Congenital orbital teratoma: a rare case with intracranial extension.

INTRODUCTION: Teratoma is the most common congenital tumor, but the orbital location is rare. It is composed of tissues from ectoderm, mesoderm, and endoderm. CLINICAL PRESENTATION: Congenital orbital teratoma commonly presents as unilateral proptosis, with rapid growth, leading to exposure keratopathy. DIAGNOSIS: Prenatal ultrasound may detect the orbital mass, computed tomography (CT) scans, and magnetic resonance (MR) imaging are better in demonstrating multilocular cystic and solid mass, without bone erosion. Laboratory tests should include alfa-fetoprotein (AFP) and B-human chorionic gonadotropin (B-HCG), and histopathologically, it contains all three germ cell layers components. The management is surgical removal of the lesion, the mature teratoma has a benign behavior, and the immature has a poor prognostic. We describe a rare case of congenital orbital teratoma with intracranial extension of the lesion, in which was treated with orbital exenteration. After surgery, AFP levels decreased, the middle face displacement has improved and development milestones were appropriate.

Cognitive Performance and Diabetic Retinopathy: What Your Eyes Can Reveal About Your Brain.

BACKGROUND: Diabetic retinopathy (DR) is a chronic diabetes complication. People with Type 2 Diabetes Mellitus (T2DM) have two times the risk for dementia, suggesting it is a new chronic diabetes complication. OBJECTIVE: Evaluate the association of DR with cognitive performance in a T2DM population. METHODS: Cross-sectional study with 400 T2DM adults from whom socio-demographic, clinical, laboratory data were collected, and screening test for depression symptoms (Patient Health Questionaire- 9 (PHQ-9)), Mini-Mental State Examination (MMSE), Semantic Verbal Fluency Test, Trail Making Test A and B, Word Memory test were performed. All cognitive test scores were converted into Global Cognition z-Score (GCS(z)). The association between GCS(z) < 0 with DR was performed using a multivariate binary logistic regression model adjusted for age ≥ 65 years, school years ≤ 6 years, DM duration ≥ 10 years, depression symptoms score > 9 at PHQ-9, arterial hypertension, physical activity, diabetic retinopathy, macular edema, and cardiovascular disease. RESULTS: After exclusions, the 251 eligible patients were 56.6% female, with a mean age of 61.1 (±9.8) years, DM duration of 12.6 (±8.9) years, and 7.6 (±4.2) years of school education. DR prevalence was 46.5%. Multivariate Logistic Regression Model showed an association between DR and GCS(z) < 0, with odds ratio (CI95%) of 2.50 (1.18-5.34), adjusted for age, low education level, arterial hypertension and depression symptoms (OD and CI95% respectively: 5.46(2.42-12.34); 12.19 (5.62-26.46); 2.55 (0.88-7.39); 3.53 (1.55-8.07)). CONCLUSION: In this T2DM population, having DR increased the chance for worse cognitive performance even when adjusted for age, low education level, presence of arterial hypertension, and depression symptoms.

Risk factors for cognitive decline in type 2 diabetes mellitus patients in Brazil: a prospective observational study.

