rodenburg J; Hutten BA; Wiegman A; Kastelein JJ; Sijbrands EJ;2005Circulation1Not a direct comparison of DNA positive and negative participants
2770Genetic screening of patients with familial hypercholesterolaemia (FH), A New Zealand perspective.Laurie AD; Scott RS; George PM;2004Atherosclerosis Supplements1Description of a programme -letter to the editor
555Apolipoprotein E phenotype and lipoprotein(a) in familial hypercholesterolaemia, implication for lipoprotein(a) metabolism.Lindahl G; Mailly F; Humphries S; Seed M;1994Clinical Investigator1Did not answer question
2460Tracking of high-and low-density-lipoprotein cholesterol from childhood to young adulthood in a single large kindred with familial hypercholesterolemiaMellies MJ; Laskarzewski PM; Tracy T; Glueck CJ;1985Metabolism, Clinical & Experimental1Case study of one family
1923Clinically applicable mutation screening in familial hypercholesterolemia.Nissen H; Guldberg P; Hansen AB; Petersen NE; Horder M;1996Human Mutation1Study does not answer the question - assessment of a screening assay
1800Evaluation of a clinically applicable mutation screening technique for genetic diagnosis of familial hypercholesterolemia and familial defective apolipoprotein B.Nissen H; Hansen AB; Guldberg P; Hansen TS; Petersen NE; Horder M;1998Clinical Genetics1Description of an assay
1714An update on familial hypercholesterolaemia.Ose L;1999Annals of Medicine1Narrative review
438Familial hypercholesterolemia in children.Rodenburg J; Vissers MN; Wiegman A; Trip MD; Bakker HD; Kastelein JJ;2004Current Opinion in Lipidology1Narrative review
1115Screening for A Prevalent LDL Receptor Mutation in Patients with Severe Hypercholesterolemia.Savolainen MJ; Korhonen T; Aaltosetala K; Kontula K; Kesaniemi YA;1991Human Genetics1Study of DNA assay. Out of date paper
3317High risk/high priority, familial hypercholesterolemia--a paradigm for molecular medicine.Schuster H;2002Atherosclerosis Supplements1Narrative review
1250Familial hypercholesterolemia, a challenge of diagnosis and therapy.Sibley C; Stone NJ;2006Cleveland Clinic Journal of Medicine1Narrative review
1489DNA testing for familial hypercholesterolemia, improving disease recognition and patient care.Vergopoulos A; Knoblauch H; Schuster H;2002American Journal of PharmacoGenomics1Narrative review
3331Familial hypercholesterolaemia in Finland, common, rare and mild mutations of the LDL receptor and their clinical consequences. Finnish FH-group.Vuorio AF; alto-Setala K; Koivisto UM; Turtola H; Nissen H; Kovanen PT; Miettinen TA; Gylling H; Oksanen H; Kontula K;2001Annals of Medicine1Not a direct comparison of DNA positive and negative participants
2105Genetic basis of familial dyslipidemia and hypertension, 15-year results from Utah.Williams RR; Hunt SC; Hopkins PN; Wu LL; Hasstedt SJ; Berry TD; Barlow GK; Stults BM; Schumacher MC; Ludwig EH; et a;1993American Journal of Hypertension1This paper discusses the general risk for HTN as an outcome
5327Corneal arcus, case finding and definition of individual clinical risk in heterozygous familial hypercholesterolaemia.Winder AF; Jolleys JC; Day LB; Butowski PF;1998Clinical Genetics1Study not written in English
1558Relation between coronary artery disease, risk factors and intima-media thickness of carotid artery, arterial distensibility, and stiffness indexAlan S; Ulgen MS; Ozturk O; Alan B; Ozdemir L; Toprak N;2003Angiology2Not FH population
439Families with familial combined hyperlipidemia and families enriched for coronary artery disease share genetic determinants for the atherogenic lipoprotein phenotypeAllayee H; Aouizerat BE; Cantor RM; linga- Thie GM; Krauss RM; Lanning CD; Rotter JI; Lusis AJ; de B;1998American Journal of Human Genetics2Not FH population
2612High ‘population attributable fraction’ for coronary heart disease mortality among relatives in monogenic familial hypercholesterolemiaAustin MA; Zimmern RL; Humphries SE;2002Genetics in Medicine2Does not answer the question
318The C766T low-density lipoprotein receptor related protein polymorphism and coronary artery disease, plasma lipoproteins, and longevity in the Czech populationBenes P; Muzik J; Benedik J; Elbl L; Vask A; Siskova L; Znojil V; Vacha J;2001Journal of Molecular Medicine2Does not answer question
678Risk factor variability and coronary heart disease.Berg K;1990Acta Geneticae Medicae et Gemellologiae2Report, not a trial
394P1A1/A2 polymorphism of platelet glycoprotein IIIa and risk of acute coronary syndromes in heterozygous familial hypercholesterolemiaCenarro A; Casao E; Civeira F; Jensen HK; Faergeman O; Pocovi M;1999Atherosclerosis2Study within DNA positive group
1545Tendon xanthomas in familial hypercholesterolemia are associated with cardiovascular risk independently of the low- density lipoprotein receptor gene mutationCiveira F; Castillo S; Alonso R; Merino-Ibarra E; Cenarro A; Artied M; Martin-Fuentes P; Ros E; Pocovi M; Mata P; Spanish F;2005Arteriosclerosis, Thrombosis & Vascular Biology2DNA testing not done in all participants
3297Family history of coronary heart disease, evidence-based applicationsCrouch MA; Gramling R;2005Primary Care, Clinics in Office Practice2Narrative review
3288Family history of cardiovascular events and endothelial dysfunction in children with familial hypercholesterolemiade Jongh S; Lilien MR; Bakker HD; Hutten BA; Kastelein JJ; Stroes ES;2002Atherosclerosis2Not all participants DNA tested
1465Prevalence and significance of cardiovascular risk factors in a large cohort of patients with familial hypercholesterolaemiade Sauvage Nolting PR; Defesche JC; Buirma RJ; Hutten BA; Lansberg PJ; Kastelein JJ;2003Journal of Internal Medicine2This study did not provide a comparison between mutation positive and mutation negative individuals
1369Advanced method for the identification of patients with inherited hypercholesterolemia.Defesche JC; Lansberg PJ; Umans- Eckenhausen MA; Kastelein JJ;2004Seminars in Vascular Medicine2Description of a model
2736Genetic tests may have additional predictive power over and above the accepted risk factors in patients with severe hypercholesterolemiaDescamps OS;2005Louvain Medical2Not RCT
2002Coronary artery disease in heterozygous familial hypercholesterolemia patients with the same LDL receptor gene mutationFerrieres J; Lambert J; Lussier-Cacan S; Davignon J;1995Circulation2DNA testing not done in all participants
1762LDL receptor mutations and ApoB mutations are not risk factors for ischemic cerebrovascular disease of the young, but lipids and lipoproteins areFrikke-Schmidt R; rlien-Soborg P; Thorsen S; Jensen HK; Vorstrup S;1999European Journal of Neurology2No FH patients
1741Ethical issues in molecular screening for heterozygous familial hypercholesterolemia, the complexity of dealing with genetic susceptibility to coronary artery diseaseGaudet D; Gagne C; Perron P; Couture P; Tonstad S;1999Community Genetics2Does not answer the question asked
393Contribution of receptor negative versus receptor defective mutations in the LDL-receptor gene to angiographically assessed coronary artery disease among young (25–49 years) versus middle-aged (50–64 years) menGaudet D; Vohl MC; Couture P; Moorjani S; Tremblay G; Perron P; Gagne C; Despres JP;1999Atherosclerosis2Not all participants tested for mutations
5223Relative contribution of low-density lipoprotein receptor and lipoprotein lipase gene mutations to angiographically assessed coronary artery disease among French CanadiansGaudet D; Vohl MC; Julien P; Tremblay G; Perron P; Gagne C; Bergeron J; Moorjani S; Despres JP;1998American Journal of Cardiology2Within DNA positive group comparison
666Genetic and environmental factors affecting the incidence of coronary artery disease in heterozygous familial hypercholesterolemiaHill JS; Hayden MR; Frohlich J; Pritchard PH;1991Arteriosclerosis & Thrombosis2Patients not DNA tested
1273Lipoprotein(a) is an independent risk factor for cardiovascular disease in heterozygous familial hypercholesterolemiaHolmes DT; Schick BA; Humphries KH; Frohlich J;2005Clinical Chemistry2Did not answer question
1611Evaluation of coronary risk factors in patients with heterozygous familial hypercholesterolemiaHopkins PN; Stephenson S; Wu LL; Riley WA; Xin Y; Hunt SC;2001American Journal of Cardiology2DNA testing not done in all participants
2428Familial hypercholesterolaemia as an example of early diagnosis of coronary artery disease risk by DNA techniquesHumphries SE;1986British Heart Journal2This is an editorial
99Mutational analysis in UK patients with a clinical diagnosis of familial hypercholesterolaemia, relationship with plasma lipid traits, heart disease risk and utility in relative tracingHumphries SE; Cranston T; Allen M; Middleton PH; Fernandez MC; Senior V; Hawe E; Iversen A; Wray R; Crook MA; Wierzbicki AS;2006Journal of Molecular Medicine (Berlin Germany)2Does not answer question asked
82Genetic determinants of cardiovascular disease risk in familial hypercholesterolemiaJansen AC; Van AC; Tanck MW; Cheng S; Fontecha MR; Li J; Defesche JC; Kastelein JJ;2005Arteriosclerosis, Thrombosis & Vascular Biology2
5224Effect of common methylenetetrahydrofolate reductase gene mutation on coronary artery disease in familial hypercholesterolemiaKawashiri M; Kajinami K; Nohara A; Yagi K; Inazu A; Koizumi J; Mabuchi H;2000American Journal of Cardiology2Not a direct comparison of DNA positive and negative participants
368Paraoxonase gene polymorphisms are associated with carotid arterial wall thickness in participants with familial hypercholesterolemiaLeus FR; Wittekoek ME; Prins J; Kastelein JJ; Voorbij HA;2000Atherosclerosis2Does not answer the question, not a direct comparison of DNA positive and negative participants
325PON2 gene variants are associated with clinical manifestations of cardiovascular disease in familial hypercholesterolemia patientsLeus FR; Zwart M; Kastelein JJ; Voorbij HA;2001Atherosclerosis2Not a direct comparison of DNA positive and negative participants
198Apolipoprotein E genotype is not associated with cardiovascular disease in heterozygous participants with familial hypercholesterolemiaMozas P; Castillo S; Reyes G; Tejedor D; Civeira F; Garcia-Alvarez I; Puzo J; Cenarro A; Alonso R; Mata P; Pocovi M; Spanish g;2003American Heart Journal2Did not answer the question
3316Prevalence and significance of cardiovascular risk factors in a large cohort of patients with familial hypercholesterolaemiaNolting PRWD; Defesche JC; Buirma RJA; Hutten BA; Lansberg PJ; Kastelein JJP;2003Journal of Internal Medicine2Outcome measures were not those identified by GDG
1445Importance of LDL/HDL cholesterol ratio as a predictor for coronary heart disease events in patients with heterozygous familial hypercholesterolaemia, a 15-year follow-up (1987–2002)Panagiotakos DB; Pitsavos C; Skoumas J; Chrysohoou C; Toutouza M; Stefanadis CI; Toutouzas PK;2002Current Medical Research & Opinion2DNA testing not done in all participants
106Coronary artery disease prevention in childhood, Results of management of children with familial hypercholesterolemiaRose V; Cullen-Dean G; Regelink-Helden E; Kay-Soroka S;1991Annals of the New York Academy of Sciences2Does not answer question
1081Coronary Risk-Factors and the Severity of Angiographic Coronary-Artery Disease in Members of High-Risk PedigreesSharp SD; Williams RR; Hunt SC; Schumacher MC;1992American Heart Journal2DNA testing not done in all participants
1575Genetic and metabolic factors predicting risk of cardiovascular disease in familial hypercholesterolemia. [Review] [125 refs]Smilde TJ; van W; Wollersheim H; Kastelein JJ; Stalenhoef AF;2001Netherlands Journal of Medicine2Narrative review
1552Influence of LDL-receptor mutation type on age at first cardiovascular event in patients with familial hypercholesterolaemiaSouverein OW; Defesche JC; Zwinderman AH; Kastelein JJ; Tanck MW;2006European Heart Journal2Does not answer the question
3318Identification and treatment of individuals at high risk of coronary heart disease. [Review] [30 refs]Stein EA;2002American Journal of Medicine2Narrative review
2575Plasma high-density lipoproteins and ischemic heart disease, studies in a large kindred with familial hypercholesterolemiaStreja D; Steiner G; Kwiterovich POJ;1978Annals of Internal Medicine2DNA testing not done in all participants
1098Risk of Fatal Coronary Heart-Disease in Familial HypercholesterolemiaThorogood M;1991British Medical Journal2DNA testing not done in all participants
1454Family history and cardiovascular risk in familial hypercholesterolemia, data in more than 1000 childrenWiegman A; Rodenburg J; de J; Defesche JC; Bakker HD; Kastelein JJ; Sijbrands EJ;2003Circulation2Patients were not all DNA tested
5283Efficacy and safety of cholesterol-lowering treatment, prospective meta-analysis of data from 90,056 participants in 14 randomised trials of statinsBaigent C; Keech A; Kearney PM; Blackwell L; Buck G; Pollicino C; Kirby A; Sourjina T; Peto R; Collins R; Simes R;2005Lancet3Used for background only
1550Diagnosis and screening for familial hypercholesterolaemia, Finding the patients, finding the genesBhatnagar D;2006Annals of Clinical Biochemistry3Narrative review
2409Variables affecting apolipoprotein B measurements in 3- to 5-day-old babies, a study of 4491 neonatesBlades BL; Dudman NP; Wilcken DE;1987Pediatric Research3Not relevant population
2376Screening for familial hypercholesterolemia in 5000 neonates, a recall studyBlades BL; Dudman NP; Wilcken DE;1988Pediatric Research3Not relevant population
1369Advanced method for the identification of patients with inherited hypercholesterolemia.Defesche JC; Lansberg PJ; Umans-Eckenhausen MA; Kastelein JJ;2004Seminars in Vascular Medicine3Narrative review
5284A comprehensive description of muscle symptoms associated with lipid-lowering drugsFranc S; Dejager S; Bruckert E; Chauvenet M; Giral P; Turpin G;2003Cardiovascular Drugs and Therapy3Used for background information only
26Implementation of cascade testing for the detection of familial hypercholesterolaemiaHadfield SG; Humphries SE;2005 AugCurrent Opinion in Lipidology3Narrative review
5287Genetic screening by DNA technology, a systematic review of health economic evidence.Rogowski W;2006International Journal of Technology Assessment in Health Care3See health economics review
5288Systematic family screening for familial hypercholesterolemia in IcelandThorsson B; Sigurdsson G; Gudnason V;2003Arteriosclerosis, Thrombosis & Vascular Biology3Does not answer the question
181Screening for familial hypercholesterolaemia in relativesTonstad S; Vollebaek LE; Ose L;1995Lancet3Narrative review
