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  1. Watts GF, Gidding S, Wierzbicki AS, Toth PP, Alonso R, Brown WV, et al.
    J Clin Lipidol, 2014 Mar-Apr;8(2):148-72.
    PMID: 24636175 DOI: 10.1016/j.jacl.2014.01.002
    Familial hypercholesterolemia (FH) is a dominantly inherited disorder present from birth that markedly elevates plasma low-density lipoprotein cholesterol and causes premature coronary heart disease. There are at least 20 million people with FH worldwide, but the majority remains undetected, and current treatment is often suboptimal. To address this major gap in coronary prevention we present, from an international perspective, consensus-based guidance on the care of FH. The guidance was generated from seminars and workshops held at an international symposium. The recommendations focus on the detection, diagnosis, assessment, and management of FH in adults and children and set guidelines for clinical purposes. They also refer to best practice for cascade screening and risk notifying and testing families for FH, including use of genetic testing. Guidance on treatment is based on risk stratification, management of noncholesterol risk factors, and the safe and effective use of low-density lipoprotein-lowering therapies. Recommendations are given on lipoprotein apheresis. The use of emerging therapies for FH is also foreshadowed. This international guidance acknowledges evidence gaps but aims to make the best use of contemporary practice and technology to achieve the best outcomes for the care of FH. It should accordingly be used to inform clinical judgment and be adjusted for country-specific and local healthcare needs and resources.
    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy*
  2. EAS Familial Hypercholesterolaemia Studies Collaboration, Vallejo-Vaz AJ, Akram A, Kondapally Seshasai SR, Cole D, Watts GF, et al.
    Atheroscler Suppl, 2016 Dec;22:1-32.
    PMID: 27939304 DOI: 10.1016/j.atherosclerosissup.2016.10.001
    The potential for global collaborations to better inform public health policy regarding major non-communicable diseases has been successfully demonstrated by several large-scale international consortia. However, the true public health impact of familial hypercholesterolaemia (FH), a common genetic disorder associated with premature cardiovascular disease, is yet to be reliably ascertained using similar approaches. The European Atherosclerosis Society FH Studies Collaboration (EAS FHSC) is a new initiative of international stakeholders which will help establish a global FH registry to generate large-scale, robust data on the burden of FH worldwide.
    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy*
  3. Chua YA, Razman AZ, Ramli AS, Mohd Kasim NA, Nawawi H
    J Atheroscler Thromb, 2021 Oct 01;28(10):1095-1107.
    PMID: 33455995 DOI: 10.5551/jat.57026
    AIM: Familial hypercholesterolaemia (FH) is the most common autosomal dominant lipid disorder, leading to severe hypercholesterolaemia. Early detection and treatment with lipid-lowering medications may reduce the risk of premature coronary artery disease in FH patients. However, there is scarcity of data on FH prevalence, detection rate, treatment and control with lipid-lowering therapy in the Malaysian community.

    METHODS: Community participants (n=5130) were recruited from all states in Malaysia. Blood samples were collected for lipid profiles and glucose analyses. Personal and family medical histories were collected by means of assisted questionnaire. Physical examination for tendon xanthomata and premature corneal arcus were conducted on-site. FH were clinically screened using Dutch Lipid Clinic Network Criteria.

    RESULTS: Out of 5130 recruited community participants, 55 patients were clinically categorised as potential (Definite and Probable) FH, making the prevalence FH among the community as 1:100. Based on current total population of Malaysia (32 million), the estimated number of FH patients in Malaysia is 320,000, while the detection rates are estimated as 0.5%. Lipid-lowering medications were prescribed to 54.5% and 30.5% of potential and possible FH patients, respectively, but none of them achieved the therapeutic LDL-c target.

    CONCLUSION: Clinically diagnosed FH prevalence in Malaysian population is much higher than most of the populations in the world. At community level, FH patients are clinically under-detected, with majority of them not achieving target LDL-c level for high-risk patients. Therefore, public health measures are warranted for early detection and treatment, to enhance opportunities for premature CAD prevention.

