Survival Probability in Patients with Sickle Cell Anemia Using the Competitive Risk Statistical Model.

The clinical picture of patients with sickle cell anemia (SCA) is associated with several complications some of which could be fatal. The objective of this study is to analyze the causes of death and the effect of sex and age on survival of Brazilian patients with SCA. Data of patients with SCA who were seen and followed at HEMORIO for 15 years were retrospectively collected and analyzed. Statistical modeling was performed using survival analysis in the presence of competing risks estimating the covariate effects on a sub-distribution hazard function. Eight models were implemented, one for each cause of death. The cause-specific cumulative incidence function was also estimated. Males were most vulnerable for death from chronic organ damage (p = 0.0005) while females were most vulnerable for infection (p=0.03). Age was significantly associated (p ≤ 0.05) with death due to acute chest syndrome (ACS), infection, and death during crisis. The lower survival was related to death from infection, followed by death due to ACS. The independent variables age and sex were significantly associated with ACS, infection, chronic organ damage and death during crisis. These data could help Brazilian authorities strengthen public policies to protect this vulnerable population

On the stability of solutions of semilinear elliptic equations with Robin boundary conditions on Riemannian manifolds

We investigate existence and nonexistence of stationary stable nonconstant solutions, i.e. patterns, of semilinear parabolic problems in bounded domains of Riemannian manifolds satisfying Robin boundary conditions. These problems arise in several models in applications, in particular in Mathematical Biology. We point out the role both of the nonlinearity and of geometric objects such as the Ricci curvature of the manifold, the second fundamental form of the boundary of the domain and its mean curvature. Special attention is devoted to surfaces of revolution and to spherically symmetric manifolds, where we prove refined results

EEG-based grading of Immune Effector Cell-Associated Neurotoxicity Syndrome

CAR-T cell therapy is an effective cancer therapy for multiple refractory/relapsed hematologic malignancies but is associated with substantial toxicity, including Immune Effector Cell Associated Neurotoxicity Syndrome (ICANS). Improved detection and assessment of ICANS could improve management and allow greater utilization of CAR-T cell therapy, however, an objective, specific biomarker has not been identified. We hypothesized that the severity of ICANS can be quantified based on patterns of abnormal brain activity seen in electroencephalography (EEG) signals. We conducted a retrospective observational study of 120 CAR-T cell therapy patients who had received EEG monitoring. We determined a daily ICANS grade for each patient through chart review. We used visually assessed EEG features and machine learning techniques to develop the Visual EEG-Immune Effector Cell Associated Neurotoxicity Syndrome (VE-ICANS) score and assessed the association between VE-ICANS and ICANS. We also used it to determine the significance and relative importance of the EEG features. We developed the Visual EEG-ICANS (VE-ICANS) grading scale, a grading scale with a physiological basis that has a strong correlation to ICANS severity (R = 0.58 [0.47-0.66]) and excellent discrimination measured via area under the receiver operator curve (AUC = 0.91 for ICANS ≥ 2). This scale shows promise as a biomarker for ICANS which could help to improve clinical care through greater accuracy in assessing ICANS severity

Mortality in children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil.

OBJECTIVE: To determine the mortality rate of children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil. METHODS: The number of deaths, the mortality rate and the causes of deaths in patients with sickle cell anemia who were treated and followed up at our institution for 15 years were determined and compared to data available for the Brazilian population. RESULTS: The overall number of deaths was 281 patients with a mortality rate of 16.77%. Survival probability was significantly higher in females. The number of deaths and the mortality rate were age-specific with a significant increase in the 19- to 29-year-old age group. The remaining life expectancy of the patients with sickle cell anemia was less than that of Brazilians at large. The gap between the two was about 20 years for ages between one and five years with this gap decreasing to ten years after the age of 65 years. The most common causes of death were infection, acute chest syndrome, overt stroke, organ damage and sudden death during painful crises. CONCLUSION: To the best of our knowledge, this is the first Brazilian study in a single institution in Rio de Janeiro; the mortality rate was 18.87% among adult patients with sickle cell anemia. The mortality rates in children and adults are higher than those reported in developed countries of the northern hemisphere

SYNTHESIS OF Au-NANOPARTICLES USING GOLD RECOVERED FROM ELECTRONIC WASTE

This work was supported by the Araucária Foundation, a Research Founding Agency from Paraná State – Brazil, CAPES and CNPq

Clouds over IMD? Perspectives for inherited metabolic diseases in adults from a retrospective cohort study in two Swiss adult metabolic clinics.

