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  1. Article ; Online: Therapeutic Plasmapheresis. A Gate to an Effective Treatment of Severe Pathological Conditions

    Claudia Stefanutti

    Advances in Bioscience and Clinical Medicine, Vol 2, Iss 2, Pp 1-

    2014  Volume 3

    Abstract: There are two ways to perform therapeutic plasmapheresis (TP). The simplest form is therapeutic plasma exchange (TPE) where the blood taken from the patient is separated in a centrifuge into cells and plasma. While the plasma is discarded, the cells ... ...

    Abstract There are two ways to perform therapeutic plasmapheresis (TP). The simplest form is therapeutic plasma exchange (TPE) where the blood taken from the patient is separated in a centrifuge into cells and plasma. While the plasma is discarded, the cells together with a substitution fluid (e.g. fresh frozen plasma; albumin solution) are returned to the patient. The second form of treatment specifically removes the pathogenic substance alone by filtration on a specific substrate. A column is attached to the plasma line, selectively eliminating the pathogenic molecule and returning the patient’s own plasma. Nowadays, practitioners of centrifugal TP tend to be hematologists and transfusionists, whilst the membrane-based approach appeals primarily to nephrologists or other specialists (experts in hemodialysis, surgeons). Adsorption, an apheresis technique conceivably with the highest selectivity, is founded on the principles of affinity chromatography in which plasma is passed through an insoluble matrix bound firmly to a ligand. The ligands could be either comparatively nonspecific chemical binders, e.g. heparin or charcoal, or specific recombinant protein antigens or monoclonal antibodies. A wide range of medical disorders of varying pathologies and end-organ involvement may be managed by TPE (1). TPE has still a crucial role in emergency. Critical care units staffed by highly trained medical and paramedical personnel using highly technical and sophisticated equipment might be supported by the provision of TPE. Timing is frequently of the essence in disorders in which plasma exchange (PEX) has a role, with decisions being based on a set of facts or clinical circumstances which may be rapidly changing (2) (Table 1). Severe Hypertriglyceridemia (sHTG) could be associated with acute pancreatitis (AP), a fatal and dreaded complication. Technique Pathology Emergency Long-term Plasma-Exchange Sepsis, MODS X Plasma-Exchange Hyperlipidemic Pancreatitis Hyperchylomicronemia X Plasma-Exchange Familial Hypercholesterolemia X Lipoprotein Apheresis (Dextran sulphate) Familial Hypercholesterolemia Hyper Lp(a) lipoproteinemia X Lipoprotein Apheresis (Dextran sulphate) Preeclampsia X Lipoprotein Apheresis (H.E.L.P.) Familial Hypercholesterolemia HyperLp(a)lipoproteinemia X Lipoprotein Apheresis (H.E.L.P.) Sepsis X Table 1. Different clinical use of conventional and selective apheresis techniques in relation to the pathology to be treated in emergency or on long (chronic)-term. Throughout the acute phase of hyperlipidemic pancreatitis (HLP), PEX (Lipid-apheresis) could be of considerable assistance not only in lowering TG levels but also in the prevention of recurrent HLP (3). Furthermore, patients with homo-, double- compound-, and heterozygous Familial Hypercholesterolemia and HyperLp(a) lipoproteinemia would benefit lipoprotein-apheresis (LA) as an extracorporeal procedure providing selective removal of lipids and lipoproteins including Low Density Lipoproteins (LDL) and other apolipoprotein B100-containing lipoproteins (4, 5). Having numerous metabolic and clinical superiorities, LA characterizes an upgraded selective form of conventional extracorporeal therapies, e.g. plasma-exchange (PEX- Lipid-apheresis), which were broadly-used for managing severe hypercholesterolemia in the seventies. However, LA is primarily used in the treatment of previously-mentioned severe forms of dyslipidemia. FH patients are particularly prone to coronary ischemic events necessitating a tailored, intensive, efficient, continuous, and unceasing form of treatment. Obviously, a therapeutic approach exclusively relying on existing accessible medications would not lead to preferred clinical outcomes. The above reported clinical examples clearly suggest what differences exist between the non-selective and selective apheresis techniques as far as different pathologies exhibiting affinity are concerned. In particular, the clinical presentation, emergency or not, greatly affects the use of a given extracorporeal technique. However, depending upon the indication, a selective technique not usually utilized in emergency, such as selective dextran sulfate cellulose LA was recently suggested to treat acutely preeclampsia, where targeted therapies to stabilize the clinical manifestations and prolong pregnancy do not exist (6). A further complication of the apheresis issue is represented by the indication of sepsis with multi organ dysfunction syndrome (MODS) as the most common cause of death in patients in non-coronary intensive care units. Presently, an effective treatment to reduce mortality in sepsis and MODS patients is still not available. Therapeutic PEX in the management of sepsis and MODS was suggested several years ago and more recently (7, 8). On the other side, even a selective LA technique such as Heparin induced Extracorporeal Lipoprotein Precipitation (H.E.L.P.) was used in the treatment of sepsis and it is still on use (9). In conclusion, extracorporeal therapeutic techniques can be clinically helpful in emergency, in deferred urgency and in chronic, long-term use. As these techniques are invasive and relatively complex, appropriate equipment and highly skillful medical and non-medical staff personnel is a necessary complement. Key words: Therapeutic Plasmapheresis, Plasma Exchange, Lipoprotein Apheresis, Hyperlipidemic Pancreatitis, Acute Pancreatitis, Homozygous- Heterozygous Familial Hypercholesterolemia, Hyper Lp(a) lipoproteinemia
    Keywords Medicine ; R ; Biology (General) ; QH301-705.5
    Subject code 610
    Language English
    Publishing date 2014-07-01T00:00:00Z
    Publisher Australian International Academic Centre PTY.LTD.
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  2. Article ; Online: Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous Familial Hypercholesterolaemia. A Position Paper of the Associations of Preventive Pediatrics of Serbia, Mighty Medic and International Lipid Expert Panel

