Artigos e Materiais de Revistas Científicas - LIM/36

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https://observatorio.fm.usp.br/handle/OPI/3739

A coleção de Artigos e Materiais de Revistas Científicas engloba artigos originais, artigos de revisão, artigos de atualização, artigos técnicos, relatos de experiências, resenhas, ensaios, editoriais, cartas ao editor, debates, notas científicas e técnicas, depoimentos, entrevistas e pontos de vista. Consideram-se como artigos científicos originais os trabalhos redigidos para divulgação de informações e resultados sobre determinada pesquisa científica, publicados em periódico científico após avaliação por outros pesquisadores.

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Data-driven, cross-disciplinary collaboration: lessons learned at the largest academic health center in Latin America during the COVID-19 pandemic
(2024) RITTO, Ana Paula; ARAUJO, Adriana Ladeira de; CARVALHO, Carlos Roberto Ribeiro de; SOUZA, Heraldo Possolo De; FAVARETTO, Patricia Manga e Silva; SABOYA, Vivian Renata Boldrim; GARCIA, Michelle Louvaes; KULIKOWSKI, Leslie Domenici; KALLAS, Esper Georges; PEREIRA, Antonio Jose Rodrigues; COBELLO JUNIOR, Vilson; SILVA, Katia Regina; ABDALLA, Eidi Raquel Franco; SEGURADO, Aluisio Augusto Cotrim; SABINO, Ester Cerdeira; RIBEIRO JUNIOR, Ulysses; FRANCISCO, Rossana Pulcineli Vieira; MIETHKE-MORAIS, Anna; LEVIN, Anna Sara Shafferman; SAWAMURA, Marcio Valente Yamada; FERREIRA, Juliana Carvalho; SILVA, Clovis Artur; MAUAD, Thais; GOUVEIA, Nelson da Cruz; LETAIF, Leila Suemi Harima; BEGO, Marco Antonio; BATTISTELLA, Linamara Rizzo; DUARTE, Alberto Jose da Silva; SEELAENDER, Marilia Cerqueira Leite; MARCHINI, Julio; FORLENZA, Orestes Vicente; ROCHA, Vanderson Geraldo; MENDES-CORREA, Maria Cassia; COSTA, Silvia Figueiredo; CERRI, Giovanni Guido; BONFA, Eloisa Silva Dutra de Oliveira; CHAMMAS, Roger; BARROS FILHO, Tarcisio Eloy Pessoa de; BUSATTO FILHO, Geraldo
Introduction The COVID-19 pandemic has prompted global research efforts to reduce infection impact, highlighting the potential of cross-disciplinary collaboration to enhance research quality and efficiency.Methods At the FMUSP-HC academic health system, we implemented innovative flow management routines for collecting, organizing and analyzing demographic data, COVID-related data and biological materials from over 4,500 patients with confirmed SARS-CoV-2 infection hospitalized from 2020 to 2022. This strategy was mainly planned in three areas: organizing a database with data from the hospitalizations; setting-up a multidisciplinary taskforce to conduct follow-up assessments after discharge; and organizing a biobank. Additionally, a COVID-19 curated collection was created within the institutional digital library of academic papers to map the research output.Results Over the course of the experience, the possible benefits and challenges of this type of research support approach were identified and discussed, leading to a set of recommended strategies to enhance collaboration within the research institution. Demographic and clinical data from COVID-19 hospitalizations were compiled in a database including adults and a minority of children and adolescents with laboratory confirmed COVID-19, covering 2020-2022, with approximately 350 fields per patient. To date, this database has been used in 16 published studies. Additionally, we assessed 700 adults 6 to 11 months after hospitalization through comprehensive, multidisciplinary in-person evaluations; this database, comprising around 2000 fields per subject, was used in 15 publications. Furthermore, thousands of blood samples collected during the acute phase and follow-up assessments remain stored for future investigations. To date, more than 3,700 aliquots have been used in ongoing research investigating various aspects of COVID-19. Lastly, the mapping of the overall research output revealed that between 2020 and 2022 our academic system produced 1,394 scientific articles on COVID-19.Discussion Research is a crucial component of an effective epidemic response, and the preparation process should include a well-defined plan for organizing and sharing resources. The initiatives described in the present paper were successful in our aim to foster large-scale research in our institution. Although a single model may not be appropriate for all contexts, cross-disciplinary collaboration and open data sharing should make health research systems more efficient to generate the best evidence.
