Discrimination of acute lymphoblastic leukemia from systemic-onset juvenile idiopathic arthritis at disease onset

OBJECTIVE: To assess clinical and laboratory features that differentiate acute lymphoblastic leukemia from systemic juvenile idiopathic arthritis at disease onset. METHODS: Fifty-seven leukemia patients with musculoskeletal involvement, without blasts on peripheral blood and without glucocorticoid therapy at disease onset and 102 systemic juvenile idiopathic arthritis patients (International League of Associations for Rheumatology criteria) were retrospectively evaluated. The following features were examined: fever, rheumatoid rash, arthritis, limb pain, hepatomegaly, splenomegaly, pericarditis, myocarditis, pleuritis, weight loss, bleeding, anemia, leukopenia, neutropenia, thrombocytopenia, erythrocyte sedimentation rate, and lactic dehydrogenase levels. RESULTS: The median age at disease onset was significantly higher in leukemia patients than in those with systemic-onset juvenile idiopathic arthritis (5.8 vs. 3.8 years). In addition, the frequencies of limb pain, hepatomegaly, weight loss and hemorrhagic manifestations were significantly higher in leukemia patients than in systemic-onset juvenile idiopathic arthritis patients (70% vs. 1%, 54% vs. 32%, 30% vs. 8%, and 9% vs. 0%, respectively). Likewise, the frequencies of anemia, leukopenia, neutropenia, thrombocytopenia and high lactic dehydrogenase levels were statistically higher in leukemia patients than in patients with systemic-onset juvenile idiopathic arthritis (88% vs. 57%, 39% vs. 1%, 60% vs. 1%, 77% vs. 1%, and 56% vs. 14%, respectively). Remarkably, multivariate analysis revealed that limb pain (OR = 553; 95% CI =46.48-6580.42) and thrombocytopenia (OR = 754.13; 95% CI =64.57-8806.72) were significant independent variables that differentiated leukemia from systemic-onset juvenile idiopathic arthritis. The R2 of the Nagelkerke test was 0.91, and the Kaplan-Meier survival curves were similar for acute lymphoblastic leukemia patients with and without limb pain. CONCLUSION: Our study emphasizes the importance of investigating leukemia in patients presenting with musculoskeletal manifestations and, in particular, limb pain associated with thrombocytopenia

Circulating inflammatory mediators and organ dysfunction after cardiovascular surgery with cardiopulmonary bypass: a prospective observational study

INTRODUCTION: Cardiovascular surgery with cardiopulmonary bypass (CPB) has improved in past decades, but inflammatory activation in this setting is still unpredictable and is associated with several postoperative complications. Perioperative levels of macrophage migration inhibitory factor (MIF) and other inflammatory mediators could be implicated in adverse outcomes in cardiac surgery. METHODS: Serum levels of MIF, monocyte chemoattractant protein (MCP)-1, soluble CD40 ligand, IL-6 and IL-10 from 93 patients subjected to CPB were measured by enzyme-linked immunosorbent assay and compared with specific and global postoperative organ dysfunctions through multiple organ dysfunction score (MODS) and sequential organ failure assessment (SOFA). RESULTS: Most of the cytokines measured had a peak of production between 3 and 6 hours after CPB, but maximum levels of MIF occurred earlier, at the cessation of CPB. Among specific organ dysfunctions, the most frequent was hematological, occurring in 82% of the patients. Circulatory impairment was observed in 73.1% of the patients, and 51% of these needed inotropics or vasopressors within the first 24 hours after surgery. The third most frequent dysfunction was pulmonary, occurring in 48.4% of the patients. Preoperative levels of MIF showed a relevant direct correlation with the intensity of global organ dysfunction measured by SOFA (ρ = 0.46, p < 0.001) and MODS (ρ = 0.50, p < 0.001) on the third day after surgery. MCP-1 production was associated with postoperative thrombocytopenia, and MIF was related to the use of a high dose of vasopressors in patients with cardiovascular impairment and also to lower values of the ratio of partial arterial oxygen tension (PaO(2)) to fraction of inspired oxygen (FiO(2)) registered in the first 24 hours after CPB. CONCLUSION: Despite the multifactorial nature of specific or multiple organ dysfunctions, MIF should be explored as a predicting factor of organ dysfunction, or even as a potential therapeutic target in decreasing postoperative complications