    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy
  4. Masaany M, Siti HS, Nurliza I, Mazita A
    Otolaryngol Head Neck Surg, 2008 Jun;138(6):803-4.
    PMID: 18503863 DOI: 10.1016/j.otohns.2008.02.020
    Cholesterol granuloma (CG) is a histologic description of foreign body giant cell formation toward cholesterol crystals. The majority of temporal bone CG is unilateral and most common in the petrous apex. Middle ear CG is usually the result of underlying ear diseases. Primary middle ear CG is very rare. Most reported CG has not been associated with familial hypercholesterolemia (FH). FH, an autosomal dominant disorder, manifests as high levels of serum cholesterol and low density lipoprotein (LDL) cholesterol. We report a rare case of FH and bilateral aggressive primary middle ear CG. This publication has been approved by the IRB, Hospital Alor Setar.
    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy
  5. Khoo KL, Page MM, Liew YM, Defesche JC, Watts GF
    J Clin Lipidol, 2016 05 13;10(5):1188-94.
    PMID: 27678436 DOI: 10.1016/j.jacl.2016.05.006
    BACKGROUND: Familial hypercholesterolemia (FH) leads to premature coronary artery disease and aortic stenosis, with undertreated severe forms causing death at a young age. Lipoprotein apheresis (LA) is often required for lowering low-density lipoprotein cholesterol levels in severe FH.

    OBJECTIVES: The objective of this study was to present the first experiences with LA in Malaysia, between 2004 and 2014.

    METHODS: We retrospectively collected data from patient records to assess the effectiveness, adverse effects, patient quality of life, and costs associated with an LA service for genetically confirmed homozygous and heterozygous FH.

    RESULTS: We treated 13 women and 2 men aged 6 to 59 years, 10 with homozygous and 5 with heterozygous FH, all on maximally tolerated cholesterol-lowering drug therapy, for a total of 65 patient-years. Acute lowering of low-density lipoprotein cholesterol post apheresis was 56.3 ± 7.2%, with time-averaged mean lowering of 34.9 ± 13.9%. No patients experienced any cardiovascular events during the period of receiving LA. Patients receiving LA experienced few side effects and enjoyed reasonable quality of life, but inability to continue treatment was frequent because of cost.

    CONCLUSION: LA for severe FH can be delivered effectively in the short term in developing nations, but costs are a major barrier to sustaining this mode of treatment for this high-risk group of patients. New drug therapies for FH, such as the proprotein convertase subtilisin/kexin type 9 inhibitors, microsomal triglyceride transfer protein inhibitors, and apolipoprotein-B100 antisense oligonucleotides may allow improved care for these patients, but costs and long-term safety remain as issues to be addressed.

    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy*
  6. Pang J, Hu M, Lin J, Miida T, Nawawi HM, Park JE, et al.
    BMJ Open, 2017 Oct 25;7(10):e017817.
    PMID: 29074516 DOI: 10.1136/bmjopen-2017-017817
    OBJECTIVE: To determine physicians' knowledge, awareness and preferences regarding the care of familial hypercholesterolaemia (FH) in the Asia-Pacific region.

    SETTING: A formal questionnaire was anonymously completed by physicians from different countries/regions in the Asia-Pacific. The survey sought responses relating to general familiarity, awareness of management guidelines, identification (clinical characteristics and lipid profile), prevalence and inheritance, extent of elevation in risk of cardiovascular disease (CVD) and practice on screening and treatment.

    PARTICIPANTS: Practising community physicians from Australia, Japan, Malaysia, South Korea, Philippines, Hong Kong, China, Vietnam and Taiwan were recruited to complete the questionnaire, with the UK as the international benchmark.

    PRIMARY OUTCOME: An assessment and comparison of the knowledge, awareness and preferences of FH among physicians in 10 different countries/regions.

    RESULTS: 1078 physicians completed the questionnaire from the Asia-Pacific region; only 34% considered themselves to be familiar with FH. 72% correctly described FH and 65% identified the typical lipid profile, with a higher proportion of physicians from Japan and China selecting the correct FH definition and lipid profile compared with those from Vietnam and Philippines. However, less than half of the physician were aware of national or international management guidelines; this was significantly worse than physicians from the UK (35% vs 61%, p<0.001). Knowledge of prevalence (24%), inheritability (41%) and CVD risk (9%) of FH were also suboptimal. The majority of the physicians considered laboratory interpretative commenting as being useful (81%) and statin therapy as an appropriate cholesterol-lowering therapy (89%) for FH management.

    CONCLUSIONS: The study identified important gaps, which are readily addressable, in the awareness and knowledge of FH among physicians in the region. Implementation of country-specific guidelines and extensive work in FH education and awareness programmes are imperative to improve the care of FH in the region.

