Background and Aims: The aim of the familial hypercholesterolemia
research forum (FHRF) is to collect date about the clinical and laboratory
phenotypes of the Egyptian patients with FH. We present preliminary
results of the Egyptian registry
Methods: An online electronic case report form (e-CRF) was prepared to
collect data matching the protocol of the familial hypercholesterolemia
Studies Collaboration (FHSC) of the European Atherosclerosis Society (EAS)
Results: From August 2017 to September 2018, 49 cases with FH (33%
males, mean age 45 ±15 years) were enrolled. Median time from diagnosis
to enrollment was 8 (range 1-20) years. Dutch Lipid Network criteria was
used in all patients, with 31%, 12% and 57% in the definite, probable and
possible categories respectively. Mean baseline levels for total cholesterol
was 339±100 mg/dl, for triglycerides was 217±137 mg/dl, for LDL-C was
249±98 mg/dl and for HDL-C was 46±15 mg/dl. For economic reasons, no
genetic tests were done for diagnosis confirmation. All patients received
lipid-lowering therapy (41% monotherapy and 59% combination with
Ezetimibe). Fibrates were added in 10% of cases. Only one patient received
lipoprotein apheresis. No patients received PCSK-9 inhibitors
Conclusions: The Egyptian FHRF registry, to our knowledge, is the first FH
registry in Egypt. The preliminary results showed that the e-CRF system is
feasible and reliable. The phenotype of enrolled FH cases showed very high
lipoprotein levels, aggressive atherosclerosis and inadequate therapeutic
interventions. Further registry data will provide detailed insights about the
magnitude of the problem in Egypt |