Major discrepancy between clinical diagnosis of death and anatomopathological findings in adolescents with chronic diseases during 18-years

Objectives: To evaluate the inconsistency between clinical diagnosis of death and autopsy findings in adolescents with chronic diseases. Methods: A cross-sectional study including a sample of adolescents’ autopsies who died in a pediatric and adolescent tertiary hospital over&nbsp;18&nbsp;consecutive years. During this period, there were n&nbsp;=&nbsp;2912 deaths, and n&nbsp;=&nbsp;581/2912(20%) occurred in adolescents. Of these, n&nbsp;=&nbsp;85/581(15%) underwent autopsies and were analyzed. Further results were divided into two groups: Goldman classes&nbsp;I&nbsp;or&nbsp;II (high disagreement between main clinical diagnosis of death and anatomopathological findings, n&nbsp;=&nbsp;26) and Goldman classes&nbsp;III, IV or&nbsp;V (low or no disagreement between these two parameters, n&nbsp;=&nbsp;59). Results: Median age at death (13.5&nbsp;[10‒19] vs. 13&nbsp;[10‒19] years, p&nbsp;=&nbsp;0.495) and disease duration (22&nbsp;[0‒164]&nbsp;vs.&nbsp;20&nbsp;[0‒200] months, p&nbsp;=&nbsp;0.931), and frequencies for males (58%&nbsp;vs.&nbsp;44%, p&nbsp;=&nbsp;0.247) were similar between class I/II&nbsp;vs.&nbsp;class&nbsp;III/IV/V. The frequency of pneumonia (73%&nbsp;vs.&nbsp;48%, p&nbsp;=&nbsp;0.029), pulmonary abscess (12%&nbsp;vs.&nbsp;0%, p&nbsp;=&nbsp;0.026), as well as isolation of yeast (27%&nbsp;vs.&nbsp;5%, p&nbsp;=&nbsp;0.008), and virus (15%&nbsp;vs.&nbsp;2%, p&nbsp;=&nbsp;0.029) identified in the autopsy, were significantly higher in adolescents with Goldman class&nbsp;I/II compared to those with Goldman class&nbsp;III/IV/V. In contrast, cerebral edema was significantly lower in adolescents of the first group (4%&nbsp;vs.&nbsp;25%, p&nbsp;=&nbsp;0.018). Conclusion: This study showed that&nbsp;30% of the adolescents with chronic diseases had major discrepancies between clinical diagnosis of death and autopsy findings. Pneumonia, pulmonary abscess, as well as isolation of yeast and virus were more frequently identified at autopsy findings in the groups with major discrepancies

Emotional, hyperactivity and inattention problems in adolescents with immunocompromising chronic diseases during the COVID-19 pandemic

Objective: To assess factors associated with emotional changes and Hyperactivity/Inattention (HI) motivated by COVID-19 quarantine in adolescents with immunocompromising diseases. Methods: A cross-sectional study included&nbsp;343&nbsp;adolescents with immunocompromising diseases and 108&nbsp;healthy adolescents. Online questionnaires were answered including socio-demographic data and self-rated healthcare routine during COVID-19 quarantine and validated surveys: Strengths and Difficulties Questionnaire (SDQ), Pittsburgh Sleep Quality Index (PSQI), Pediatric Quality of Life Inventory 4.0 (PedsQL4.0). Results: The frequencies of abnormal emotional SDQ scores from adolescents with chronic diseases were similar to those of healthy subjects (110/343&nbsp;[32%] vs.&nbsp;38/108 [35%], p&nbsp;=&nbsp;0.548), as well as abnormal hyperactivity/inattention SDQ scores (79/343 [23%] vs.&nbsp;29/108 [27%], p&nbsp;=&nbsp;0.417). Logistic regression analysis of independent variables associated with abnormal emotional scores from adolescents with chronic diseases showed: female sex (Odds Ratio [OR&nbsp;=&nbsp;3.76]; 95%&nbsp;Confidence Interval (95%&nbsp;CI) 2.00‒7.05; p &lt; 0.001), poor sleep quality (OR&nbsp;=&nbsp;2.05; 95%&nbsp;CI&nbsp;1.08‒3.88; p&nbsp;=&nbsp;0.028) and intrafamilial violence during pandemic (OR&nbsp;=&nbsp;2.17; 95%&nbsp;CI&nbsp;1.12‒4.19; p&nbsp;=&nbsp;0.021) as independently associated with abnormal emotional scores, whereas total PedsQL score was inversely associated with abnormal emotional scores (OR&nbsp;=&nbsp;0.95; 95%&nbsp;CI&nbsp;0.93‒0.96; p &lt; 0.0001). Logistic regression analysis associated with abnormal HI scores from patients evidenced that total PedsQL score (OR&nbsp;=&nbsp;0.97; 95%&nbsp;CI&nbsp;0.95‒0.99; p&nbsp;=&nbsp;0.010], changes in medical appointments during the pandemic (OR&nbsp;=&nbsp;0.39; 95%&nbsp;CI&nbsp;0.19-0.79; p&nbsp;=&nbsp;0.021), and reliable COVID-19 information (OR&nbsp;=&nbsp;0.35; 95%&nbsp;CI&nbsp;0.16‒0.77; p&nbsp;=&nbsp;0.026) remained inversely associated with abnormal HI scores. Conclusion: The present study showed emotional and HI disturbances in adolescents with chronic immunosuppressive diseases during the COVID-19 pandemic. It reinforces the need to promptly implement a longitudinal program to protect the mental health of adolescents with and without chronic illnesses during future pandemics