BACKGROUND: Type 2 Diabetes Mellitus (T2DM) patients are twice as likely to develop dementia. The study's goal was to evaluate cognitive performance and risk factors for cognitive decline in this population. METHODS: Prospective observational study was conducted with 400 T2DM adults, of whom, during routine baseline and follow-up appointments, had socio-demographic, clinical, and laboratory data collected, and underwent physical examination, screening for depression symptoms (Patient Health Questionaire-9-PHQ-9), and cognitive tests: Mini-Mental State Examination (MMSE), Semantic Verbal Fluency Test, Trail Making Test A/B, and Word Memory Tests. Each cognitive test score was converted to a z-score and its average resulted in a new variable called Global Cognitive z-Score [GCS(z)]. Averages of the cognitive test scores and GCS(z) at both moments were compared by the Student's T-Test for paired samples. Multivariate binary logistic regression models were built to assess the association of GCS(z) < zero with risk factors for cognitive decline at the baseline and follow-up. RESULTS: After exclusions, 251 patients were eligible, being 56.6% female, mean age of 61.1 (± 9.8) years, 12.6 (± 8.9) years of DM duration, and 7.6 (± 4.2) years of school education. Follow-up had 134 patients reevaluated and took place after a mean of 18.4(± 5.0) months. Eleven (14%) patients with a GCS(z) ≥ 0 at baseline turned into a GCS(z) < 0 at follow-up. There were no significant differences between the means of cognitive test scores and GCS(z) at the two evaluation moments. At the baseline, the multivariate logistic regression model identified five risk factors associated with GCS(z) < zero: age ≥ 65 years, schooling ≤ 6 years, arterial hypertension, depression symptoms, and diabetic retinopathy (DR), with odds ratio (OR) and 95% confidence interval (CI95%) respectively: 5.46 (2.42-12.34); 12.19 (5.62-26.46); 2.55 (0.88-7.39); 3.53 (1.55-8.07) e 2.50 (1.18-5.34). At follow-up, the risk factors for GCS(z) < zero were: schooling ≤ 6 years, DM duration ≥ 10 years, depression symptoms, arterial hypertension, and cardiovascular disease (CVD), OR and CI95% respectively: 10.15 (3.68-28.01); 2.68 (0.96-7.48); 4.92 (1.77-13.70); 7.21 (1.38-35.71) e 5.76 (1.93-17.18). CONCLUSIONS: Based on our results, cognitive evaluation and follow-up should be incorporated on the routine of T2DM patients, especially for those with advanced age, low education level, prolonged DM duration, arterial hypertension, depression symptoms, CVD, and DR.

Perfil epidemiológico da Covid-19 no Estado do Paraná / Epidemiological profile of Covid-19 in the State of Paraná

A pandemia de Covid-19 já causou mais de 990 mil óbitos pelo mundo. A doença, causada pelo coronavírus SARS-CoV-2, é um importante problema de saúde pública. Desde 12 de março até 28 de setembro de 2020, foram registrados 174.124 casos e 4.335 óbitos pela doença no Paraná. O estudo teve como objetivo traçar o perfil epidemiológico da Covid-19 no Paraná. Fez--se análise descritiva com dados do informe epidemiológico da Secretaria de Saúde do Estado. Até 28 de setembro de 2020, 1,5% da população paranaense havia sido contaminada e 72,6% dos pacientes já estavam recuperados. A média de idade dos casos e óbitos foram, respectivamente, de 39,8 e 68,6 anos. Dos confirmados, 91.676 (53%) eram do sexo feminino e dos óbitos, 2.599 (60%) do masculino. Dos 906 (0,5%) pacientes internados, 446 (49,2%) estavam em UTI. A taxa de letalidade era de 2,5%. É possível delinear o com-portamento da doença no Estado. (AU)

Covid-19 pandemic has already caused more than 990 thousand deaths worldwide. The coronavirus disease, due to SARS-CoV-2, is an important public health problem. From March 12 to September 28, 2020, Paraná has already registered 174.124 cases and 4.335 deaths. The aim of this study is to describe the epidemiological profile of Covid-19 in the state of Paraná. Data collection were informed by Health Secretary of Paraná. As of September 28, 1,5% of Paraná population had been infected and 72,6% had recovered. The mean age of confirmed cases and deaths were 39,8 and 68,6 years old, respectively. Among confirmed cases, 91.676 (53%) were female and among deaths, 2.599 (60%) were male. 906 (0,5%) were hospitalized, of which 446 (49,2%) were in ICU bed. Mortality rate was 2,5%. It is possible to determinate the disease's behavior in Paraná. (AU)

Schistosoma mansoni FES Tyrosine Kinase Involvement in the Mammalian Schistosomiasis Outcome and Miracidia Infection Capability in Biomphalaria glabrata.

