Background and objectives: 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 data from the
pilot phase 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 June 2018, 19 cases with FH (26% males,
mean age 36.6 ±10.4 years) were enrolled. Median time from diagnosis to
enrolment was 4 (range 1-13) years. Dutch Lipid Network criteriawas used
in all patients, with 63%, 11% and 26% in the definite, probable and possible
categories respectively. Mean baseline levels for total cholesterol was
393±129 mg/dl, for triglycerides was 281±181 mg/dl, for LDL-C was
293±131 mg/dl and for HDL-C was 40±16 mg/dl. For economic reasons, no
genetic tests were done for diagnosis confirmation. All patients received
lipid-lowering therapy (32% monotherapy and 68% combination with
Ezetimibe). Fibrates were added in 26% of cases. Only one patient received
lipoprotein apheresis. No patients received PCSK-9 inhibitors.
Conclusion: The pilot phase of the Egyptian FHRF registry, to our knowledge,
is the first FH registry in Egypt. The preliminary data 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. |