Poor Sleep quality and health-related quality of life impact in adolescents with and without chronic immunosuppressive conditions during COVID-19 quarantine

OBJECTIVE: To assess the possible factors that influence sleep quality in adolescents with and without chronic immunosuppressive conditions quarantined during the coronavirus disease 2019 (COVID-19) pandemic. METHODS: This cross-sectional study included 305 adolescents with chronic immunocompromised conditions and 82 healthy adolescents. Online surveys were completed, which included questions on socio-demographic data and self-rated healthcare routine during COVID-19 quarantine and the following validated questionnaires: the Pittsburgh Sleep Quality Index (PSQI), Pediatric Quality of Life Inventory 4.0 (PedsQL4.0), and Pediatric Outcome Data Collection Instrument (PODCI). RESULTS: The median current age [14 (10-18)&nbsp;vs. 15 (10-18) years,&nbsp;p=0.847] and frequency of female sex (62%&nbsp;vs. 58%,&nbsp;p=0.571) were similar in adolescents with chronic conditions compared with healthy adolescents. The frequency of poor sleep quality was similar in both groups (38%&nbsp;vs. 48%,&nbsp;p=0.118). Logistic regression analysis, including both healthy adolescents and adolescents with chronic conditions (n=387), demonstrated that self-reported increase in screen time (odds ratio [OR] 3.0; 95% confidence interval [CI] 1.3-6.8;&nbsp;p=0.008) and intrafamilial violence report (OR 2.1; 95% CI 1.2-3.5;&nbsp;p=0.008) were independently associated with poor sleep quality in these adolescents. However, the PODCI global function score was associated with a lower OR for poor sleep quality (OR 0.97; 95% CI 0.94-0.99;&nbsp;p=0.001). Further logistic regression, including only adolescents with chronic conditions (n=305), demonstrated that self-reported increase in screen time (OR 3.1; 95% CI 1.4-6.8;&nbsp;p=0.006) and intrafamilial violence report (OR 2.0; 95% CI 1.2-3.4;&nbsp;p=0.011) remained independently associated with poor quality of sleep, whereas a lower PODCI global function score was associated with a lower OR for sleep quality (OR 0.96; 95% CI 0.94-0.98;&nbsp;p&lt;0.001). CONCLUSION: Self-reported increases in screen time and intrafamilial violence report impacted sleep quality in both healthy adolescents and those with chronic conditions. Decreased health-related quality of life was observed in adolescents with poor sleep quality

Brazilian multicenter study of 71 patients with juvenile-onset Takayasu's arteritis: clinical and angiographic features

