The WAA apheresis registry contains data on more than 140,000 apheresis procedures conducted in 12 different countries. The aim is to give an update of indications, type and number of procedures and adverse events (AEs). MATERIAL AND METHODS: The WAA-registry is used for registration of apheresis procedures and is free of charge. The responsible person for a center can apply at the site www.waa-registry.org RESULTS: Data includes reported AEs from 2012 and various procedures and diagnoses during the years 2018-2022; the latter in total from 27 centers registered a total of 9500 patients (41% women) that began therapeutic apheresis (TA) during the period. A total of 58,355 apheresis procedures were performed. The mean age was 50 years (range 0-94). The most common apheresis procedure was stem cell collection for which multiple myeloma was the most frequent diagnosis (51%). Donor cell collection was done in 14% and plasma exchange (PEX) in 28% of patients; In relation to all performed procedures PEX, using a centrifuge (35%) and LDL-apheresis (20%) were the most common. The main indication for PEX was TTP (17%). Peripheral veins were used in 56% as the vascular access. The preferred anticoagulant was ACD. AEs occurred in 2.7% of all procedures and were mostly mild (1%) and moderate 1.5% (needed supportive medication) and, only rarely, severe (0.15%). CONCLUSION: The data showed a wide range of indications and variability in apheresis procedures with low AE frequency.
- MeSH
- dárci tkání MeSH
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- novorozenec MeSH
- plazmaferéza MeSH
- předškolní dítě MeSH
- registrace MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- separace krevních složek * metody MeSH
- výměna plazmy škodlivé účinky MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- kojenec MeSH
- lidé středního věku MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- novorozenec MeSH
- předškolní dítě MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
BACKGROUND: The aim of this study was to compare trends in mortality and incidence, clinicopathological features and survival of patients diagnosed with pancreatic ductal adenocarcinoma under 50 years of age (early-onset pancreatic cancer [EOPC]) with patients diagnosed over 50 years of age (late-onset pancreatic cancer [LOPC]). METHODS: The national oncological registry of the Czech Republic was reviewed to identify all patients with histologically confirmed pancreatic ductal adenocarcinoma diagnosed between the years 1985 and 2015. Incidence, mortality, clinicopathological and survival data were analyzed and compared between patients with EOPC and LOPC. RESULTS: From a total of 18 888 patients included in the study, 1324 patients were under the age of 50 years (7.0%). The average annual percentage changes (AAPC) in incidence of all patients with EOPC was -1.0%. The APPC for male patients with EOPC was -2.0% and for female patients was +0.6%. The AAPC in incidence for LOPC was +1.3%. There were no differences in tumor stage, grade or location between EOPC and LOPC. Young patients were more frequently male (64.4% vs. 52.9%), more frequently underwent treatment and had better overall survival. The median survival interval for EOPC was 5.9 months and for LOPC was 4.5 months (p < .001). CONCLUSIONS: The clinicopathological features of pancreatic ductal adenocarcinoma were similar in patients under and over the age of 50 years. Patients with EOPC survived longer than patients with LOPC. Continued efforts should be made to diagnose early and treat young patients aggressively.
- MeSH
- duktální karcinom pankreatu * epidemiologie terapie MeSH
- incidence MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory slinivky břišní * epidemiologie terapie diagnóza MeSH
- registrace MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- přehledy MeSH
- Geografické názvy
- Česká republika MeSH
- MeSH
- COVID-19 * epidemiologie mortalita MeSH
- hospitalizace statistika a číselné údaje MeSH
- lidé MeSH
- riziko MeSH
- vakcinace statistika a číselné údaje MeSH
- věkové faktory MeSH
- Check Tag
- lidé MeSH
- Geografické názvy
- Česká republika MeSH
Lipoprotein apheresis (LA) is a therapeutic option for patients with severe hypercholesterolemia who have persistently elevated LDL-C levels despite attempts at drug therapy. MicroRNAs (miRNAs), important posttranscriptional gene regulators, are involved in the pathogenesis of atherosclerosis. Our study aimed to monitor the dynamics of twenty preselected circulating miRNAs in patients under long-term apheresis treatment. Plasma samples from 12 FH patients (men = 50%, age = 55.3 ± 12.2 years; mean LA overall treatment time = 13.1 ± 7.8 years) were collected before each apheresis therapy every sixth month over the course of four years of treatment. Eight complete follow-up (FU) samples were measured in each patient. Dynamic changes in the relative quantity of 6 miRNAs (miR-92a, miR-21, miR-126, miR-122, miR-26a, and miR-185; all p < 0.04) during FU were identified. Overall apheresis treatment time influenced circulating miR-146a levels (p < 0.04). In LDLR mutation homozygotes (N = 5), compared to heterozygotes (N = 7), we found higher plasma levels of miR-181, miR-126, miR-155, and miR-92a (all p < 0.03). Treatment with PCSK9 inhibitors (N = 6) affected the plasma levels of 7 miRNAs (miR-126, miR-122, miR-26a, miR-155, miR-125a, miR-92a, and miR-27a; all p < 0.04). Long-term monitoring has shown that LA in patients with severe familial hypercholesterolemia influences plasma circulating miRNAs involved in endothelial dysfunction, cholesterol homeostasis, inflammation, and plaque development. The longer the treatment using LA, the better the miRNA milieu depicting the potential cardiovascular risk.
