LIVIVO - The Search Portal for Life Sciences

zur deutschen Oberfläche wechseln
Advanced search

Search results

Result 1 - 10 of total 116

Search options

  1. Article: Incidence of Antiphospholipid Syndrome: Is Estimation Currently Possible?

    Ioannou, Yiannis / Beukelman, Timothy / Murray, Miranda / Erkan, Doruk

    European journal of rheumatology

    2023  Volume 10, Issue 1, Page(s) 39–44

    Abstract: Antiphospholipid syndrome is a systemic autoimmune disorder characterized by vascular thrombosis and/or obstetric events in association with persistently elevated antiphospholipid antibodies. Antiphospholipid syndrome is typically considered a rare ... ...

    Abstract Antiphospholipid syndrome is a systemic autoimmune disorder characterized by vascular thrombosis and/or obstetric events in association with persistently elevated antiphospholipid antibodies. Antiphospholipid syndrome is typically considered a rare disease, but the true incidence is uncertain owing to the diverse antiphospholipid antibody-related clinical manifestations, inconsistent definitions of antiphospholipid antibody positivity, under-recognition of the disease, and limited population-based studies. Published estimates of the incidence of antiphospholipid syndrome range from approximately 2 to 80 per 100 000 person-years. A targeted literature review and applied methodology were performed to derive a best available estimate. Significant limitations of the published literature were observed, some of which have been previously reported. The incidence of antiphospholipid syndrome in the United States was estimated to be approximately 7.1 to 13.7 per 100 000 person-years in the general population. Although this estimate is likely more accurate than previously reported estimates, large, contemporary, population-based studies that reasonably adhere to the antiphospholipid syndrome classification criteria are needed to further refine estimates of the incidence of antiphospholipid syndrome.
    Language English
    Publishing date 2023-02-08
    Publishing country Turkey
    Document type Journal Article
    ZDB-ID 2873727-1
    ISSN 2148-4279 ; 2147-9720
    ISSN (online) 2148-4279
    ISSN 2147-9720
    DOI 10.5152/eurjrheum.2023.22012
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  2. Article: The Differences Between Childhood and Adult Onset Antiphospholipid Syndrome.

    Wincup, Chris / Ioannou, Yiannis

    Frontiers in pediatrics

    2018  Volume 6, Page(s) 362

    Abstract: Antiphospholipid syndrome (APS) is a rare autoimmune disease of unknown etiology that represents a leading cause of acquired thromboembolism and recurrent miscarriage. It is characterized by the persistent elevated presence of pathogenic antiphospholipid ...

    Abstract Antiphospholipid syndrome (APS) is a rare autoimmune disease of unknown etiology that represents a leading cause of acquired thromboembolism and recurrent miscarriage. It is characterized by the persistent elevated presence of pathogenic antiphospholipid auto-antibodies directed against cardiolipin, ß2-glycoprotein-I, and/or a positive lupus anticoagulant test. As with many autoimmune disorders, the pathogenesis of APS is believed to be the result of a complex interaction between environmental triggers and genetic predisposition. Although more common in adults, APS occasionally manifests in the neonatal period and throughout childhood. Adut-onset APS classification criteria are poorly validated to the pediatric population (in which pregnancy related complications are seldom seen) and as a result, assessment of the prevalence of the disease in childhood is difficult. Thromboembolic events seen in children include deep venous thrombosis in addition to stroke and pulmonary embolism, which can lead to significant long-term disability. The disease can be classified as either primary (when occurring in isolation) or secondary, in which the disease is diagnosed in the context of another underlying disease, most commonly systemic lupus erythematosus. A variety of laboratory and clinical difference are seen between pediatric and adult-onset APS. The marked female predominance seen in adult-onset disease is less evident in childhood where the gender split is more evenly spread. In addition, children with APS are at a higher risk of recurrent thromboembolism than adults. The treatment of childhood-onset APS is challenging due to a lack of large-scale prospective studies in the pediatric population. Therapeutic options are often based upon treatment guidelines that have been based upon literature from the adult-onset form of the disease. In the majority of cases, treatment is focused on the prevention of further thrombosis through treatment with long-term anti-coagulation therapy. The evidence for the use of antiplatelet agents (such as aspirin) and hydroxychloroquine is inconclusive. It is important to remember that anti-coagulation can have significant lifestyle implications for the child with APS and it is essential to consider potential implications relating to school and recreational activities, with contact sports often discouraged due to the increased risk of bleeding.
    Language English
    Publishing date 2018-11-27
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2018.00362
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  3. Article ; Online: The Michael Mason Prize: Pathogenic antiphospholipid antibodies, stressed out antigens and the deployment of decoys.