    Bojko Bjelakovic / Claudia Stefanutti / Željko Reiner / Gerald F. Watts / Patrick Moriarty / David Marais / Kurt Widhalm / Hofit Cohen / Mariko Harada-Shiba / Maciej Banach

    Journal of Clinical Medicine, Vol 10, Iss 4930, p

    2021  Volume 4930

    Abstract: Heterozygous familial hypercholesterolaemia (FH) is among the most common genetic metabolic lipid disorders characterised by elevated low-density lipoprotein cholesterol (LDL-C) levels from birth and a significantly higher risk of developing premature ... ...

    Abstract Heterozygous familial hypercholesterolaemia (FH) is among the most common genetic metabolic lipid disorders characterised by elevated low-density lipoprotein cholesterol (LDL-C) levels from birth and a significantly higher risk of developing premature atherosclerotic cardiovascular disease. The majority of the current pediatric guidelines for clinical management of children and adolescents with FH does not consider the impact of genetic variations as well as characteristics of vascular phenotype as assessed by recently developed non-invasive imaging techniques. We propose a combined integrated approach of cardiovascular (CV) risk assessment and clinical management of children with FH incorporating current risk assessment profile (LDL-C levels, traditional CV risk factors and familial history) with genetic and non-invasive vascular phenotyping. Based on the existing data on vascular phenotype status, this panel recommends that all children with FH and cIMT ≥0.5 mm should receive lipid lowering therapy irrespective of the presence of CV risk factors, family history and/or LDL-C levels Those children with FH and cIMT ≥0.4 mm should be carefully monitored to initiate lipid lowering management in the most suitable time. Likewise, all genetically confirmed children with FH and LDL-C levels ≥4.1 mmol/L (160 mg/dL), should be treated with lifestyle changes and LLT irrespective of the cIMT, presence of additional RF or family history of CHD.
    Keywords familial hypercholesterolaemia ; children ; cardiovascular risk ; vascular phenotype ; Medicine ; R
    Subject code 610
    Language English
    Publishing date 2021-10-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  3. Article: Effects of selective H.E.L.P. LDL-apheresis on plasma inflammatory markers concentration in severe dyslipidemia: Implication for anti-inflammatory response

    Maria Grazia, Zenti / Claudia, Stefanutti

    Cytokine. 2011 Dec., v. 56, no. 3

    2011  

    Abstract: Therapeutic plasmapheresis is a recognized medical procedure in which various techniques are used to separate and remove undesirable or excessively elevated plasma elements from blood. The main purpose of the procedure is to remove the substances ... ...