Schistosomiasis is a neglected tropical disease (NTD) caused by helminthes from the Schistosoma genus. This NTD can cause systemic symptoms induced by the deposition of parasite eggs in the host liver, promoting severe complications. Functional studies to increase knowledge about parasite biology are required for the identification of new drug targets, because the treatment is solely based on praziquantel administration, a drug in which the mechanism of action is still unknown. Protein kinases are important for cellular adaptation and maintenance of many organisms homeostasis and, thus, are considered good drug targets for many pathologies. Accordingly, those proteins are also important for Schistosoma mansoni, as the parasite relies on specific environmental signals to develop into its different stages. However, the specific roles of protein kinases in S. mansoni biology are not well understood. This work aims at investigating the tyrosine-protein kinase FES (Feline Sarcoma) functions in the maintenance of S. mansoni life cycle, especially in the establishment of mammalian and invertebrate hosts' infection. In this regard, the verification of Smfes expression among S. mansoni stages showed that Smfes is more expressed in infective free-living stages: miracidia and cercariae. Schistosomula exposed to SmFES-dsRNA in vitro presented a reduction in movement and size and increased mortality. Mice infected with Smfes-knocked-down schistosomula exhibited a striking reduction in the area of liver granuloma and an increased rate of immature eggs in the intestine. Female adult worms recovered from mice presented a reduced size and changes in the ovary and vitellarium; and males exhibited damage in the gynecophoral canal. Subsequently, miracidia hatched from eggs exposed to SmFES-dsRNA presented changes in its capability to infect and to sense the snail mucus. In addition, the SmFES RNAi effect was stable from miracidia to cercariae. The establishment of infection with those cercariae reproduced the same alterations observed for the knocked-down schistosomula infection. Our findings show that SmFES tyrosine kinase (1) is important in schistosomula development and survival; (2) has a role in adult worms pairing and, consequently, female maturation; (3) might be essential for egg antigen expression, thus responsible for inducing granuloma formation and immunomodulation; and (4) is essential for miracidia infection capability. In addition, this is the first time that a gene is kept knocked down during three different S. mansoni life stages and that a tyrosine kinase is implicated in the parasite reproduction and infection establishment in the mammalian host. Accordingly, SmFES should be explored as an alternative to support schistosomiasis treatment and morbidity control.

Kounis syndrome. Apropos of a clinical case. / Síndrome de Kounis. A propósito de um caso clínico.

Kounis syndrome, while an acute coronary syndrome, occurs in the context of a hypersensitivity reaction, allergies, or anaphylaxis and is subdivided into three types: coronary spasm in normal arteries, instability of plaques in atherosclerotic coronary arteries, and thrombosis of coronary stents. Herein, the case of a 73-year-old patient who, after administration of amoxicillin/clavulanic acid, went into cardiorespiratory arrest with evidence of ST-T segment elevation on electrocardiogram is reported. Coronarography revealed no obstructive lesions, and spontaneous resolution of electrocardiographic abnormalities was observed. A review of anamnesis with the family revealed a previous allergy to penicillin. The tryptase dosage was strongly positive. Kounis syndrome type 2 was diagnosed, and the clinical outcome was good.

Síndrome de Kounis. A propósito de um caso clínico / Kounis syndrome. Apropos of a clinical case

RESUMO A síndrome de Kounis, enquanto síndrome coronária aguda, ocorre em um contexto de reação de hipersensibilidade, alergia ou anafilaxia, e subdivide-se em três tipos: o espasmo coronário em artérias normais, a instabilidade de placas em artérias coronárias ateroscleróticas e a trombose de stent coronário. Apresenta-se o caso de uma doente de 73 anos que, após administração de amoxicilina/ácido clavulânico, entra em parada cardiorrespiratória, com evidência de supradesnivelamento do segmento ST-T em eletrocardiograma. Realiza coronariografia com ausência de lesões obstrutivas, verificando-se resolução espontânea das alterações eletrocardiográficas. Revisão da anamnese com a família documenta alergia prévia à penicilina. O doseamento de triptase foi fortemente positivo. Foi admitida provável síndrome de Kounis tipo 2, com boa evolução clínica posterior.