    Ioannou, Yiannis

    Rheumatology (Oxford, England)

    2011  Volume 51, Issue 1, Page(s) 32–36

    Abstract: The antiphospholipid syndrome is a common autoimmune cause of vascular thrombosis and recurrent miscarriages. aPLs that target the N-terminal domain [Domain I (DI)] of the phospholipid binding protein ß2-glycoprotein I (ß2GPI) represent the key sub- ... ...

    Abstract The antiphospholipid syndrome is a common autoimmune cause of vascular thrombosis and recurrent miscarriages. aPLs that target the N-terminal domain [Domain I (DI)] of the phospholipid binding protein ß2-glycoprotein I (ß2GPI) represent the key sub-population of aPLs that promote thrombosis. This review describes two research arms relating to the study of this autoantigen. The first arm describes recent novel biochemical and functional insights into the molecular structure of ß2GPI in vivo and how this may be altered in APS. These findings support the emerging hypothesis that redox modification of ß2GPI may be relevant to the pathogenesis of APS and the development of pathogenic anti-ß2GPI antibodies. The second arm describes how a recombinant DI peptide engineered using a bacterial expression system was used to delineate the fine immunodominant epitopes on DI that pathogenic anti-ß2GPI antibodies target. The epitope was found to be conformational and revolve around arginine (R) 39 within DI. Thus, whole recombinant DI was used in an in vivo mouse model as a novel decoy peptide inhibitor of anti-ß2GPI antibodies. DI and a high binding mutant completely abrogated the pathogenic effects of aPL in this murine model, with loss of inhibition of pathogenicity observed upon mutating the residue R39 to serine. This proof-of-principle study supports the ongoing development of recombinant DI as a novel therapeutic inhibitory peptide for patients with APS.
    MeSH term(s) Animals ; Antibodies, Antiphospholipid/immunology ; Antiphospholipid Syndrome/immunology ; Awards and Prizes ; History, 21st Century ; Humans ; Immunodominant Epitopes/immunology ; Mice ; Oxidation-Reduction ; Recombinant Proteins/immunology ; Rheumatology/history ; beta 2-Glycoprotein I/immunology ; beta 2-Glycoprotein I/metabolism
    Chemical Substances Antibodies, Antiphospholipid ; Immunodominant Epitopes ; Recombinant Proteins ; beta 2-Glycoprotein I
    Language English
    Publishing date 2011-11-24
    Publishing country England
    Document type Historical Article ; Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 1464822-2
    ISSN 1462-0332 ; 1462-0324
    ISSN (online) 1462-0332
    ISSN 1462-0324
    DOI 10.1093/rheumatology/ker353
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  4. Article: Niemann-Pick C proteins in sterol transport and absorption: flies in the ointment.

    Ioannou, Yiannis A

    Developmental cell

    2007  Volume 12, Issue 4, Page(s) 481–483

    Abstract: The Niemann-Pick C proteins have slowly emerged as regulators of subcellular lipid transport and sterol absorption at the small intestine. A recent article in Cell Metabolism suggests that in addition to their significant structural and sequence homology, ...

    Abstract The Niemann-Pick C proteins have slowly emerged as regulators of subcellular lipid transport and sterol absorption at the small intestine. A recent article in Cell Metabolism suggests that in addition to their significant structural and sequence homology, these proteins may orchestrate their functions in a previously unappreciated fashion (Voght et al., 2007).
    MeSH term(s) Absorption ; Animals ; Biological Transport ; Cholesterol/pharmacokinetics ; Drosophila melanogaster/metabolism ; Mice ; Models, Biological ; Niemann-Pick Diseases/metabolism ; Sterols/pharmacokinetics
    Chemical Substances Sterols ; Cholesterol (97C5T2UQ7J)
    Language English
    Publishing date 2007-04
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2054967-2
    ISSN 1878-1551 ; 1534-5807
    ISSN (online) 1878-1551
    ISSN 1534-5807
    DOI 10.1016/j.devcel.2007.03.017
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  5. Article: Comparison of juvenile and adult onset systemic lupus erythematosus.