5380Child-parent screening for familial hypercholesterolaemia, screening strategy based on a meta-analysisWald DS; Bestwick JP; Wald NJ;2007British Medical Journal3Does not answer our question-the FH guideline is not evaluating population screening
217Prevention of familial cardiovascular disease by screening for family history and lipids in youthsWilliams RR; Hunt SC; Barlow GK; Wu LL; Hopkins PN; Schumacher MC; Hasstedt SJ; Ware J; Chamberlain RM; Weinberg AD; et a;1992Clinical Chemistry3Does not answer the question
2116Documented need for more effective diagnosis and treatment of familial hypercholesterolemia according to data from 502 heterozygotes in UtahWilliams RR; Schumacher MC; Barlow GK; Hunt SC; Ware JL; Pratt M; Latham BD;1993American Journal of Cardiology3Not RCT
5289A World Wide Web site for low-density lipoprotein receptor gene mutations in familial hypercholesterolemia, sequence-based, tabular, and direct submission data handlingWilson DJ; Gahan M; Haddad L; Heath K; Whittall RA; Williams RR; Humphries SE; Day IN;1998American Journal of Cardiology3Does not answer the question
5330Familial hypercholesterolemia, ethical, practical and psychological problems from the perspective of patientsAgård A; Bolmsjö IA; Hermerén G; Wahlstöm J;2005Patient Education and Counseling6Outcomes did not answer the question
62Guidelines for the diagnosis and management of heterozygous familial hypercholesterolemia.Civeira F; International P;2004Atherosclerosis6Method of review not provided
1138Quality of life, anxiety and concerns among statin-treated children with familial hypercholesterolaemia and their parentsde Jongh S; Kerckhoffs MC; Grootenhuis MA; Bakker HD; Heymans HSA; Last BF;2003Acta Paediatrica6Does not answer the question
1741Ethical issues in molecular screening for heterozygous familial hypercholesterolemia, the complexity of dealing with genetic susceptibility to coronary artery diseaseGaudet D; Gagne C; Perron P; Couture P; Tonstad S;1999Community Genetics6Narrative review
954A genetic screening programme for familial hypercholesterolaemia in the RSA, Results and recommendationsHitzeroth HW; Op TH;1986South African Medical Journal6Narrative review
1232Disease knowledge and adherence to treatment in patients with familial hypercholesterolemiaHollman G; Olsson AG; Ek AC;2006Journal of Cardiovascular Nursing6
369Effects of a soy-protein beverage on plasma lipoproteins in children with familial hypercholesterolemiaLaurin D; Jacques H; Moorjani S; Steinke FH; Gagné C; Brun D; Lupien PJ;1991American Journal of Clinical Nutrition6In a systematic review on diet in children with FH
144Getting insurance after genetic screening on familial hypercholesterolaemia, the need to educate both insurers and the public to increase adherence to national guidelines in the NetherlandsMarang-van de Mheen PJ; Van Maarle MC; Stouthard MEA;2002Journal of Epidemiology & Community Health6Not relevant population - Dutch study
568Psychological impact of genetic testing for familial hypercholesterolemia within a previously aware population, A randomized controlled trialMarteau T; Senior V; Humphries SE; Bobrow M; Cranston T; Crook MA; Day L; Fernandez M; Horne R; Iversen A; Jackson Z; Lynas J; Price H; Savine R; Sikorski J; Watson M; Weinman J; Wierzbicki AS; Wray R;2004American Journal of Medical Genetics6Does not answer the question
438Familial hypercholesterolemia in children.Rodenburg J; Vissers MN; Wiegman A; Trip MD; Bakker HD; Kastelein JJ;2004Current Opinion in Lipidology6Narrative review which included psychological considerations
527Treatment of familial hypercholesterolaemia in childrenSegall MM; Fosbrooke AS; Lloyd JK; Wolff OH;1970Lancet6Case series 1970
5342Guidelines for the diagnosis and management of familial hypercholesterolaemiaSullivan D;2007Heart Lung and Circulation6Methodology not explained
139Stratification of risk in children with familial hypercholesterolemia with focus on psychosocial issuesTonstad S;2001Nutrition Metabolism and Cardiovascular Diseases6Does not answer the question
263Psychosocial function during treatment for familial hypercholesterolemiaTonstad S; Nøvik TS; Vandvik IH;1996Pediatrics6Outcomes did not answer the question
125Long-term compliance with lipid-lowering medication after genetic screening for familial hypercholesterolemiaUmans-Eckenhausen-Marina AW; Defesche JC; van-Dam MJ; Kastelein-John JP;2003Archives of Internal Medicine6Does not answer the question
5361Family communication regarding inherited high cholesterol, why and how do patients disclose genetic risk?van den Nieuwenhoff HW; Mesters I; Gielen C; de Vries NK;2007Social Science and Medicine6Does not answer the question
32The importance of written information packages in support of case-finding within families at risk for inherited high cholesterolvan den Nieuwenhoff HWP; Mesters I; Nellissen-Joyce JTM; Stalenhoef AF; de Vries NK;2006Journal of Genetic Counseling6Does not answer the question
1582Prenatal diagnosis of familial hypercholesterolemia, importance of DNA analysis in the high-risk South African populationVergotine J; Thiart R; Langenhoven E; Hillermann R; De J; Kotze MJ;2001Genetic Counseling6Does not answer the question
137Baseline lipid values partly determine the response to high-dose simvastatin in patients with familial hypercholesterolemia. The examination of probands and relatives in Statin studies with familial hypercholesterolemia (ExPRESS FH)de Sauvage Nolting PR; Buirma RJ; Hutten BA; Kastelein JJ; Dutch ExPRESS investigators Group;2002Atherosclerosis7Not RCT
1068Efficacy and Safety of Once-Daily Vs Twice-Daily Dosing with Fluvastatin, A Synthetic Reductase Inhibitor, in Primary HypercholesterolemiaInsull W; Black D; DuJovne C; Hosking JD; Hunninghake D; Keilson L; Knopp R; McKenney J; Stein E; Troendle AJ; Wright JT;1994Archives of Internal Medicine7Does not answer question - not high dose low dose but rather studies dosing schedules
1534Comparative dose efficacy study of atorvastatin versus simvastatin, pravastatin, lovastatin, and fluvastatin in patients with hypercholesterolemia (the CURVES study)Jones P; Kafonek S; Laurora I; Hunninghake D;1998American Journal of Cardiology7Not FH population
395Ten-year follow-up of familial hypercholesterolemia patients after intensive cholesterol-lowering therapyMasaki N; Tatami R; Kumamoto T; Izawa A; Shimada Y; Takamatsu T; Katsushika S; Ishise S; Maruyama Y; Yoshimoto N;2005International Heart Journal7This is a duplicate report of the study in question
1284Comparative Pharmacokinetics and Pharmacodynamics of Pravastatin and LovastatinPan HY; Devault AR; Wangiverson D; Ivashkiv E; Swanson BN; Sugerman AA;1990Journal of Clinical Pharmacology7Primary outcome not LDL level
748High dose atorvastatin induces regression of carotid atherosclerosis in patients with familial hypercholesterolaemia (FH), The atorvastatin versus simvastatin on atherosclerosis progression (ASAP) studySmilde TJ; Trip MD; Wissen S; Wollersheim H; Kastelein JJP; Stalenhoef AFH;2000Circulation7Abstract only
1432Statins, Within-group comparisons, statin escape and combination therapyTikkanen MJ;1996Current Opinion in Lipidology7Narrative review
120Effect of atorvastatin (80 mg) and simvastatin (40 mg) on plasma fibrinogen levels and on carotid intima media thickness in patients with familial hypercholesterolemiaTrip MD; van W; Smilde TJ; Hutten BA; Stalenhoef AF; Kastelein JJ;2003American Journal of Cardiology7Not LDL outcome
1448High dose of simvastatin normalizes postprandial remnant-like particle response in patients with heterozygous familial hypercholesterolemiaTwickler TB; linga-Thie GM; de Valk HW; Schreuder PCNJ; Jansen H; Cabezas MC; Erkelens DW;2000Arteriosclerosis, Thrombosis & Vascular Biology7Not RCT
807Pravastatin and lovastatin similarly reduce serum cholesterol and its precursor levels in familial hypercholesterolaemiaVanhanen H; Miettinen TA;1992European Journal of Clinical Pharmacology7Not RCT
235Atorvastatin compared with simvastatin-based therapies in the management of severe familial hyperlipidaemiasWierzbicki AS; Lumb PJ; Semra Y; Chik G; Christ ER; Crook MA;1999Quarterly Journal of Medicine7Not RCT
241High-dose atorvastatin therapy in severe heterozygous familial hypercholesterolaemiaWierzbicki AS; Lumb PJ; Semra YK; Crook MA;1998Quarterly Journal of Medicine7Not RCT
637Statin use benefits children2004Clinical Advisor8Narrative review
436Rosuvastatin. Opt for statins with evidence of efficacy on clinical outcome2004Prescrire International8Narrative review
593Ezetimibe, new preparation. A cholesterol-lowering drug with no clinical advantage2004Prescrire International8To be deferred pending ezetimibe TA
340Bile acid sequestrants.Ast M; Frishman WH;1990Journal of Clinical Pharmacology8Narrative review
685Relationship between LDL-C and non-HDL-C levels and clinical outcome in the GREek Atorvastatin and Coronary-heart- disease Evaluation (GREACE) studyAthyros VG; Mikhailidis DP; Papageorgiou AA; Symeonidis AN; Daskalopoulou SS; Kakafika AI; Pehlivanidis AN; Bouloukos VI; Langer A;2004Current Medical Research & Opinion8Not FH population
847Effect of atorvastatin on high density lipoprotein cholesterol and its relationship with coronary events, a subgroup analysis of the GREek Atorvastatin and Coronary heart-disease Evaluation (GREACE) StudyAthyros VG; Mikhailidis DP; Papageorgiou AA; Symeonidis AN; Mercouris BR; Pehlivanidis AN; Bouloukos VI; Elisaf M;2004Current Medical Research and Opinion8Not FH patients
397One-year atorvastatin treatment in hypercholesterolemic patients with or without carotid artery diseaseAvellone G; Di Garbo V; Abruzzese G; Campisi D; De Simone R; Raneli G; Licata G;2006International Angiology8Not RCT
5379A systematic review and meta-analysis of statin therapy in children with familial hypercholesterolemiaAvis HJ; Vissers MN; Stein EA; Wijburg FA; Trip MD; Kastelein JP; Hutten BA;2007Arteriosclerosis, Thrombosis & Vascular Biology8This study essentially repeats information in recently published systematic review in the UK by Arambepola et al, 2007
290Efficacy and safety of fluvastatin, a new HMG CoA reductase inhibitor, in elderly hypercholesterolaemic womenBaggio G; De Candia O; Forte PL; Mello F; Andriolli A; Donazzan S; Valerio G; Milani M; Crepaldi G;1994Drugs8Not RCT
5283Efficacy and safety of cholesterol-lowering treatment, prospective meta-analysis of data from 90,056 participants in 14 randomised trials of statinsBaigent C; Keech A; Kearney PM; Blackwell L; Buck G; Pollicino C; Kirby A; Sourjina T; Peto R; Collins R; Simes R;2005Lancet8Used for background only
711Early statin therapy induces complete normalization of endothelial dysfunction in children with familial hypercholesterolemia [abstract]Bakker HD; de JS; Lilien MR; Stroes ESG; Kastelein JJP;2002Journal of Inherited Metabolic Disease8Abstract only
135Familial hypercholesterolaemia, optimum treatment strategies.Ballantyne CM;2002International Journal of Clinical Practice Supplement8Narrative review
511Ezetimibe, Efficacy and safety in clinical trialsBallantyne CM;2002European Heart Journal Supplements8Not our population of homozygous or children
858Apolipoprotein E genotypes and response of plasma lipids and progression-regression of coronary atherosclerosis to lipid-lowering drug therapyBallantyne CM; Herd JA; Stein EA; Ferlic LL; Dunn JK; Gotto AM; Marian AJ;2000Journal of the American College of Cardiology8Not FH patients
97Pharmacotherapy for dyslipidaemia--current therapies and future agents.Bays H; Stein EA;2003Expert Opinion on Pharmacotherapy8Narrative
3320Long-term treatment of severe familial hypercholesterolemia in children, effect of sitosterol and bezafibrateBecker M; Staab D; Von B;1992Pediatrics8Not RCT
45Comparative efficacy and safety of ciprofibrate and sustained-release bezafibrate in patients with type II hyperlipidaemiaBetteridge DJ; O’Bryan-Tear CG;1996Postgraduate Medical Journal8No control in this parallel group study
382National Heart, Lung, and Blood Institute type II Coronary Intervention Study, design, methods, and baseline characteristicsBrensike JF; Kelsey SF; Passamani ER; Fisher MR; Richardson JM; Loh IK; Stone NJ; Aldrich RF; Battaglini JW; Moriarty DJ; Myrianthopoulos MB; Detre KM; Epstein SE; Levy RI;1982Controlled Clinical Trials8Protocol only
1523Long term treatment of familial hypercholesterolemia with Colestipol, a new anionic exchange resinBriani G; Fellin R; Balestrieri P; Baggio G; Baiocchi MR; Crepaldi G;1975Giornale Italiano di Cardiologia8Not RCT
5311Expert commentary, the safety of fibrates in lipid-lowering therapyBrown WV;2007American Journal of Cardiology8Commentary
1524Homozygous familial hypercholesterolemia, novel therapy with ezetimibeBruckert E; Gagne C; Gauder D; Sager P; Ponsonnet D; Lipka L; LeBeaut A; Suresh R; Abreu P; Veltri E;2002Atherosclerosis8Abstract only
136Rosuvastatin.Carswell CI; Plosker GL; Jarvis B;1986Drugs8Narrative review
877Rosuvastatin in the management of hyperlipidemia.Cheng JW;2004Clinical Therapeutics8Narrative - not systematic review
36Familial hypercholesterolemia and response to statin therapy according to LDLR genetic background.Choumerianou DM; Dedoussis GV;2005Clinical Chemistry & Laboratory Medicine8Narrative
34Efficacy and safety of lovastatin therapy in adolescent girls with heterozygous familial hypercholesterolemiaClauss SB; Holmes KW; Hopkins P; Stein E; Cho M; Tate A; Johnson-Levonas AO; Kwiterovich PO;2005Pediatrics8Included in Simon Broome SR
1525Ezetimibe for primary hypercholesterolemiaColantonio LD; Cermignani EC; Ciapponi A;20068Protocol only
3321Niacin Treatment of Hypercholesterolemia in ChildrenColletti RB; Neufeld EJ; Roff NK; Mcauliffe TL; Baker AL; Newburger JW;1993Pediatrics8Not RCT
437Familial combined hyperlipidemia in children, clinical expression, metabolic defects, and management.Cortner JA; Coates PM; Liacouras CA; Jarvik GP;1993Journal of Pediatrics8Not relevant population
218Association of specific LDL receptor gene mutations with differential plasma lipoprotein response to simvastatin in young French Canadians with heterozygous familial hypercholesterolemiaCouture P; Brun LD; Szots F; Lelievre M; Gaudet D; Despres JP; Simard J; Lupien PJ; Gagne C;1998Arteriosclerosis, Thrombosis & Vascular Biology8Did not answer the question. Included in Simon Broome SR
3322Loss of dental enamel in a patient taking cholestyramineCurtis DM; Driscoll DJ; Goldman DH; Weidman WH;1991Mayo Clinic Proceedings8Not RCT
133Combination treatment with cholestyramine and bezafibrate for heterozygous familial hypercholesterolaemiaCurtis LD; Dickson AC; Ling KL; Betteridge J;1988British Medical Journal8No control in parallel group study using six treatment sequences with three patients in each sequence.