    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy*
  7. Hagger MS, Hardcastle SJ, Hu M, Kwok S, Lin J, Nawawi HM, et al.
    Eur J Prev Cardiol, 2018 06;25(9):936-943.
    PMID: 29592531 DOI: 10.1177/2047487318766954
    Background High rates of inadequate health literacy are associated with maladaptive health outcomes in chronic disease including increased mortality and morbidity rates, poor treatment adherence and poor health. Adequate health literacy may be an important factor in the effective treatment and management of familial hypercholesterolemia, and may also be implicated in genetic screening for familial hypercholesterolemia among index cases. The present study examined the prevalence and predictors of health literacy in familial hypercholesterolemia patients attending clinics in seven countries. Design Cross-sectional survey. Methods Consecutive FH patients attending clinics in Australia, Brazil, China, Hong Kong, Malaysia, Taiwan and the UK completed measures of demographic variables (age, gender, household income and highest education level) and a brief three-item health literacy scale. Results Rates of inadequate health literacy were lowest in the UK (7.0%), Australia (10.0%), Hong Kong (15.7%) and Taiwan (18.0%) samples, with higher rates in the Brazil (22.0%), Malaysia (25.0%) and China (37.0%) samples. Income was an independent predictor of health literacy levels, accounting for effects of age. Health literacy was also independently related to China national group membership. Conclusions Findings indicate non-trivial levels of inadequate health literacy in samples of familial hypercholesterolemia patients. Consistent with previous research in chronic illness, inadequate health literacy is related to income as an index of health disparities. Chinese familial hypercholesterolemia patients are more likely to have high rates of inadequate health literacy independent of income. Current findings highlight the imperative of education interventions targeting familial hypercholesterolemia patients with inadequate health literacy.
    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy*
  8. Hagger MS, Hamilton K, Hardcastle SJ, Hu M, Kwok S, Lin J, et al.
    Soc Sci Med, 2019 12;242:112591.
    PMID: 31630009 DOI: 10.1016/j.socscimed.2019.112591
    RATIONALE: Familial Hypercholesterolemia (FH) is a genetic condition that predisposes patients to substantially increased risk of early-onset atherosclerotic cardiovascular disease. FH risks can be minimized through regular participation in three self-management. BEHAVIORS: physical activity, healthy eating, and taking cholesterol lowering medication.

    OBJECTIVE: The present study tested the effectiveness of an integrated social cognition model in predicting intention to participate in the self-management behaviors in FH patients from seven countries.

    METHOD: Consecutive patients in FH clinics from Australia, Hong Kong, Brazil, Malaysia, Taiwan, China, and UK (total N = 726) completed measures of social cognitive beliefs about illness from the common sense model of self-regulation, beliefs about behaviors from the theory of planned behavior, and past behavior for the three self-management behaviors.

    RESULTS: Structural equation models indicated that beliefs about behaviors from the theory of planned behavior, namely, attitudes, subjective norms, and perceived behavioral control, were consistent predictors of intention across samples and behaviors. By comparison, effects of beliefs about illness from the common sense model were smaller and trivial in size. Beliefs partially mediated past behavior effects on intention, although indirect effects of past behavior on intention were larger for physical activity relative to taking medication and healthy eating. Model constructs did not fully account for past behavior effects on intentions. Variability in the strength of the beliefs about behaviors was observed across samples and behaviors.

    CONCLUSION: Current findings outline the importance of beliefs about behaviors as predictors of FH self-management behaviors. Variability in the relative contribution of the beliefs across samples and behaviors highlights the imperative of identifying sample- and behavior-specific correlates of FH self-management behaviors.

    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy
  9. EAS Familial Hypercholesterolaemia Studies Collaboration, Vallejo-Vaz AJ, De Marco M, Stevens CAT, Akram A, Freiberger T, et al.
    Atherosclerosis, 2018 10;277:234-255.
    PMID: 30270054 DOI: 10.1016/j.atherosclerosis.2018.08.051
    BACKGROUND AND AIMS: Management of familial hypercholesterolaemia (FH) may vary across different settings due to factors related to population characteristics, practice, resources and/or policies. We conducted a survey among the worldwide network of EAS FHSC Lead Investigators to provide an overview of FH status in different countries.

    METHODS: Lead Investigators from countries formally involved in the EAS FHSC by mid-May 2018 were invited to provide a brief report on FH status in their countries, including available information, programmes, initiatives, and management.

    RESULTS: 63 countries provided reports. Data on FH prevalence are lacking in most countries. Where available, data tend to align with recent estimates, suggesting a higher frequency than that traditionally considered. Low rates of FH detection are reported across all regions. National registries and education programmes to improve FH awareness/knowledge are a recognised priority, but funding is often lacking. In most countries, diagnosis primarily relies on the Dutch Lipid Clinics Network criteria. Although available in many countries, genetic testing is not widely implemented (frequent cost issues). There are only a few national official government programmes for FH. Under-treatment is an issue. FH therapy is not universally reimbursed. PCSK9-inhibitors are available in ∼2/3 countries. Lipoprotein-apheresis is offered in ∼60% countries, although access is limited.