    Baqai, Tanya / Ioannou, Yiannis

    British journal of hospital medicine (London, England : 2005)

    2012  Volume 73, Issue 10, Page(s) 558–563

    MeSH term(s) Adolescent ; Adult ; Age of Onset ; Antibodies, Antinuclear/analysis ; Genetic Predisposition to Disease ; Humans ; Lupus Erythematosus, Systemic/classification ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/epidemiology ; Lupus Erythematosus, Systemic/genetics ; Lupus Erythematosus, Systemic/therapy ; Prognosis ; Treatment Outcome ; Young Adult
    Chemical Substances Antibodies, Antinuclear
    Language English
    Publishing date 2012-10-30
    Publishing country England
    Document type Comparative Study ; Journal Article
    ISSN 1750-8460
    ISSN 1750-8460
    DOI 10.12968/hmed.2012.73.10.558
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  6. Article: Guilty until proven innocent: the case of NPC1 and cholesterol.

    Ioannou, Yiannis A

    Trends in biochemical sciences

    2005  Volume 30, Issue 9, Page(s) 498–505

    Abstract: Cholesterol accumulation in the endosomes and lysosomes of Niemann-Pick C (NPC) cells is considered to be the hallmark of this disorder, so the main focus of NPC research has revolved around cholesterol and its role in disease pathogenesis. However, ... ...

    Abstract Cholesterol accumulation in the endosomes and lysosomes of Niemann-Pick C (NPC) cells is considered to be the hallmark of this disorder, so the main focus of NPC research has revolved around cholesterol and its role in disease pathogenesis. However, recent data indicate that cholesterol is not the primary culprit in this human lipidosis. I propose a new hypothesis for the potential action or function of the NPC1 protein in the endosome. In this context, the relationship of NPC2 and NPC1 is also discussed.
    MeSH term(s) Carrier Proteins/physiology ; Cholesterol/metabolism ; Cholesterol/pharmacokinetics ; Humans ; Membrane Glycoproteins/physiology ; Models, Chemical ; Niemann-Pick Diseases/genetics ; Niemann-Pick Diseases/physiopathology
    Chemical Substances Carrier Proteins ; Membrane Glycoproteins ; NPC1 protein, human ; Cholesterol (97C5T2UQ7J)
    Language English
    Publishing date 2005-09
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S.
    ZDB-ID 194216-5
    ISSN 1362-4326 ; 0968-0004 ; 0376-5067
    ISSN (online) 1362-4326
    ISSN 0968-0004 ; 0376-5067
    DOI 10.1016/j.tibs.2005.07.007
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  7. Article ; Online: Ofatumumab: a novel treatment for severe systemic lupus erythematosus.

    Thornton, C Clare / Ambrose, Nicola / Ioannou, Yiannis

    Rheumatology (Oxford, England)

    2015  Volume 54, Issue 3, Page(s) 559–560

    MeSH term(s) Adult ; Antibodies, Monoclonal/immunology ; Antibodies, Monoclonal/therapeutic use ; Antigens, CD20/immunology ; Female ; Humans ; Lupus Erythematosus, Systemic/drug therapy ; Severity of Illness Index ; Treatment Outcome
    Chemical Substances Antibodies, Monoclonal ; Antigens, CD20 ; ofatumumab (M95KG522R0)
    Language English
    Publishing date 2015-03
    Publishing country England
    Document type Case Reports ; Letter
    ZDB-ID 1464822-2
    ISSN 1462-0332 ; 1462-0324
    ISSN (online) 1462-0332
    ISSN 1462-0324
    DOI 10.1093/rheumatology/keu475
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  8. Article ; Online: New therapeutic targets for the antiphospholipid syndrome.

    Pericleous, Charis / Ioannou, Yiannis

    Expert opinion on therapeutic targets

    2010  Volume 14, Issue 12, Page(s) 1291–1299

    Abstract: Importance of the field: The antiphospholipid syndrome (APS) is an autoimmune condition whereby pathogenic antiphospholipid antibodies (aPL) cause vascular thrombosis and/or recurrent miscarriage, and carries a high burden of morbidity and mortality. ... ...