743Effect of simvastatin on remnant lipoproteins in heterozygous familial hypercholesterolaemiaDane-Stewart CA; Watts GF; Mamo JCL; Redgrave TG; Barrett PHR; Dimmitt S;2000Atherosclerosis Supplements8Abstract only
13Ezetimibe.Darkes MJ; Poole RM; Goa KL;2003American Journal of Cardiovascular Drugs8Narrative review
5312Safety considerations with fibrate therapyDavidson MH; Armani A; McKenney JM; Jacobson TA;2007American Journal of Cardiology8Used for background information only
455European guidelines on cardiovascular disease prevention in clinical practice, Third Joint Task Force of European and other Societies on Cardiovascular Disease Prevention in Clinical PracticeDe Backer G; Ambrosioni E; Borch-Johnsen K; Brotons C; Cifkova R; Dallongeville J; Ebrahim S; Faergeman O; Graham I; Mancia G; Cats VM; Orth-Gomer K; Perk J; Pyorala K; Rodicio JL; Sans S; Sansoy V; Sechtem U; Silber S; Thomsen T; Wood D;2003European Journal of Cardiovascular Prevention & Rehabilitation8General guideline/narrative
300The effect of simvastatin treatment on the low-density lipoprotein subfraction profile and composition in familial hypercholesterolaemiade Graaf J; Demacker PN; Stalenhoef AF;1993Netherlands Journal of Medicine8Not RCT
943Early statin therapy restores endothelial function in children with familial hypercholesterolemiade Jongh S; Lilien MR; Roodt JO; Stroes ESG; Bakker HD; Kastelein JJP;2002Journal of the American College of Cardiology8Included in Simon Broome SR
649Efficacy and safety of statin therapy in children with familial hypercholesterolemia, a randomized, double-blind, placebo-controlled trial with simvastatinde Jongh S; Ose L; Szamosi T; Gagne C; Lambert M; Scott R; Perron P; Dobbelaere D; Saborio M; Tuohy MB; Stepanavage M; Sapre A; Gumbiner B; Mercuri M; van-Trotsenburg ASP; Bakker HD; Kastelein JJP;2002Circulation8Included in Simon Broome SR
142Two-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])de Sauvage Nolting PR; Buirma RJ; Hutten BA; Kastelein JJ; Dutch ExPRESS Investigator Group;2002American Journal of Cardiology8Not RCT
499The effect of low-dose simvastatin in children with familial hypercholesterolaemia, A 1-year observationDirisamer A; Hachemian N; Bucek RA; Wolf F; Reiter M; Widhalm K;2003European Journal of Pediatrics8Not RCT. Included in Simon Broome SR
962Changes in Serum Lipoprotein(A) in Hyperlipidemic Participants Undergoing Long-Term Treatment with Lipid-Lowering DrugsDobs AS; Prasad M; Goldberg A; Guccione M; Hoover DR;1995Cardiovascular Drugs and Therapy8Not specified outcome measure
6Statins in hypercholesterolaemia, a dose-specific meta-analysis of lipid changes in randomised, double blind trialsEdwards JE; Moore RA;2003BMC Family Practice8Not FH population
3323A study of the dose-effect relationship of cholestyramine in children with familial hypercholesterolemiaFarah JR; Kwiterovich PO; Neill CA;1977Advances in Experimental Medicine & Biology8Not a long term study
417Rosuvastatin, A risk-benefit assessment for intensive lipid loweringFerdinand KC;2005Expert Opinion on Pharmacotherapy8Not RCT
123The effect of cholestyramine on serum lipids and platelet aggregation of hypercholesterolemic children (type II A) while on high linoleic acid dietFernandes J; jkhuis-Stoffelsma R; Grose WF;1977Acta Paediatrica Scandinavica8Not a long term study
5284A comprehensive description of muscle symptoms associated with lipid-lowering drugsFranc S; Dejager S; Bruckert E; Chauvenet M; Giral P; Turpin G;2003Cardiovascular Drugs and Therapy8Used for background only
288Pravastatin effectively lowers LDL cholesterol in familial combined hyperlipidemia without changing LDL subclass patternFranceschini G; Cassinotti M; Vecchio G; Gianfranceschi G; Pazzucconi F; Murakami T; Sirtori M; D’Acquarica AL; Sirtori CR;1994Arteriosclerosis & Thrombosis8Not RCT
998The Affinity of Low-Density Lipoproteins and of Very-Low-Density Lipoprotein Remnants for the Low-Density-Lipoprotein Receptor in Homozygous Familial Defective Apolipoprotein B-100Gallagher JJ; Myant NB;1995Atherosclerosis8Does not answer question
1009Effect of ezetimibe on low-density lipoprotein subtype distribution, results of a placebo-controlled, double-blind trial in patients treated by regular low-density lipoprotein apheresis and statinsGeiss HC; Otto C; Parhofer KG;2006Metabolism-Clinical and Experimental8Used for background only
3324Therapy of familial and acquired hyperlipoproteinemia in children and adolescentsGlueck CJ;1983Preventive Medicine8Narrative review
709Safety and efficacy of long-term diet and diet plus bile acid-binding resin cholesterol-lowering therapy in 73 children heterozygous for familial hypercholesterolemiaGlueck CJ; Mellies MJ; Dine M; et a;1986Pediatrics8This is a case series of 33 children with FH who were treated with diet and resins and compared to children treated with resins only and also to normal children. Individual data is presented.
66Therapy of familial hypercholesterolemia in childhood, diet and cholestyramine resin for 24 to 36 monthsGlueck CJ; Tsang RC; Fallat RW; Mellies M;1977Pediatrics8Not RCT
1014Atorvastatin lowers lipoprotein(a) but not apolipoprotein(a) fragment levels in hypercholesterolemic participants at high cardiovascular riskGonbert S; Malinsky S; Sposito AC; Laouenan H; Doucet C; Chapman MJ; Thillet J;2002Atherosclerosis8No control group
667Efficacy and safety of statin therapy in children with familial hypercholesterolemia, a randomized controlled trialGotto AM;2005The Journal of Pediatrics8Comment only
3325Clinical management of children and young adults with heterozygous familial hypercholesterolaemia in the UKGreene O; Durrington P;2004Journal of the Royal Society of Medicine8Does not answer the question
143Dose-dependent action of atorvastatin in type IIB hyperlipidemia, preferential and progressive reduction of atherogenic apoB-containing lipoprotein subclasses (VLDL-2, IDL, small dense LDL) and stimulation of cellular cholesterol effluxGuerin M; Egger P; Soudant C; Le G; van T; Dupuis R; Chapman MJ;2002Atherosclerosis8Not FH population
338The effects of simvastatin on plasma lipoproteins and cholesterol homeostasis in patients with heterozygous familial hypercholesterolaemiaHagemenas FC; Pappu AS; Illingworth DR;1990European Journal of Clinical Investigation8Not RCT
1041Pharmacokinetics and response to pravastatin in paediatric patients with familial hypercholesterolaemia and in paediatric cardiac transplant recipients in relation to polymorphisms of the SLCO1B1 and ABCB1 genesHedman M; Antikainen M; Holmberg C; Neuvonen M; Eichelbaum M; Kivisto KT; Neuvonen PJ; Niemi M;2006British Journal of Clinical Pharmacology8Case series, not GDG outcome measures
433Efficacy and safety of pravastatin in children and adolescents with heterozygous familial hypercholesterolemia, A prospective clinical follow-up studyHedman M; Matikainen T; Fohr A; Lappi M; Piippo S; Nuutinen M; Antikainen M;2005Journal of Clinical Endocrinology & Metabolism8Case series, not RCT.
95Lipid and lipoprotein profiles in children with familial hypercholesterolaemia, Effects of therapyHennermann JB; Herwig J; Marz W; Asskali F; Bohles HJ;1998European Journal of Pediatrics8Not a long term study
210Effects of atorvastatin on serum lipids of patients with familial hypercholesterolaemiaHoogerbrugge N;1998Journal of Internal Medicine8Not RCT
39Comparative-Evaluation of the Safety and Efficacy of Hmg-Coa Reductase Inhibitor Monotherapy in the Treatment of Primary HypercholesterolemiaHsu I; Spinler SA; Johnson NE;1995Annals of Pharmacotherapy8Narrative report
1060Effects of One-Year of Treatment with Pravastatin, An Hmg-Coa Reductase Inhibitor, on Lipoprotein-AHunninghake DB; Stein EA; Mellies MJ;1993Journal of Clinical Pharmacology8Not FH population
304How effective is drug therapy in heterozygous familial hypercholesterolemia?.Illingworth DR;1993American Journal of Cardiology8Narrative only
323Hypocholesterolaemic effects of lovastatin in familial defective apolipoprotein B-100.Illingworth DR; Vakar F; Mahley RW; Weisgraber KH;1992Lancet8Not RCT
96Pharmacology and therapeutics of ezetimibe (SCH 58235), a cholesterol-absorption inhibitor.Jeu L; Cheng JW;2003Clinical Therapeutics8Not our population of homozygous or children
5Effects of statin therapy on the progression of carotid atherosclerosis, a systematic review and meta-analysisKang S; Wu Y; Li X;2004Atherosclerosis8Does not answer question
1101Efficacy, Safety, and Tolerability of Pravastatin for the Long-Term Treatment of Patients with Diverse Types of Hyperlipidemia - A Multicenter Prospective-StudyKatoh K; Mizuno K; Niimura S; Fukuchi S;1993Current Therapeutic Research-Clinical and Experimental8Not FH patients
11043-Year Follow-Up of the Oxford- Cholesterol-Study - Assessment of the Efficacy and Safety of Simvastatin in Preparation for A Large Mortality StudyKeech A; Collins R; Macmahon S; Armitage J; Lawson A; Wallendszus K; Fatemian M; Kearney E; Lyon V; Mindell J; Mount J; Painter R; Parish S; Slavin B; Sleight P; Youngman L; Peto R;1994European Heart Journal8Not FH population
598A 4-year trial of simvastatin in the treatment of patients with heterozygous familial hypercholesterolemiaKitatani M; Koizumi J; Inazu A; Kajinami K; Mabuchi H;1996Current Therapeutic Research, Clinical & Experimental8Not RCT
250Short-term efficacy and safety of pravastatin in 72 children with familial hypercholesterolemia.[erratum appears in Pediatr Res 1996 Dec, 40(6):866]Knipscheer HC; Boelen CC; Kastelein JJ; van D; Groenemeijer BE; van d; Buller HR; Bakker HD;1996Pediatric Research8Included in Simon Broome SR
5384Evaluation of the efficacy, safety, and tolerability of ezetimibe in primary hypercholesterolaemia, a pooled analysis from two controlled phase III clinical studiesKnopp RH; Dujovne CA; Le BA; Lipka LJ; Suresh R; Veltri EP;2003International Journal of Clinical Practice8Used for background only
1540Multiple-dose safety and pharmacokinetics of ezetimibe in adolescent childrenKosoglou T; Kakkar T; Statkevich P; Anderson L; Pai S; Boutros T; Veltri EP; Affrime MB;2001Clinical Pharmacology & Therapeutics8Abstract only
253Treatment of familial hypercholesterolemia in children and adolescents, effect of lovastatin. Canadian Lovastatin in Children Study GroupLambert M; Lupien PJ; Gagne C; Levy E; Blaichman S; Langlois S; Hayden M; Rose V; Clarke JT; Wolfe BM; Clarson C; Parsons H; Stephure DK; Potvin D; Lambert J;1996Pediatrics8Included in Simon Broome SR
87The management of familial hypercholesterolaemia in childhoodLee PJ;2002Current Paediatrics8Narrative only
1155Gender-Related Response to Fluvastatin in Patients with Heterozygous Familial HypercholesterolemiaLeitersdorf E;1994Drugs8Gender response not a question
317Beneficial effect of simvastatin in patients with drug resistant familial hypercholesterolaemiaLewis-Barned NJ; Ball MJ;1992New Zealand Medical Journal8Case series - no control
76Treatment of Primary Hypercholesterolemia with Simvastatin - New-Zealand Multicenter EvaluationLintott CJ; Scott RS; Sharpe DN; Nye ER; Charleson H; French JK; White HD; Reuben S; Maling TJB; Lewis GRB; Sutherland WHF; Robertson MC; Frampton C;1991Medical Journal of Australia8Not RCT
1203Ezetimibe for management of hypercholesterolemiaMauro VF; Tuckerman CE;2003Annals of Pharmacotherapy8Not our population of homozygous and children
148A randomized crossover trial of combination pharmacologic therapy in children with familial hyperlipidemiaMcCrindle BW; Helden E; Cullen-Dean G; Conner WT;2002Pediatric Research8Included in Simon Broome SR
116Acceptability and compliance with two forms of cholestyramine in the treatment of hypercholesterolemia in children, a randomized, crossover trialMcCrindle BW; O’Neill MB; Cullen-Dean G; Helden E;1997The Journal of Pediatrics8Does not answer the question
107Efficacy and safety of atorvastatin in children and adolescents with familial hypercholesterolemia or severe hyperlipidemia, a multicenter, randomized, placebo-controlled trialMcCrindle BW; Ose L; Marais AD;2003Journal of Pediatrics8Included in Simon Broome SR
2The use of ezetimibe in achieving low density lipoprotein lowering goals in clinical practice, position statement of a United Kingdom consensus panelMikhailidis DP; Wierzbicki AS; Daskalopoulou SS; Al-Saady N; Griffiths H; Hamilton G; Monkman D; Patel V; Pittard J; Schachter M;2005Current Medical Research and Opinion8Not our population - children and homozygous
114A comparative study of colestipol and colestyramine in children and adolescents with familial hypercholesterolaemiaMordasini R; Twelsick F; Oster P; Schellenberg B; Raetzer H; Heuck CC; Schlierf G;1978Monatsschrift fur Kinderheilkunde8Non English language study
176Abnormal low density lipoproteins in children with familial hypercholesterolemia--effect of polyanion exchange resinsMordasini R; Twelsiek F; Oster P; Schellenberg B; Raetzer H; Heuck CC; Schlierf G;1978Klinische Wochenschrift8This study is not randomised, blinded and there is no allocation concealment reported. The results described in the text do not correlate with the tables included.