Establishment of oral microbiome in very low birth weight infants during the first weeks of life and the impact of oral diet implementation
(2023) VANZELE, Pedro A. R.; SPARVOLI, Luiz Gustavo; CAMARGO, Patricia P. de; TRAGANTE, Carla R.; BEOZZO, Glenda P. N. S.; KREBS, Vera L. J.; CORTEZ, Ramon V.; TADDEI, Carla R.
Very low birth weight (VLBW) infants, mostly preterm, have many barriers to feeding directly from the mother's breast, and need to be fed alternatively. Feeding is a major influencer in oral microbial colonization, and this colonization in early life is crucial for the promotion of human health. Therefore, this research aimed to observe the establishment of oral microbiome in VLBW infants during their first month of life through hospitalization, and to verify the impact caused by the implementation of oral diet on the colonization of these newborns. We included 23 newborns followed during hospitalization and analyzed saliva samples collected weekly, using 16S rRNA gene sequencing. We observed a significant decrease in richness and diversity and an increase in dominance over time (q-value < 0.05). The oral microbiome is highly dynamic during the first weeks of life, and beta diversity suggests a microbial succession in early life. The introduction of oral diet does not change the community structure, but affects the abundance, especially of Streptococcus. Our results indicate that although time is related to significant changes in the oral microbial profile, oral feeding benefits genera that will remain colonizers throughout the host's life.
A randomized, double-blind, non-inferiority trial comparing the immunogenicity and safety of two seasonal inactivated influenza vaccines in adults
(2023) VANNI, Tazio; SALOMAO, Maria da Grada; VISCONDI, Juliana Yukari Kodaira; BRAGA, Patricia Emilia; SILVA, Anderson da; PIORELLI, Roberta de Oliveira; SANTOS, Joane do Prado; GATTAS, Vera Lucia; LUCCHESI, Maria Beatriz Bastos; OLIVEIRA, Mayra Martho Moura de; KOIKE, Marcelo Eiji; CAMPOS, Lucia M. A.; COELHO, Eduardo B.; WECKX, Lily Yin; LARA, Amanda Nazareth; PAIVA, Terezinha M.; TIMENETSKY, Maria do Carmo S. T.; PRECIOSO, Alexander Roberto
Background: To enhance the production and availability of influenza vaccines in different regions of the world is paramount to mitigate the global burden of this disease. Instituto Butantan developed and man-ufactured an embryonated egg-based inactivated split-virion trivalent seasonal influenza vaccine as part of a technology transfer partnership with Sanofi Pasteur.Methods: This is a phase IV, randomized, double-blind, active-controlled, multicenter clinical trial includ-ing adults 18-60 and > 60 years recruited during the 2019 southern hemisphere influenza season. Subjects were randomized 1:1 to receive either the Sanofi Pasteur Trivalent Seasonal Influenza Vaccine (SP-TIV) or Instituto Butantan Trivalent Seasonal Influenza Vaccine (IB-TIV). Hemagglutinin inhibition antibody titers were assessed pre-vaccination and 21 days post-vaccination.Results: 624 participants were randomized and vaccinated. In both intention-to-treat and per-protocol analysis, non-inferiority of the SP-TIV versus IB-TIV was demonstrated for the three influenza strains. In the per-protocol analysis, the SP-GMT/IB-GMT ratios for H1N1, H3N2, and B were 0.9 (95%CI, 0.7- 1.1), 1.2 (95%CI, 1.0-1.4), and 1.1 (95%CI, 0.9-1.3), respectively. Across vaccination groups, the most com-mon adverse reactions (AR) were limited to the injection-site, including pain and tenderness. The major-ity of the ARs were graded 1 and/or 2 and lasted less than one day. No serious adverse reaction was observed.Conclusion: This study demonstrated the non-inferiority of the immunogenicity of a single-dose of Instituto Butantan versus a single dose of the Sanofi Pasteur Seasonal Trivalent Influenza Vaccine in adults. Both vaccines were well tolerated and presented similar safety profiles.(c) 2023 Elsevier Ltd. All rights reserved.
