Cascade screening program for familial hypercholesterolemia

Endocrinol Diabetes Nutr (Engl Ed). 2018 May;65(5):280-286. doi: 10.1016/j.endinu.2017.12.009. Epub 2018 Mar 6.
[Article in English, Spanish]

Abstract

Introduction and objective: Early detection of heterozygous familial hypercholesterolemia (HFH) is needed to prevent premature cardiovascular events. Our aim isto describe the course of an HFH screening detection day in the Northern Cadiz Health Area in Spain and to analyze the data recorded.

Subjects and methods: Descriptive study of an FH cascade screening program. Index cases (ICs) and their 1st and 2nd grade relatives were appointed during a weekend by the FH Foundation. Venous blood samples were taken from the subjects for genetic, blood, and chemistry tests; specialized medical consultation and physical examination were performed.

Results: The study sample consisted of 132 subjects: 21 ICs and 111 relatives (16 under 18years old), with a mean age of 11.4years (SD4.57). Mean age of subjects over 18years was 45.2years. A gene mutation was found in 90 relatives. Mean age at diagnosis was 25years (SD17.7) for relatives and for 36.4years (SD17.2; P=.01) for ICs. Smoking rate was higher in relatives than in ICs (26.3% vs 4.8%; P=.02) and corneal arcus was more common in ICs as compared to relatives (47.6% vs 12.6%; P<.001). Prior myocardial infarction was recorded in 14.3% of ICs and 4.2% of relatives respectively (P=.07). Maximum lipid lowering treatment was being administered to 43.1%.

Conclusions: The screening detection approach identified the estimated 4% population with HFH in the area, and allows for diagnosing HFH 11.4years earlier.

Keywords: Aterosclerosis; Atherosclerosis; Cardiovascular disease; Cascade screening; Cribado en cascada; Detección genética; Enfermedad cardiovascular; Familial hypercholesterolemia; Genetic testing; Hipercolesterolemia familiar.

MeSH terms

  • Adolescent
  • Adult
  • Anticholesteremic Agents / therapeutic use
  • Apolipoproteins B / genetics
  • Catchment Area, Health
  • Child
  • Cross-Sectional Studies
  • Early Diagnosis
  • Female
  • Genetic Carrier Screening*
  • Genetic Testing / methods*
  • Humans
  • Hydroxymethylglutaryl-CoA Reductase Inhibitors / therapeutic use
  • Hyperlipoproteinemia Type II / diagnosis*
  • Hyperlipoproteinemia Type II / drug therapy
  • Hyperlipoproteinemia Type II / epidemiology
  • Hyperlipoproteinemia Type II / genetics
  • Male
  • Middle Aged
  • Myocardial Infarction / epidemiology
  • Proprotein Convertase 9 / genetics
  • Receptors, LDL / genetics
  • Smoking / epidemiology
  • Socioeconomic Factors
  • Spain / epidemiology

Substances

  • Anticholesteremic Agents
  • Apolipoproteins B
  • Hydroxymethylglutaryl-CoA Reductase Inhibitors
  • LDLR protein, human
  • Receptors, LDL
  • PCSK9 protein, human
  • Proprotein Convertase 9