65Current management of severe homozygous hypercholesterolaemias.Naoumova RP; Thompson GR; Soutar AK;2004Current Opinion in Lipidology8Narrative only
606Long-term treatment with simvastatin in patients with familial combined hyperlipidemiaNapoli C; Lepore S; Condorelli M; Chiariello M;1995Current Therapeutic Research, Clinical & Experimental8Not FH population
638Effect of statin treatment for familial hypercholesterolaemia on life assurance, results of consecutive surveys in 1990 and 2002Neil HAW; Hammond T; Mant D; Humphries SE;2004British Medical Journal8Not RCT
145Long-term treatment with pitavastatin (NK-104), a new HMG-CoA reductase inhibitor, of patients with heterozygous familial hypercholesterolemiaNoji Y; Higashikata T; Inazu A; Nohara A; Ueda K; Miyamoto S; Kajinami K; Takegoshi T; Koizumi J; Mabuchi H; Hokuriku NK;2002Atherosclerosis8Pitavastatin not available in UK
1513Effect of rosuvastatin on low- density lipoprotein cholesterol in patients with hypercholesterolemiaOlsson AG; Pears J; McKellar J; Mizan J; Raza A;2001American Journal of Cardiology8Not FH population
171Determinants of variable response to statin treatment in patients with refractory familial hypercholesterolemiaO’Neill FH; Patel DD; Knight BL; Neuwirth CK; Bourbon M; Soutar AK; Taylor GW; Thompson GR; Naoumova RP;2001Arteriosclerosis, Thrombosis & Vascular Biology8Not RCT
56Diagnostic, clinical, and therapeutic aspects of familial hypercholesterolemia in children.Ose L;2004Seminars in Vascular Medicine8Narrative review
267Fluvastatin in severe hypercholesterolemia, analysis of a clinical trial databasePeters TK;1995American Journal of Cardiology8Source of studies included in review not provided
523Efficacy and safety of atorvastatin 10 mg every other day in hypercholesterolemiaPiamsomboon C; Saguanwong S; Nasawadi C; Pongsiri K; Laothavorn P; Chatlaong B; Tanprasert P;2002Journal of the Medical Association of Thailand8Not RCT
1311One Year Experience in the Treatment of Familial Hypercholesterolemia with SimvastatinQuiney J; Watts GF; Kerrmuir M; Slavin B; Lewis B;1992Postgraduate Medical Journal8Not RCT
776Treatment of patients with homozygous familial hypercholesterolemia with expanded doses of simvastatin [abstract]Raal FJ; Pilcher GJ; Pieterse AC; Stein EA; Laskarzewski P; Illingworth DR; Melino MR; Mitchel YB;1997Atherosclerosis Supplements8Abstract only
438Familial hypercholesterolemia in children.Rodenburg J; Vissers MN; Wiegman A; Trip MD; Bakker HD; Kastelein JJ;2004Current Opinion in Lipidology8Narrative review
334Therapeutic efficacy of the HMG- CoA-reductase inhibitor pravastatin in hyperlipoproteinaemia type IISaxenhofer H; Weidmann P; Riesen WF; Beretta-Piccoli C; Fragiacomo C; Wunderlin R; Noseda G;1990European Journal of Clinical Pharmacology8Not FH population
165‘Low dose’ colestipol in children, adolescents and young adults with familial hypercholesterolemiaSchlierf G; Mrozik K; Heuck CC; Middelhoff G; Oster P; Riesen W; Schellenberg B;1982Atherosclerosis8Poor study quality - not randomised or controlled, dropout rate high
19A meta-analysis to evaluate the efficacy of statins in children with familial hypercholesterolemiaShafiq N; Bhasin B; Pattanaik S; Pandhi P; Venkateshan SP; Singh M; Malhotra S;2007International Journal of Clinical Pharmacology and Therapeutics8An Indian study which repeats information in Arambepola et al, 2007
1374Simvastatin in Severe Primary Hypercholesterolemia - Efficacy, Safety, and Tolerability in 595 Patients Over 18 WeeksSimons LA; Abraham R; Beilin L; Masarei J; Beng C; Pannall P; Cain H; Bradfield R; Calvert GD; Cornish G; England J; Craig IH; Heller RF; Smith AJ; Hunt D; Janus ED; Best JD; Lloyd B; Hung J; Newnham H; Barter P; Simons LA; Sullivan D; Thomas DW; Tallis G; Wahlqvist M; Wilcken DE;1993Clinical Cardiology8Not RCT
187The effect of cholesterol lowering on carotid and femoral artery wall stiffness and thickness in patients with familial hypercholesterolaemiaSmilde TJ; van d; Wollersheim H; van L; Kastelein JJ; Stalenhoef AF;2000European Journal of Clinical Investigation8Not LDL outcome
566Diet only and diet plus simvastatin in the treatment of heterozygous familial hypercholesterolemia in childhoodStefanutti C; Lucani G; Vivenzio A; Di G;1999Drugs Under Experimental & Clinical Research8Included in Simon Broome review
737Rosuvastatin (20, 40 and 80 MG) reduces LDL-C, raises HDL-C and achieves treatment goals more effectively than atorvastatin (20, 40 and 80 mg) in patients with heterozygous familial hypercholesterolaemiaStein E; Strutt KL; Miller E; Southworth H;2001Atherosclerosis Supplements8Abstract only
159Statins in children. Why and when.Stein EA;2001Nutrition Metabolism & Cardiovascular Diseases8Narrative review
206Efficacy and safety of lovastatin in adolescent males with heterozygous familial hypercholesterolemia, a randomized controlled trial.[see comment]Stein EA; Illingworth DR; Kwiterovich POJ; Liacouras CA; Siimes MA; Jacobson MS; Brewster TG; Hopkins P; Davidson M; Graham K; Arensman F; Knopp RH; DuJovne C; Williams CL; Isaacsohn JL; Jacobsen CA; Laskarzewski PM; Ames S; Gormley GJ;1999JAMA8Included in Simon Broome SR
128More Western Hypercholesterolemic Patients Achieve Japan Atherosclerosis Society LDL-C Goals with Rosuvastatin Therapy Than with Atorvastatin, Pravastatin, or Simvastatin TherapyStrutt K; Caplan R; Hutchison H; Dane A; Blasetto J;2004Circulation Journal8We did not review statin versus statin
237Effect of dietary fish supplementation on lipoprotein levels in patients with hyperlipoproteinemiaSucic M; Katica D; Kovacevic V;1998Collegium Antropologicum8Not FH population
414Effects of rosuvastatin on endothelial function in patients with familial combined hyperlipidaemia (FCH)Ter Avest E; Abbink EJ; Holewijn S; de Graaf J; Tack CJ; Stalenhoef AFH;2005Current Medical Research & Opinion8Not FH population
3329A rational approach to treating hypercholesterolaemia in children - Weighing the risks and benefitsTonstad S;1997Drug Safety8Narrative review
184Role of lipid-lowering pharmacotherapy in children.Tonstad S;2000Paediatric Drugs8Narrative
3328Colestipol tablets in adolescents with familial hypercholesterolaemiaTonstad S; Ose L;1996Acta Paediatrica86 month follow up only- require long term follow up
78Management of hyperlipidemia in the pediatric populationTonstad S; Thompson GR;2004Current Treatment Options in Cardiovascular Medicine8Narrative review
111Long term statin treatment reduces lipoprotein(a) concentrations in heterozygous familial hypercholesterolaemiavan Wissen S; Smilde TJ; Trip MD; de Boo T; Kastelein JJ; Stalenhoef AF;2003Heart8Not outcome specified
50Long-term safety and efficacy of high-dose atorvastatin treatment in patients with familial hypercholesterolemiavan Wissen S; Smilde TJ; Trip MD; Stalenhoef AF; Kastelein JJ;2005American Journal of Cardiology8Not RCT
154Influence of LDL receptor gene mutation and apo E polymorphism on lipoprotein response to simvastatin treatment among adolescents with heterozygous familial hypercholesterolemiaVohl MC; Szots F; Lelievre M; Lupien PJ; Bergeron J; Gagne C; Couture P;2002Atherosclerosis8Not outcome identified by GDG
233Drug therapy of severe hypercholesterolemia.Weizel A; Richter WO;1997European Journal of Medical Research8Narrative review
3330Use of cholestyramine in treatment of children with familial hypercholesterolaemiaWest RJ; Lloyd JK;1973Archives of disease in childhood8Not RCT
1487Arterial intima-media thickness in children heterozygous for familial hypercholesterolaemiaWiegman A; de Groot E; Hutten BA; Rodenburg J; Gort J; Bakker HD; Sijbrands EJG; Kastelein JJP;2004Lancet8Not LDL outcome after treatment
1488Efficacy and safety of statin therapy in children with familial hypercholesterolemia, a randomized controlled trial.Wiegman A; Hutten BA; de G; Rodenburg J; Bakker HD; Buller HR; Sijbrands EJ; Kastelein JJ;2004JAMA8Included in Simon Broome SR
452Effects of statin therapy in children with familial hypercholesterolemiaWiegman A; Hutten BA; de G; Roumie CL;2004Journal of Clinical Outcomes Management8Narrative
48Efficacy of ezetimibe in patients with statin-resistant and statin- intolerant familial hyperlipidaemiasWierzbicki AS; Doherty E; Lumb PJ; Chik G; Crook MA;2005Current Medical Research & Opinion8Not our population - homozygous or children
5385Comparison of therapy with simvastatin 80 mg and 120 mg in patients with familial hypercholesterolaemiaWierzbicki AS; Lumb PJ; Chik G;2001International Journal of Clinical Practice8Not FH population
1496Dose-response effects of atorvastatin and simvastatin on high-density lipoprotein cholesterol in hypercholesterolaemic patients, a review of five comparative studiesWierzbicki AS; Mikhailidis DP;2002International Journal of Cardiology8Non FH patients
239Atorvastatin in the treatment of primary hypercholesterolemia and mixed dyslipidemias. [Review] [99 refs]Yee HS; Fong NT;1998Annals of Pharmacotherapy8Narrative, not systematic review
535Atorvastatin versus four statin-fibrate combinations in patients with familial combined hyperlipidaemiaAthyros VG; Papageorgiou AA; Athyrou VV; Demitriadis DS; Pehlivanidis AN; Kontopoulos AG;2002Journal of Cardiovascular Risk9Not FH population
595Pilot study of the effect of the simvastatin-ciprofibrate combination on myocardial infarction risk profile in patients with refractory familial combined hyperlipidaemiaAthyros VG; Papageorgiou AA; Basagiannis EC; Lafaras CT; Kontopoulos AG;2006Clinical Drug Investigation9Non RCT - no control
536Atorvastatin plus pravastatin for the treatment of heterozygous familial hypercholesterolaemia -A pilot studyAthyros VG; Papageorgiou AA; Demitriadis DS; Kontopoulos AG;2001Current Medical Research & Opinion9Not RCT
733Efficacy and tolerability of four statin-fibrate combinations during long-term (5 years) administration in patients with combined familial hyperlipidemiaAthyros VG; Papageorgiou AA; Demitriadis DS; Pehlivanidis AN; Doukelis PV; Karakasis TD; Kontopoulos AG;2001Atherosclerosis Supplements9Abstract only
616Combined treatment with pravastatin and gemfibrozil in patients with refractory familial combined hyperlipidaemia, A clinical studyAthyros VG; Papageorgiou AA; Hagikonstantinou HJ; Papadopoulos GV; Zamboulis CX; Kontopoulos AG;1994Drug Investigation9Not FH population
580Safety and efficacy of long-term statin-fibrate combinations in patients with refractory familial combined hyperlipidemiaAthyros VG; Papageorgiou AA; Hatzikonstandinou HA; Didangelos TP; Carina MV; Kranitsas DF; Kontopoulos AG;1997American Journal of Cardiology9Not FH population
252Fish oil supplementation in patients with heterozygous familial hypercholesterolemiaBalestrieri GP; Maffi V; Sleiman I; Spandrio S; Di S; Salvi A; Scalvini T1996Recenti Progressi in Medicina9This is not a placebo controlled trial but compared fish oil and statins to olive oil and statins
861Efficacy and safety of rosuvastatin alone and in combination with cholestyramine in patients with severe hypercholesterolemia, A randomized, open-label, multicenter trialBallantyne CM; Miller E; Chitra R;2004Clinical Therapeutics9Not FH population. This study included adults with severe hypercholesterolemia but excluded homozygous FH and familial dysbetalipoproteinemia. There is no specific inclusion diagnosis for heterozygous FH and the patient clinical characteristics indicate
865A Multicenter Comparison of the Effects of Simvastatin and Fenofibrate Therapy in Severe Primary Hypercholesterolemia, with Particular Emphasis on Lipoproteins Defined by Their Apolipoprotein CompositionBard JM; Parra HJ; Camare R; Luc G; Ziegler O; Dachet C; Bruckert E; Dousteblazy P; Drouin P; Jacotot B; Degennes JL; Keller U; Fruchart JC;1992Metabolism-Clinical and Experimental9Does not answer question
875Effect of ezetimibe and/or simvastatin on coenzyme Q10 levels in plasma - A randomised trialBerthold HK; Naini A; Di Mauro S; Hallikainen M; Gylling H; Krone W; Gouni- Berthold I;2006Drug Safety9Not our population of homozygous and children
605Comparison of gemfibrozil versus simvastatin in familial combined hyperlipidemia and effects on apolipoprotein-B-containing lipoproteins, low- density lipoprotein subfraction profile, and low-density lipoprotein oxidizabilityBredie SJH; De B; Demacker PNM; Kastelein JJP; Stalenhoef AFH;1995American Journal of Cardiology9Not FH population
876Effects of gemfibrozil or simvastatin on apolipoprotein-B- containing lipoproteins, apolipoprotein-CIII and lipoprotein(a) in familial combined hyperlipidaemiaBredie SJH; Westerveld HT; Knipscheer HC; De B; Kastelein JJP; Stalenhoef AFH;1996Netherlands Journal of Medicine9Not FH population. Not primary outcome measure of LDL cholesterol.
256Comparison of the efficacy of simvastatin and standard fibrate therapy in the treatment of primary hypercholesterolemia and combined hyperlipidemiaBruckert E; De Gennes JL; Malbecq W; Baigts F;1995Clinical Cardiology9Not a question asked
597Pravastatin, cholestyramine, and bezafibrate in patients with heterozygous familial hypercholesterolemia, The Spanish Multicenter Pravastatin StudyCarmena R; De Oya M; Gomez-Gerique J; Mata P; Serrano S; Franco M; Martinez- Triguero ML; varez-Sala L; Matesanz J; Rubio MJ; Gras X; Olivan J;1996Cardiovascular Risk Factors9Not randomised in follow up study
37Lipid altering-efficacy of ezetimibe co-administered with simvastatin compared with rosuvastatin, a meta-analysis of pooled data from 14 clinical trialsCatapano A; Brady WE; King TR; Palmisano J;2005Current Medical Research & Opinion9Not our population of homozygous and children
1538Treatment of familial hypercholesterolemia with ezetimibe/simvastatin in a child less than 10 years of ageCathey V; Beck J; Bond A; Placket P;2006Journal of Pediatric Nursing9This is an abstract of a case study of one patient
5383Efficacy and safety of ezetimibe coadministered with statins, randomised, placebo-controlled, blinded experience in 2382 patients with primary hypercholesterolemiaDavidson MH; Ballantyne CM; Kerzner B; Melani L; Sager PT; Lipka L; Strony J; Suresh R; Veltri E;2004International Journal of Clinical Practice9Used for background only
758Effects of equal doses of pravastatin, simvastatin and atorvastatin in heterozygous patients with familial hypercholesterolemiaDe Mattei S; Masturzo P; Fascetti P; Elicio N; Bertolini S;1999Atherosclerosis Supplements9Abstract only
956Effects of bezafibrate and simvastatin on endothelial activation and lipid peroxidation in hypercholesterolemia, evidence of different vascular protection by different lipid- lowering treatmentsDesideri G; Croce G; Tucci M; Passacquale G; Broccoletti S; Valeri L; Santucci A; Ferri C;2003Journal of Clinical Endocrinology and Metabolism9Not FH population
81Comparison between lovastatin and cholestyramine in the treatment of moderate to severe primary hypercholesterolaemiaEbeling T; Turtola H; Voutilainen E; Uusitupa M; Pyörälä K; Reijonen T;1992Annals of Medicine9Not question asked
341Efficacy and safety of simvastatin (alone or in association with cholestyramine). A 1-year study in 66 patients with type II hyperlipoproteinaemiaEmmerich J; Aubert I; Bauduceau B; Dachet C; Chanu B; Erlich D; Gautier D; Jacotot B; Rouffy J;1990European Heart Journal9Not an RCT
346Plasma lipoprotein changes after treatment with pravastatin and gemfibrozil in patients with familial hypercholesterolemiaFranceschini G; Sirtori M; Vaccarino V; Gianfranceschi G; Chiesa G; Sirtori CR;1989Journal of Laboratory & Clinical Medicine9There is no control in this parallel group study. The study does not answer question 9.