Use of elexacaftor plus tezacaftor plus ivacaftor in individuals with cystic fibrosis and at least one F508del allele: a systematic review and meta-analysis
(2023) SILVA FILHO, Luiz Vicente Ribeiro Ferreira da; ATHANAZIO, Rodrigo Abensur; TONON, Carolina Rodrigues; FERREIRA, Juliana Carvalho; TANNI, Suzana Erico
Objective: To evaluate the effect of treatment with the combination of three cystic fibrosis transmembrane conductance regulator (CFTR) modulators-elexacaftor+tezac aftor+ivacaftor (ETI)-on important clinical endpoints in individuals with cystic fibrosis. Methods: This was a systematic review and meta-analysis of randomized clinical trials that compared the use of ETI in individuals with CF and at least one F508del allele with that of placebo or with an active comparator such as other combinations of CFTR modulators, following the Preferred Reporting Items for Systematic Reviews and MetaAnalyses (PRISMA) recommendations and the Patients of interest, Intervention to be studied, Comparison of interventions, and Outcome of interest (PICO) methodology. We searched the following databases: MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, and ClinicalTrials.gov from their inception to December 26th, 2022. The risk of bias was assessed using the Cochrane risk-of-bias tool, and the quality of evidence was based on the Grading of Recommendations Assessment, Development and Evaluation (GRADE). Results: We retrieved 54 studies in the primary search. Of these, 6 met the inclusion criteria and were analyzed (1,127 patients; 577 and 550 in the intervention and control groups, respectively). The meta-analysis revealed that the use of ETI increased FEV 1% [risk difference (RD), +10.47%; 95% CI, 6.88-14.06], reduced the number of acute pulmonary exacerbations (RD, -0.16; 95% CI, -0.28 to -0.04), and improved quality of life (RD, +14.93; 95% CI, 9.98-19.89) and BMI (RD, +1.07 kg/m(2); 95% CI, 0.90-1.25). Adverse events did not differ between groups (RD, -0.03; 95% CI, -0.08 to 0.01), and none of the studies reported deaths. Conclusions: Our findings demonstrate that ETI treatment substantially improves clinically significant, patientcentered outcomes.
Rothmund-Thomson syndrome, a disorder far from solved
(2023) MARTINS, Davi Jardim; LAZZARO FILHO, Ricardo Di; BERTOLA, Debora Romeo; HOCH, Nicolas Carlos
Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive disorder characterized by a range of clinical symptoms, including poikiloderma, juvenile cataracts, short stature, sparse hair, eyebrows/eyelashes, nail dysplasia, and skeletal abnormalities. While classically associated with mutations in the RECQL4 gene, which encodes a DNA helicase involved in DNA replication and repair, three additional genes have been recently identified in RTS: ANAPC1, encoding a subunit of the APC/C complex; DNA2, which encodes a nuclease/helicase involved in DNA repair; and CRIPT, encoding a poorly characterized protein implicated in excitatory synapse formation and splicing. Here, we review the clinical spectrum of RTS patients, analyze the genetic basis of the disease, and discuss molecular functions of the affected genes, drawing some novel genotype-phenotype correlations and proposing avenues for future studies into this enigmatic disorder.
Evaluation of pediatric diabetes mellitus after SARS-CoV-2 infection: A long-term prospective case series
(2023) FINK, Thais T.; CANTON, Ana P. M.; PEREIRA, Maria Fernanda B.; BAIN, Vera; MATSUO, Olivia; ASTLEY, Camilla; MARQUES, Heloisa H. S.; CORREA-SILVA, Simone; MONTENEGRO, Marilia M.; PALMEIRA, Patricia; GARANITO, Marlene P.; DUARTE, Alberto J. S.; CARNEIRO-SAMPAIO, Magda; LATRONICO, Ana C.; SILVA, Clovis A.
