NMR-based lipoprotein analysis for patients with severe hypercholesterolemia undergoing lipoprotein apheresis or PCSK9-inhibitor therapy (NAPALI-Study).

Taking into account the discordance between low-density lipoprotein cholesterol (LDL-C) and LDL particle (LDL-P) number, cardiovascular risk more closely correlates with LDL-P in patients. The aim of our study was to evaluate the number of lipid particles in patients with severe hypercholesterolemia treated with different lipid-lowering regimens. Four groups of patients differing with respect to lipid-lowering therapy were recruited from hypercholesterolemic outpatients and lipoprotein apheresis (LA) facilities, and were treated with statins alone (group A), with statins and proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors (PCSK9i) (group B), with statins and LA (group C), or with statins, PCSK9i, and LA (group D). Cholesterol, triglycerides, LDL-C, high-density lipoprotein cholesterol (HDL-C), LDL-P number and size, HDL-P number and size were determined using nuclear magnetic resonance spectroscopy. The lowest LDL-P number was achieved at the end of LA sessions in combination with statins or in combination with statins and a monoclonal PCSK9i (median; 25th and 75th percentile) (group C: 244 nmoL/L: 237, 244, P < 0.05; group D: 244 nmoL/L: 99, 307, P < 0.05). Comparing LDL-P number at the start of LA (group C: 978 nmoL/L: 728, 1404; group D: 954 nmoL/L: 677, 1521) to the other patient groups (groups A and B), the lowest LDL-P number was measured in patients treated with PCSK9i and a statin (group B): LDL-P (762 nmoL/L: 604, 1043, P < 0.05), large LDL-P (472 nmoL/L: 296, 574, P < 0.05), and small LDL-P (342 nmoL/L: 152, 494, P < 0.05). Very low-density lipoprotein and HDL particle sizes remained approximately the same in all groups. LA in combination with statins or in combination with statins and PCSK9i most reduced LDL-P numbers in hypercholesterolemic patients.

Schettler VJ ，Muellendorff F ，Schettler E ，Platzer C ，Norkauer S ，Julius U ，Neumann CL ... -  《-》

Lipoprotein Apheresis and Proprotein Convertase Subtilisin/Kexin Type 9 Inhibitors in Patients With Heterozygous Familial Hypercholesterolemia: A One Center Study.

Kolovou V ，Katsiki N ，Makrygiannis S ，Mavrogieni S ，Karampetsou N ，Manolis A ，Melidonis A ，Mikhailidis DP ，Kolovou GD ... -  《-》

Experience with proprotein convertase subtilisin/kexine type 9 inhibitors (PCSK9i) in patients undergoing lipoprotein apheresis.

We analyzed efficacy and safety of PCSK9i in patients undergoing lipoprotein apheresis (LA) and in patients treated at our outpatient department for metabolic disorders. The medical records of 40 LA patients, taking PCSK9i were reviewed with respect to LDL-cholesterol (LDL-C) and lipoprotein(a) (Lp(a)) lowering as well as occurrence of adverse events. Furthermore, we analyzed the data of 152 patients of our outpatient department, undergoing PCSK9i therapy. Mean pre-apheresis LDL-C value was reduced by PCSK9i from 3.71 ± 1.19 to 1.78 ± 0.84 mmol/l (53 ± 12%). The relative lowering of the pre-apheresis Lp(a) was 20 ± 12% (from 191 ± 63.5 to 152 ± 51.9 nmol/l). 25% of LA patients could stop LA after reaching LDL-C target after initiation of PCSK9i. 75% of the patients are continuing the regular LA therapy, showing an insufficient LDL-C lowering following PCSK9i injections or/and additionally elevated Lp(a) or/and adverse effects of PCSK9i, leading to the discontinuation of injections. The number of LA patients has grown from 112 in 2016 to 128 nowadays due to an increasing percentage of patients with elevated Lp(a) (79% and 89% respectively). The mean reduction rate of LDL-C under PCSK9i therapy in outpatients was 53.03%. In 34% of patients the target value could not be reached. 43% of persons suffered from adverse effects. 3/4 of LA patients could not stop extracorporeal treatment after PCSK9i administration. In hypercholesterolemic patients with coexisting elevated Lp(a) and progressive cardiovascular disease the combination of LA and PCSK9i could be beneficial. The total patients' number in LA units increases due to persons with Lp(a)-hyperlipoproteinemia.

Tselmin S ，Julius U ，Weinert N ，Bornstein SR ，Schatz U ... -  《-》

[The role of PCSK9-inhibitors and of lipoprotein apheresis in the treatment of homozygous and severe heterozygous familial hypercholesterolemia: A rivalry, or are things quite different?].

Bláha V ，Bláha M ，Lánská M ，Havel E ，Vyroubal P ，Zadák Z ，Žák P ... -  《-》

Real-World Efficacy of Proprotein Convertase Subtilisin/Kexin Type 9 Inhibitors (PCSK9i) in Heterozygous Familial Hypercholesterolemia Patients Referred for Lipoprotein Apheresis.

Matta A ，Bongard V ，Bouisset F ，Taraszkiewicz D ，Rabès JP ，Ferrières J ... -  《MEDICAL SCIENCE MONITOR》