{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T14:26:10Z","timestamp":1762093570612,"version":"build-2065373602"},"reference-count":42,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","license":[{"start":{"date-parts":[[2023,9,1]],"date-time":"2023-09-01T00:00:00Z","timestamp":1693526400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:p>Light chain amyloidosis (AL) is a complex disorder defined by the extracellular deposition of insoluble amyloid fibrils formed by intact or fragmented immunoglobulin light chains, leading to cell dysfunction, rapid organ deterioration, and, ultimately, death. Although the clinical presentation of AL is directly connected to organ involvement, signs and symptoms of AL are frequently nonspecific, misinterpreted, and late recognized. Thus, an early diagnosis combined with effective therapies to cease disease progression and rescue organ function is essential. The aim of this study was to assess the knowledge and characterize the current clinical practice regarding AL diagnosis and referral among Portuguese physicians. A Delphi-like panel (one round only) with a group of national experts from different medical specialties (cardiology, hematology, internal medicine, nephrology, and neurology) was carried out online, in which 30 statements were classified using a 4-point Likert scale. For each statement, the consensus level was set at 70% for \u201cfully agree\/disagree\u201d and the majority level was defined as &gt;70% in agreement or disagreement. Although the results suggest the existence of adequate general knowledge of AL amyloidosis, they also disclosed the necessity to raise awareness for this disease. Overall, this Delphi panel revealed a high lack of consensus regarding the diagnosis and early management of patients with AL among different specialties despite the qualified majority obtained in 26 statements. An optimized strategy for AL early diagnosis, transversal to several medical fields, is urgently needed. Moreover, referral centers with access to diagnostic technology and a network of diverse specialties should be established to foster an early diagnosis and better disease approach to boost the possibility of a better outcome for patients with AL.<\/jats:p>","DOI":"10.1097\/j.pbj.0000000000000231","type":"journal-article","created":{"date-parts":[[2023,10,17]],"date-time":"2023-10-17T15:15:54Z","timestamp":1697555754000},"update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":2,"title":["Diagnosis and referral of patients with AL amyloidosis in Portugal: results from a Delphi panel"],"prefix":"10.1097","volume":"8","author":[{"given":"Rui","family":"Bergantim","sequence":"first","affiliation":[{"name":"Faculty of Medicine of the University of Porto (FMUP), Porto, Portugal"},{"name":"i3S - Institute for Research and Innovation in Health, University of Porto, Porto, Portugal"},{"name":"Cancer Drug Resistance Group, Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Porto, Portugal"},{"name":"Department of Hematology, Centro Hospitalar Universit\u00e1rio de S\u00e3o Jo\u00e3o, Porto, Portugal"}]},{"given":"Andr\u00e9","family":"Caetano","sequence":"additional","affiliation":[{"name":"Hospital de Egas Moniz, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal"}]},{"given":"Francisco F.","family":"Silva","sequence":"additional","affiliation":[{"name":"Hospital da Cruz Vermelha, Lisbon, Portugal"}]},{"given":"Isabel","family":"Tavares","sequence":"additional","affiliation":[{"name":"Faculty of Medicine of the University of Porto (FMUP), Porto, Portugal"},{"name":"i3S - Institute for Research and Innovation in Health, University of Porto, Porto, Portugal"},{"name":"Nephrology Service, Centro Hospitalar e Universit\u00e1rio de S\u00e3o Jo\u00e3o, Porto, Portugal"}]},{"given":"Manuela","family":"Ferreira","sequence":"additional","affiliation":[{"name":"Hospital de Santa Maria, Centro Hospitalar Universit\u00e1rio Lisboa Norte, Lisbon, Portugal"}]},{"given":"Ana R.","family":"Jaime","sequence":"additional","affiliation":[{"name":"Medical Department\u2014Hematology, Janssen-Cilag, S.A., Lisbon, Portugal"}]},{"given":"Gra\u00e7a V.","family":"Esteves","sequence":"additional","affiliation":[{"name":"Hospital de Santa Maria, Centro Hospitalar Universit\u00e1rio Lisboa Norte, Lisbon, Portugal"}]}],"member":"276","published-online":{"date-parts":[[2023,9]]},"reference":[{"issue":"1","key":"R1-20241001","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1038\/s41572-018-0034-3","article-title":"Systemic immunoglobulin light chain amyloidosis","volume":"4","author":"Merlini","year":"2018","journal-title":"Nat Rev Dis Primers"},{"issue":"4","key":"R2-20241001","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1016\/j.jmb.2019.12.015","article-title":"Inherent biophysical properties modulate the toxicity of soluble amyloidogenic light 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