ROBERTA SHCOLNIK SZOR
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Unidades Organizacionais
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conferenceObject Treating multiple myeloma in a resource-limited setting: real-world outcomes(2022) MATINEZ, Gracia; SEGURO, Fernanda; JACOMASSI, Mayara; VISNADI, Helena; ATANAZIO, Marcelo; SZOR, Roberta; NEFFA, Pedro; PEREIRA, Thales; SILVA, Wellington; DORLHIAC, Pedro; VELASQUES, Rodrigo; BASSOLI, Lucas; ROCHA, Vanderson- Systemic amyloidosis journey from diagnosis to outcomes: a twelve-year real-world experience of a single center in a middle-income country(2022) SZOR, Roberta Shcolnik; FERNANDES, Fabio; LINO, Angelina Maria Martins; MENDONCA, Leonardo Oliveira; SEGURO, Fernanda Salles; FEITOSA, Valkercyo Araujo; CASTELLI, Jussara Bianchi; JORGE, Lecticia Barbosa; ALVES, Lucas Bassolli de Oliveira; NEVES, Precil Diego Miranda de Menezes; SOUZA, Evandro de Oliveira; CAVALCANTE, Livia Barreira; MALHEIROS, Denise; KALIL, Jorge; MARTINEZ, Gracia Aparecida; ROCHA, VandersonBackground: Systemic amyloidosis is caused by the deposition of misfolded protein aggregates in tissues, leading to progressive organ dysfunction and death. Epidemiological studies originate predominantly from high-income countries, with few data from Latin America. Due to the non-specific clinical manifestations, diagnosing amyloidosis is often challenging and patients experience a long journey and delay in diagnosis. This study aimed to assess clinical and laboratory characteristics, the diagnostic journey, and outcomes of patients with biopsy-proven systemic amyloidosis diagnosed between 2009 and 2020 at a university referral center in a middle-income Latin American country. Patients' medical records were retrospectively reviewed. Results: One hundred and forty-three patients were included. The median age at diagnosis was 60 years and 54% were male. Until the diagnosis, most of the patients (52%) were seen by at least 3 specialists, the main ones being: general practitioners (57%), nephrologists (45%), and cardiologists (38%). The most common manifestations were renal (54%) and cardiac (41%) disorders, and cachexia was seen in 36% of patients. In 72% of the cases, & GE; 2 biopsies were required until the final diagnosis. The median time from symptoms onset to diagnosis was 10.9 months, and most patients (75%) had & GE; 2 organs involved. The following subtypes were identified: AL (68%), ATTR (13%), AA (8%), AFib (4%), and inconclusive (7%). Median OS was 74.3 months in the non-AL subgroup and 18.5 months in AL. Among AL patients, those with advanced cardiac stage had the worst outcome [median OS 8.6 months versus 52.3 for stage III versus I-II, respectively (p < 0.001)]. AL subtype, cardiac involvement, and ECOG & GE; 2 were identified as independent risk factors for reduced survival. Conclusions: Systemic amyloidosis is still an underdiagnosed condition and the delay in its recognition leads to poor outcomes. Medical education, better diagnostic tools, improvement in access to therapies, and establishment of referral centers may improve patient outcomes in middle-income countries.
conferenceObject Coronary flow reserve by PET 13N-ammonia in patients with hereditary transthyretin amyloidosis with and without cardiac involvement(2022) ALENCAR NETO, A. C.; CAFEZEIRO, C. R. F.; BUENO, B. V. K.; SOUZA, F. Ribeiro De; RISSATO, J. H. S. Henrique; BORGES, T. Souza; CARVALHAL, S. Freitas; LIMA, M. Santos; BUCHPIGUEL, C. Alberto; CHALELA, W. Azem; RAMIRES, F. J. Alvarez; SZOR, R. Shcolnik; KALIL FILHO, R.; ROCHITTE, C. E.; FERNANDES, F.conferenceObject Gemcitabine-Induced Non-Cardiogenic Pulmonary Edema(2022) SAAVEDRA, N. Medina; BRIDI, G. D.; SANTOS, G. C.; MARANGONI, K. F.; NASCIMENTO, Y. P. P.; SCIORTINO, A. D.; VISNADI, H.; SZOR, R. S.; CURY-MARTINS, J.; KAWASSAKI, A. M.- Clinical, Laboratory, and Imaging Profile in Patients with Systemic Amyloidosis in a Brazilian Cardiology Referral Center(2022) FERNANDES, Fabio; ALENCAR NETO, Aristoteles Comte de; BUENO, Bruno Vaz Kerges; CAFEZEIRO, Caio Reboucas Fonseca; RISSATO, Joao Henrique; SZOR, Roberta Shcolnik; CARVALHO, Mariana Lombardi Peres de; MATHIAS JUNIOR, Wilson; LINO, Angelina Maria Martins; CASTELLI, Jussara Bianchi; SOUZA, Evandro de Oliveira; RAMIRES, Felix Jose Alvarez; HOTTA, Viviane Tiemi; SOARES JUNIOR, Jose; TAVARES, Caio de Assis Moura; KRIEGER, Jose Eduardo; ROCHITTE, Carlos Eduardo; DABARIAN, Andre; HAJJAR, Ludhmila Abrahao; KALIL FILHO, Roberto; MADY, CharlesBackground: Systemic amyloidosis is a disease with heterogeneous clinical manifestations. Diagnosis depends on clinical suspicion combined with specific complementary methods. Objective: To describe the clinical, laboratory, electrocardiographic, and imaging profile in patients with systemic amyloidosis with cardiac involvement. Methods: This study was conducted with a convenience sample, analyzing clinical, laboratory, electrocardiographic, echocardiographic, nuclear medicine, and magnetic resonance data. Statistical significance was set at p < 0.05. Results: A total of 105 patients were evaluated (median age of 66 years), 62 of whom were male. Of all patients, 83 had transthyretin (ATTR) amyloidosis, and 22 had light chain (AL) amyloidosis. With respect to ATTR cases, 68.7% were the hereditary form (ATTRh), and 31.3% were wild type (ATTRw). The most prevalent mutations were Val142Ile (45.6%) and Val50Met (40.3%). Time from onset of symptoms to diagnosis was 0.54 and 2.15 years, in the AL and ATTR forms, respectively (p < 0.001). Cardiac involvement was observed in 77.9% of patients with ATTR and in 90.9% of those with AL. Alterations were observed in atrioventricular and intraventricular conduction in 20% and 27.6% of patients, respectively, with 33.7% in ATTR and 4.5% in AL (p = 0.006). In the ATTRw form, there were more atrial arrhythmias than in ATTRh (61.5% versus 22.8%; p = 0.001). On echocardiogram, median septum thickness in ATTRw, ATTRh, and AL was 15 mm, 12 mm, and 11 mm, respectively (p = 0.193). Elevated BNP was observed in 89.5% of patients (median 249, ICR 597.7), and elevated troponin was observed in 43.2%. Conclusion: In this setting, it was possible to characterize cardiac involvement in systemic amyloidosis in its different subtypes by means of clinical history and the diagnostic methods described.