title: A systematic review of current studies in patients with familial hypercholesterolemia by use of national familial hypercholesterolemia registries.
abstract: More than 25 million are expected to have familial hypercholesterolemia worldwide. The risk of cardiovascular disease (CVD) in familial hypercholesterolemia is not clear and register studies represent a valuable tool to get such data, which will be discussed in the present paper.
  
  
  
  A systematic review of current familial hypercholesterolemia studies, by use of National familial hypercholesterolemia registries was performed from 1980 to 2016. This review shows that familial hypercholesterolemia patients have a high prevalence of CVD also in the time period after statins became available. The patient group does not reach recommended target values for lipids, and have a significantly higher CVD mortality as compared with the general population according to age and sex.
  
  
  
  The review underscores the importance of establishing National familial hypercholesterolemia registries with complete datasets on familial hypercholesterolemia patients to improve early diagnostics, therapeutics and long-term follow-up to prevent the premature CVD events and deaths in this patient group.
authors.affiliation: aThe Lipid Clinic, Oslo University Hospital bDepartment of Nutrition, Institute of Basic Medical Sciences, University of Oslo, Oslo, Norway.
authors.forename: Liv
authors.initials: L
authors.lastname: Mundal
authors.forename: Kjetil
authors.initials: K
authors.lastname: Retterstøl
mesh_terms: D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000453:epidemiology* / Q000401:mortality; D012042:Registries