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  1. Article ; Online: Empowering young voices: navigating the complexities of minors in healthcare decisions.

    de Winter, J Peter / Toelen, Jaan / Milani, Gregorio Paolo

    European journal of pediatrics

    2024  

    Language English
    Publishing date 2024-03-19
    Publishing country Germany
    Document type Editorial
    ZDB-ID 194196-3
    ISSN 1432-1076 ; 0340-6199 ; 0943-9676
    ISSN (online) 1432-1076
    ISSN 0340-6199 ; 0943-9676
    DOI 10.1007/s00431-024-05524-4
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  2. Article ; Online: Individualized Approach to Management of Light Chain Amyloidosis.

    Palladini, Giovanni / Milani, Paolo

    Journal of the National Comprehensive Cancer Network : JNCCN

    2023  Volume 21, Issue 1, Page(s) 91–98

    Abstract: Systemic light chain (AL) amyloidosis is caused by a B-cell (most commonly plasma cell) clone that produces a toxic light chain that forms amyloid fibrils in tissues and causes severe, progressive organ dysfunction. The clinical presentation is protean, ... ...

    Abstract Systemic light chain (AL) amyloidosis is caused by a B-cell (most commonly plasma cell) clone that produces a toxic light chain that forms amyloid fibrils in tissues and causes severe, progressive organ dysfunction. The clinical presentation is protean, and patients are usually extremely frail, thus requiring careful adaptation of the treatment approach. However, the severity of organ involvement can be accurately assessed with biomarkers that allow a sharp prognostic stratification and precise tailoring of the treatment strategy. Moreover, the availability of biomarker-based response criteria also allows adjustment of the treatment approach over time. The recent completion of 3 large randomized clinical trials has offered new evidence for designing appropriate treatments. All this information has recently been integrated in the joint guidelines of the International Society of Amyloidosis and the European Hematology Association for the treatment of AL amyloidosis. Other clinical trials are underway testing new agents directed against the amyloid clone and the amyloid deposits. Our understanding of the peculiarities of the amyloid clone, as well as our ability to detect residual clonal disease and improve organ dysfunction, are also being refined and will result in more precise personalization of the treatment approach.
    MeSH term(s) Humans ; Multiple Organ Failure ; Amyloidosis/diagnosis ; Amyloidosis/etiology ; Amyloidosis/therapy ; Immunoglobulin Light-chain Amyloidosis/diagnosis ; Immunoglobulin Light-chain Amyloidosis/etiology ; Immunoglobulin Light-chain Amyloidosis/therapy ; Amyloid/therapeutic use ; Prognosis ; Biomarkers
    Chemical Substances Amyloid ; Biomarkers
    Language English
    Publishing date 2023-01-12
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2250759-0
    ISSN 1540-1413 ; 1540-1405
    ISSN (online) 1540-1413
    ISSN 1540-1405
    DOI 10.6004/jnccn.2022.7092
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  3. Article ; Online: Diagnosis and Treatment of AL Amyloidosis.

    Palladini, Giovanni / Milani, Paolo

    Drugs

    2023  Volume 83, Issue 3, Page(s) 203–216

    Abstract: Systemic light chain (AL) amyloidosis is caused by an usually small B cell clone that produces a toxic light chain forming amyloid deposits in tissue. The heart and kidney are the major organs affected, but all others, with the exception of the CNS, can ... ...