Mental health among children and adolescents after SARS-CoV-2 infection: A prospective study in a tertiary university hospital
(2023) MATSUO, Olivia Mari; LINDOSO, Livia; MARQUES, Heloisa Helena de Sousa; V, Guilherme Polanczyk; FARHAT, Sylvia Costa Lima; BAIN, Vera; FINK, Thais T.; MARTINS, Fernanda; ASTLEY, Camilla; SUGUITA, Priscila; TRINDADE, Vitor; CORREA-SILVA, Simone; PALMEIRA, Patricia; SANSON, Camila; PAULA, Yoshino de; LITVINOV, Nadia; FERREIRA, Juliana Caires O. A.; SAKITA, Neusa Keico; GUALANO, Bruno; SILVA, Clovis Artur A.; PEREIRA, Maria Fernanda Badue
Impaired cardiorespiratory fitness and endothelial function after SARS-CoV-2 infection in a sample of mainly immunocompromised youth
(2023) ASTLEY, Camilla; PRADO, Danilo Marcelo Leite do; SIECZKOWSKA, Sofia Mendes; ESTEVES, Gabriel P.; SUGUITA, Priscila; FINK, Thais; LINDOSO, Livia; MATSUO, Olivia; MARTINS, Fernanda; BAIN, Vera; PEREIRA, Maria Fernanda Badue; MARQUES, Heloisa Helena; MALLUF, Adriana; LEAL, Gabriela Nunes; SILVA, Clovis Artur; ROSCHEL, Hamilton; GUALANO, Bruno
This study aimed to compare cardiopulmonary fitness and endothelial function 6 months after hospital diagnosis in a sample mainly comprising immunocompromised patients with confirmed severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection versus noninfected controls. Youth (n = 30; age: 14 yr; 60% females) with confirmed SARS-CoV-2 seen in a tertiary hospital of Sao Paulo, Brazil, were matched by propensity score based on BMI, age, sex, and pre-existing diseases with a control group who had not been tested positive for SARS-CoV-2 infection (n = 30; age: 15 yr; 50% females). Cardiopulmonary fitness (by means of a cardiopulmonary exercise test: CPET) and brachial flow-mediated dilation (%b-FMD) were assessed 3-6 mo after diagnosis. Patients were matched by propensity score based on BMI, age, sex and pre-existing diseases, if any, with a control group who had not been tested positive for SARS-CoV-2. Compared with controls, patients with COVID-19 showed reduced ventilatory anaerobic threshold (VAT) and peak exercise time and minute ventilation/maximum voluntary ventilation (V_E/MVV) (all P < 0.01). Brachial endothelial function variables were all adjusted for body surface area (BSA). Patients with COVID-19 had decreased %b-FMD (3.6 vs. 5.4; P = 0.03) mean and positive flow (P = 0.02 and P = 0.03, respectively) versus controls. Adjusted linear regression models exploring associations between CPET variables, %b-FMD and the potential predictors post-COVID-19 syndrome, number of symptoms, hospitalization, and COVID severity did not detect significant associations, except for total shear rate in hospitalization (coefficient: -65.07 [95%CI -119.5;-10.5], P = 0.02). Immunocompromised and previously healthy children and adolescents with COVID-19 presented with impaired exercise capacity and endothelial dysfunction when compared with their noninfected counterparts, but the mechanisms remain unknown.
Risk factors for mortality in 1528 Brazilian childhood-onset systemic lupus erythematosus patients
(2023) SAKAMOTO, Ana P.; SILVA, Clovis A.; PITA, Ana C.; TRINDADE, Vitor C.; ISLABAO, Aline G.; FIOROT, Fernanda J.; LOPES, Sandra R. M.; PEREIRA, Rosa M. R.; SAAD-MAGALHAES, Claudia; RUSSO, Gleice C. S.; LEN, Claudio A.; PRADO, Rogerio do; CAMPOS, Lucia M. A.; AIKAWA, Nadia E.; APPENZELLER, Simone; FERRIANI, Virginia P. L.; SILVA, Marco F.; FELIX, Marta; FONSECA, Adriana R.; ASSAD, Ana P. L.; SZTAJNBOK, Flavio R.; SANTOS, Maria C.; BICA, Blanca E.; SENA, Evaldo G.; MORAES, Ana J.; FRAGA, Melissa M.; ROBAZZI, Teresa C.; SPELLING, Paulo F.; SCHEIBEL, Iloite M.; CAVALCANTI, Andre S.; MATOS, Erica N.; GUIMARAES, Luciano J.; SANTOS, Flavia P.; MOTA, Licia M. H.; BONFA, Eloisa; TERRERI, Maria T.
Objectives: To identify associations between mortality in cSLE patients and their characteristics: clinical and laboratory features, disease activity and damage scores, and treatment; to evaluate risk factors associated with mortality in cSLE; and to determine the most frequent causes of death in this group of patients.Methods: We performed a multicenter retrospective cohort using data from 1,528 cSLE patients followed in 27 pediatric rheumatology tertiary centers in Brazil. Patients' medical records were reviewed according to a standardized protocol, in which information regarding demographic and clinical features, disease activity and damage scores, and treatment were collected and compared between deceased cSLE patients and survivors. Univariate and multivariate analyses by Cox regression model were used to calculate risk factors for mortality, whereas survival rates were analyzed by Kaplan-Meier plots.Results: A total of 63/1,528 (4.1%) patients deceased, 53/63 were female (84.1%), median age at death was 11.9 (9.4-13.1) years and median time interval between cSLE diagnosis and death was 3.2 (0.5-5.3) years. Sepsis was the main cause of death in 27/63 (42.8%) patients, followed by opportunistic infections in 7/63 (11.1%), and alveolar hemorrhage in 6/63 (9.5%) patients. The regression models resulted in neuropsychiatric lupus (NP-SLE) (HR = 2.56, 95% CI = 1.48-4.42) and chronic kidney disease (CKD) (HR = 4.33, 95% CI = 2.33-4.72), as risk factors significantly associated with mortality. Overall patient survival after cSLE diagnosis at 5, 10, and 15 years were 97%, 95.4%, and 93.8%, respectively.Conclusions: This study confirmed that the recent mortality rate in cSLE in Brazil is low, but still of concern. NP-SLE and CKD were the main risk factors for mortality, indicating that the magnitude of these manifestations was significantly high.
