Extramedullary Imaging Manifestations of Leukemia/Lymphoblastic Lymphoma in Pediatrics: A Case Series.

Leukemia is the most common childhood malignancy and often presents with nonspecific symptoms. Occasionally, it presents with extramedullary manifestations, which have been more frequent in cases of myeloid lineage or T cells. However, precursor B-cell leukemia/lymphoblastic lymphoma can also have extramedullary manifestations in some patients. Describing certain clinical features along with diagnostic imaging can establish a presentation pattern and suggest a diagnosis in the pediatric population. Herein, we present a series of four patients with extramedullary manifestations of B-cell lymphoblastic leukemia, describing their clinical imaging and histopathological characteristics.

Clinical Profile of SARS-CoV-2 Infection: Mechanisms of the Cellular Immune Response and Immunogenetic Markers in Patients from Brazil.

OBJECTIVES: The aim of this study is to evaluate some mechanisms of the immune response of people infected with SARS-CoV-2 in both acute infection and early and late convalescence phases. METHODS: This is a cohort study of 70 cases of COVID-19, confirmed by RT-PCR, followed up to 60 days. Plasma Samples and clinical data were. Viral load, blood count, indicators inflammation were the parameters evaluated. Cellular immune response was evaluated by flow cytometry and Luminex immunoassays. RESULTS: In the severe group, hypertension was the only reported comorbidity. Non severe patients have activated memory naive CD4+ T cells. Critically ill patients have central memory CD4+ T cell activation. Severe COVID-19 patients have both central memory and activated effector CD8+ T cells. Non-severe COVID-19 cases showed an increase in IL1ß, IL-6, IL-10 and TNF and severely ill patients had higher levels of the cytokines IL-6, IL-10 and CXCL8. CONCLUSIONS: The present work showed that different cellular responses are observed according to the COVID-19 severity in patients from Brazil an epicenter the pandemic in South America. Also, we notice that some cytokines can be used as predictive markers for the disease outcome, possibility implementation of strategies effective by health managers.

Hemocromatosis: actualización clínica y diagnóstica / Hemochromatosis: clinical and diagnostic update

La hemocromatosis es un desorden en el cual la sobrecarga progresiva de hierro puede llevar a complicaciones sistémicas con gran morbimortalidad. Es una entidad clinicopatológica, con múltiples genes comprometidos y una fisiopatología común, con una expresión clínica y fenotípica variable, que depende de múltiples factores, tanto individuales como ambientales. Para su diagnóstico y seguimiento adecuado es necesario tener en cuenta elementos clínicos, bioquímicos y moleculares. En esta revisión, se presentan las generalidades de la hemocromatosis, además de sus mecanismos fisiopatológicos y moleculares, teniendo en cuenta su valor para el diagnóstico de la enfermedad. Adicionalmente, se describe la clasificación y un algoritmo diagnóstico propuestos recientemente por grupos de trabajo de expertos, así como las opciones de manejo y seguimiento de los pacientes con hemocromatosis

Hemochromatosis is a disorder in which progressive iron overload may lead to systemic complications with potential morbidity and mortality. It is a clinicopathologic entity that involves multiple genes and common pathophysiology, and has a variable clinical and phenotypic expression that depends on several individual and environmental factors. To make the diagnosis and perform a proper follow-up, clinical, biochemical, and molecular elements must be considered. This review aims to present the general characteristics of hemochromatosis, its molecular and pathophysiologic mechanisms, and their significance in the diagnosis of this disorder. In addition, a new classification and a proposed diagnostic algorithm by an expert working group are described, as well as management and follow-up options for patients with hemochromatosis

Linfoma de Hodgkin clásico: diferentes caras, una misma entidad / Classic Hodgkin lymphoma: different faces, same entity

El linfoma de Hodgkin clásico es una neoplasia linfoide maligna derivada de las células B del centro germinal, que corresponde aproximadamente al 85 % de los casos de linfoma de Hodgkin. Esta entidad afecta principalmente a pacientes jóvenes, y cuenta con un excelente pronóstico gracias a los avances en los métodos diagnósticos para su estadificación y tratamiento. Su enfoque diagnóstico correcto y completo requiere de una historia clínica exhaustiva y una biopsia de ganglio linfático adecuada para el análisis e identificación de los hallazgos histopatológicos e inmunohistoquímicos característicos, ya que a diferencia de otros linfomas donde las células neoplásicas son una población importante o dominante, las células de Hodgkin y Reed-Sternberg generalmente representan menos del 10 % de la lesión tumoral. Aunque todavía falta mucho por entender sobre la naturaleza biológica de este linfoma y sus diferentes subtipos, en los últimos años se ha avanzado considerablemente en la comprensión de su linfomagénesis, especialmente cuando está relacionada con la infección por el virus de Epstein-Barr. Su alta heterogeneidad y posible superposición morfológica, obligan a continuar su estudio para poder identificarlo, al igual que a sus posibles diagnósticos diferenciales en aquellos casos donde se presente con una variante o patrón infrecuente. Este artículo pretende ofrecer una descripción integral resumida y actualizada sobre la fisiopatología, la clínica, el diagnóstico histopatológico con énfasis en aquellos patrones raros que podrían llegar a ser factores distractores y de confusión, y el pronóstico del linfoma de Hodgkin clásico, buscando lograr una mejor comprensión de la enfermedad