    Abstract Systemic light chain (AL) amyloidosis is caused by an usually small B cell clone that produces a toxic light chain forming amyloid deposits in tissue. The heart and kidney are the major organs affected, but all others, with the exception of the CNS, can be involved. The disease is rapidly progressive, and it is still diagnosed late. Screening programs in patients followed by hematologists for plasma cell dyscrasias should be considered. The diagnosis requires demonstration in a tissue biopsy of amyloid deposits formed by immunoglobulin light chains. The workup of patients with AL amyloidosis requires adequate technology and expertise, and patients should be referred to specialized centers whenever possible. Stagings are based on cardiac and renal biomarkers and guides the choice of treatment. The combination of daratumumab, cyclophosphamide, bortezomib and dexamethasone (dara-CyBorD) is the current standard of care. Autologous stem cell transplant is performed in eligible patients, especially those who do not attain a satisfactory response to dara-CyBorD. Passive immunotherapy targeting the amyloid deposits combined with chemo-/immune-therapy targeting the amyloid clone is currently being tested in controlled clinical trials. Response to therapy is assessed based on validated criteria. Profound hematologic response is the early goal of treatment and should be accompanied over time by deepening organ response. Many relapsed/refractory patients are also treated with daratumumab combination, but novel regimens will be needed to rescue daratumumab-exposed subjects. Immunomodulatory drugs are the current cornerstone of rescue therapy, while immunotherapy targeting B-cell maturation antigen and inhibitors of Bcl-2 are promising alternatives.
    MeSH term(s) Humans ; Immunoglobulin Light-chain Amyloidosis/diagnosis ; Immunoglobulin Light-chain Amyloidosis/drug therapy ; Plaque, Amyloid/complications ; Bortezomib ; Kidney ; Cyclophosphamide
    Chemical Substances Bortezomib (69G8BD63PP) ; Cyclophosphamide (8N3DW7272P)
    Language English
    Publishing date 2023-01-18
    Publishing country New Zealand
    Document type Journal Article ; Review
    ZDB-ID 120316-2
    ISSN 1179-1950 ; 0012-6667
    ISSN (online) 1179-1950
    ISSN 0012-6667
    DOI 10.1007/s40265-022-01830-z
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    Zs.A 762: Show issues Location:
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  4. Article ; Online: Advances in the treatment of light chain amyloidosis.

    Palladini, Giovanni / Milani, Paolo

    Current opinion in oncology

    2022  Volume 34, Issue 6, Page(s) 748–756

    Abstract: Purpose of review: After many years, the management of systemic light chain (AL) amyloidosis is entering the era of evidence-based medicine, with three recently published randomized clinical trials, a regimen (daratumumab, cyclophosphamide, bortezomib, ... ...

    Abstract Purpose of review: After many years, the management of systemic light chain (AL) amyloidosis is entering the era of evidence-based medicine, with three recently published randomized clinical trials, a regimen (daratumumab, cyclophosphamide, bortezomib, and dexamethasone, daratumumab-CyBorD) labeled for upfront therapy, more clinical trials ongoing, and published guidelines. In this review, we discuss how current practice is changing based on this data.
    Recent findings: Daratumumab-CyBorD grants unprecedentedly high rates of hematologic and organ response and became the novel standard-of-care in AL amyloidosis. The International Society of Amyloidosis and the European Hematology Association issued common guidelines for autologous stem cell transplant (ASCT) in this disease. Improved patient selection and effective induction regimens greatly reduced ASCT-related mortality. Venetoclax is emerging as a very effective option in patients harboring the common t(11;14) abnormality. Rapid and profound reduction of the amyloid free light chain can improve survival also at advanced stages.
    Summary: Daratumumab-CyBorD is being integrated into the treatment flow-chart whereas the role of ASCT is being redefined. New approaches are being tested in clinical trials. Treatment of daratumumab-refractory patients and validation of criteria of hematologic progression to be used in clinical trials and in individual patient management are current areas of research.
    MeSH term(s) Amyloidosis/drug therapy ; Bortezomib/therapeutic use ; Cyclophosphamide ; Dexamethasone ; Humans ; Immunoglobulin Light Chains/therapeutic use ; Treatment Outcome
    Chemical Substances Immunoglobulin Light Chains ; Bortezomib (69G8BD63PP) ; Dexamethasone (7S5I7G3JQL) ; Cyclophosphamide (8N3DW7272P)
    Language English
    Publishing date 2022-08-04
    Publishing country United States
    Document type Journal Article ; Review ; Research Support, Non-U.S. Gov't
    ZDB-ID 1049384-0
    ISSN 1531-703X ; 1040-8746
    ISSN (online) 1531-703X
    ISSN 1040-8746
    DOI 10.1097/CCO.0000000000000881
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    Zs.A 3046: Show issues Location:
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  5. Article ; Online: Role of autologous haematopoietic cell transplantation in the treatment of systemic light chain amyloidosis in the era of anti-CD38 monoclonal antibodies.

