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  1. Article ; Online: Left Ventricular Non-Compaction in Children

    Emanuele Monda / Gianantonio De Michele / Gaetano Diana / Federica Verrillo / Marta Rubino / Annapaola Cirillo / Adelaide Fusco / Federica Amodio / Martina Caiazza / Francesca Dongiglio / Giuseppe Palmiero / Pietro Buono / Maria Giovanna Russo / Giuseppe Limongelli

    Diagnostics, Vol 14, Iss 1, p

    Aetiology and Diagnostic Criteria

    2024  Volume 115

    Abstract: Left ventricular non-compaction (LVNC) is a heterogeneous myocardial disorder characterized by prominent trabeculae protruding into the left ventricular lumen and deep intertrabecular recesses. LVNC can manifest in isolation or alongside other heart ... ...

    Abstract Left ventricular non-compaction (LVNC) is a heterogeneous myocardial disorder characterized by prominent trabeculae protruding into the left ventricular lumen and deep intertrabecular recesses. LVNC can manifest in isolation or alongside other heart muscle diseases. Its occurrence among children is rising due to advancements in imaging techniques. The origins of LVNC are diverse, involving both genetic and acquired forms. The clinical manifestation varies greatly, with some cases presenting no symptoms, while others typically manifesting with heart failure, systemic embolism, and arrhythmias. Diagnosis mainly relies on assessing heart structure using imaging tools like echocardiography and cardiac magnetic resonance. However, the absence of a universally agreed-upon standard and limitations in diagnostic criteria have led to ongoing debates in the scientific community regarding the most reliable methods. Further research is crucial to enhance the diagnosis of LVNC, particularly in early life stages.
    Keywords left ventricular non-compaction ; diagnosis ; etiology ; Medicine (General) ; R5-920
    Subject code 610
    Language English
    Publishing date 2024-01-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  2. Article ; Online: OCT angiography indices and the choroidal vascularity index in wild-type transthyretin (TTR) amyloidosis (ATTRwt)

    Michele Rinaldi / Fausto Tranfa / Flavia Chiosi / Giuseppe Campagna / Maddalena De Bernardo / Marco Gioia / Francesco Natale / Martina Caiazza / Francesca Dongiglio / Federica Verrillo / Giuseppe Palmiero / Giuseppe Limongelli / Ciro Costagliola

    Frontiers in Medicine, Vol

    2024  Volume 10

    Abstract: PurposeRetinal angiopathy represents a well-known ocular manifestation of hereditary transthyretin amyloidosis (ATTRv). Until recently, there have been no reports on retinal changes in ATTRwt. In this retrospective observational clinical study, we aimed ... ...

    Abstract PurposeRetinal angiopathy represents a well-known ocular manifestation of hereditary transthyretin amyloidosis (ATTRv). Until recently, there have been no reports on retinal changes in ATTRwt. In this retrospective observational clinical study, we aimed to determine whether vessel density (VD) indices and the choroidal vascularity index (CVI) could offer insights into retinal and choroidal vascular changes among patients affected by ATTRwt.MethodsEighteen patients with a confirmed diagnosis of ATTRwt underwent structural optical coherence tomography (OCT) and OCT angiography (OCTA). We established a control group consisting of 16 healthy subjects for statistical comparisons. The 3D OCT and OCTA datasets were analyzed to assess retinal and choroidal thickness and VD. For measuring CVI, we obtained measurements for the total choroid area (TCA), luminal area (LA), and stromal area (SA).ResultsThe mean VD exhibited a statistically significant reduction in the superficial capillary plexus (SCP), deep capillary plexus (DCP), and choriocapillaris (CC) among the ATTRwt group in comparison to the control group (p < 0.0001). Notably, ATTRwt patients displayed decreased choroidal thickness (p = 0.08). Additionally, the median CVI was lower in the ATTRwt group than in the control group (p = 0.04).ConclusionThe indices from OCTA and CVI have the potential to serve as non-invasive biomarkers for the quantitative evaluation of retinal and choroidal vascular involvement in patients with ATTRwt.
    Keywords choroidal vascularity index (CVI) ; retinal and choroidal microangiopathy ; retinal vessel density ; wild-type (ATTRwt) transthyretin amyloidosis ; OCT angiography (OCTA) ; Medicine (General) ; R5-920
    Subject code 610
    Language English
    Publishing date 2024-01-01T00:00:00Z
    Publisher Frontiers Media S.A.
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  3. Article ; Online: Pathophysiology, Functional Assessment and Prognostic Implications of Nutritional Disorders in Systemic Amyloidosis

    Francesca Dongiglio / Emanuele Monda / Giuseppe Palmiero / Federica Verrillo / Marta Rubino / Gaetano Diana / Annapaola Cirillo / Adelaide Fusco / Erica Vetrano / Michele Lioncino / Martina Caiazza / Giuseppe Cerciello / Laura Capodicasa / Flavia Chiosi / Vincenzo Simonelli / Maria Luisa De Rimini / Francesco Natale / Alessandro Di Santo / Elisabetta Moscarella /
    Paolo Calabrò / Giuseppe Limongelli

    Journal of Clinical Medicine, Vol 12, Iss 528, p

    2023  Volume 528

    Abstract: Gastrointestinal involvement is a common clinical feature of patients with systemic amyloidosis. This condition is responsible for invalidating gastrointestinal symptoms, a significant macro and micronutrient deficit, and is a marker of disease severity. ...

    Abstract Gastrointestinal involvement is a common clinical feature of patients with systemic amyloidosis. This condition is responsible for invalidating gastrointestinal symptoms, a significant macro and micronutrient deficit, and is a marker of disease severity. Gastrointestinal involvement should be actively sought in patients with systemic amyloidosis, while its diagnosis is challenging in patients with isolated gastrointestinal symptoms. The nutritional status in systemic amyloidosis plays an essential role in the clinical course and is considered a significant prognostic factor. However, the definition of nutritional status is still challenging due to the lack of internationally accepted thresholds for anthropometric and biochemical variables, especially in specific populations such as those with systemic amyloidosis. This review aims to elucidate the fundamental steps for nutritional assessment by using clinical and instrumental tools for better prognostic stratification and patient management regarding quality of life and outcomes.
    Keywords amyloidosis ; gastrointestinal disorders ; prognosis ; nutritional assessment ; Medicine ; R
    Subject code 610
    Language English
    Publishing date 2023-01-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

    Full text online

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    Kategorien

    Inter-library loan at ZB MED

    Your chosen title can be delivered directly to ZB MED Cologne location if you are registered as a user at ZB MED Cologne.

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