LDL-C–Lowering Therapies for Adults and Children With Homozygous Familial Hypercholesterolemia: Challenges and Successes

Evolving Concepts of the SCORE System: Subtracting Cholesterol from Risk Estimation: A Way for a Healthy Longevity?

“…Thus, early and intense reduction is mandatory to achieve regression of atherosclerosis and, consequently, a reduction in coronary events and mortality [ 86 ]. LLT should be initiated in children [ 89 ]. According to ESC/EAS guidelines, at diagnosis, the first step of FH treatment in children is a healthy diet and lifestyle, followed by statins from the age of 6 to 10 years, starting with increasing doses (low to high) in order to reach the targets.…”

“…According to ESC/EAS guidelines, at diagnosis, the first step of FH treatment in children is a healthy diet and lifestyle, followed by statins from the age of 6 to 10 years, starting with increasing doses (low to high) in order to reach the targets. The goal in children >10 years of age is an LDL-C < 135 mg/dL, and at younger ages, a ≥50% reduction in LDL-C [ 86 , 89 ]. A very high LDL-C or additional CV risk factors may decrease this target or the age recommended for starting statin therapy [ 86 , 89 ].…”

“…The goal in children >10 years of age is an LDL-C < 135 mg/dL, and at younger ages, a ≥50% reduction in LDL-C [ 86 , 89 ]. A very high LDL-C or additional CV risk factors may decrease this target or the age recommended for starting statin therapy [ 86 , 89 ]. According to the 2023 Updated EAS consensus statement, in HoFH children and/or adolescents, the LDL-C goal is <115 mg/dL if treatment is initiated before 18 years, in the absence of ASCVD at imaging assessment, with a lower goal in those with established ASCVD [ 86 ].…”

“…With respect to this analysis and this point of view, several different studies have shown and replicated the major relationship between LDL-C and ASCVD [ 1 , 4 , 8 , 10 , 60 , 104 , 105 , 106 ]. Moreover, interventional trials have clearly indicated that LLT, not only statins, reduce LDL-C and, consequently, MACEs [ 6 , 9 , 20 , 22 , 23 , 37 , 60 , 74 , 78 , 89 , 107 , 108 , 109 , 110 , 111 ]. Certainly, there is a residual risk, non-LDL-C-related, that should be taken into account and opportunely treated [ 112 , 113 , 114 , 115 ].…”