1017Treatment of Primary Hypercholesterolemia - Fluvastatin Versus BezafibrateGreten H; Beil FU; Schneider J; Weisweiler P; Armstrong VW; Keller C; Klor HU; Vonhodenberg E; Weidinger G; Eskotter H; Farber L; Assmann G;1994American Journal of Medicine9Not a question
215Combination therapy with statins. [Review] [51 refs]Gylling H; Miettinen TA;2002Current Opinion in Investigational Drugs9Narrative only
1539Homozygous hypercholesterolaemia and ezetimibe, a case reportHendriksz CJ; Norbury G; Tabrah S; Taylor A; Humphries SE;2004Acta Paediatrica9This is a single case study
75Treatment of dyslipidemia in children and adolescentsHolmes KW; Kwiterovich J;2005Current Cardiology Reports9Narrative review
333Influence of lovastatin plus gemfibrozil on plasma lipids and lipoproteins in patients with heterozygous familial hypercholesterolemiaIllingworth DR; Bacon S;1989Circulation9RCT
1070Short-Term and Long-Term Effects of Lovastatin and Pravastatin Alone and in Combination with Cholestyramine on Serum-Lipids, Lipoproteins and Apolipoproteins in Primary HypercholesterolemiaJacob BG; Mohrle W; Richter WO; Schwandt P;1992European Journal of Clinical Pharmacology9Not RCT
1071Long-Term Treatment (2 Years) with the Hmg Coa Reductase Inhibitors Lovastatin Or Pravastatin in Combination with Cholestyramine in Patients with Severe Primary HypercholesterolemiaJacob BG; Richter WO; Schwandt P;1993Journal of Cardiovascular Pharmacology9Not RCT
43Low-Dose Combination Therapy with Colestipol and Simvastatin in Patients with Moderate to Severe HypercholesterolemiaJohansson J;1995Nutrition Metabolism and Cardiovascular Diseases9Non FH population
46Comparison of ezetimibe plus simvastatin versus simvastatin monotherapy on atherosclerosis progression in familial hypercholesterolemia. Design and rationale of the Ezetimibe and Simvastatin in Hypercholesterolemia Enhances Atherosclerosis Regression (ENHKastelein JJ; Sager PT; de G; Veltri E;2005American Heart Journal9Used for background only
1529Efficacy of colestimide coadministered with atorvastatin in japanese patients with heterozygous familial hypercholesterolemia (FH)Kawashiri MA; Higashikata T; Nohara A; Kobayashi J; Inazu A; Koizumi J; Mabuchi H;2005Circulation Journal9Not RCT
587Effects of simvastatin and ciprofibrate alone and in combination on lipid profile, plasma fibrinogen and low density lipoprotein particle structure and distribution in patients with familial combined hyperlipidaemia and coronary artery diseaseKontopoulos AG; Athyros VG; Papageorgiou AA; Hatzikonstandinou HA; Mayroudi MC; Boudoulas H;1996Coronary Artery Disease9Not FH population
71The Use of Pravastatin Alone and in Combination with Colestipol Or Probucol in the Treatment of Primary HypercholesterolemiaKostis JB; Wilson AC; Pan HY; Kuo PT; Tannenbaum AK;1992Current Therapeutic Research-Clinical and Experimental9Not RCT
265Efficacy and safety of triple therapy (fluvastatin-bezafibrate- cholestyramine) for severe familial hypercholesterolemiaLeitersdorf E; Muratti EN; Eliav O; Peters TK;1995American Journal of Cardiology9Not RCT
1530Effects of lovastatin alone and in combination with cholestyramine on serum lipids and apolipoproteins in heterozygotes for familial hypercholesterolemiaLeren TP; Hjermann I; Berg K; Leren P; Foss OP; Viksmoen L;1988Atherosclerosis9Not RCT
660Long-term effect of lovastatin alone and in combination with cholestyramine on lipoprotein (a) level in familial hypercholesterolemic participantsLeren TP; Hjermann I; Foss OP; Leren P; Berg K;1992Clinical Investigator9Not RCT
659Long term treatment with pravastatin, simvastatin and gemfibrozil in patients with primary hypercholesterolaemia, A controlled studyMuggeo M; Travia D; Querena M; Zenti MG; Bagnani M; Branzi P; Cigolini M;1992Drug Investigation9Does not answer the question
291Fluvastatin in familial hypercholesterolemia, a cohort analysis of the response to combination treatmentMuratti EN; Peters TK; Leitersdorf E;1994American Journal of Cardiology9Not RCT
190Ezetimibe/Simvastatin, a review of its use in the management of hypercholesterolemia. [Review] [60 refs]Murdoch D; Scott LJ;2004American Journal of Cardiovascular Drugs9Not our population of homozygous and children
1239Effects of Simvastatin, Bezafibrate and Gemfibrozil on the Quantity and Composition of Plasma-LipoproteinsNakandakare E; Garcia RC; Rocha JC; Sperotto G; Oliveira HCF; Quintao ECR;1990Atherosclerosis9Does not answer question
584Long-term treatment with pravastatin alone and in combination with gemfibrozil in familial type IIB hyperlipoproteinemia or combined hyperlipidemiaNapoli C; Lepore S; Chiariello P; Condorelli M; Chiariello M;1997Journal of Cardiovascular Pharmacology & Therapeutics9Not FH population
1248Meeting national cholesterol education goals in clinical practice - A comparison of lovastatin and fluvastatin in primary preventionNash DT;1996American Journal of Cardiology9Not FH population
676Comparison of raloxifene and atorvastatin effects on serum lipids composition of healthy post-menopausal womenPiperi C; Kalofoutis C; Lagogianni I; Troupis G; Kalofoutis A;2004Molecular & Cellular Biochemistry9Not FH patients
675Beneficial effects of raloxifene and atorvastatin on serum lipids and HDL phospholipids levels of postmenopausal womenPiperi C; Kalofoutis C; Skenderi K; Economidou O; Kalofoutis A;2004Journal of Obstetrics and Gynaecology, the journal of the Institute of Obstetrics and Gynaecology9Not FH
1327Comparative Effects of 2 Hmg- Coa Reductase Inhibitors (Lovastatin and Pravastatin) on Serum-Lipids and LipoproteinsRichter WO; Jacob BG; Schwandt P;1991International Journal of Tissue Reactions- Experimental and Clinical Aspects9Not RCT
617Effects of fish oil on serum lipids and lipoprotein(A) levels in heterozygous familial hypercholesterolemiaSalvi A; Di S; Sleiman I; Spandrio S; Balestrieri GP; Scalvini T;1993Current Therapeutic Research, Clinical & Experimental9Not RCT
83Efficacy, safety and tolerability of lovastatin and bezafibrate retard in patients with hypercholesterolemiaSchumacher M; Eber B; Silberbauer K; Breier C; St³hlinger W; Schmidt P; Gaul G; Klein W;1992Acta medica Austriaca9Not question asked
721Fluvastatin and fish oil are more effective on cardiovascular risk factors than fluvastatin aloneSinger P; sal2002Medizinische Welt9Non English article (German)
1381Rationale, design and baseline characteristics of a clinical trial comparing the effects of robust vs conventional cholesterol lowering and intima media thickness in patients with familial hypercholesterolaemiaSmilde TJ; Trip MD; Wollersheim H; van Wissen S; Kastelein JJP; Stalenhoef AFH;2000Clinical Drug Investigation9Protocol only
1531Low-Dose Combined Therapy with Fluvastatin and Cholestyramine in Hyperlipidemic PatientsSprecher DL; Abrams J; Allen JW; Keane WF; Chrysant SG; Ginsberg H; Fischer JJ; Johnson BF; Theroux P; Jokubaitis L;1994Annals of Internal Medicine9Not FH population
1532Achieving lipoprotein goals in patients at high risk with severe hypercholesterolemia, efficacy and safety of ezetimibe co-administered with atorvastatinStein E; Stender S; Mata P; Sager P; Ponsonnet D; Melani L; Lipka L; Suresh R; Maccubbin D; Veltri E;2004American Heart Journal9Not our population of homozygous and children
740ZD4522 is superior to atorvastatin in the treatment of patients with heterozygous familial hypercholesterolemia [abstract]Stein E; Strutt KL; Miller E;2001Atherosclerosis Supplements9Abstract only
1541Treatment of dyslipidemia with lovastatin and ezetimibe in an adolescent with cholesterol ester storage diseaseTadiboyina VT; Liu DM; Miskie BA; Wang J; Hegele RA;2005Lipids in Health & Disease9This is a single case study
77Familial hypercholesterolemia in a paediatric patientUllah M; Ahmad SA;2005Journal of the College of Physicians & Surgeons Pakistan9Case study
274Comparison of lovastatin (20 mg) and nicotinic acid (1.2 g) with either drug alone for type II hyperlipoproteinemiaVacek JL; Dittmeier G; Chiarelli T; White J; Bell HH;1995American Journal of Cardiology9No control in this parallel group study which utilized four treatment sequences.
723Heterozygous familial hypercholesterolemia, coadministration of ezetimibe plus atorvastatinVermaak W; Pinto X; Ponsonnet D; Sager P; Lipka L; Suresh R; Veltri E;2002Atherosclerosis Supplements9Abstract only
1482Simvastatin Plus Low-Dose Colestipol in the Treatment of Severe Familial HypercholesterolemiaWeisweiler P;1988Current Therapeutic Research-Clinical and Experimental9No control group
351Simvastatin and bezafibrate, effects on serum lipoproteins and lecithin, cholesterol acyltransferase activity in familial hypercholesterolaemiaWeisweiler P;1988European Journal of Clinical Pharmacology9Does not answer question
771Fenofibrate-simvastatin therapy compared to simvastatin-resin therapy and atorvastatin for familial hypercholesterolaemiaWierzbicki AS; Lumb PJ; Cheung J; Crook MA;1997Atherosclerosis Supplements9Abstract only
651Treatment of severe, resistant familial combined hyperlipidemia with a bezafibrate-lovastatin combinationYeshurun D; Abukarshin R; Elias N; Lanir A; Naschitz JE;1993Clinical Therapeutics9Not FH population
5231Effectiveness of association of plasma exchange and cholestyramine in treatment of homozygous familial hypercholesterolaemiaAlfano G; Salera P; Rossi PL; et a;1986Acta Medica Romana10Case report
86Impact of the characteristics of patients and their clinical management on outcomes in children with homozygous familial hypercholesterolemiaAl-Shaikh AM; Abdullah MH; Barclay A; Cullen-Dean G; McCrindle BW;2002Cardiology in the Young10Not the question
91Low-density lipoprotein apheresis, clinical results with different methodsBambauer R;2002Artificial Organs10Does not answer the question - comparison of different systems
5236Is lipoprotein (a)-apheresis useful?Bambauer R;2005Therapeutic Apheresis & Dialysis, Official Peer- Reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy10Narrative review
5232Low-density lipoprotein apheresis for prevention and regression of atherosclerosis, clinical resultsBambauer R; Olbricht CJ; Schoeppe E;1997Therapeutic Apheresis10Population not all FH, no absolute numbers reported, use of concomitant drugs unclear
5235Low-density lipoprotein apheresis, an overview.Bambauer R; Schiel R; Latza R;2003Therapeutic Apheresis & Dialysis, Official Peer- Reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy10Narrative
5233Apheresis technologies for prevention and regression of atherosclerosis, clinical resultsBambauer R; Schneidewind JM; Latza R;1999ASAIO Journal10Repeat of 1997 report
98Atorvastatin improves blood rheology in patients with familial hypercholesterolemia (FH) on long-term LDL apheresis treatmentBanyai S; Banyai M; Falger J; Jansen M; Alt E; Derfler K; Koppensteiner R;2001Atherosclerosis10Does not answer the question
5237Therapeutic efficiency of lipoprotein(a) reduction by low- density lipoprotein immunoapheresisBanyai S; Streicher J; Strobl W; Gabriel H; Gottsauner-Wolf M; Rohac M; Weidinger F; Horl WH; Derfler K;1998Metabolism, Clinical & Experimental1016 patients
166Pregnancy outcome in familial homozygous hypercholesterolemic females treated with long-term plasma exchangeBeigel Y; Bar J; Cohen M; Hod M;1998Acta Obstetricia et Gynecologica Scandinavica102 FH patients - 5 pregnancies
398Regression of atherosclerosis in patients with familial hypercholesterolaemia under LDL-apheresisBorberg H; Gaczkowski A; Hombach V; Oette K; Stoffel W;1988Progress in Clinical & Biological Research1010 patients only
5239Direct adsorption of low-density lipoprotein by DALI-LDL-apheresis, results of a prospective long-term multicenter follow-up covering 12,291 sessionsBosch T; Gahr S; Belschner U; Schaefer C; Lennertz A; Rammo J; for t;2006Therapeutic Apheresis & Dialysis, Official Peer- Reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy10FH sub population report not provided
5240Plasma exchange versus an affinity column for cholesterol reductionBurgstaler EA; Pineda AA;1992Journal of Clinical Apheresis10Case study
390Selective and semiselective low-density lipoprotein apheresis in familial hypercholesterolemiaBusnach G; Cappelleri A; Vaccarino V; Franceschini G; Dal C; Perrino ML; Brando B; Sirtori C; Minetti L;1988Blood Purification107 patients
365Double-filtration plasmapheresis in heterozygous familial hypercholesterolemia, our experience over 25 treatmentsCandrina R; Spandrio S; Di S; Scalvini T; Cotelli M; Tosoni M; Giustina G;1988Beitrage zur Infusionstherapie106 patients - study of technique only
5264Comparison between treatments of severe forms of familial hypercholesterolemia by total plasma exchange and selective removal of low density lipoproteins (LDLapheresis)].Dairou F; Rottembourg J; De G; Assogba U; Bruckert E; Jacobs C; Truffert J;1988Presse Medicale1010 patients
538Combined LDL-apheresis and statin treatment in homozygous and heterozygous familial hyperlipoproteinaemiaDerfler K; Goldammer A;2001Journal of Clinical & Basic Cardiology1014 patients
5241One year experience with a low density lipoprotein apheresis systemDurst R; Rund D; Schurr D; Eliav O; Ben-Yehuda D; Shpizen S; Ben-Avi L; Schaap T; Pelz I; Leitersdorf E;2002Israel Medical Association Journal1013 patients only
5242Long-term clinical experience with HELP-LDL-apheresis in combination with HMG-CoA- reductase inhibitors for maximum treatment of coronary heart disease associated with severe hypercholesterolemiaEisenhauer T; Armstrong VW; Schuff-Werner P; Schutz E; Thiery J; Scheler F; Seidel D;1989ASAIO Transactions1010 patients
407Long-term experience with the HELP system for treatment of severe familial hypercholesterolemiaEisenhauer T; Schuff- Werner P; Armstrong VW; Talartschik J; Scheler F; Seidel D;1987ASAIO Transactions1010 patients
630Lipoprotein particles in homozygous familial hypercholesterolemic patients treated with portacaval shunt and LDL apheresisGairin D; Monard F; Cachera C; Bard JM; Amouyel P; Duriez P; Tacquet A; Fruchart JC;2003Clinica Chimica Acta102 patients
5243Composition and immunoreactivity of serum low density lipoproteins (LDL) before and after LDL-apheresis on dextran sulfate-cellulose columnsGandjini H; Gambert P; Athias A; Mousson C; Rifle G; Lallemant C;1994Transfusion Science104 patients only
