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Maria Stella Graziani

Deputy Director
Martina Zaninotto

Associate Editors
Ferruccio Ceriotti
Davide Giavarina
Bruna Lo Sasso
Giampaolo Merlini
Martina Montagnana
Andrea Mosca
Paola Pezzati
Rossella Tomaiuolo
Matteo Vidali

International Advisory Board Khosrow Adeli Canada
Sergio Bernardini Italy
Marcello Ciaccio Italy
Eleftherios Diamandis Canada
Philippe Gillery France
Kjell Grankvist Sweden
Hans Jacobs The Netherlands
Eric Kilpatrick UK
Magdalena Krintus Poland
Giuseppe Lippi Italy
Mario Plebani Italy
Sverre Sandberg Norway
Ana-Maria Simundic Croatia
Tommaso Trenti Italy
Cas Weykamp The Netherlands
Maria Willrich USA
Paul Yip Canada

Biomedia srl
Via L. Temolo 4, 20126 Milano

Responsible Editor
Giuseppe Agosta

Editorial Secretary
Andrea di Bello
Biomedia srl
Via L. Temolo 4, 20126 Milano
Tel. 0245498282


ISSN print: 0393 – 0564
ISSN digital: 0392- 7091

BC: Articoli scritti da M. Vidus Rosin

Identificazione di danno renale reversibile e di precoce risposta alla chemioterapia in pazienti con amiloidosi AL
Identification of reversible renal damage and early response to chemotherapy in AL amyloidosis
<p>The kidney&nbsp;is involved in 70% of patients with immunoglobulin light-chain (AL) amyloidosis, but little is known on progression or&nbsp;reversibility of renal involvement. Furthermore, criteria for renal response have never been validated. We designed a&nbsp;staging system for renal damage and identified criteria for renal response and progression in a population of 732&nbsp;newly diagnosed patients with AL amyloidosis. The population was composed of 461 patients from Pavia (testing&nbsp;cohort) and 271 subjects from Heidelberg (validation cohort). Baseline proteinuria &gt;5 g/24 h and estimated glomerular&nbsp;filtration rate (eGFR) &lt;50 mL/min/1.73 m<sup>2</sup> were independently associated with poorer renal survival and discriminated&nbsp;between 3 stages (with none, one or two markers above the cut-off) with significant different renal survival. At 6-month&nbsp;follow-up, a &ge;25% eGFR decrease predicted poor renal survival in both cohorts and was adopted as criterion for renal&nbsp;progression. A decrease in proteinuria &ge;30% or below the cut-off of 0.5 g/24 h in absence of renal progression were&nbsp;the criteria for renal response, being associated with longer renal survival in the testing and validation cohorts. These&nbsp;endpoints can be used as validated response criteria in renal AL amyloidosis, allowing early assessment of treatment&nbsp;efficacy.</p>
Biochimica Clinica ; 40(1) 21-27
Contributi scientifici - Scientific Papers
Amiloidosi AL: il cuore del problema
AL amyloidosis: the heart of the problem
<p>Immunoglobulin light chain amyloidosis (AL) is characterized by the&nbsp;production of immunoglobulin light chains with conformational abnormalities that cause systemic toxicity with rapid&nbsp;deterioration of the function of vital organs. When the heart is involved, as it is the case in ~3/4 of patients, clinical signs&nbsp;and symptoms often appear when organ damage is already irreversible and the treatment cannot longer change the&nbsp;course of disease. Although in recent years new powerful therapeutic regimens have become available, which are able&nbsp;to significantly improve long-term survival, the mortality rate in the first year after diagnosis has indeed not improved, still&nbsp;being 25-30%. Cardiac involvement is responsible for almost all of these deaths. Early diagnosis based on biochemical&nbsp;markers of the disease rather than on clinical symptoms and signs can allow for early detection of patients with cardiac&nbsp;amyloidosis and to establish an effective therapy. To this end, our group has proposed the introduction of the&nbsp;measurement of natriuretic peptides that can identify the presence of amyloid cardiomyopathy with a sensitivity of 100%&nbsp;in the monitoring of subjects with monoclonal gammopathies of undetermined significance (MGUS) and altered ratio of&nbsp;circulating free light chains (FLC). Individuals with MGUS and altered FLC ratio are at intermediate/high risk of&nbsp;developing a malignant disease (AL amyloidosis in 10-15% of cases) and, according to the guidelines of the International&nbsp;Myeloma Working Group, they should be monitored regularly for their entire life. Here we describe a case where the&nbsp;application of these recent recommendations has allowed the timely recognition of amyloid cardiomyopathy.</p>
Biochimica Clinica ; 39(3) 220-222
Casi clinici - Case report