Association between Serum Lactate and Morbidity and Mortality in Neonates: A Systematic Review and Meta-Analysis
(2023) MATSUSHITA, Felipe Yu; KREBS, Vera Lucia Jornada; CARVALHO, Werther Brunow De
Objective: Lactate is a marker of hypoperfusion in critically ill patients. Whether lactate is useful for identifying and stratifying neonates with a higher risk of adverse outcomes remains unknown. This study aimed to investigate the association between lactate and morbidity and mortality in neonates. Methods: A meta-analysis was performed to determine the association between blood lactate levels and outcomes in neonates. Ovid MEDLINE, EMBASE, Cochrane Library, and ClinicalTrials.gov were searched from inception to 1 May 2021. A total of 49 observational studies and 14 data accuracy test studies were included. The risk of bias was assessed using the Newcastle-Ottawa Scale for observational studies and the QUADAS-2 tool for data accuracy test studies. The primary outcome was mortality, while the secondary outcomes included acute kidney injury, necessity for renal replacement therapy, neurological outcomes, respiratory morbidities, hemodynamic instability, and retinopathy of prematurity. Results: Of the 3184 articles screened, 63 studies fulfilled all eligibility criteria, comprising 46,069 neonates. Higher lactate levels are associated with mortality (standard mean difference, -1.09 [95% CI, -1.46 to -0.73]). Using the estimated sensitivity (0.769) and specificity (0.791) and assuming a prevalence of 15% for adverse outcomes (median of prevalence among studies) in a hypothetical cohort of 10,000 neonates, assessing the lactate level alone would miss 346 (3.46%) cases (false negative) and wrongly diagnose 1776 (17.76%) cases (false positive). Conclusions: Higher lactate levels are associated with a greater risk of mortality and morbidities in neonates. However, our results do not support the use of lactate as a screening test to identify adverse outcomes in newborns. Research efforts should focus on analyzing serial lactate measurements, rather than a single measurement.
Identifying two distinct subphenotypes of patent ductus arteriosus in preterm infants using machine learning
(2023) MATSUSHITA, Felipe Yu; KREBS, Vera Lucia Jornada; CARVALHO, Werther Brunow de
To use unsupervised machine learning to identify potential subphenotypes of preterm infants with patent ductus arteriosus (PDA). The study was conducted retrospectively at a neonatal intensive care unit in Brazil. Patients with a gestational age < 28 weeks who had undergone at least one echocardiogram within the first two weeks of life and had PDA size > 1.5 or LA/AO ratio > 1.5 were included. Agglomerative hierarchical clustering on principal components was used to divide the data into different clusters based on common characteristics. Two distinct subphenotypes of preterm infants with hemodynamically significant PDA were identified: ""inflamed,"" characterized by high leukocyte, neutrophil, and neutrophil-to-lymphocyte ratio, and ""respiratory acidosis,"" characterized by low pH and high pCO(2) levels. Conclusions: This study suggests that there may be two distinct subphenotypes of preterm infants with hemodynamically significant PDA: ""inflamed"" and ""respiratory acidosis."" By dividing the population into different subgroups based on common characteristics, it is possible to get a more nuanced understanding of the effectiveness of PDA interventions. What is Known: Treatment of PDA in preterm infants has been controversial. Stratification of preterm infants with PDA into subgroups is important in order to determine the best treatment. What is New: Unsupervised machine learning was used to identify two subphenotypes of preterm infants with hemodynamically significant PDA. The 'inflamed' cluster was characterized by higher values of leukocyte, neutrophil, and neutrophil-to-lymphocyte ratio. The 'respiratory acidosis' cluster was characterized by lower pH values and higher pCO2 values.