38In vivo metabolism of LDL subfractions in patients with heterozygous FH on statin therapy, rebound analysis of LDL subfractions after LDL apheresisGeiss HC; Bremer S; Barrett PH; Otto C; Parhofer KG;2004Journal of Lipid Research10Does not answer the question
1009Effect of ezetimibe on low-density lipoprotein subtype distribution, results of a placebo-controlled, double-blind trial in patients treated by regular low-density lipoprotein apheresis and statinsGeiss HC; Otto C; Parhofer KG;2006Metabolism-Clinical and Experimental10Not our population of homozygous and children
784Atorvastatin compared with simvastatin in patients with severe LDL hypercholesterolaemia treated by regular LDL apheresisGeiss HC; Parhofer KG; Schwandt P;1999Journal of Internal Medicine10Does not answer the question
80Atorvastatin in low-density lipoprotein apheresis-treated patients with homozygous and heterozygous familial hypercholesterolemiaGoldammer A; Wiltschnig S; Heinz G; Jansen M; Stulnig T; Horl WH; Derfler K;2002Metabolism, Clinical & Experimental1014 patients
801Effect of different LDL-apheresis methods on parameters involved in atherosclerosisHershcovici T; Schechner V; Orlin J; Harell D; Beigel Y;2004Journal of Clinical Apheresis106 patients only, does not answer the question
61Long-term effect of low-density lipoprotein apheresis in patients with heterozygous familial hypercholesterolemia.Higashikata T; Mabuchi H;2003Therapeutic Apheresis & Dialysis, Official Peer- Reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy10Narrative review
70Effects of combined low-density lipoprotein apheresis and aggressive statin therapy on coronary calcified plaque as measured by computed tomographyHoffmann U; Derfler K; Haas M; Stadler A; Brady TJ; Kostner K;2003American Journal of Cardiology108 patients only
423Comparison of selectivity of LDL removal by double filtration and dextran-sulfate cellulose column plasmapheresisHomma Y; Mikami Y; Tamachi H; Nakaya N; Nakamura H; Araki G; Goto Y;1986Atherosclerosis104 patients only
5244Clinical outcome of patients with familial hypercholesterolemia and coronary artery disease undergoing partial ileal bypass surgeryIssa JS; Garrido AJ; Giannini SD; Forti N; Diament J; Pinotti HW;2000Arquivos Brasileiros de Cardiologia10Case study
271Therapy of severe familial heterozygous hypercholesterolemia by low- density lipoprotein apheresis with immunoadsorption, effects of the addition of 3-hydroxy-3- methylglutaryl coenzyme A reductase inhibitors to therapyJacob BG; Richter WO; Schwandt P;1993Clinical Investigator108 patients only
822Lipid reductions by low-density lipoprotein apheresis, A comparison of three systemsJovin IS; Taborski U; Stehr A; Muller- Berghaus G;2000Metabolism-Clinical and Experimental10Case study
5245Therapeutic effects of LDL apheresis in the prevention of atherosclerosis.Kajinami K; Mabuchi H;1999Current Opinion in Lipidology10Narrative review
315LDL-apheresis, results of longterm treatment and vascular outcome.Keller C;1991Atherosclerosis10Narrative review
5246Indication of low-density lipoprotein apheresis in severe hypercholesterolemia and its atherosclerotic vascular complications, dextran sulfate cellulose low-density lipoprotein apheresis.Keller C;2003Therapeutic Apheresis & Dialysis10Narrative review
392Changes of atherosclerosis of the carotid arteries due to severe familial hypercholesterolemia following long-term plasmapheresis, assessed by duplex scanKeller C; Spengel FA;1988Klinische Wochenschrift103 patients only
5247Nonpharmacologic approaches for the treatment of hyperlipidemia.Kermani T; Frishman WH;2005Cardiology in Review10Narrative report
254Coronary atherosclerosis reduced in patients with familial hypercholesterolemia after intensive cholesterol lowering with low-density lipoprotein- apheresis, 1-year follow-up study. The Osaka LDL-Apheresis Multicenter Trial GroupKitabatake A; Sato H; Hori M; Kamada T; Kubori S; Hoki N; Minamino T; Yamada M; Kato T;1994Clinical Therapeutics1013 patients
174The familial hypercholesterolemia regression study, a randomized comparison of therapeutic reduction of both low-density lipoprotein and lipoprotein(a) versus low-density lipoprotein aloneKitano Y; Thompson GR;1997Therapeutic Apheresis10Repeat of Thomson et al study
55Differential indication of lipoprotein apheresis during pregnancy.Klingel R; Gohlen B; Schwarting A; Himmelsbach F; Straube R;2003Therapeutic Apheresis & Dialysis, Official Peer- Reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy10Narrative review
175The retardation of progression, stabilization, and regression of coronary and carotid atherosclerosis by low-density lipoprotein apheresis in patients with familial hypercholesterolemia.Koga N;1997Therapeutic Apheresis10Narrative review
5249Efficacy and safety measures for low density lipoprotein apheresis treatment using dextran sulfate cellulose columns.Koga N;1999Therapeutic Apheresis10Narrative review
283Reduction of lipoprotein(a) by LDL-apheresis using a dextran sulfate cellulose column in patients with familial hypercholesterolemiaKoizumi J; Koizumi I; Uno Y; Inazu A; Kajinami K; Haraki T; Yagi K; Kamon N; Miyamoto S; Takegoshi T; et a;1993Atherosclerosis1013 patients
5250Retrospective comparison of 5 different methods for long-term LDL-apheresis in 20 patients between 1986 and 2001Krebs A; Krebs K; Keller F;2004International Journal of Artificial Organs10Not the question - a comparison of methods
871Effect of apheresis of low-density lipoprotein on peripheral vascular disease in hypercholesterolemic patients with coronary artery diseaseKroon AA; vanAsten WNJC; Stalenhoef AFH;1996Annals of Internal Medicine10Repeat of article published elsewhere
202Treatment of hypercholesterolemia with heparin-induced extracorporeal low-density lipoprotein precipitation (HELP).Lees RS; Holmes NN; Stadler RW; Ibrahim SF; Lees AM;1996Journal of Clinical Apheresis10Narrative review
44Clinical applications of long-term LDL-apheresis on and beyond refractory hypercholesterolemia.Mabuchi H; Higashikata T; Kawashiri MA;2004Transfusion & Apheresis Science10Narrative review
5252Long-term effect of low-density lipoprotein apheresis in patients with homozygous familial hypercholesterolemia.Makino H; Harada- Shiba M;2003Therapeutic Apheresis & Dialysis10Narrative review
5253Changes in lipoprotein profile after selective LDL apheresisMatsunaga T; Takasaki S; Masakane I; Okazaki M; Tomoike H;2004Internal Medicine10Case study
17Hypercholesterolemia and LDL apheresis.Morelli F; Carlier P; Giannini G; De L; Dejana AM; Ruzzenenti MR;2005International Journal of Artificial Organs10Narrative
65Current management of severe homozygous hypercholesterolaemias.Naoumova RP; Thompson GR; Soutar AK;2004Current Opinion in Lipidology10Narrative review
311LDL cholesterol apheresis by dextran sulfate cellulose adsorption. Long-term experience in patients with familial hypercholesterolemiaOlbricht CJ; Schulzeck P;1991ASAIO Transactions105 patients
932Effect of HELP-LDL-apheresis on outcomes in patients with advanced coronary- atherosclerosis and severe hypercholesterolemiaPark JW; Merz M; Braun P;1998Atherosclerosis10Not clear is this is an FH population
167Long-term effect of low-density lipoprotein apheresis on plasma lipoproteins and coronary heart disease in native vessels and coronary bypass in severe heterozygous familial hypercholesterolemiaRichter WO; Donner MG; Hofling B; Schwandt P;1998Metabolism, Clinical & Experimental10Repeat of Donner study
680Short- and long-term effects on serum lipoproteins by three different techniques of apheresisRichter WO; Donner MG; Schwandt P;1996Artificial Organs10Comparison of three types of systems in 19 patients - not the question
141Three low density lipoprotein apheresis techniques in treatment of patients with familial hypercholesterolemia, a long- term evaluationRichter WO; Donner MG; Schwandt P;1999Therapeutic Apheresis10Does not answer the question - we did not review apheresis techniques
619Treatment of severe hypercholesterolemia with heparin-induced LDL apheresisRichter WO; Jacob B; Ritter MM; Suhler K; Vierneisel K; Schwandt P;1992Current Therapeutic Research, Clinical & Experimental105 patients
280Three-year treatment of familial heterozygous hypercholesterolemia by extracorporeal low-density lipoprotein immunoadsorption with polyclonal apolipoprotein B antibodiesRichter WO; Jacob BG; Ritter MM; Suhler K; Vierneisel K; Schwandt P;1993Metabolism, Clinical & Experimental108 patients
375Lipoprotein changes in familial hypercholesterolemia after extracorporeal immunoadsorption of low density lipoproteinsRiesen WF; Jaross W; Descoeudres C; Mordasini R; Koban F; Thulin H; Fisher S; Bergmann S;1988Annales de Biologie Clinique102 patients
307Treatment of severe hypercholesterolaemia by LDL- apheresisScarpato N; Gnasso A; Nappi G; Falco C; Postiglione A; Formisano S; Mancini M;2001Biomaterials, Artificial Cells, & Immobilization Biotechnology1014 patients
5257Impact of chronic LDL-apheresis treatment on Achilles tendon affection in patients with severe familial hypercholesterolemia, a clinical and ultrasonographic 3- year follow-up studyScheel AK; Schettler V; Koziolek M; Koelling S; Werner C; Muller GA; Strutz F;2004Atherosclerosis10Does not answer the question
211H.E.L.P. apheresis therapy in the treatment of severe hypercholesterolemia, 10 years of clinical experience.Seidel D;1996Artificial Organs10Review
5259A review of randomized controlled trials using therapeutic apheresisShehata N; Kouroukis C; Kelton JG;2002Transfusion Medicine Reviews10Narrative review
547Plasmapheresis by using secondary membrane filters, Twelve years of experienceSiami FS; Siami GA;2000ASAIO Journal10Does not answer the question - type of system only
5265LDL-apheresis, current status.Stefanutti C; Vivenzio A; Lucani G; Di G;2001Clinica Terapeutica1011 children in study
5313Guidelines on the use of therapeutic apheresis in clinical practice-Evidence-based approach from the apheresis applications committee of the American society for apheresisSzczepiorkowski ZM; Bandarenko N; Kim HC; Linenberger ML; Marques MB; Sarode R; Schwartz J; Shaz BH; Weinstein R; Wirk A; Winters JL;2007Journal of Clinical Apheresis10Guidelines on apheresis technique not appropriate for this question
5314The new approach to assignment of ASFA categories-Introduction to the fourth special issue, Clinical applications of therapeutic apheresisSzczepiorkowski ZM; Shaz BH; Bandarenko N; Winters JL;2007Journal of Clinical Apheresis10Does not answer the question - new model for grading clients
297Regression of coronary atherosclerosis by combined LDL-apheresis and lipid-lowering drug therapy in patients with familial hypercholesterolemia, a multicenter study. The LARS InvestigatorsTatami R; Inoue N; Itoh H; Kishino B; Koga N; Nakashima Y; Nishide T; Okamura K; Saito Y; Teramoto T; et a;1992Atherosclerosis10Repeat of Koga study
266LDL-apheresis, clinical experience and indications in the treatment of severe hypercholesterolemia.Thiery J; Seidel D;1993Transfusion Science10Narrative review
5260A systematic review of LDL apheresis in the treatment of cardiovascular disease.Thompsen J; Thompson PD;2006Atherosclerosis10Not specific to FH. Review for FH not complete
5262LDL apheresis.Thompson GR;2003Atherosclerosis10Narrative review
430Improved survival of patients with homozygous familial hypercholesterolaemia treated with plasma exchangeThompson GR; Miller JP; Breslow JL;1985British Medical Journal105 homozygous atients only
301Treatment of children with homozygous familial hypercholesterolemia, safety and efficacy of low-density lipoprotein apheresisUauy R; Zwiener RJ; Phillips MJ; Petruska ML; Bilheimer DW;1992Journal of Pediatrics103 children
374The treatment of familial hypercholesterolaemia by partial ileal bypass surgery. A review of the literature. [Review] [40 refs]Van Niekerk JL; Hendriks T; De Boer HH;1984Netherlands Journal of Medicine10Narrative review
101Low-density lipoprotein apheresis for the treatment of refractory hyperlipidemia.Vella A; Pineda AA; O’Brien T;2001Mayo Clinic Proceedings10Narrative review
243The effect of LDL apheresis on progression of coronary artery disease in patients with familial hypercholesterolemia. Results of a multicenter LDL apheresis studyWaidner T; Franzen D; Voelker W; Ritter M; Borberg H; Hombach V; Hopp HW;1994Clinical Investigator10Repeat of study by Borberg
100Apheresis technology for prevention and regression of atherosclerosis.Yamamoto A; Harada-Shiba M; Kawaguchi A; Tsushima M;2001Therapeutic Apheresis10Narrative review
179Apheresis technology for prevention and regression of atherosclerosis, an overview.Yamamoto A; Kawaguchi A; Harada-Shiba M; Tsushima M; Kojima S;1997Therapeutic Apheresis10Narrative review
402LDL-apheresis, potential procedure for prevention and regression of atheromatous vascular lesionYokoyama S; Yamamoto A; Hayashi R; Satani M;1987Japanese Circulation Journal109 patients
492Screening for hypercholesterolaemia in 10,000 neonates in a multi-ethnic populationBeeso J; Wong N; Ayling R; Eldridge P; Marshall W; Sherwood R; Peters T;1999European Journal of Pediatrics11Does not answer the question
9[Pathogenetic bases and surgical alternatives in the treatment of familial hypercholesterolemia]. [Review] [104 refs] [Spanish]Benito Fernandez C; Cienfuegos Suarez JA; Voltas Baro J;1991Medicina Clinica11Narrative review
35Metabolic effects of portacaval shunt surgery and liver transplantation in familial hypercholesterolemia. [Review] [48 refs]Bilheimer DW;1988Beitrage zur Infusionstherapie11Narrative review
40Portacaval shunt surgery and liver transplantation in the treatment of homozygous familial hypercholesterolemia. [Review] [24 refs]Bilheimer DW;1988Progress in Clinical & Biological Research11Narrative review
5270Liver transplantation to provide low-density-lipoprotein receptors and lower plasma cholesterol in a child with homozygous familial hypercholesterolemiaBilheimer DW; Goldstein JL; Grundy SM; Starzl TE; Brown MS;1984New England Journal of Medicine11Case study
3287The validity of screening for hypercholesterolaemia at different ages from 2 to 17 yearsBoulton TJC;1979Australian & New Zealand Journal of Medicine11Does not answer the question