ABSTRACT Kounis syndrome, while an acute coronary syndrome, occurs in the context of a hypersensitivity reaction, allergies, or anaphylaxis and is subdivided into three types: coronary spasm in normal arteries, instability of plaques in atherosclerotic coronary arteries, and thrombosis of coronary stents. Herein, the case of a 73-year-old patient who, after administration of amoxicillin/clavulanic acid, went into cardiorespiratory arrest with evidence of ST-T segment elevation on electrocardiogram is reported. Coronarography revealed no obstructive lesions, and spontaneous resolution of electrocardiographic abnormalities was observed. A review of anamnesis with the family revealed a previous allergy to penicillin. The tryptase dosage was strongly positive. Kounis syndrome type 2 was diagnosed, and the clinical outcome was good.

Avaliação da terceiro ventriculostomia endoscópica em lactentes: uma revisão sistemática da literatura e metanálise / Evaluation of endoscopic third ventriculostomy in infants: a systematic literature review and meta-analysis

Introdução: A hidrocefalia é um distúrbio da fisiologia do líquido cefalorraquidiano (LCR), usualmente associada ao aumento a pressão intracraniana (PIC) e expansão dos ventrículos cerebrais. Representa uma das condições mais comuns em neurocirurgia pediátrica. A terceiro ventriculostomia endoscópica (TVE) é uma opção de tratamento à hidrocefalia que visa eliminar a dependência do paciente às derivações ventrículo peritoneais. Embora a baixa idade seja associada à falha da TVE, ainda não existe consenso sobre a realização da TVE em crianças abaixo de 24 meses. Objetivos: Mensurar a taxa de falha da TVE em crianças até 24 meses; medir a associação entre menor idade, diferentes etiologias da hidrocefalia e a falha da TVE. Metodologia: Seguindo a metodologia do PRISMA-P foram identificados estudos nas bases com os descritores relacionados à TVE, falha e lactentes, sem limite de data. Várias etapas foram realizadas por dois pesquisadores de forma cega e independente. Nas leituras de resumo e texto completo aplicou-se um formulário de elegibilidade com critérios elaborados para esta RSL. Dois instrumentos - Escala de Newcastle-Ottawa e Formulário de análise de qualidade - viabilizaram a análise qualitativa que permitiu a inclusão e a extração de dados. Os testes Q e I2 avaliaram a heterogeneidade e o modelo de efeito randômico foi utilizado para o cálculo das estimativas metanalíticas. Os programas OpenMeta [Analyst] e Review Mananger versão 5.4 foram utilizados. Resultados: Foram identificados 440 estudos nas bases Pubmed, Embase e Lilacs e 1 por referência de artigo. Foram retirados 134 títulos duplicados, por não serem elegíveis 118 na leitura de resumos e 155 na leitura de textos completos. Dos 34 estudos analisados 8 foram excluídos pela análise de qualidade e após a retirada de uma coorte repetida foram incluídos 25 artigos. A taxa de falha da TVE variou de 14,6 a 77,8%. Não foi possível calcular uma estimativa sumária da taxa de falha dos estudos em função da alta heterogeneidade (I²: 82,2). Optou-se então pela metanálise em subgrupos. Não foi detectada heterogeneidade (I²=0, p=0,56) portanto, a medida metanalítica para a falha de acordo com a idade, comparando lactentes menores de 6 meses com lactentes de 6 a 24 meses foi RR=1,73 IC95%[1,22-2,45]. Ao considerar estenose de aqueduto a categoria de base para análise de outros subgrupos o RR metanalítico com significância estatística foi obtido para infecção 2,07 IC95%[1,13-3,79], para hemorragia intraventricular: 2,27 IC95%[1,40-3,68], para mielomeningocele : 2,13, IC95%[1,01-4,48]. Apesar da ausência de heterogeneidade, o RR para lesão de fossa posterior foi de 1,31 IC95%:[0,69-2,40] incluiu o valor nulo e não foi significativo (p= 0,41). A taxa de falha da repetição de TVE variou de 0-90%, as etiologias foram estenose de aqueduto, infecção, hemorragia e outros. Os principais achados na repetição do procedimento foram membrana adicional e estoma fechado. Discussão e Conclusões: A medida metanalítica sumária para taxa de falha não foi obtida devido à grande heterogeneidade dos estudos. Foram feitas tentativas de agrupamento por tamanho da casuística e por data de realização que poderiam sugerir maior domínio da técnica mas a heterogeneidade se manteve. As principais complicações relatadas foram fístula liquórica e hemorragia intraoperatória, relacionando-se com a falha da cirurgia. Não foi possível avaliar os lactentes que foram submetidos à TVE em associação com reservatório de LCR. Os resultados desta RSL e metanálise confirmam que a idade menor de 6 meses é um fator prognóstico para a falha da TVE e demonstram o valor prognóstico de outras etiologias da hidrocefalia como a infecção, a hemorragia intraventricular e a mielomeningocele onde o risco para a falha da TVE foi 2 vezes maior.

