A nation-wide survey of patients with homozygous familial hypercholesterolemia phenotype undergoing LDL-apheresis in Turkey (A-HIT 1 registry)

Lipoprotein apheresis efficacy, challenges and outcomes: A descriptive analysis from the UK Lipoprotein Apheresis Registry, 1989–2017

“…Ideally this should be two plasma or blood volumes per procedure (acute reduction > 80%) at weekly intervals. HoFH patients in the Turkish registry achieved a mean acute reduction in LDL of 68.2% in the last four sessions of LA [17] which is similar to the 69.22% reduction seen in the HoFH patients in the UK registry. Data from a Norwegian study of seven patients with HoFH showed comparable results with an average acute reduction in LDL of 62.71%.…”

“…Recent registry data from Germany presented data from 1435 patients in 71 centres over 4 years [6]. In Turkey there is a national registry for HoFH patients which, in 2018, included 88 patients undergoing regular LA in 19 specialised centres [17]. None of these patients had genetic confirmation of HoFH and the diagnosis is based on clinical criteria alone.…”

“…There is limited information in the registry, some of which was entered several years ago and it would be beneficial to include some additional fields such as length of each LA treatment and adverse effects to provide a more comprehensive report. LA is generally felt to be a safe treatment with a low incidence of adverse events [17]. Adverse events are currently not collected in the apheresis registry and therefore it is unknown if the incidence in the UK is comparable with other centres around the world.…”

A cross-national investigation of cardiovascular survival in homozygous familial hypercholesterolemia: The Sino-Roman Study

“…This has been well demonstrated by our study, as well as experience from centers in Turkey. 48 We classified genetic mutations according to the methodology used in previous research. 14,49 Studies in hoFH from the United States, 50 Spain, 51 and France 14 have demonstrated that patients with receptor negativemutations exhibit earlier onset of CVD and reduced survival compared with those with receptor defective mutations; our results accord with this ( Fig.…”

Negative impact of COVID-19 pandemic on the lifestyle and management of patients with homozygous familial hypercholesterolemia

“…Of note, we have been collaborating with these centers for a nationwide registry provided insight into the real-life management of patients with HoFH undergoing LA in Turkey. 3 Information received from other centers revealed that LA was accessible in 61% of the centers. However, most of the patients (75%) were not attending their LA sessions with the fear of getting infected.…”