Objective: To describe the clinical and angiographic characteristics of Takayasu's arteritis in Brazilian children and adolescents. Methods: A retrospective data collection was performed in 71 children and adolescents followed in 10 Brazilian reference centers in Pediatric Rheumatology. The evaluation was carried out in three different time points: from onset of symptoms to diagnosis, from the 6th to 12th month of diagnosis, and in the last visit. Results: Of 71 selected patients, 51 (71.8%) were girls. The mean age of onset of symptoms and of time to diagnosis was 9.2 (+/- 4.2) years and 1.2 (+/- 1.4) years, respectively. At the end of the study, 20 patients were in a state of disease activity, 39 in remission and 5 had evolved to death. The most common symptoms in baseline assessment, second evaluation, and final evaluation were, respectively: constitutional, musculoskeletal, and neurological symptoms. A decrease in peripheral pulses was the most frequent cardiovascular signal, and an increase in erythrocyte sedimentation rate was the most frequent laboratory finding in all three evaluation periods. The tuberculin test was positive in 41% of those tested. Stenosis was the most frequent angiographic lesion, abdominal artery was the most affected segment, and angiographic type IV the most frequent. Most (90%) participants were treated with glucocorticoids, 85.9% required another immunosuppressive drug, and 29.6% underwent angioplasty. Conclusion: This is the largest study on juvenile-onset Takayasu arteritis, and a high number of patients under the age of 10 years, with predominance of constitutional symptoms early in the disease, was observed. (C) 2016 Elsevier Editora Ltda. All rights reserved.Univ Fed Sao Paulo, Dept Pediat, Sect Pediat Rheumatol, Sao Paulo, SP, BrazilUniv Sao Paulo, Inst Crianca, Pediat Rheumatol Sect, Sao Paulo, SP, BrazilSanta Casa Misericordia Sao Paulo, Sao Paulo, SP, BrazilUniv Sao Paulo, Sch Med, Ribeirao Preto, SP, BrazilUniv Estado Rio de Janeiro, BR-20550011 Rio De Janeiro, RJ, BrazilUniv Fed Rio de Janeiro, Inst Pediat Martagao Gesteira, Rio De Janeiro, RJ, BrazilUniv Fed Rio de Janeiro, Rheumatol Unit, Rio De Janeiro, RJ, BrazilUniv Fed Pernambuco, Rheumatol Unit, Recife, PE, BrazilUniv Fed Bahia, Salvador, BA, BrazilHosp Pequeno Principe, Curitiba, Parana, BrazilUniv Fed Sao Paulo, Dept Pediat, Sect Pediat Rheumatol, Sao Paulo, SP, BrazilWeb of Scienc

Incidence and risk factors for acute gastroenteritis among pilgrims following the French way to Santiago de Compostela (Spain) in summer 2008.

[ES] Conocer la incidencia de gastroenteritis aguda en los peregrinos del Camino de Santiago, los factores de riesgo asociados y su caracterización microbiológica. Se diseñaron dos estudios simultáneos, uno transversal mediante encuestas autocumplimentadas de peregrinos llegados a Santiago y otro de casos y controles a los peregrinos en el camino. Se hizo un análisis multivariado mediante regresión logística. En el estudio transversal la densidad de incidencia fue de 23,5 episodios de gastroenteritis aguda por 1.000 peregrinos-día (intervalo de confianza del 95% [IC95%]: 18,9–29,4/103). En el estudio de casos y controles los factores de mayor riesgo fueron la edad <20 años (odds ratio [OR]=4,72; IC95%: 2,16–10,28), viajar en grupo (tres personas o más) (OR=1,49; IC95%: 0,98–2,28) y consumir agua no embotellada (OR=2,09; IC95%: 0,91–4,82). Norovirus fue el microorganismo aislado con más frecuencia (56%). Ser peregrino menor de 20 años, realizar el camino en grupo y consumir agua no embotellada se asocian con un mayor riesgo de presentar gastroenteritis aguda. [EN] To determine the incidence of acute gastroenteritis in pilgrims on St. James' Way, as well as associated risk factors and microbiological characteristics. Two studies were designed simultaneously: a cross-sectional study through self-completed questionnaires among pilgrims reaching Santiago, and a case-control study of pilgrims traveling along the Way. Multivariate analysis was performed using logistic regression. In the cross-sectional study, the incidence rate was 23.5 episodes of acute gastroenteritis/10³ pilgrims-day (95% CI: 18.9-2.4/10³. In the case-control study, the major risk factors were age <20 years (OR=4.72; 95% CI: 2.16-10.28), traveling in groups (three or more) (OR=1.49; 95% CI: 0.98-2.28), and drinking unbottled water (OR=2.09; 95% CI: 0.91-4.82). The most frequent etiologic agent was norovirus (56%). Age less than 20 years, traveling in groups and drinking unbottled water were important risk factors for acute gastroenteritis.Para desarrollar el trabajo de campo, el Centro Nacional de Epidemiología (Instituto de Salud Carlos III) financió el desplazamiento y las dietas de los miembros del PEAC, la Consellería de Sanidade de Galicia aportó el material técnico necesario y cedió un vehículo para los desplazamientos a lo largo del Camino, y S.A. de Xestion do Xacobeo facilitó el alojamiento de los investigadores de campo.S