- MeSH
- cirkulující mikroRNA * genetika MeSH
- dospělí MeSH
- hyperlipoproteinemie typ II * genetika terapie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mikro RNA * genetika MeSH
- proproteinkonvertasa subtilisin/kexin typu 9 genetika MeSH
- senioři MeSH
- separace krevních složek * MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- časopisecké články MeSH
- práce podpořená grantem MeSH
Elevated low-density lipoprotein (LDL) cholesterol levels lead to atherosclerosis and platelet hyperaggregability, both of which are known culprits of arterial thrombosis. Normalization of LDL cholesterol in familial hypercholesterolemia (FH) is not an easy task and frequently requires specific treatment, such as regularly performed lipid apheresis and/or novel drugs such as proprotein convertase subtilisin kexin 9 monoclonal antibodies (PCSK9Ab). Moreover, a high resistance rate to the first-line antiplatelet drug acetylsalicylic acid (ASA) stimulated research of novel antiplatelet drugs. 4-methylcatechol (4-MC), a known metabolite of several dietary flavonoids, may be a suitable candidate. The aim of this study was to analyse the antiplatelet effect of 4-MC in FH patients and to compare its impact on two FH treatment modalities via whole-blood impedance aggregometry. When compared to age-matched, generally healthy controls, the antiplatelet effect of 4-MC against collagen-induced aggregation was higher in FH patients. Apheresis itself improved the effect of 4-MC on platelet aggregation and blood from patients treated with this procedure and pretreated with 4-MC had lower platelet aggregability when compared to those solely treated with PCKS9Ab. Although this study had some inherent limitations, e.g., a low number of patients and possible impact of administered drugs, it confirmed the suitability of 4-MC as a promising antiplatelet agent and also demonstrated the effect of 4-MC in patients with a genetic metabolic disease for the first time.
- MeSH
- hyperlipoproteinemie typ II * farmakoterapie MeSH
- LDL-cholesterol MeSH
- lidé MeSH
- monoklonální protilátky farmakologie terapeutické užití MeSH
- proproteinkonvertasa subtilisin/kexin typu 9 MeSH
- proproteinkonvertasy terapeutické užití MeSH
- separace krevních složek * metody MeSH
- subtilisin MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
Cíle: Hodnocení dlouhodobého vlivu léčby rheoferézou na suchou formu věkem podmíněné makuární degenerace. Materiál a metody: Do hodnocení jsme zařadili 65 pacientů a do kontrolní skupiny 55 pacientů s minimální dobou sledování 60 měsíců. Základní léčba se skládala z 8 procedur rheoferézy, přídatná léčba (booster therapy) ze 2 rheoferéz za 1,5-2 roky po základní léčbě. Hodnotili jsme změny nejlépe korigované zrakové ostrosti, anatomických poměrů a elektrické aktivity sítnice, hematologické, biochemické a imunologické parametry. Výsledky: Léčba rheoferézou významně přispěla: 1) Ke stabilizaci nejlépe korigované zrakové ostrosti léčených pacientů, která se nejprve nevýznamně zvyšovala do 2 let sledování a následně jen mírně klesala. Naproti tomu v kontrolní skupině se zraková ostrost snižovala, do 4 let nevýznamně, poté již statisticky významně. 2) Ke zlepšení morfologického nálezu u 62,4 % léčených pacientů v porovnání se 7,5 % kontrol, naproti tomu k progresi onemocnění do 3. stadia (vlhká forma onemocnění nebo geografická atrofie) s významným poklesem zrakových funkcí došlo jen u 7,1 % léčených pacientů oproti 37,0 % kontrol. 3) K regresi, dokonce i k přiložení drúzového odchlípení retinálního pigmentového epitelu (DPED). Ke zmenšení plochy DPED u 80,4 % léčených pacientů, naproti tomu ke zvětšení plochy DPED u 47,1 % kontrol a rozvoji nového DPED jen u 2 očí léčených pacientů oproti 16 očím kontrol. 4) K udržení integrity vrstvy elipsoidů ve fovee u 68,2 % léčených pacientů, naproti tomu defektní elipsoidní vrstvu ve fovee jsme zaznamenali u 66,6 % kontrol. 5) Ke stabilizaci aktivity gangliových buněk, čípkového systému a aktivity centrální oblasti sítnice s excentricitou mezi 1,8° a 30° u léčených pacientů v porovnání s její alterací v kontrolní skupině projevující se zejména od 3,5 let sledování. 