Statins Treatment and Oro-Dental Aspects in a Case of Hereditary Hypercholesterolemia in a Child Under 6 Years

Abstract

Familial hypercholesterolemia (FH) is a genetic disease with autosomal dominant transmission, characterised by high blood cholesterol levels. The evolution of this disease leads to primary atherosclerosis and cardiovascular disease. Patients with HF develop atherosclerosis by the age of 20 and usually do not survive past the age of 30. We present the case and oro-dental aspects of a preschooler that was diagnosed at the age of 4 with FH, compound heterozygote (mutation/genotype1 LDLR: C20IX, exon 4; mutation/ genotype2 LDLR: G571E, exon 12) and the experience of our clinic in the management of this patient that received offlabel treatment with statins. When diagnosed, his cholesterol level was 932 mg/dL and his LDL-cholesterol level was 792 mg/dL. Treatment with rosuvastatin and ezetimibe was prescribed. Both substances (rosuvastatin and ezetimibe) are not approved for children under the age of 6 in Europe. Taking into considerations the diagnosis and prognosis for unfavorable evolution, treatment with statins was started at the age of 5 years.

Changes in Rosuvastatin Pharmacokinetics During Postnatal Ontogenesis in Rats

“…Constantin et al described the case of a 5-year-old boy, in whom rosuvastatin treatment for 2.5 years has led to the reduction of LDL and total cholesterol levels by 42.4 and 36.4%, respectively. No concurrent adverse effects were observed in this patient (2). Miyagi et al came with similar findings in 4-yearold twins treated with pitavastatin combined with ezetimibe.…”

“…Familial hypercholesterolemia (FH) represents a frequent genetic disorder phenotypically characterized by increased serum cholesterol and low-density lipoprotein (LDL) levels (1). The prevalence of FH is estimated to range between 1/200 -1:300 in western societies, while the prevalence of homozygous FH is approximately 1:160,000 -300,000 (2).…”

Atherosclerosis from Newborn to Adult—Epidemiology, Pathological Aspects, and Risk Factors

“…Statins (3-hydroxy-3-methyl-glutaryl-CoA reductase inhibitors), the most common type of substance prescribed, can reduce the LDL cholesterol level in 20-49% of cases, and thereby, the risk of atherosclerotic cardiovascular events is reduced by 22% [146]. Regarding treatment with statins in pediatric patients, there are very few studies, although guidelines recommend starting treatment at the age of 8-10 years, after 6-12 months of diet intervention [147]. Next to lipid-lowering treatments, developing and testing new anti-inflammatory drugs is needed in the future therapeutic approach to cardiovascular disease caused by ATS [148].…”