    Abstract Importance of the field: The antiphospholipid syndrome (APS) is an autoimmune condition whereby pathogenic antiphospholipid antibodies (aPL) cause vascular thrombosis and/or recurrent miscarriage, and carries a high burden of morbidity and mortality. Currently the only proven treatment is long-term anticoagulation, which is not effective in all patients and carries risk of haemorrhage.
    Areas covered in this review: Novel therapeutic targets that are currently being explored for APS in order to address the unmet needs of better, safer and ideally targeted therapy. These include B cell depletion, new-generation anticoagulants, interfering with aPL cell-mediated activation of endothelial cells and platelets both at the cell surface level and intracellularly, targeting components of the complement system and the novel concept of using decoy peptides to target only the pathogenic sub-population of aPL.
    What the reader will gain: An overview of the potential targets and rationale underpinning them.
    Take home message: Though current options remain limited for the treatment of APS, the future holds much promise with the identification of multiple targets, many of which are currently being explored. The challenge will be to undertake carefully designed prospective multi-centre trials to generate the evidence necessary to support integration of such candidates into clinical practice.
    MeSH term(s) Abortion, Habitual/prevention & control ; Antibodies, Antiphospholipid/immunology ; Anticoagulants/therapeutic use ; Antiphospholipid Syndrome/drug therapy ; Antiphospholipid Syndrome/immunology ; B-Lymphocytes/drug effects ; B-Lymphocytes/metabolism ; Blood Platelets ; Endothelial Cells/metabolism ; Endothelial Cells/pathology ; Female ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use ; Molecular Targeted Therapy ; Pregnancy ; Pregnancy Complications/drug therapy ; Pregnancy Complications/immunology ; Thrombosis/drug therapy ; Thrombosis/etiology ; Thrombosis/immunology
    Chemical Substances Antibodies, Antiphospholipid ; Anticoagulants ; Hydroxymethylglutaryl-CoA Reductase Inhibitors
    Language English
    Publishing date 2010-09-28
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 2055208-7
    ISSN 1744-7631 ; 1472-8222
    ISSN (online) 1744-7631
    ISSN 1472-8222
    DOI 10.1517/14728222.2010.524207
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  9. Article ; Online: Activation of mitochondrial TRAP1 stimulates mitochondria-lysosome crosstalk and correction of lysosomal dysfunction.

    Chen, Fannie W / Davies, Joanna P / Calvo, Raul / Chaudhari, Jagruti / Dolios, Georgia / Taylor, Mercedes K / Patnaik, Samarjit / Dehdashti, Jean / Mull, Rebecca / Dranchack, Patricia / Wang, Amy / Xu, Xin / Hughes, Emma / Southall, Noel / Ferrer, Marc / Wang, Rong / Marugan, Juan J / Ioannou, Yiannis A

    iScience

    2022  Volume 25, Issue 9, Page(s) 104941

    Abstract: Numerous studies have established the involvement of lysosomal and mitochondrial dysfunction in the pathogenesis of neurodegenerative disorders such as Alzheimer's and Parkinson diseases. Building on our previous studies of the neurodegenerative ... ...

    Abstract Numerous studies have established the involvement of lysosomal and mitochondrial dysfunction in the pathogenesis of neurodegenerative disorders such as Alzheimer's and Parkinson diseases. Building on our previous studies of the neurodegenerative lysosomal lipidosis Niemann-Pick C1 (NPC1), we have unexpectedly discovered that activation of the mitochondrial chaperone tumor necrosis factor receptor-associated protein 1 (TRAP1) leads to the correction of the lysosomal storage phenotype in patient cells from multiple lysosomal storage disorders including NPC1. Using small compound activators specific for TRAP1, we find that activation of this chaperone leads to a generalized restoration of lysosomal and mitochondrial health. Mechanistically, we show that this process includes inhibition of oxidative phosphorylation and reduction of oxidative stress, which results in activation of AMPK and ultimately stimulates lysosome recycling. Thus, TRAP1 participates in lysosomal-mitochondrial crosstalk to maintain cellular homeostasis and could represent a potential therapeutic target for multiple disorders.
    Language English
    Publishing date 2022-08-14
    Publishing country United States
    Document type Journal Article
    ISSN 2589-0042
    ISSN (online) 2589-0042
    DOI 10.1016/j.isci.2022.104941
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

  10. Article ; Online: An innovative interactive medical student induction programme in paediatrics.

    Williams, Nia / Ioannou, Yiannis / Stewart, Charles / Gulati, Pooja / Singh, Suveer

    Medical teacher

    2015  Volume 37, Issue 2, Page(s) 200–201

    MeSH term(s) Clinical Competence ; Education, Medical/methods ; Educational Measurement/methods ; Humans ; Pediatrics/education ; Physical Examination/methods
    Language English
    Publishing date 2015-02
    Publishing country England
    Document type Comment ; Letter
    ZDB-ID 424426-6
    ISSN 1466-187X ; 0142-159X
    ISSN (online) 1466-187X
    ISSN 0142-159X
    DOI 10.3109/0142159X.2014.940872
    Database MEDical Literature Analysis and Retrieval System OnLINE

    More links

    Kategorien

To top