Introduction: Hydrocephalus is a disorder of the cerebrospinal fluid (CSF) physiology, usually associated with increased intracranial pressure (ICP) and expansion of the cerebral ventricles. It represents one of the most common conditions treated in pediatric neurosurgery. Third endoscopic ventriculostomy (ETV) is a treatment option for hydrocephalus that aims to eliminate the patient's dependence on shunt. Young age is associated with surgery failure, and it is not clear whether or not ETV should be performed in children under 24 months old. Objectives: Measure the failure rate of TVE in children under 24 months old; measure is a relation between failure and younger age and hydrocephalus etiology. Methodology: This review was developed observing the parameters of the PRISMA-P guidelines. Systematic search using PubMed Embase and Lilacs using respective entry terms for each mesh terms ETV, failure and infants was carried out. the articles were chosen by reading abstracts, full text, applying the eligibility form and quality analysis - newcastle scale and quality form. The data were analyzed in OpenMeta[Analyst] and Review Mananger version 5.4, The Q and I² tests and random effect model were used to derive the meta-analytic estimates. Results: 440 studies were identified in Pubmed, Embase and Lilacs databases and 1 by article reference. 134 duplicates were removed, as 118 were not eligible when reading abstracts and 155 when reading full text. Of the 34 studies analyzed, 8 were excluded by the quality analysis and, after the removal of a repeated cohort e cohort, 25 articles were included. The failure rate of TVE ranged from 14.6 to 77.8%. It was not possible to calculate a summary estimate of the failure rate of the studies due to the high heterogeneity (I²: 82.2). Subgroup analysis was chosen The RR for age 1.73 CI95%:[1.22-2.45]; the RR for etiology, comparing each onde with aqueduct stenosis, were: infection 2.07 CI95%[1.13-3.79], intraventricular hemorrhage RR: 2.27 CI95%[1.40-3.68], RR for spinal dysraphism: 2.13 CI95%[1.01-4.48]. The RR for posterior fossa-space occupying lesions was 1.31 IC95%:[0.69-2.40], and not statistically significant. The failure rate for re-do ETV ranged from 0- to 90%, the re-do ETV etiologies were aqueduct stenosis, intraventricular infection, hemorrhage and others. The main intraoperative findings were extra membrane and closed stoma. Discussion and Conclusions: Due to the great heterogeneity of the sample, it was not possible to calculate the failure rate of infants. The most reported complications were CSF fistula and hemorrhage, related to the failure of the surgery. It was not possible to evaluate infants who underwent ETV in association with a CSF reservoir. The results of this systematic review and meta-analysis confirm that the age untill 6 months old is a prognostic factor for the failure of ETV and demonstrate the prognostic value of other aetiologies of hydrocephalus such as infection, intraventricular hemorrhage and myelomeningocele, in which the risk for ETV failure was 2 times higher.