    Chakraborty, Rajshekhar / Milani, Paolo / Palladini, Giovanni / Gertz, Morie

    The Lancet. Haematology

    2023  Volume 10, Issue 11, Page(s) e936–e940

    Abstract: The primary goal of the initial treatment in systemic light chain amyloidosis is to obtain a rapid and profound haematological response as safely as possible, coupled with supportive care by a multidisciplinary team. The treatment landscape has evolved ... ...

    Abstract The primary goal of the initial treatment in systemic light chain amyloidosis is to obtain a rapid and profound haematological response as safely as possible, coupled with supportive care by a multidisciplinary team. The treatment landscape has evolved with the introduction of highly effective therapies targeting the plasma cell clones, which can attain high rates of haematological complete response with minimal treatment-related morbidity and mortality. Consequently, the role of high-dose melphalan followed by autologous haematopoietic cell transplantation (HDM-AHCT) is being analysed, particularly considering the absence of randomised controlled trial data supporting its superiority over standard-dose therapies in systemic light chain amyloidosis treatment. In this Viewpoint, we will explore the role of HDM-AHCT in the management of patients with systemic light chain amyloidosis who are eligible for transplantation, and the unresolved questions surrounding HDM-AHCT use as both front-line and salvage therapy.
    MeSH term(s) Humans ; Antineoplastic Agents/therapeutic use ; Immunoglobulin Light-chain Amyloidosis/drug therapy ; Amyloidosis/drug therapy ; Hematopoietic Stem Cell Transplantation ; Antibodies, Monoclonal/therapeutic use ; Transplantation, Autologous ; Treatment Outcome
    Chemical Substances Antineoplastic Agents ; Antibodies, Monoclonal
    Language English
    Publishing date 2023-10-03
    Publishing country England
    Document type Journal Article ; Review
    ISSN 2352-3026
    ISSN (online) 2352-3026
    DOI 10.1016/S2352-3026(23)00175-8
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  6. Article ; Online: The quest for validated treatment endpoints in light chain (AL) amyloidosis: composite criteria for a composite disease.

    Palladini, Giovanni / Milani, Paolo

    Leukemia & lymphoma

    2021  Volume 62, Issue 8, Page(s) 1793–1794

    MeSH term(s) Humans ; Immunoglobulin Light Chains ; Immunoglobulin Light-chain Amyloidosis/diagnosis ; Immunoglobulin Light-chain Amyloidosis/therapy
    Chemical Substances Immunoglobulin Light Chains
    Language English
    Publishing date 2021-04-21
    Publishing country United States
    Document type Journal Article ; Comment
    ZDB-ID 1042374-6
    ISSN 1029-2403 ; 1042-8194
    ISSN (online) 1029-2403
    ISSN 1042-8194
    DOI 10.1080/10428194.2021.1913152
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    Zs.A 2997: Show issues Location:
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  7. Article ; Online: Systematic review shows that suction-based airway clearance devices for foreign body airway obstruction are promising.

    Parri, Niccolò / Madera, Anna / D'Aiuto, Francesca / Zampogna, Stefania / Milani, Gregorio Paolo

    Acta paediatrica (Oslo, Norway : 1992)

    2024  

    Language English
    Publishing date 2024-04-02
    Publishing country Norway
    Document type Journal Article
    ZDB-ID 203487-6
    ISSN 1651-2227 ; 0365-1436 ; 0803-5253
    ISSN (online) 1651-2227
    ISSN 0365-1436 ; 0803-5253
    DOI 10.1111/apa.17229
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  8. Article ; Online: Efecto del riesgo cardiovascular sobre la limpieza del ARN del SARS-CoV-2 en hisopos nasofaríngeos de pacientes con COVID-19.

