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  1. Article: A Straining Heart: Transthyretin Amyloidosis as a Cause of Heart Failure.

    Tender-Vieira, Joana / Pinto, Claudemira / Matias, Paula / Marques, Pedro / Almeida, Jorge S

    Cureus

    2023  Volume 15, Issue 12, Page(s) e50957

    Abstract: Cardiac amyloidosis is a disease caused by the deposition of amyloid fibrils in the extracellular space of the heart, most often by immunoglobulin light chains or by transthyretin. It is often underdiagnosed because the signs and symptoms are nonspecific ...

    Abstract Cardiac amyloidosis is a disease caused by the deposition of amyloid fibrils in the extracellular space of the heart, most often by immunoglobulin light chains or by transthyretin. It is often underdiagnosed because the signs and symptoms are nonspecific or due to the false perception that the diagnosis always requires an endomyocardial biopsy. Transthyretin amyloidosis is being increasingly recognized as a cause of heart failure, particularly in patients with heart failure with preserved ejection fraction (HFpEF). We present the clinical case of an 86-year-old man whose diagnosis was based on signs and symptoms compatible with cardiac amyloidosis and in which imaging performed a preponderant role. This case reminds clinicians to consider the diagnosis in older patients with HFpEF, left ventricular hypertrophy and rhythm disturbances. It highlights the importance of evaluating global longitudinal strain (GLS) in a standard echocardiographic evaluation.
    Language English
    Publishing date 2023-12-22
    Publishing country United States
    Document type Case Reports
    ZDB-ID 2747273-5
    ISSN 2168-8184
    ISSN 2168-8184
    DOI 10.7759/cureus.50957
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: New insights in cryoglobulinemic vasculitis.

    Silva, Filipa / Pinto, Claudemira / Barbosa, Arsénio / Borges, Tiago / Dias, Carlos / Almeida, Jorge

    Journal of autoimmunity

    2019  Volume 105, Page(s) 102313

    Abstract: Cryoglobulins are antibodies that precipitate at low temperatures and dissolve after rewarming. Cryoglobulinemia refers to the presence of circulating cryoglobulins and generally leads to a systemic inflammatory syndrome characterized by fatigue, ... ...

    Abstract Cryoglobulins are antibodies that precipitate at low temperatures and dissolve after rewarming. Cryoglobulinemia refers to the presence of circulating cryoglobulins and generally leads to a systemic inflammatory syndrome characterized by fatigue, arthralgia, purpura, ulcers, neuropathy and/or glomerulonephritis. The disease mainly involves small to medium-sized blood vessels and causes vasculitis due to cryoglobulin-containing immune complexes. Cryoglobulinemia is classified into three types (I, II and III) on the basis of immunoglobulin composition. Predisposing conditions include lymphoproliferative, autoimmune diseases and hepatitis C virus infection. The diagnosis of cryoglobulinemic syndrome is predominantly based on the presence of clinical features and laboratorial demonstration of serum cryoglobulins. The treatment strategy depends on the cause of cryoglobulinemia. For patients with chronic HCV infection, antiviral therapy is indicated. Immunosuppressive or immunomodulatory therapy, including steroids, plasmapheresis and cytotoxic agents, is reserved for organ-threatening manifestations. In this review, we discuss the main clinical presentations, diagnostic approach and treatment options.
    MeSH term(s) Antiviral Agents/therapeutic use ; Cryoglobulinemia/diagnosis ; Cryoglobulinemia/drug therapy ; Hepacivirus/drug effects ; Hepatitis C, Chronic/diagnosis ; Hepatitis C, Chronic/drug therapy ; Humans ; Immunosuppressive Agents/therapeutic use ; Vasculitis/diagnosis ; Vasculitis/drug therapy
    Chemical Substances Antiviral Agents ; Immunosuppressive Agents
    Language English
    Publishing date 2019-08-02
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 639452-8
    ISSN 1095-9157 ; 0896-8411
    ISSN (online) 1095-9157
    ISSN 0896-8411
    DOI 10.1016/j.jaut.2019.102313
    Database MEDical Literature Analysis and Retrieval System OnLINE

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