Inherited metabolic diseases (IMD) are complex medical conditions. Thanks to improvements in diagnosis and treatment, a growing number of pediatric IMD patients reach adulthood. Thus, clinical care of adults with IMD has emerged as a new and challenging reality. This purpose of this study of adults with IMD in an adult metabolic clinic at two academic hospitals (Lausanne and Geneva) was to help inform decisions on the future organization of health care for this group of patients. All adult patients with a biochemical and/or genetic diagnosis of IMD followed at the clinics were included in the study. Electronic patient records were reviewed for clinical features, diagnostic studies, treatment and long-term outcome. Data of undiagnosed patients referred for suspected IMD were analyzed separately. 126 patients were included in the study. The most prevalent group of diseases was small molecules disorders with 82 (65%) patients, followed by energy defects disorders with 29 (23%) patients and complex molecules disorders with 15 (12%) patients. Overall, 64% of patients were diagnosed before, and 36% after the age 16 years. Among the 126 cases, 51% suffered from medical complications. 79% of the patients were receiving a specific treatment for their disease. Among the 138 undiagnosed patients referred for suspicion of IMD, investigations lead to a genetic diagnosis in 24 (17%) patients. 19 had confirmation of an IMD, 5 were found to have another genetic condition. This retrospective study reveals significant features of adult IMD cohort. The disorders are heterogeneous, and there is no one-size-fits-all approach - treatment must be tailored to fit each specific disorder in each individual patient. Even patients who are followed at the dedicated clinic are not protected from metabolic decompensations and/or chronic organ-specific complications. While it is commonly assumed that patients with IMD are more stable once they become adults, our data show that the diseases continue to exact a lifelong toll. A coordinated monitoring of target organs by a multidisciplinary team is needed. To ensure that the success in diagnosis and treatment of individuals with IMD is sustained, there is a clear requirement for adequately staffed adult IMD clinics

Evaluation of chickenpox susceptibility in children with chronic renal failure

OBJETIVO: Avaliar a suscetibilidade natural à varicela de crianças e adolescentes portadores de insuficiência renal crônica (IRC). MÉTODOS: Estudo transversal de 83 pacientes com idade acima de 18 meses e inferior a 18 anos, durante 2000 e 2001, com ritmo de filtração glomerular (RFG) abaixo de 70mL/min/1,73m², portando cartão vacinal preconizado pela Fundação Nacional de Saúde e que não receberam nenhuma dose da vacina específica. Do total, três pacientes (3/83) foram excluídos, por terem recebido doses da vacina por meio de órgãos não governamentais. A sorologia foi realizada pelo método Enzyme-Linked Immuno Sorbent Assay, considerando-se títulos sorológicos protetores acima de 100mUA/mL. RESULTADOS: Os pacientes renais crônicos tinham idade mediana de 11 anos, 66% eram masculinos, 60% procedentes do próprio município de São Paulo, com RFG médio de 33,6mL/min/1,73m². O diagnóstico clínico de varicela por profissional médico ocorreu em 39 pacientes; destes, 10% se mostraram soronegativos. Dos 80 pacientes restantes, 21 (26%) apresentaram títulos não protetores para varicela. A prevalência de suscetibilidade em menores de seis anos foi 7,93 (IC95%=3,29-19,12) vezes superior à de maiores de seis anos. CONCLUSÕES: Houve diminuição da suscetibilidade à varicela com a idade. Pacientes abaixo de seis anos foram cerca de oito vezes mais suscetíveis à varicela que os renais crônicos com idade mais avançada e duas vezes mais suscetíveis do que a população pediátrica brasileira de mesma idade.OBJECTIVE: To evaluate the immune response to chickenpox natural infection in pediatric patients with chronic renal insufficiency. METHODS: This cross-sectional study enrolled 83 patients between 18 months and 18 years old, with glomerular filtration rate below 70mL/min/1.73m², during the years 2000 and 2001, who did not received specific immunization according to official documentation. Three patients (3/83) had been previously vaccinated by non-governmental agencies and were excluded. Varicella antibodies were evaluated by Enzyme-Linked Immuno Sorbent Assay and antibody titers above 100mUA/mL were considered protective. RESULTS: The median age was 11 years old, 66% were male, 60% lived in the city of São Paulo, with an average glomerular filtration rate of 33.6mL/min/1.73m². Clinical diagnosis of chickenpox was done by pediatricians in 39 patients and four of these 39 were seronegative for varicella. Susceptibility to chickenpox was found in 21/80 (26%) patients and the relative prevalence of susceptibility to the disease was 7.93 (95%CI=3.29-19.12) higher in patients under six years of age. CONCLUSIONS: Susceptibility to varicella infection decreased with age. Patients younger than six years were eight times more susceptible than older patients and twice more susceptible compared to the Brazilian pediatric population of the same age group

Gauge Invariant Effective Potential for Abelian Maxwell-Chern-Simons Systems

We investigate the effective potential for Abelian Maxwell--Chern--Simons systems. The calculations follow an alternate approach, recently proposed as a gauge invariant formulation of the effective potential, constructed in terms of a gauge invariant order parameter. We compare the results with another investigation, obtained within a standard route of calculating the effective potential.Comment: 10 pages. Revtex. To appear in Phys. Rev.