Clinical Aspects and Etiologic Investigation of Pediatric Patients With Acute Liver Failure
(2023) LUGLIO, Michele; MARQUES, Tatiana de Carvalho Silva; PEREIRA, Maria Fernanda Badue; DELGADO, Artur Figueiredo; CARVALHO, Werther Brunow de; TANNURI, Ana Cristina Aoun; SANDY, Natascha Silva; LITVINOV, Nadia; PAULA, Camila Sanson Yoshino de; SANTOS, Ariane Guissi dos; LAZARI, Carolina dos Santos; GOUVEA, Michele Soares Gomes; PAULA, Anderson Vicente de; MENDOZA, Tania Regina Tozetto; TANIGAWAH, Ryan Yukimatsu; LIMAH, Fabiana Roberto; HIRAYAMAH, Andre Bubna; SANTOS, Isabela Gusson Galdino dos; PINHOG, Joao Renato Rebello; SABINOG, Ester Cerdeira; MENDES-CORREAHG, Maria Cassia; ALVESH, Venancio Avancini Ferreira; MARQUESC, Heloisa Helena de Sousa
A new outbreak of hepatitis of unknown origin raised awareness in the international community. A few reports have attempted to associate new cases with adenovirus infection and the immunologic effects of previous SARS-CoV-2 infections through a superantigen mechanism. Moreover, according to a case series, viral isolates were identified in 7 of 10 cases of pediatric patients with hepatitis of unknown origin and acute liver failure. Adenovirus was detected by respiratory secretion polymerase chain reaction in 2 patients, with neither presenting with SARS-CoV-2 acute infection. Clinical and laboratory descriptions and cross-referencing epidemiologic and pathophysiological data can help identify possible disease etiologies.
Clinical Presentation and Outcomes of Kawasaki Disease in Children from Latin America: A Multicenter Observational Study from the REKAMLATINA Network
(2023) NARAYAN, Hari K.; LIZCANO, Anel; LAM-HINE, Tracy; ULLOA-GUTIERREZ, Rolando; BAINTO, Emelia V.; GARRIDO-GARCIA, Luis M.; ESTRIPEAUT, Dora; AGUILA, Olguita del; GOMEZ, Virgen; FAUGIER-FUENTES, Enrique; MINO-LEON, Greta; BELTRAN, Sandra; COFRE, Fernanda; CHACON-CRUZ, Enrique; SALTIGERAL-SIMENTAL, Patricia; MARTINEZ-MEDINA, Lucila; DUENAS, Lourdes; LUCIANI, Kathia; RODRIGUEZ-QUIROZ, Francisco J.; MORENO, German Camacho; VIVIANI, Tamara; ALVAREZ-OLMOS, Martha I.; MARQUES, Heloisa Helena de Sousa; LOPEZ-MEDINA, Eduardo; PIREZ, Maria C.; TREMOULET, Adriana H.
Objectives To describe the clinical presentation, management, and outcomes of Kawasaki disease (KD) in Latin America and to evaluate early prognostic indicators of coronary artery aneurysm (CAA). Study design An observational KD registry-based study was conducted in 64 participating pediatric centers across 19 Latin American countries retrospectively between January 1, 2009, and December 31, 2013, and prospectively from June 1, 2014, to May 31, 2017. Demographic and initial clinical and laboratory data were collected. Logistic regression incorporating clinical factors and maximum coronary artery z-score at initial presentation (between 10 days before and 5 days after intravenous immunoglobulin [IVIG]) was used to develop a prognostic model for CAA during follow-up (>5 days after IVIG). Results Of 1853 patients with KD, delayed admission (>10 days after fever onset) occurred in 16%, 25% had incomplete KD, and 11% were resistant to IVIG. Among 671 subjects with reported coronary artery z-score during follow-up (median: 79 days; IQR: 36, 186), 21% had CAA, including 4% with giant aneurysms. A simple prognostic model utilizing only a maximum coronary artery z-score >= 2.5 at initial presentation was optimal to predict CAA during follow-up (area under the curve: 0.84; 95% CI: 0.80, 0.88). Conclusion From our Latin American population, coronary artery z-score >= 2.5 at initial presentation was the most important prognostic factor preceding CAA during follow-up. These results highlight the importance of early echocardiography during the initial presentation of KD. (J Pediatr 2023;263:113346).