Human leptospirosis: seroreactivity and genetic susceptibility in the population of São Miguel Island (Azores, Portugal).

BACKGROUND: Leptospirosis is a worldwide zoonotic and recognized neglected infectious disease. It has been observed that only a proportion of individuals exposed to pathogenic species of Leptospira become infected and develop clinically evident disease. Moreover, little information is available in subsequent reinfections. In the present study, we determine if a first infection with leptospirosis protects against subsequent reinfection, and investigate which of the host genetic factors are involved in the susceptibility and resistance to leptospirosis. METHODOLOGY AND FINDINGS: We conducted, in 2011, a retrospective hospital-based case-control study in the São Miguel Island population (Azores archipelago). In order to determine the seropositivity against pathogenic Leptospira after the first episode of leptospirosis, we performed a serological evaluation in 97 unrelated participants diagnosed with leptospirosis between 1992 and 2011. The results revealed that 46.4% of the 97 participants have circulating anti-Leptospira antibodies, and from these participants 35.6% maintained the seroprevalence for the same serogroup. Moreover, three of them were reinfected with unrelated Leptospira serovars. The genetic study was carried out by adding a control group composed of 470 unrelated healthy blood donors, also from São Miguel Island. Twenty five SNPs among twelve innate immune genes - IL1α, IL1ß, IL6, IL10, IL12RB1, TLR2, TLR4, TLR9, CD14, CISH, LTA and TNF - were genotyped, as well as HLA class I (-A and -B) genes. Association analysis indicates that genotypes -511GG (OR=1.6, 95%CI 1.01-2.56, p=0.04) in IL1ß, +1196CG (OR=2.0, 95%CI 1.26-3.27, p=0.003) in IL12RB1, -292TA (OR=1.8, 95% CI 1.06-2.1, p=0.03) and +3415CG (OR=1.8, 95% CI 1.08-3.08, p=0.02), both in CISH confer susceptibility to pathogenic Leptospira. CONCLUSION: The present study suggests some degree of long-term protection against leptospires with an attenuation of symptoms in case of reinfection. Moreover, our data supports the genetic influence of IL1ß, IL12RB1 and CISH genes and the susceptibility to leptospirosis infection.

First isolation of human Leptospira strains, Azores, Portugal.

OBJECTIVES: The aim of this study was the first identification of Leptospira isolates from Azorean inpatients. METHODS: Whole blood samples from 68 inpatients attending the São Miguel Hospital between 2006 and 2008, with a clinical and epidemiological suspicion of leptospirosis, were inoculated in a transport medium broth at the patient's bedside and further processed using a serial dilution technique prior to culture. At admission, 62 (91%) patients were also analyzed for the presence of leptospiral DNA by a nested PCR and 40 (59%) for specific agglutinins by microscopic agglutination test (MAT). The isolates obtained were first assigned at the serogroup level by both MAT reactivity with hyperimmune rabbit antisera and a PCR-based assay with the single primer iRep1. The species identification was performed by DNA sequencing. The use of monoclonal antibodies allowed intraspecific discrimination at the serovar level. RESULTS: Of the 10 (14.7%) human Leptospira isolates, seven were identified as Leptospira interrogans serovar Copenhageni and three as Leptospira borgpetersenii serovar Arborea, which is in agreement with previous data from the Azorean rodent population. CONCLUSIONS: This study represents a great step towards the definitive identification of the pathogenic leptospires in Azorean patients and confirms the bacteriological human-rodent connection for the first time.