    Abstract Therapeutic plasmapheresis is a recognized medical procedure in which various techniques are used to separate and remove undesirable or excessively elevated plasma elements from blood. The main purpose of the procedure is to remove the substances responsible for the disease (autoantibodies, circulating immune complexes, lipoproteins and other molecules) from the patient’s blood. Low-Density-Lipoproteins-apheresis (LDL_a) is the selective removal of all apolipoprotein-B100-containing lipoproteins: LDL, very low-density lipoprotein, and lipoprotein (a). They are lowered acutely by 65–75%. There is little effect on other plasma lipidic and non-lipidic components. LDL_a was reported to increase resistance of LDL to oxidation, counteract procoagulatory state and relief disturbances of hemorheology associated with atherosclerosis. These effects are likely to be regarded as to be pleiotropic effects. In the sense that they are not necessarily related to the apolipoprotein-B100-containing lipoproteins level in plasma. There is robust evidence that LDL_a can induce the stabilization of atherosclerotic plaques through its lipid-lowering action. However, other effects unrelated to the apolipoprotein-B100-containing lipoproteins extracorporeal removal, such as the decrease of cytokines and adhesion molecules induced by LDL_a were also reported. Altogether these actions are thought to favorably influence regression of florid, nonfibrous atherosclerotic lesions through a blockade of lipid deposition in the vessel wall, plaque stabilization, and ultimately, coronary and extracoronary artery disease progression. This brief review provides some indication on existing evidence of Heparin-induced Extracorporeal Low-density-lipoprotein Precipitation LDL_a effects on plasma mediators of inflammation.
    Keywords adhesion ; antigen-antibody complex ; atherosclerosis ; autoantibodies ; blood ; cytokines ; disease course ; hyperlipidemia ; inflammation ; low density lipoprotein ; oxidation ; patients ; plasmapheresis
    Language English
    Dates of publication 2011-12
    Size p. 850-854.
    Publishing place Elsevier Ltd
    Document type Article
    ZDB-ID 1018055-2
    ISSN 1096-0023 ; 1043-4666
    ISSN (online) 1096-0023
    ISSN 1043-4666
    DOI 10.1016/j.cyto.2011.08.038
    Database NAL-Catalogue (AGRICOLA)

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  4. Article ; Online: Effects of selective H.E.L.P. LDL-apheresis on plasma inflammatory markers concentration in severe dyslipidemia: Implication for anti-inflammatory response.

    Zenti, Maria Grazia / Grazia, Zenti Maria / Stefanutti, Claudia / Claudia, Stefanutti

    Cytokine

    2011  Volume 56, Issue 3, Page(s) 850–854

    Abstract: Therapeutic plasmapheresis is a recognized medical procedure in which various techniques are used to separate and remove undesirable or excessively elevated plasma elements from blood. The main purpose of the procedure is to remove the substances ... ...

    Abstract Therapeutic plasmapheresis is a recognized medical procedure in which various techniques are used to separate and remove undesirable or excessively elevated plasma elements from blood. The main purpose of the procedure is to remove the substances responsible for the disease (autoantibodies, circulating immune complexes, lipoproteins and other molecules) from the patient's blood. Low-Density-Lipoproteins-apheresis (LDL_a) is the selective removal of all apolipoprotein-B100-containing lipoproteins: LDL, very low-density lipoprotein, and lipoprotein (a). They are lowered acutely by 65-75%. There is little effect on other plasma lipidic and non-lipidic components. LDL_a was reported to increase resistance of LDL to oxidation, counteract procoagulatory state and relief disturbances of hemorheology associated with atherosclerosis. These effects are likely to be regarded as to be pleiotropic effects. In the sense that they are not necessarily related to the apolipoprotein-B100-containing lipoproteins level in plasma. There is robust evidence that LDL_a can induce the stabilization of atherosclerotic plaques through its lipid-lowering action. However, other effects unrelated to the apolipoprotein-B100-containing lipoproteins extracorporeal removal, such as the decrease of cytokines and adhesion molecules induced by LDL_a were also reported. Altogether these actions are thought to favorably influence regression of florid, nonfibrous atherosclerotic lesions through a blockade of lipid deposition in the vessel wall, plaque stabilization, and ultimately, coronary and extracoronary artery disease progression. This brief review provides some indication on existing evidence of Heparin-induced Extracorporeal Low-density-lipoprotein Precipitation LDL_a effects on plasma mediators of inflammation.
    MeSH term(s) Biomarkers/blood ; Blood Component Removal ; Chemical Precipitation ; Dyslipidemias/blood ; Humans ; Inflammation/blood ; Lipoproteins, LDL/isolation & purification
    Chemical Substances Biomarkers ; Lipoproteins, LDL
    Language English
    Publishing date 2011-12
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 1018055-2
    ISSN 1096-0023 ; 1043-4666
    ISSN (online) 1096-0023
    ISSN 1043-4666
    DOI 10.1016/j.cyto.2011.08.038
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Characterisation of patients with familial chylomicronaemia syndrome (FCS) and multifactorial chylomicronaemia syndrome (MCS)