    CONCLUSIONS: FH is a recognised public health concern. Management varies widely across countries, with overall suboptimal identification and under-treatment. Efforts and initiatives to improve FH knowledge and management are underway, including development of national registries, but support, particularly from health authorities, and better funding are greatly needed.

    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy*
  10. Watts GF, Gidding S, Wierzbicki AS, Toth PP, Alonso R, Brown WV, et al.
    Eur J Prev Cardiol, 2015 Jul;22(7):849-54.
    PMID: 24776375 DOI: 10.1177/2047487314533218
    Familial hypercholesterolaemia (FH) is a dominantly inherited disorder present from birth that markedly elevates plasma low-density lipoprotein (LDL) cholesterol and causes premature coronary heart disease. There are at least 20 million people with FH worldwide, but the majority remains undetected and current treatment is often suboptimal.To address this major gap in coronary prevention we present, from an international perspective, consensus-based guidance on the care of FH. The guidance was generated from seminars and workshops held at an international symposium. The recommendations focus on the detection, diagnosis, assessment and management of FH in adults and children, and set guidelines for clinical purposes. They also refer to best practice for cascade screening and risk notifying and testing families for FH, including use of genetic testing. Guidance on treatment is based on risk stratification, management of non-cholesterol risk factors and safe and effective use of LDL lowering therapies. Recommendations are given on lipoprotein apheresis. The use of emerging therapies for FH is also foreshadowed.This international guidance acknowledges evidence gaps, but aims to make the best use of contemporary practice and technology to achieve the best outcomes for the care of FH. It should accordingly be employed to inform clinical judgment and be adjusted for country-specific and local healthcare needs and resources.
    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy*
  11. Pang J, Chan DC, Hu M, Muir LA, Kwok S, Charng MJ, et al.
    J Clin Lipidol, 2019 01 25;13(2):287-300.
    PMID: 30797720 DOI: 10.1016/j.jacl.2019.01.009
    BACKGROUND: There is a lack of information on the health care of familial hypercholesterolemia (FH).

    OBJECTIVE: The objective of this study was to compare the health care of FH in countries of the Asia-Pacific region and Southern Hemisphere.

    METHODS: A series of questionnaires were completed by key opinion leaders from selected specialist centers in 12 countries concerning aspects of the care of FH, including screening, diagnosis, risk assessment, treatment, teaching/training, and research; the United Kingdom (UK) was used as the international benchmark.

    RESULTS: The estimated percentage of patients diagnosed with the condition was low (overall <3%) in all countries, compared with ∼15% in the UK. Underdetection of FH was associated with government expenditure on health care (ϰ = 0.667, P type 9 inhibitors. A deficit of FH registries, training programs, and publications were identified in less economically developed countries. The demonstration of cost-effectiveness for cascade screening, genetic testing, and specialized treatments were significantly associated with the availability of subsidies from the health care system (ϰ = 0.571-0.800, P 

    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy
  12. Azraii AB, Ramli AS, Ismail Z, Abdul-Razak S, Mohd-Kasim NA, Ali N, et al.
    Atherosclerosis, 2018 10;277:508-516.
    PMID: 30270092 DOI: 10.1016/j.atherosclerosis.2018.08.018
    BACKGROUND AND AIMS: This study aimed to determine knowledge, awareness and practice (KAP) regarding familial hypercholesterolaemia (FH) among Malaysian primary care physicians (PCP), and to compare KAP between PCP with postgraduate qualification (PCP-PG-Qual) and PCP without PG qualification (PCP-noPG-Qual).

    METHODS: This was a cross-sectional study involving PCP with ≥1-year working experience in Malaysian primary care settings. An adapted and validated 25-item FH-KAP questionnaire was disseminated during primary care courses. Total score for each domain was calculated by summing-up the correct responses, converted into percentage scores. Normality distribution was examined and comparisons of mean/median percentage scores were made between the two groups of PCP.

    RESULTS: A total of 372 PCP completed the questionnaire. Regarding knowledge, 77.7% correctly defined FH. However, only 8.3% correctly identified coronary artery disease risk in untreated FH. The mean percentage knowledge score was significantly higher in PCP-PG-Qual compared to PCP-noPG-Qual (48.9, SD ± 13.92 vs. 35.2, SD ± 14.13), t(370) = 8.66, p 

    Matched MeSH terms: Hyperlipoproteinemia Type II/therapy*
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