1118Cholesterol in Childhood - How High Is Ok - Recommendations for Screening, Case-Finding and InterventionBoulton TJC; Seal JA; Magarey AM;1991Medical Journal of Australia11Recommendations only
5272Orthotopic liver transplantation as a successful treatment for familial hypercholesterolemiaCienfuegos JA; Turrion V; Pardo F; Ardaiz J; Mora NP; Escartin P; Barrios C; Cuervas-Mons V; Garrido A;1988Transplantation Proceedings11Transplantation technique not our question
62Guidelines for the diagnosis and management of heterozygous familial hypercholesterolemia.Civeira F; International P;2004Atherosclerosis11Methodology not described
1465Prevalence and significance of cardiovascular risk factors in a large cohort of patients with familial hypercholesterolaemiade Sauvage Nolting PR; Defesche JC; Buirma RJ; Hutten BA; Lansberg PJ; Kastelein JJ;2003Journal of Internal Medicine11Does not answer the question
2736Genetic tests may have additional predictive power over and above the accepted risk factors in patients with severe hypercholesterolemiaDescamps OS;2005Louvain Medical11Does not answer the question
1516Heterozygous familial hypercholesterolemia. Relationship between plasma lipids, lipoproteins, clinical manifestations and ischaemic heart disease in men and womenGagne C; Moorjani S; Brun D; Toussaint M; Lupien PJ;1979Atherosclerosis11Does not answer the question
1741Ethical issues in molecular screening for heterozygous familial hypercholesterolemia, the complexity of dealing with genetic susceptibility to coronary artery diseaseGaudet D; Gagne C; Perron P; Couture P; Tonstad S;1999Community Genetics11Does not answer the question
5275Pharmacologic and surgical treatment of dyslipidemic children and adolescents. [Review] [55 refs]Hoeg JM;1991Annals of the New York Academy of Sciences11Narrative review
1232Disease knowledge and adherence to treatment in patients with familial hypercholesterolemiaHollman G; Olsson AG; Ek AC;2006Journal of Cardiovascular Nursing11Does not answer the question
75Treatment of dyslipidemia in children and adolescentsHolmes KW; Kwiterovich J;2005Current Cardiology Reports11Does not answer the question
5244Clinical outcome of patients with familial hypercholesterolemia and coronary artery disease undergoing partial ileal bypass surgeryIssa JS; Garrido AJ; Giannini SD; Forti N; Diament J; Pinotti HW;2000Arquivos Brasileiros de Cardiologia11Case review of three patients
2526Apolipoprotein B measurement in blood spotted on paper (a screening test for familial type II A hyperlipoproteinemia)Koffigan M; Fruchart JC; Dhondt JL; Moschetto Y; Farriaux JP;1982Ricerca in Clinica e in Laboratorio11Does not answer the question
2531Assessment of plasma total cholesterol as a test to detect elevated low density (beta) lipoprotein cholesterol levels (type IIa hyperlipoproteinemia) in young participants from a population-based sampleKwiterovich POJ; Heiss G; Johnson N; Chase GA; Tamir I; Rifkind B;1982American Journal of Epidemiology11Does not answer the question
87The management of familial hypercholesterolaemia in childhoodLee PJ;2002Current Paediatrics11
1294Lipid profile of children with a family history of coronary heart disease or hyperlipidemia, 9-year experience of an outpatient clinic for the prevention of cardiovascular diseasesMakedou A; Kourti M; Makedou K; Lazaridou S; Varlamis G;2005Angiology11Not our population
5277Homozygous familial hypercholesterolemia and its management. [Review] [64 refs]Marais AD; Firth JC; Blom DJ;2004Seminars in Vascular Medicine11Narrative review
1508A review on the diagnosis, natural history, and treatment of familial hypercholesterolaemiaMarks D; Thorogood M; Neil HA; Humphries SE;2003Atherosclerosis11A narrative review
1335Exercise testing in asymptomatic patients with heterozygous familial hypercholesterolaemiaMichaelides AP; Fourlas CA; Pitsavos C; Andrikopoulos GK; Skoumas I; Kartalis A; Katsaros A; Stougiannos P; Stefanadis CI;2004Coronary Artery Disease11Does not answer the question
65Current management of severe homozygous hypercholesterolaemias.Naoumova RP; Thompson GR; Soutar AK;2004Current Opinion in Lipidology11Narrative review
56Diagnostic, clinical, and therapeutic aspects of familial hypercholesterolemia in children.Ose L;2004Seminars in Vascular Medicine11Narrative review
1112Screening for Familial Hypercholesterolemia by Measurement of Apolipoproteins in Capillary BloodSkovby F; Micic S; Jepsen B; Larsen SO; Hansen B; Tegllund L; Pedersen BN;1991Archives of Disease in Childhood11Does not answer the question
1675Hereditary dyslipidemias and combined risk factors in children with a family history of premature coronary artery diseaseSveger T; Flodmark CE; Nordborg K; Nilsson-Ehle P; Borgfors N;2000Archives of disease in childhood11Does not answer the question
301Treatment of Children with Homozygous Familial Hypercholesterolemia - Safety and Efficacy of Low-Density- Lipoprotein ApheresisUauy R; Zwiener RJ; Phillips MJ; Petruska ML; Bilheimer DW;1992Journal of Pediatrics11Case reports of three children
58Clinical, diagnostic, and therapeutic aspects of familial hypercholesterolemia.van Aalst Cohen ES; Jansen AC; de Jongh S; de Sauvage Nolting P.R.; Kastelein JJ;2004Seminars in Vascular Medicine11Narrative review
5292Noninvasive assessment of arterial stiffness and risk of atherosclerotic events in children. [Review] [50 refs]Aggoun Y; Szezepanski I; Bonnet D;2005Pediatric Research12Narrative review
543Sustained long-term improvement of arterial endothelial function in heterozygous familial hypercholesterolemia patients treated with simvastatinAlonso R; Mata P; De A; Villacastin BP; Martinez-Gonzalez J; Badimon L;2001Atherosclerosis12Not an RCT
5293Familial homozygous hypercholesterolemia, clinical and cardiovascular features in 18 patientsBrook GJ; Keidar S; Boulos M; Grenadier E; Wiener A; Shehada N; Markiewicz W; Benderli A; Aviram M;1989Clinical Cardiology12Not heterozygous population
391Vascular stiffness in familial hypercholesterolaemia is associated with C-reactive protein and cholesterol burdenCheng HM; Ye ZX; Chiou KR; Lin SJ; Charng MJ;2007European Journal of Clinical Investigation12Does not answer the question
208Familial hypercholesterolaemia with supravalvular aortic stenosisChidambaram N; Prasad PV;1997Journal of the Indian Medical Association12Case Study
455European guidelines on cardiovascular disease prevention in clinical practice, Third Joint Task Force of European and other Societies on Cardiovascular Disease Prevention in Clinical PracticeDe Backer G; Ambrosioni E; Borch- Johnsen K; Brotons C; Cifkova R; Dallongeville J; Ebrahim S; Faergeman O; Graham I; Mancia G; Cats VM; Orth-Gomer K; Perk J; Pyorala K; Rodicio JL; Sans S; Sansoy V; Sechtem U; Silber S; Thomsen T; Wood D;2003European Journal of Cardiovascular Prevention & Rehabilitation12General guidelines
294Impact of genetic defects on atherosclerosis in patients suspected of familial hypercholesterolaemiaDescamps OS; Gilbeau JP; Leysen X; Van L; Heller FR;2001European Journal of Clinical Investigation12Does not answer the question - looks at risk by genotype but not ongoing evaluation
5294Secondary prevention with lipid lowering therapy in familial hypercholesterolemia, a correlation between new evolution of stenotic lesion and achieved cholesterol levels after revascularization proceduresFukuzawa S; Ozawa S; Inagaki M; Morooka S; Inoue T;1999Internal Medicine12Does not answer the question
5296Atherosclerotic plaque evolution in the descending thoracic aorta in familial hypercholesterolemic patients. A transesophageal echo studyHerrera CJ; Frazin LJ; Dau PC; DeFrino P; Stone NJ; Mehlman DJ; Vonesh MJ; Talano JV; McPherson DD;1994Arteriosclerosis & Thrombosis12Not a study of CAD
5297Detection and quantitation of calcify atherosclerosis by ultra fast computed tomography in children and young adults with homozygous familial hypercholesterolemiaHoeg JM; Feuerstein IM; Tucker EE;1994Arteriosclerosis & Thrombosis12Not heterozygous population
168Linking genotype to aorta- coronary atherosclerosis, a model using familial hypercholesterolemia and aorta- coronary calcificationJensen JM; Kruse TA; Brorholt-Petersen JU; Christiansen TM; Jensen HK; Kolvraa S; Faergeman O;1999Annals of Human Genetics12Does not answer the question
5300Cardiovascular risk reduction in high-risk pediatric patients, A scientific statement from the American Heart Association expert panel on population and prevention science, the councils on cardiovascular disease in the young, epidemiology and prevention,Kavey RE; Allada V; Daniels SR; Hayman LL; McCrindle BW; Newburger JW; Parekh RS; Steinberger J;2006Circulation12This is a study about risk and does not answer the question
164Characteristic cardiovascular manifestation in homozygous and heterozygous familial hypercholesterolemiaKawaguchi A; Miyatake K; Yutani C; Beppu S; Tsushima M; Yamamura T; Yamamoto A;1999American Heart Journal12Not heterozygous population
343Results of serial coronary angiography in patients with homozygous familial hypercholesterolaemiaKlein JM; Drobinski G; Bruckert E; Dairou F; Thomas D; De G; Grosgogeat Y;1988European Heart Journal12Not heterozygous population
5301Aortic root involvement in homozygous familial hypercholesterolemia-- transesophageal echocardiography appearances of supravalvular aortic stenosisKoh TW;2005Echocardiography12Not heterozygous population
231Development of coronary atherosclerosis in asymptomatic heterozygous patients with familial hypercholesterolemiaMiida T; Nakamura Y; Okada M;1996Journal of Cardiology12Does not answer the question
533Homodynamic and angiographic evaluation of cardiovascular involvement in patients with homozygous familial hypercholesterolemiaPiscione F; Cappelli- Bigazzi M; Postiglione A; Gnasso A; Focaccio A; Chiariello M;1990Coronary Artery Disease12Not heterozygous population
313Relative protection from cerebral atherosclerosis of young patients with homozygous familial hypercholesterolemiaPostiglione A; Nappi A; Brunetti A; Soricelli A; Rubba P; Gnasso A; Cammisa M; Frusciante V; Cortese C; Salvatore M; et a;1991Atherosclerosis12Wrong population and wrong question
5303Extent and severity of atherosclerotic involvement of the aortic valve and root in familial hypercholesterolaemiaRallidis L; Naoumova RP; Thompson GR; Nihoyannopoulos P;1998Heart12Wrong for question - not ongoing assessment or evaluation of test
234Aortic stenosis in homozygous familial hypercholesterolaemiaRallidis L; Nihoyannopoulos P; Thompson GR;1996Heart12Wrong population
347Extra coronary atherosclerosis in familial hypercholesterolemiaRubba P; De S; Postiglione A; Cortese C; Gnasso A; Mancini M;1988Atherosclerosis12Did not answer the question
1081Coronary Risk-Factors and the Severity of Angiographic Coronary-Artery Disease in Members of High-Risk PedigreesSharp SD; Williams RR; Hunt SC; Schumacher MC;1992American Heart Journal12Study wrong for the question. This study refers to coronary risk.
5304Clinical review 168, What vascular ultrasound testing has revealed about pediatric parthenogenesis, and a potential clinical role for ultrasound in pediatric risk assessment. [Review] [77 refs]Slyper AH;2004Journal of Clinical Endocrinology & Metabolism12Narrative review
5305Cardiovascular features of homozygous familial hypercholesterolemia, analysis of 16 patientsSprecher DL; Schaefer EJ; Kent KM; Gregg RE; Zech LA; Hoeg JM; McManus B; Roberts WC; Brewer HBJ;1984American Journal of Cardiology12Wrong population
188Evaluation of the aortic root by MRI, insights from patients with homozygous familial hypercholesterolemiaSummers RM; ndrasko-Bourgeois J; Feuerstein IM; Hill SC; Jones EC; Busse MK; Wise B; Bove KE; Rishforth BA; Tucker E; Spray TL; Hoeg JM;1998Circulation12Not heterozygous population
5266Carotid intima-media thickness and plaque in patients with familial hypercholesterolaemia mutations and control participants.[see comment]Tonstad S; Joakimsen O; Stensland-Bugge E; Ose L; Bonaa KH; Leren TP;1998European Journal of Clinical Investigation12CMT not our outcome measure
1487Arterial intima-media thickness in children heterozygous for familial hypercholesterolaemiaWiegman A; de Groot E; Hutten BA; Rodenburg J; Gort J; Bakker HD; Sijbrands EJG; Kastelein JJP;2004Lancet12Does not address ongoing assessment or an evaluation of testing
5331Long-term compliance and changes in plasma lipids, plant sterols and arytenoids in children and parents with FH consuming plant sterol ester-enriched spreadAmundsen AL; Ntanios F; Put Nv; Ose L;2004European Journal of Clinical Nutrition13This is an open label follow up of the 2002 study in the Cochrane systematic review
5332Plant sterol ester-enriched spread lowers plasma total and LDL cholesterol in children with familial hypercholesterolemiaAmundsen AL; Ose L; Nenseter MS; Ntanios FY;2002American Journal of Clinical Nutrition13This study is included in a systematic review
2611Tracing and treatment of children with essential familial hypercholesterolemiaAndersen GE; Clausen J;1972Zeitschrift fur Ernahrungswissenschaft13Poorly designed and reported study
252Fish oil supplementation in patients with heterozygous familial hypercholesterolemiaBalestrieri GP; Maffi V; Sleiman I; Spandrio S; Di SO; Salvi A; Scalvini T;1996Recenti Progressi in Medicina13This paper is included in a systematic review.
878Treatment of severe familial hypercholesterolemia in childhood with sitosterol and sitosterolBecker M; Staab D; Von BK;1993Journal of Pediatrics13This study in included in both systematic reviews.
880Dietary management of patients with familial hypercholesterolaemia treated with simvastatinChisholm A; Mann J; Sutherland W; Williams S; Ball M;1992Quarterly Journal of Medicine13This study is included in a systematic review.
5333The effect of dietary fat content on plasma no cholesterol sterol concentrations in patients with familial hypercholesterolemia treated with simvastatinChisholm A; Sutherland W; Ball M;1994Metabolism, Clinical and Experimental13This study is included in a systematic review.
925Dietary treatment of familial hypercholesterolemiaConnor WE; Connor SL;1989Arteriosclerosis13This is a narrative paper.