A comprehensive model for assessing and classifying patients with thrombotic microangiopathy: the TMA-INSIGHT score
(2023) ADDAD, Vanessa Vilani; PALMA, Lilian Monteiro Pereira; VAISBICH, Maria Helena; BARBOSA, Abner Macola Pacheco; ROCHA, Naila Camila da; CARDOSO, Marilia Mastrocolla de Almeida; ALMEIDA, Juliana Tereza Coneglian de; SORDI, Monica A. P. de Paula de; MACHADO-RUGOLO, Juliana; ARANTES, Lucas Frederico; ANDRADE, Luis Gustavo Modelli de
Background Thrombotic Microangiopathy (TMA) is a syndrome characterized by the presence of anemia, thrombocytopenia and organ damage and has multiple etiologies. The primary aim is to develop an algorithm to classify TMA (TMA-INSIGHT score). Methods This was a single-center retrospective cohort study including hospitalized patients with TMA at a single center. We included all consecutive patients diagnosed with TMA between 2012 and 2021. TMA was defined based on the presence of anemia (hemoglobin level < 10 g/dL) and thrombocytopenia (platelet count < 150,000/mu L), signs of hemolysis, and organ damage. We classified patients in eight categories: infections; Malignant Hypertension; Transplant; Malignancy; Pregnancy; Thrombotic Thrombocytopenic Purpura (TTP); Shiga toxin-mediated hemolytic uremic syndrome (STEC-SHU) and Complement Mediated TMA (aHUS). We fitted a model to classify patients using clinical characteristics, biochemical exams, and mean arterial pressure at presentation. Results We retrospectively retrieved TMA phenotypes using automatic strategies in electronic health records in almost 10 years (n = 2407). Secondary TMA was found in 97.5% of the patients. Primary TMA was found in 2.47% of the patients (TTP and aHUS). The best model was LightGBM with accuracy of 0.979, and multiclass ROC-AUC of 0.966. The predictions had higher accuracy in most TMA classes, although the confidence was lower in aHUS and STEC-HUS cases. Conclusion Secondary conditions were the most common etiologies of TMA. We retrieved comorbidities, associated conditions, and mean arterial pressure to fit a model to predict TMA and define TMA phenotypic characteristics. This is the first multiclass model to predict TMA including primary and secondary conditions.
Global access to affordable CFTR modulator drugs: Time for action!
(2022) ZAMPOLI, Marco; KASHIRSKAYA, Nataliya; KARADAG, Bulent; SILVA FILHO, Luiz Vicente Ribeiro Ferreira da; PAUL, Grace R.; NOKE, Christine
Children and adolescents with non-Hodgkin lymphoma in Brazil: A national survey identifies the challenges encountered and documents the diversity of care
(2023) COSTA, Ana Rosa S.; COSTA, Juliana T.; BREVIGLIERI, Carla Nolasco Monteiro; MELGARES, Elia M. P.; GODINHO, Patrick R.; METZGER, Monika L.
Introduction: Brazil has many inequities in the healthcare provided nationwide. Therefore, in order to access challenges in treatment, available resources and current practices, to identify barriers in delivering a good quality of care among Brazilian centers treating children and adolescents with Non-Hodgkin Lymphoma (NHL) and to generate a future prospective guideline, a customized online survey was distributed to pediatric hematologists and oncologists across the country. Results: A total of 97 surveys were completed (35% response rate), from 47 cities in all Brazilian regions and 79 units of care, with a median of 1 answer by the center (range 1 - 5). Most respondents work at an institution supported exclusively by public/philanthropic resources (58%), with an average of 5 to 9 new cases/year (49%), and 41% have 4 to 6 oncologists/centers. Additionally, 22% have no easy access to the intensive care unit, 26% have no access to Rasburicase, 28% have no access to Rituximabe as front-line therapy and 41% have unreliable methotrexate monitoring levels. Those differences cannot be explained thoroughly by regional wealth variances, nor by the financing model. Regarding the pathology service, 70% consider having reasonable quality assistance, but the timeframe to deliver diagnosis is satisfactory to 46%. There is no uniform management of care, with the current guideline from the Sociedade Brasileira de Oncologia Pediatrica being adopted by 54 to 59%, depending on the NHL subtype. Conclusion: This study provides insights into the heterogeneity of care among Brazilian centers. Recognizing those diversities will support the design of effective strategies and collaboration nationwide. (C) 2021 Associacao Brasileira de Hematologia, Hemoterapia e Terapia Celular.
