Τίτλος:
Characteristics and management of 1093 patients with clinical diagnosis of familial hypercholesterolemia in Greece: Data from the Hellenic Familial Hypercholesterolemia Registry (HELLAS-FH)
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Background and aims: Although familial hypercholesterolemia (FH) is one of the most common genetic disorders, it remains largely underdiagnosed and undertreated. The Hellenic Atherosclerosis Society has established the Hellenic Familial Hypercholesterolemia (HELLAS-FH) Registry, part of the Familial Hypercholesterolemia Studies Collaboration (FHSC), to evaluate the characteristics and management of patients with FH in Greece. Methods: Patients with diagnosed FH were recruited by a network of sites throughout Greece. The prevalence of cardiovascular disease (CVD) risk factors, as well as management of FH, was recorded. Results: This interim analysis included 1093 patients (556 male; 950 adults). The median age of FH diagnosis was 42.2 years (interquartile range 27.2–53.0). A family history of CVD was present in 47.8%, while 21.1% of patients had a personal history of CVD. At diagnosis, low-density lipoprotein cholesterol (LDL-C) was 241 ± 76 mg/dL in adults and 229 ± 57 mg/dL in children. Overall, 63.1% of the patients were receiving hypolipidemic drug treatment, mainly statins, at inclusion in the registry. Mean LDL-C of patients receiving drug treatment was 154 ± 76 mg/dL in adults and 136 ± 47 mg/dL in children. The majority of treated patients (87.9%) did not achieve LDL-C targets. Conclusions: FH in Greece is characterized by a significant delay in diagnosis and a high prevalence of both family and personal history of established CVD. The vast majority of FH patients do not achieve LDL-C targets. Improved awareness and management of FH are definitely needed. © 2018 Elsevier B.V.
Συγγραφείς:
Rizos, C.V.
Elisaf, M.S.
Skoumas, I.
Tziomalos, K.
Kotsis, V.
Rallidis, L.
Garoufi, A.
Athyros, V.G.
Skalidis, E.
Kolovou, G.
Koutagiar, I.
Papagianni, M.
Antza, C.
Katsiki, N.
Ganotakis, E.
Liberopoulos, E.N.
Περιοδικό:
Atherosclerosis
Εκδότης:
Elsevier Ireland Ltd
Λέξεις-κλειδιά:
atorvastatin; ezetimibe; high density lipoprotein cholesterol; lipoprotein A; low density lipoprotein cholesterol; rosuvastatin; stanozolol; biological marker; hypocholesterolemic agent; low density lipoprotein cholesterol, adult; Article; cardiovascular risk; child; cholesterol blood level; clinical feature; familial hypercholesterolemia; family history; female; Greece; human; lipoprotein blood level; major clinical study; male; priority journal; register; school child; blood; cardiovascular disease; clinical trial; down regulation; familial hypercholesterolemia; genetic predisposition; genetics; heredity; middle aged; multicenter study; pedigree; phenotype; prevalence; risk assessment; risk factor; time factor; treatment outcome, Adult; Anticholesteremic Agents; Biomarkers; Cardiovascular Diseases; Cholesterol, LDL; Down-Regulation; Female; Genetic Predisposition to Disease; Greece; Heredity; Humans; Hyperlipoproteinemia Type II; Male; Middle Aged; Pedigree; Phenotype; Prevalence; Registries; Risk Assessment; Risk Factors; Time Factors; Treatment Outcome
DOI:
10.1016/j.atherosclerosis.2018.08.017