Objectives
We sought to describe the clinical and genetic characteristics of patients with familial hypercholesterolemia (FH) that presented to the lipid clinic at Sultan Qaboos University Hospital, Muscat, Oman.
Methods
Patients who presented with high low-density lipoprotein cholesterol (LDL-C) levels (> 189.0 mg/dL or 4.9 mmol/L) were recruited to the study. FH was diagnosed according to the Dutch Lipid Clinic Network criteria. Analyses were performed using univariate statistics.
Results
The study enrolled 450 patients with a mean age of 48.0±12.0 years, 56.0% (n = 252) were males and 11.3% (n = 51) were smokers. At admission, the proportion of ‘probable/definite’, ‘possible’, and ‘unlikely’ FH were 27.6% (n = 124), 70.0% (n = 315), and 2.4% (n = 11), respectively. Overall, 26.0% (n = 117) of patients had hypertension, 22.4% (n = 101) had a history of coronary artery disease, and 17.3% (n = 78) had diabetes mellitus. Those with ‘probable/definite’ FH were more likely to be prescribed high-intensity statin therapy (75.8% vs. 54.5%;
p
< 0.001) and statin ezetimibe combination (50.8% vs. 27.3%;
p
< 0.001) when compared to the ‘unlikely’ FH cohort. Additionally, those with very high atherosclerotic vascular disease (ASCVD) risk were also associated with high-intensity statin therapy (54.7% vs. 42.7%;
p =
0.006) and statin ezetimibe combination (26.4% vs. 17.2%;
p =
0.023). Patients with ‘probable/definite’ FH were less likely to achieve their LDL-C goal attainment compared to those with ‘unlikely’ FH (13.0% vs. 57.1%;
p
< 0.001). Furthermore, those with very high ASCVD risk were less likely to achieve their LDL-C goals compared to the high ASCVD risk cohort (9.6% vs. 32.0%;
p
< 0.001).
Conclusions
FH patients are underdiagnosed, undertreated, and less likely to attain their LDL-C goals in Oman.
We report our experience with Direct Adsorption of Lipoproteins (DALI) apheresis in an Omani pregnant
woman affected by homozygous familial hypercholesterolemia. To the best of our knowledge this is the first successful
pregnancy treated with DALI apheresis.
The patient had a history of coronary artery disease, supra-aortic valvular stenosis and severe carotid artery disease with
right carotid artery stenting. She was on a regular biweekly DALI apheresis since 2008. In May 2013, she became
pregnant and rosuvastatin and ezetimibe were stopped while she continued on DALI apheresis biweekly. This treatment
during pregnancy was successful with no major complications. The average low-density lipoprotein cholesterol reduction
during therapy was 50%. She spontaneously delivered a healthy male infant (2,400 g) at 37 weeks. We showed that DALI
apheresis therapy was safe during pregnancy with a good outcome for both mother and neonate.
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