Visceral Leishmaniasis Revealing Undiagnosed Inborn Errors of Immunity
(2023) CARVALHO, Daniel Gleison; VASCONCELOS, Dewton de Moraes; SANTOS, Andreia Cristiane Rangel; LINDOSO, Jose Angelo Lauletta
Visceral Leishmaniasis (VL) is a potentially fatal disease and may be associated with primary or acquired immunodeficiencies. There are few reports, in the literature, of inborn errors of immunity. Here, we report two cases of VL as a marker of inborn errors of immunity, namely, GATA2 and RAB27A deficiency. Our data suggest that VL patients should be screened for primary immunodeficiency, particularly in cases of VL relapse.
Establishment and Comprehensive Molecular Characterization of an Immortalized Glioblastoma Cell Line from a Brazilian Patient
(2023) SILVA, Fernanda F. da; LUPINACCI, Fernanda C. S.; ELIAS, Bruno D. S.; BESERRA, Adriano O.; SANEMATSU, Paulo; ROFFE, Martin; KULIKOWSKI, Leslie D.; COSTA, Felipe D'almeida; SANTOS, Tiago G.; HAJJ, Glaucia N. M.
Glioblastoma (GBM) is the most common and aggressive primary brain tumor in adults, with few effective treatment strategies. The research on the development of new treatments is often constrained by the limitations of preclinical models, which fail to accurately replicate the disease's essential characteristics. Herein, we describe the obtention, molecular, and functional characterization of the GBM33 cell line. This cell line belongs to the GBM class according to the World Health Organization 2021 Classification of Central Nervous System Tumors, identified by methylation profiling. GBM33 expresses the astrocytic marker GFAP, as well as markers of neuronal origin commonly expressed in GBM cells, such as beta III-tubulin and neurofilament. Functional assays demonstrated an increased growth rate when compared to the U87 commercial cell line and a similar sensitivity to temozolamide. GBM33 cells retained response to serum starvation, with reduced growth and diminished activation of the Akt signaling pathway. Unlike LN-18 and LN-229 commercial cell lines, GBM33 is able to produce primary cilia upon serum starvation. In summary, the successful establishment and comprehensive characterization of this GBM cell line provide researchers with invaluable tools for studying GBM biology, identifying novel therapeutic targets, and evaluating the efficacy of potential treatments.
Stroke in vascular Ehlers-Danlos syndrome
(2023) MARTINS, Rebecca Ranzani; PAIVA, Mauricio Leonardo da Silva; TEIXEIRA, Weverton Carlos da Silva; KAWAHIRA, Rachel Sayuri Honjo; FREUA, Fernando; CASTRO, Matheus Augusto Araujo; KIM, Chong Ae; KOK, Fernando
Three-dimensional Ultrasound Evaluation of Lung Volume in Fetuses with Abdominal Wall Defect
(2023) NISHIE, Estela Naomi; OSMUNDO-JUNIOR, Gilmar de Souza; MOHAMED, Samirah Hosney Mahmoud; TANNURI, Ana Cristina Aoun; GIBELLI, Maria Augusta Bento Cicaroni; CARVALHO, Werther Brunow de; PERES, Stela Verzinhasse; FRANCISCO, Rossana Pulcineli Vieira; BRIZOT, Maria de Lourdes
Introduction: Abdominal wall defects (AWD) interfere with postnatal respiratory parameters. We aimed to evaluate lung volume (LV) in fetuses with AWD using three-dimensional (3D) ultrasound (US) and to correlate AWD with the type (omphalocele and gastroschisis) and size of the defect and neonatal morbidity and mortality.Methods: This prospective study included 72 pregnant women with fetuses with AWD and a gestational age <25 weeks. Abdominal volume, 3D US LV, and herniated volume were acquired every 4 weeks up to 33 weeks. LV was compared with normal reference curves and correlated with abdominal and herniated volumes.Results: Omphalocele (p<0.001) and gastroschisis (p<0.001) fetuses had smaller LV than did normal fetuses. LV was positively correlated with abdominal volume (omphalocele, r=0.86; gastroschisis, r=0.88), whereas LV was negatively correlated with omphalocele-herniated volume/abdominal volume (p<0.001, r= -0.51). LV was smaller in omphalocele fetuses that died (p=0.002), were intubated (p=0.02), or had secondary closure (p<0.001). In gastroschisis, a smaller LV was observed in fetuses discharged using oxygen (p=0.002).Discussion/Conclusion: Fetuses with AWD had smaller 3D LV than normal fetuses. Fetal abdominal volume was inversely correlated with LV. In omphalocele fetuses, a smaller LV was associated with neonatal mortality and morbidity.

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