TY - JOUR
TI - Characteristics and management of 1093 patients with clinical diagnosis of familial hypercholesterolemia in Greece: Data from the Hellenic Familial Hypercholesterolemia Registry (HELLAS-FH)
AU - Rizos, C.V.
AU - Elisaf, M.S.
AU - Skoumas, I.
AU - Tziomalos, K.
AU - Kotsis, V.
AU - Rallidis, L.
AU - Garoufi, A.
AU - Athyros, V.G.
AU - Skalidis, E.
AU - Kolovou, G.
AU - Koutagiar, I.
AU - Papagianni, M.
AU - Antza, C.
AU - Katsiki, N.
AU - Ganotakis, E.
AU - Liberopoulos, E.N.
JO - Atherosclerosis
PY - 2018
VL - 277
TODO - null
SP - 308-313
PB - Elsevier Ireland Ltd
SN - 0021-9150
TODO - 10.1016/j.atherosclerosis.2018.08.017
TODO - 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
TODO - 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.
ER -