The 15th Annual Scientific Meeting of the College of Pathologists, Academy of Medicine Malaysia

It has been well established that atherosclerosis begins in childhood. In the Bogalusa heart study, by the age of 15 years, 50% of young people had coronary fatty streaks, and by age 20, 33% had coronary atheroma. Therefore, prevention of atherosclerosis in the paediatric age group is of paramount importance. To understand lipid problems in children more clearly, one needs an understanding of lipid and lipoprotein metabolism. The exogenous pathway, involving lipid ingestion and chylomicron formation, will be described, as will the endogenous pathway which starts with the synthesis of very low density lipoprotein (VLDL) in the liver. Recently, the important role of the peptide PCSK9 in metabolism of low density lipoprotein cholesterol (LDLC) has been elucidated. This has led to exciting new developments in the treatment of hypercholesterolaemia. Primary hyperlipidaemia in childhood can involve increases in both total cholesterol and/or triglycerides. One of the most important conditions to recognise is familial hypercholesterolaemia (FH) which can affect one in 500 of the population. This condition is often not recognised, leading to preventable early deaths. When an index case of FH is recognised, “cascade” screening of first and second degree relatives can lead to detection of new cases and institution of life saving treatment. Hyperlipidaemia, secondary to other diseases, also occurs in paediatrics, so investigation for causative disorders, such as hypothyroidism, is important in the assessment of the hyperlipidaemic child. Integrated guidelines for cardiovascular health and risk reduction in children and adolescents, endorsed by the American Academy of Pediatrics, have been published. In addition to treatment of hyperlipidaemia, other important factors in risk reduction include diet, exercise, tobacco exposure, blood pressure and obesity. The American guidelines recommend screening of all children between the ages of 9 and 11 years for hypercholesterolaemia. An Australian model of care for management of paediatric FH was published in 2013. An important part of management includes the early introduction of statin treatment, as this has been shown to prevent early cardiovascular events and premature death.