6) K statisticky významnému zlepšení rheologických ukazatelů, a tím ke zvýšení průtoku v mikrocirkulaci a pozitivnímu ovlivnění metabolizmu v sítnici. K pozitivnímu vlivu na klasickou, alternativní i lektinovou cestu aktivace komplementu, snížení hladiny PCSK9 (proprotein konvertáza subticilin kexin 9), a tím i hladiny LDL-cholesterolu a 7) Přídatná léčba 2 procedurami RHF (tzv. „booster therapy“) se zdá být bezpečnou a vhodnou metodou prodloužení fáze stabilizace, či dokonce zlepšení zrakové ostrosti, anatomického a funkčního nálezu. Závěr: Prokázali jsme pozitivní změny anatomických, funkčních i humorálních ukazatelů při léčbě VPMD rheoferézou. Jejich korelace skýtá reálnou možnost ohrožené nemocné vytipovat a řídit individualizovanou intenzitu rheoterapie. Metodika je účinná a bezpečná s nízkým procentem nezávažných vedlejších účinků.
Purpose: Evaluation of the long-term effect of rheopheresis treatment of dry form of age-related macular degeneration (AMD). Materials and Methods: The treatment group consisted of 65 patients and 55 patients in the control group, with a minimum follow-up period of 60 months. The basic treatment consisted of 8 rheopheresis procedures, and the additional treatment (booster therapy) of 2 rheopheresis procedures 1.5–2 years after the basic treatment. We evaluated changes in best corrected visual acuity, anatomical effect, electrical activity of the retina, haematological, biochemical and immunological parameters. Results: Rheopheresis treatment contributed significantly: 1) to stabilisation of best corrected visual acuity of the treated patients, which initially showed an insignificant increased during the 2-years follow-up period, and then slightly decreased. By contrast, visual acuity decreased in the control group, to an insignificant degree up to 4 years, then statistically significantly. 2) to an improvement of the morphological findings in 62.4% of treated patients compared to 7.5% in the control group, while disease progression to stage 3 (neovascular form of the disease or geographic atrophy) with a significant decrease of visual acuity occurred in only 7.1% of treated patients, versus 37.0% in the control group. 3) to regression, even to the attachment of drusenoid pigment epithelial detachment (DPED). To a reduction of the area of DPED in 80.4% of treated patients, in contrast with an steaincrease in the area of DPED in 47.1% of patients in the control group, and the development of new DPED in only 2 eyes of treated patients compared with 16 eyes of patients in the control group. 4) to a preservation of the integrity of the ellipsoid layer in the fovea in 68.2% of the treated patients, while by contrast we found a damaged ellipsoid layer in the fovea in 66.6% of the control patients. 5) to a stabilisation of the activity of ganglion cells, the pineal system and the activity of the central area of the retina, with eccentricity between 1.8° and 30° in the treated patients, compared to alteration in the control group manifested mainly after 3.5 years of the follow-up period. 6) to a statistically significant improvement in rheological parameters, thereby increasing flow in microcirculation and positively influencing the metabolism in the retina. Also to a positive effect on the classical, alternative and lectin pathway of complement activation, a reduction in the level of proprotein convertase subtilisin kexin 9 (PCSK9), and thus also the level of LDLcholesterol, and 7) Additional treatment with 2 RHF procedures (so-called "booster therapy") seems to be a safe and suitable method of prolonging the stabilisation phase, or even improving visual acuity, anatomical and functional findings. Conclusion: We demonstrated positive changes in anatomical, functional and humoral parameters upon rheopheresis treatment of AMD. Their correlation provides a real possibility to identify patients at risk and to manage an individualised regime of rheopheresis therapy. This method of treatment is effective and safe, with a low percentage of non-serious adverse effects.
- MeSH
- geografická atrofie patologie terapie MeSH
- lidé MeSH
- makulární degenerace patologie terapie MeSH
- plazmaferéza * metody škodlivé účinky MeSH
- proproteinkonvertasa subtilisin/kexin typu 9 terapeutické užití MeSH
- retina patologie MeSH
- separace krevních složek metody škodlivé účinky MeSH
- výzkum MeSH
- Check Tag
- lidé MeSH