Glucocorticoid: Major Factor for Reduced Immunogenicity of 2009 Influenza A (H1N1) Vaccine in Patients with Juvenile Autoimmune Rheumatic Disease

Objective. To assess the immunogenicity and safety of non-adjuvanted influenza A H1N1/2009 vaccine in patients with juvenile autoimmune rheumatic disease (ARD) and healthy controls, because data are limited to the adult rheumatologic population. Method's. A total of 237 patients with juvenile ARD [juvenile systemic lupus erythematosus (JSLE), juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM), juvenile scleroderma, and vasculitis] and 91 healthy controls were vaccinated. Serology for anti-H1N1 was performed by hemagglutination inhibition assay. Seroprotection rate, seroconversion rate, and factor-increase in geometric mean titer (GMT) were calculated. Adverse events were evaluated. Results. Age was comparable in patients and controls (14.8 +/- 3.0 vs 14.6 +/- 3.7 years, respectively; p = 0.47). Three weeks after immunization, seroprotection rate (81.4% vs 95.6%; p = 0.0007), seroconversion rate (74.3 vs 95.6%; p &lt; 0.0001), and the factor-increase in GMT (12.9 vs 20.3; p = 0.012) were significantly lower in patients with juvenile ARD versus controls. Subgroup analysis revealed reduced seroconversion rates in JSLE (p &lt; 0.0001), JIA (p = 0.008), JDM (p = 0.025), and vasculitis (p = 0.017). Seroprotection (p &lt; 0.0001) and GMT (p &lt; 0.0001) were decreased only in JSLE. Glucocorticoid use and lymphopenia were associated with lower seroconversion rates (60.4 vs 82.9%; p = 0.0001; and 55.6 vs 77.2%; p = 0.012). Multivariate logistic regression including diseases, lymphopenia, glucocorticoid, and immunosuppressants demonstrated that only glucocorticoid use (p = 0.012) remained significant. Conclusion. This is the largest study to demonstrate a reduced but adequate immune response to H1N1 vaccine in patients with juvenile ARD. It identified current glucocorticoid use as the major factor for decreased antibody production. The short-term safety results support its routine recommendation for patients with juvenile ARD. ClinicalTrials.gov; NCT01151644. (First Release Nov 15 2011; J Rheumatol 2012;39:167-73; doi:10.3899/jrheum.110721)Fundacao de Amparo a Pesquisa do Estado de Sao Paulo (FAPESP) [2010/10749-0]Conselho Nacional de Desenvolvimento Cientifico e Tecnologico (CNPQ) [300248/2008-3, 300665/2009-1, 301411/2009-3, 300559/2009-7]Federico FoundationButantan Foundatio

Art déco e indústria: Brasil, décadas de 1930 e 1940

Este artigo analisa as características assumidas pela arquitetura de tendência art déco em construções ligadas à indústria - moradias, igrejas, escolas, clubes, fábricas etc. - erguidas no Brasil nas décadas de 1930 e 1940, investigando o repertório formal utilizado em diferentes tipologias. Faz uma análise mais detalhada das construções criadas pela Companhia Industrial Fiação de Tecidos Goyanna, em Pernambuco, no período entre 1937 e o final da década de 1940. Trata-se de um conjunto notável pela unidade formal, vinculada à linguagem art déco, e pelo emprego de soluções inovadoras em termos de forma e de programa.The aim of this article is to analyze the characteristics of Art Deco tendencies in buildings related to the industry - as houses, churches, schools, clubs, plants etc - in Brazil during the 1930s and 1940s. It studies the formal repertory used in different types of construction and develops a more detailed analysis of a complex of constructions by the firm Companhia Industrial Fiação de Tecidos Goyanna, in the state of Pernambuco, built during the period between 1937 and the end of 1940s. The presented group of constructions is notable for the formal coherence, associated with the Art Deco language, and for the use of innovative program and form solutions