    Philippe Moulin / Robert Dufour / Maurizio Averna / Marcello Arca / Angelo B. Cefalù / Davide Noto / Laura D’Erasmo / Alessia Di Costanzo / Christophe Marçais / Luis Antonio Alvarez-Sala Walther / Maciej Banach / Jan Borén / Robert Cramb / Ioanna Gouni-Berthold / Elizabeth Hughes / Colin Johnson / Xavier Pintó / Željko Reiner / Jeanine Roeters van Lennep /
    Handrean Soran / Claudia Stefanutti / Erik Stroes / Eric Bruckert

    Data in Brief, Vol 21, Iss , Pp 1334-

    Establishment of an FCS clinical diagnostic score

    2018  Volume 1336

    Abstract: Data presented in this article are supplementary material to our article entitled “Identification and diagnosis of patients with familial chylomicronaemia syndrome (FCS): expert panel recommendations and proposal of an “FCS Score” (Moulin et al., 2018, ... ...

    Abstract Data presented in this article are supplementary material to our article entitled “Identification and diagnosis of patients with familial chylomicronaemia syndrome (FCS): expert panel recommendations and proposal of an “FCS Score” (Moulin et al., 2018, in press). The data describe the genotypes of patients with familial chylomicronaemia syndrome (FCS) and multifactorial chylomicronaemia syndrome (MCS), from the validation and replication cohorts.
    Keywords Computer applications to medicine. Medical informatics ; R858-859.7 ; Science (General) ; Q1-390
    Language English
    Publishing date 2018-12-01T00:00:00Z
    Publisher Elsevier
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  6. Article: Effects of selective H.E.L.P. LDL-apheresis on plasma inflammatory markers concentration in severe dyslipidemia: Implication for anti-inflammatory response

    Maria Grazia, Zenti / Claudia, Stefanutti

    Cytokine

    Volume v. 56,, Issue no. 3

    Abstract: Therapeutic plasmapheresis is a recognized medical procedure in which various techniques are used to separate and remove undesirable or excessively elevated plasma elements from blood. The main purpose of the procedure is to remove the substances ... ...

    Abstract Therapeutic plasmapheresis is a recognized medical procedure in which various techniques are used to separate and remove undesirable or excessively elevated plasma elements from blood. The main purpose of the procedure is to remove the substances responsible for the disease (autoantibodies, circulating immune complexes, lipoproteins and other molecules) from the patient’s blood. Low-Density-Lipoproteins-apheresis (LDL_a) is the selective removal of all apolipoprotein-B100-containing lipoproteins: LDL, very low-density lipoprotein, and lipoprotein (a). They are lowered acutely by 65–75%. There is little effect on other plasma lipidic and non-lipidic components. LDL_a was reported to increase resistance of LDL to oxidation, counteract procoagulatory state and relief disturbances of hemorheology associated with atherosclerosis. These effects are likely to be regarded as to be pleiotropic effects. In the sense that they are not necessarily related to the apolipoprotein-B100-containing lipoproteins level in plasma. There is robust evidence that LDL_a can induce the stabilization of atherosclerotic plaques through its lipid-lowering action. However, other effects unrelated to the apolipoprotein-B100-containing lipoproteins extracorporeal removal, such as the decrease of cytokines and adhesion molecules induced by LDL_a were also reported. Altogether these actions are thought to favorably influence regression of florid, nonfibrous atherosclerotic lesions through a blockade of lipid deposition in the vessel wall, plaque stabilization, and ultimately, coronary and extracoronary artery disease progression. This brief review provides some indication on existing evidence of Heparin-induced Extracorporeal Low-density-lipoprotein Precipitation LDL_a effects on plasma mediators of inflammation.
    Keywords cytokines ; antigen-antibody complex ; inflammation ; plasmapheresis ; oxidation ; hyperlipidemia ; atherosclerosis ; blood ; low density lipoprotein ; patients ; autoantibodies ; adhesion ; disease course
    Language English
    Document type Article
    ISSN 1043-4666
    Database AGRIS - International Information System for the Agricultural Sciences and Technology

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