860Importance of diet in the treatment of familial hypercholesterolemiaConnor WE; Connor SL;1993American Journal of Cardiology13This is a narrative review.
5334Plant sterols lower LDL cholesterol without improving endothelial function in prepubertal children with familial hypercholesterolaemiade Jongh S; Vissers MN; Rol P; Bakker HD; Kastelein JJP; Stroes ESG;2003Journal of Inherited Metabolic Disease13This study is included in a systematic review.
377Effects of n-3 and n-6 fatty acid- enriched diets on plasma lipoproteins and apolipoproteins in heterozygous familial hypercholesterolemiaFriday KE; Failor RA; Childs MT; Bierman EL;1991Arteriosclerosis & Thrombosis13This is a case series
373Dietary treatment for familial hypercholesterolemia--differential effects of dietary soy protein according to the apolipoprotein E phenotypesGaddi A; Ciarrocchi A; Matteucci A; Rimondi S; Ravaglia G; Descovich GC; Sirtori CR;1991American Journal of Clinical Nutrition13Not RCT
517Pediatric familial type II hyperlipoproteinemia, therapy with diet and colestipol resinGlueck CJ; Fallat RW; Mellies M; Tsang RC;1976Pediatrics13Not RCT
709Safety and efficacy of long-term diet and diet plus bile acid- binding resin cholesterol-lowering therapy in 73 children heterozygous for familial hypercholesterolemiaGlueck CJ; Mellies MJ; Dine M; et a;1986Pediatrics13Not RCT
364Relationships of serum plant sterols (phytosterols) and cholesterol in 595 hypercholesterolemic participants, and familial aggregation of phytosterols, cholesterol, and premature coronary heart disease in hyperphytosterolemic probands and their first-degree rGlueck CJ; Speirs J; Tracy T; Streicher P; Illig E; Vandegrift J;1991Metabolism, Clinical and Experimental13Population not all FH, does not answer the question.
66Therapy of familial hypercholesterolemia in childhood, diet and cholestyramine resin for 24 to 36 monthsGlueck CJ; Tsang RC; Fallat RW; Mellies M;1977Pediatrics13Not RCT
516Diet in children heterozygous for familial hypercholesterolemiaGlueck CJ; Tsang RC; Fallat RW; Mellies MJ;1977American Journal of Diseases of Children13This study is in a systematic review.
478Soybean protein independently lowers plasma cholesterol levels in primary hypercholesterolemiaGoldberg AP; Lim A; Kolar JB; Grundhauser JJ; Steinke FH; Schonfeld G;1982Atherosclerosis13Not RCT, not defined FH population.
5335Effect of a rapeseed oil substituting diet on serum lipids and lipoproteins in children and adolescents with familial hypercholesterolemiaGulesserian T; Widhalm K;2002Journal of the American College of Nutrition13Not RCT
813Sitostanol ester margarine in dietary treatment of children with familial hypercholesterolemiaGylling H; Siimes MA; Miettinen TA;1995Journal of Lipid Research13This study is included in a systematic review.
1205Comparison of the effects of plant sterol ester and plant stanol ester-enriched margarines in lowering serum cholesterol concentrations in hypercholesterolaemic participants on a low-fat dietHallikainen MA; Sarkkinen ES; Gylling H; Erkkila AT; Uusitupa MIJ;2000European Journal of Clinical Nutrition13Not FH population
5336Influence of diets containing cow’s milk or soy protein beverage on plasma lipids in children with familial hypercholesterolemiaJacques H; Laurin D; Moorjani S; Steinke FH; Gagné C; Brun D; Lupien PJ;1992Journal of the American College of Nutrition13Not RCT
5338Squalene and noncholesterol sterols in serum and lipoproteins of children with and without familial hypercholesterolemiaKetomäki A; Gylling H; Siimes MA; Vuorio A; Miettinen TA;2003Pediatric Research13Does not answer the question
532The treatment of familial hypercholesterolemia with a plant sterolKNODE KT; LEVKOFF AH;1957Pediatrics13Not RCT. This is a case study
3326Treatment of hypercholesterolemia in children and adolescentsKoletzko B; Kupke I; Wendel U;1992Acta Paediatrica13Not RCT
140Safety and efficacy of treatment of children and adolescents with elevated low density lipoprotein levels with a step two diet or with lovastatinKwiterovich PO;2001Nutrition Metabolism and Cardiovascular Diseases13This is a narrative paper.
5340Cholesterol-free diet with a high ratio of polyunsaturated to saturated fatty acids in heterozygous familial hypercholesterolemia, significant lowering effect on plasma cholesterolMokuno H; Yamada N; Sugimoto T; Kobayashi T; Ishibashi S; Shimano H; Takizawa M; Kawakami M; Takaku F; Murase T;1990Hormone and Metabolic Research13Poorly designed study of 11 days duration
5344Randomised controlled trial of use by hypercholesterolaemic patients of a vegetable oil sterol- enriched fat spreadNeil HA; Meijer GW; Roe LS;2001Atherosclerosis13This study is included in a systematic review.
54Plant stanol and sterol esters in the control of blood cholesterol levels, mechanism and safety aspectsPlat J; Mensink RP;2005American Journal of Cardiology13This is a narrative paper.
5348Sitosterol in juvenile type II hyperlipoproteinemiaSchlierf G; Oster P; Heuck CC; Raetzer H; Schellenberg B;1978Atherosclerosis13This study is included in a systematic review.
566Diet only and diet plus simvastatin in the treatment of heterozygous familial hypercholesterolemia in childhoodStefanutti C; Lucani G; Vivenzio A; Di G;1999Drugs Under Experimental & Clinical Research13Not RCT
5343Stanol ester margarine alone and with simvastatin lowers serum cholesterol in families with familial hypercholesterolemia caused by the FH-North Karelia mutationVuorio AF; Gylling H; Turtola H; Kontula K; Ketonen P; Miettinen TA;2000Arteriosclerosis, Thrombosis & Vascular Biology13This study is in a systematic review.
328Effect of soy protein diet versus standard low fat, low cholesterol diet on lipid and lipoprotein levels in children with familial or polygenic hypercholesterolemiaWidhalm K; Brazda G; Schneider B; Kohl S;1993Journal of Pediatrics13Not RCT, not defined FH population.
5347Potential role of raising dietary protein intake for reducing risk of atherosclerosisWolfe BM;1995Canadian Journal of Cardiology13This study is in a systematic review.
5345High protein diet complements resin therapy of familial hypercholesterolemiaWolfe BM; Giovannetti PM;1992Clinical and Investigative Medicine13Not RCT
5320The use of a large-scale surveillance system in Planned Parenthood Federation of America clinics to monitor cardiovascular events in users of combination oral contraceptivesBurnhill MS;1999International Journal of Fertility & Womens Medicine14Not FH population
424Estrogen and progestin components of oral contraceptives, Relationship to vascular diseaseCarr BR; Ory H;1997Contraception14Narrative review only
160Hormones and cardiovascular health in womenCrosignani PG; Farley T; Fauser B; Glasier A; Greer I; Hanson MA; La V; Mishell D; Rosano G; Simon T; Baird DT; Benagiano G; Collins J; Dicfalusy E; Lanzone A; Negri E; Schmidt-Gollwitzer K; Skouby SO; Volpe A;2006Human Reproduction Update14Narrative review only
5321Safety of implantable contraceptives for women, data from observational studiesCurtis KM;2002Contraception14Not FH population. No discussion of women with cardiovascular disease
5322Contraception for women in selected circumstancesCurtis MK; Chrisman CE; Peterson HB;2002Obstetrics and Gynecology14Not FH population or women at high risk for CV disease
5323Oral contraceptive-induced changes in plasma lipids, do they have any clinical relevance?.Hoppe G;1987Clinical Reproduction & Fertility14Narrative review. No systematic strategy provided
412Estimates of the risk of cardiovascular death attributable to low-dose oral contraceptives in the United StatesSchwingl PJ; Ory HW; Visness CM;1999American Journal of Obstetrics and Gynecology14Not FH population
5325Myocardial infarction and use of low-dose oral contraceptives, A pooled analysis of 2 US studiesSidney S; Siscovick DS; Petitti DB; Schwartz SM; Quesenberry CP; Psaty BM; Raghunathan TE; Kelaghan J; Koepsell TD;1998Circulation14Not FH population
284Metabolic changes in Singapore women using NORPLANT(TM) implants, A four year reviewSingh K; Viegas OAC; Ratnam SS;1991Advances in Contraceptive Delivery Systems14Not FH or women on statins or with high CV risk
204The risk of myocardial infarction is lower with third-, compared to second-, generation OC - Meta- analysisSpitzer WO; Faith JM; MacRae KD; Dunn N;2003Evidence-Based Obstetrics & Gynecology14Commentary only
132Past use of oral contraceptives and cardiovascular disease, a meta-analysis in the context of the Nurses’ Health StudyStampfer MJ; Willett WC; Colditz GA; Speizer FE; Hennekens CH;1990American Journal of Obstetrics & Gynecology14Not FH population - a study of women free from CV disease
166Pregnancy outcome in familial homozygous hypercholesterolemic females treated with long-term plasma exchangeBeigel Y; Bar J; Cohen M; Hod M;1998Acta Obstetricia et Gynecologica Scandinavica15Case study only
5381Risks of statin use during pregnancy, a systematic reviewKazmin A; Garcia BF; Koren G;2007Journal of Obstetrics and Gynaecology Canada15Study does not add anything to the review
55Differential indication of lipoprotein apheresis during pregnancy.Klingel R; Gohlen B; Schwarting A; Himmelsbach F; Straube R;2003Therapeutic Apheresis & Dialysis, Official Peer- Reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy15Narrative review only
105Pregnancy in a patient with homozygous familial hypercholesterolemia treated with long-term low-density lipoprotein apheresisKroon AA; Swinkels DW; van D; Stalenhoef AF;1994Metabolism, Clinical & Experimental15Case study only
230Maternal hyperlipidemia in pregnancySalameh WA; Mastrogiannis DS;1994Clinical Obstetrics & Gynecology15Narrative review only
102Pregnancy in a patient with homozygous familial hypercholesterolemia undergoing low-density lipoprotein apheresis by dextran sulfate adsorptionTeruel JL; Lasuncion MA; Navarro JF; Carrero P; Ortuno J;1995Metabolism, Clinical & Experimental15Case study only
5369Systematic review on the risk and benefit of different cholesterol- lowering interventionsBucher HC; Griffith LE; Guyatt GH;1999Arteriosclerosis, Thrombosis & Vascular Biology17No quality assessment
5368N-3 polyunsaturated fatty acids in coronary heart disease, A meta- analysis of randomized controlled trialsBucher HC; Hengstler P; Schindler C; Meier G;2002American Journal of Medicine17Cholesterol not an outcome
272How effective are dietary interventions in lowering lipids in adults with dyslipedemia?Buckley D; Muench J; Hamilton A;2007Journal of Family Practice17No methodology
5365Dietary lipids and blood cholesterol, quantitative meta- analysis of metabolic ward studiesClarke R; Frost C; Collins R; Appleby P; Peto R;1997British Medical Journal17No quality assessment
158Long-term safety and efficacy of low-fat diets in children and adolescentsClauss SB; Kwiterovich PO;2002Minerva Paediatrica17No methodology
275What is the dietary treatment for low HDL cholesterol?Crawford P; Paden SL;2006Journal of Family Practice17No methodology
5370Systematic review of randomised controlled trials of multiple risk factor interventions for preventing coronary heart diseaseEbrahim S; Smith GD;1997British Medical Journal17Multiple risk factor intervention
5371Secondary prevention of coronary heart disease in primary careEnriquez PA; Khunti K;2001Journal of Clinical Governance17This is a guideline not a meta analysis
85Monounsaturated versus polyunsaturated dietary fat and serum lipids, a meta-analysisGardner CD; Kraemer HC;1995Arteriosclerosis, Thrombosis & Vascular Biology17Only Medline was searched
5373Dietary fat intake and prevention of cardiovascular disease, systematic reviewHooper L; Summerbell CD; Higgins JP; Thompson RL; Capps NE; Smith GD; Riemersma RA; Ebrahim S;2001British Medical Journal17Cholesterol not an outcome
544Omega 3 fatty acids for prevention and treatment of cardiovascular diseaseHooper L; Thompson RL; Harrison RA; Summerbell CD; Moore H; Worthington HV; Durrington PN; Ness AR; Capps NE; Davey SG; Riemersma RA; Ebrahim SBJ;200417Not relevant to guideline question - Omega 3
5376Effectiveness of individual lifestyle interventions in reducing cardiovascular disease and risk factorsKetola E; Sipila R; Makela M;2000Annals of Medicine17Not our population
445Meta-analysis, clinical trials, and transferability of research results into practice, The case of cholesterol-lowering interventions in the secondary prevention of coronary heart diseaseMarchioli R; Marfisi RM; Carinci F; Tognoni G;1996Archives of Internal Medicine17Wrong population - study looked at secondary prevention of coronary health disease
117Dietetic guidelines on food and nutrition in the secondary prevention of cardiovascular disease - evidence from systematic reviews of randomized controlled trials (second update, January 2006)Mead A; Atkinson G; Albin D; Alphey D; Baic S; Boyd O; Cadigan L; Clutton L; Craig L; Flanagan C; Greene P; Griffiths E; Lee NJ; Li M; McKechnie L; Ottaway J; Paterson K; Perrin L; Rigby P; Stone D; Vine R; Whitehead J; Wray L; Hooper L;2006Journal of human nutrition and dietetics17Wrong population - study looked at secondary prevention of cardiovascular disease
240Effect of dietary fatty acids on serum lipids and lipoproteins. A meta-analysis of 27 trialsMensink RP; Katan MB;1992Arteriosclerosis & Thrombosis17No clear methodology or quality assessment
5366Randomised trial of lipid lowering dietary advice in general practice, the effects on serum lipids, lipoproteins, and antioxidantsNeil HA; Roe L; Godlee RJ; Moore JW; Clark GM; Brown J; Thorogood M; Stratton IM; Lancaster T; Mant D;.;1995British Medical Journal17This trial was included in the Cochrane review “Reduced or modified dietary fat for preventing cardiovascular disease (Review)” Hooper et al 2000. This is an RCT, only meta analyses are to be included
5377Effects of low-carbohydrate vs low-fat diets on weight loss and cardiovascular risk factors, a meta-analysis of randomized controlled trialsNordmann AJ; Nordmann A; Briel M; Keller U; Yancy WS; Brehm BJ; Bucher HC;2006Archives of Internal Medicine17Wrong population - study only looked at over-weight population.
5364Systematic review of dietary intervention trials to lower blood total cholesterol in free-living participantsTang JL; Armitage JM; Lancaster T; Silagy CA; Fowler GH; Neil HA;1998British Medical Journal17This paper is reasonably old (1998) and a Cochrane review has been included which is more up to date
5378Review of dietary intervention studies, effect on coronary events and on total mortalityTruswell AS;1994Australian and New Zealand Journal of Medicine17No methodology

From: Appendix C, Clinical data extractions and excluded studies

Cover of Identification and Management of Familial Hypercholesterolaemia (FH)
Identification and Management of Familial Hypercholesterolaemia (FH) [Internet].
NICE Clinical Guidelines, No. 71.
National Collaborating Centre for Primary Care (UK).
Copyright © 2008, Royal College of General Practitioners.

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