    Mazzaccaro, Daniela / Piccinni, Rosangela / Milani, Valentina / Righini, Paolo / Nano, Giovanni / Gobbo, Giulia Ml

    Revista espanola de salud publica

    2024  Volume 98, Page(s) e1–e4

    Title translation Effect of cardiovascular risk on SARS-CoV-2 RNA clearance in nasopharyngeal swabs from COVID-19 patients.
    MeSH term(s) Humans ; COVID-19 ; SARS-CoV-2 ; RNA, Viral/genetics ; Cardiovascular Diseases/diagnosis ; Risk Factors ; Spain ; Nasopharynx ; Heart Disease Risk Factors
    Chemical Substances RNA, Viral
    Language Spanish
    Publishing date 2024-03-01
    Publishing country Spain
    Document type Letter
    ZDB-ID 1288657-9
    ISSN 2173-9110 ; 0034-8899 ; 1135-5727
    ISSN (online) 2173-9110
    ISSN 0034-8899 ; 1135-5727
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  9. Article ; Online: Monitoring Patients with Light Chain (AL) Amyloidosis during and after Therapy

    Paolo Milani / M. Teresa Cibeira

    Hemato, Vol 3, Iss 8, Pp 98-

    Response Assessment and Identification of Relapse

    2022  Volume 108

    Abstract: Light chain amyloidosis is a complex disease where a small B-cell clone produces a monoclonal immunoglobulin light chain that causes deposits and specific organ dysfunction. The available treatment strategies aim to reduce or eliminate amyloidogenic ... ...

    Abstract Light chain amyloidosis is a complex disease where a small B-cell clone produces a monoclonal immunoglobulin light chain that causes deposits and specific organ dysfunction. The available treatment strategies aim to reduce or eliminate amyloidogenic light chain production in order to avoid amyloid deposition and allow the repair of organ damage. An international effort allowed the definition of validated hematologic and organ response criteria based on biomarkers. Recently, new methods for the assessment of minimal residual disease were also proposed but still need international validation. Lastly, a joint effort is also required to accurately define relapse/progression criteria in order to apply timely therapeutic interventions. In this review, we describe the validated response criteria and report on the future direction for the definition of progression criteria in this disease.
    Keywords amyloidosis ; response assessment ; prognosis ; Medicine ; R
    Subject code 610
    Language English
    Publishing date 2022-01-01T00:00:00Z
    Publisher MDPI AG
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  10. Article ; Online: Salivary fatty acids in humans: a comprehensive literature review.

    Abodi, Martina / Mazzocchi, Alessandra / Risé, Patrizia / Marangoni, Franca / Agostoni, Carlo / Milani, Gregorio Paolo

    Clinical chemistry and laboratory medicine

    2024  

    Abstract: Fatty acids (FAs) exert diverse biological functions in humans, influencing physiological responses and, ultimately, health and disease risk. The analysis of FAs in human samples has significant implications and attracts interest in diagnostics and ... ...

    Abstract Fatty acids (FAs) exert diverse biological functions in humans, influencing physiological responses and, ultimately, health and disease risk. The analysis of FAs in human samples has significant implications and attracts interest in diagnostics and research. The standard method for assessing FA profiles involves the collection of blood samples, which can be inconvenient, invasive, and potentially painful, particularly for young individuals outside hospital settings. Saliva emerged as a promising alternative for evaluating FA profiles in both clinical and research settings. However, to the best of our knowledge, an updated synthesis of the related evidence is unavailable. This comprehensive review aims to summarize data on FA analysis and highlight the potential of the use of salivary FAs as a biomarker in health and disease. Over the past decade, there has been a growing interest in studying salivary FAs in chronic diseases, and more recently, researchers have explored the prognostic value of FAs in acute conditions to check the availability of a non-invasive sampling methodology. A deeper understanding of salivary FAs could have relevant implications both for healthy individuals and patients, particularly in elucidating the correlation between the dietary lipidic content and salivary FA level, Finally, it is crucial to address the standardization of the methods as the sampling, processing, and analysis of saliva are heterogeneous among studies, and limited correlation between blood FAs and salivary FAs is available.
    Language English
    Publishing date 2024-04-19
    Publishing country Germany
    Document type Journal Article ; Review
    ZDB-ID 1418007-8
    ISSN 1437-4331 ; 1434-6621 ; 1437-8523
    ISSN (online) 1437-4331
    ISSN 1434-6621 ; 1437-8523
    DOI 10.1515/cclm-2024-0177
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