Classic Hodgkin lymphoma is a malignant lymphoid neoplasm derived from B cells in the germinal center, and accounts for approximately 85% of all Hodgkin lymphoma cases. This disease mainly affects young patients and has an excellent prognosis due to advances in diagnostic methods for staging and treatment. A correct and complete diagnostic approach requires a thorough clinical history and an adequate lymph node biopsy for the analysis and identification of characteristic histopathological and immunohistochemical findings. Unlike other lymphomas where neoplastic cells are an important or dominant population, Reed-Sternberg/ Hodgkin cells generally represent less than 10% of the tumor lesion. Although much remains to be understood about the biological nature of this lymphoma and its different subtypes, considerable progress has been made in understanding its lymphomagenesis in recent years, especially when it is related to Epstein-Barr virus infection. Its high heterogeneity and possible morphological overlap require ongoing study to identify it and its possible differential diagnoses in cases where it presents with a rare variant or pattern. This article aims to provide a comprehensive updated summary on the pathophysiology, clinical presentation, histopathological diagnosis, with emphasis on rare patterns that could become distracting and confusing factors, and prognosis of classic Hodgkin lymphoma, seeking to achieve a better understanding of the disease

Myeloproliferative Syndrome With Eosinophilia Associated With Translocation t(8; 13) and T-cell Lymphoblastic Lymphoma: A Case Report and Review of the Literature.

The 8p11 myeloproliferative syndrome (EMS) is an aggressive neoplasm associated with chromosomal translocations involving the fibroblast growth factor receptor 1 tyrosine kinase gene on chromosome 8p11. We report the case of a 31-year-old man with no prior medical history who presents with two weeks of sore throat and cervical lymphadenopathy up to 5 cm. Initial peripheral blood examination showed leukocytosis with predominantly neutrophils and eosinophilia. A CT scan demonstrated mediastinal lymphadenopathies, liver enlargement and splenomegaly. An excisional biopsy of a cervical lymph node demonstrated findings consistent with a diagnosis of T-cell lymphoblastic lymphoma. Bone marrow aspirate and biopsy revealed hypercellular marrow with granulocytic predominance, left-shifted granulopoiesis, eosinophilia and the cytogenetic analysis showed the following karyotype: 46, XY, t(8;13). The final diagnosis was a myeloproliferative syndrome with eosinophilia related to t(8;13) and T-cell acute lymphoblastic lymphoma (8p11 myeloproliferative syndrome). We review the relevant literature about this unusual entity.

Hemofilia A: una enfermedad huérfana / Hemophilia A: a rare disease

La hemofilia A es una enfermedad hereditaria ligada al cromosoma X, causada por mutaciones en el gen F8 del factor VIII de la coagulación. Se considera una enfermedad huérfana, ya que su prevalencia es baja, de 26,6 por cada 100.000 nacidos vivos de sexo masculino. Los pacientes con hemofilia A tienen fases de inicio y amplificación de la coagulación relativamente normales y son capaces de formar el tapón plaquetario inicial en el lugar de la hemorragia, pero debido a la deficiencia del factor VIII, son incapaces de generar una cantidad de trombina en la superficie de las plaquetas, que sea suficiente para estabilizar el coágulo de fibrina. En un paciente masculino con hemorragias inusuales debe descartarse un trastorno de coagulación tipo hemofilia A, y se debe solicitar un recuento de plaquetas y un tiempo de protrombina (TP), los cuales usualmente son normales, y un tiempo de tromboplastina parcial activado (TPT) que se presenta prolongado. Para el diagnóstico diferencial con otras coagulopatías se realiza la medición de factores de coagulación, y pruebas de corrección cuando existe la sospecha de un inhibidor o de una hemofilia adquirida. Los pacientes afectados pueden presentar formas leves, moderadas o severas de la enfermedad, según el nivel plasmático del factor. En Colombia y en el mundo, la hemofilia fue reconocida como una enfermedad huérfana que representa un problema de salud pública, debido a su proceso de atención altamente especializado, que incrementa los costos asociados con la asistencia sanitaria, y afecta la calidad de vida de los pacientes y de aquellos que los rodean, además de que representa un reto diagnóstico que requiere constante actualización, para que pueda ser tratada de manera efectiva

Hemophilia A is an X-linked inherited disease caused by mutations in the coagulation factor VIII F8 gene. It is considered a rare disease, as its prevalence is 26.6 per 100,000 live male births. Patients with hemophilia A have a relatively normal coagulation onset and amplification phases, and are able to form the initial platelet plug at the site of hemorrhage; but due to factor VIII deficiency, they are unable to generate a sufficient amount of thrombin on the platelet surface to stabilize the fibrin clot. In a male patient with unusual bleeding, a hemophilia A-type coagulation disorder should be ruled out, and blood tests such as a platelet count and prothrombin time (PT), which are usually normal, and an activated partial thromboplastin time (APTT), which is prolonged, should be requested immediately. For differential diagnosis with other coagulopathies, measurement of coagulation factors and correction tests are performed when there is suspicion of an inhibitor or acquired hemophilia. Affected patients may present mild, moderate or severe forms of the disease, depending on the plasma level of the factor. In Colombia and worldwide, hemophilia was recognized as a rare disease that represents a public health problem due to its highly specialized care, which increases the costs associated with health care, and affects the quality of life of patients and those around them, as well as representing a diagnostic challenge that requires constant updating, so that it can be treated effectively

Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome With IgG Kappa/IgG Lambda Biclonal Gammopathy: A Rare Presentation of a Rare Disease.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a low prevalence multisystemic paraneoplastic disease. The name is an acronym composed by its most relevant clinical manifestations, which are polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. More than 95% of the POEMS syndrome cases are monoclonal for lambda light chains; however, few cases have been reported in the literature with a biclonal component. In this paper, we report a rare case of a patient who has POEMS syndrome with biclonal gammopathy. To the best of our knowledge, this is the first reported case in the literature of POEMS syndrome with expression of IgG kappa/IgG lambda biclonal gammopathy.

Prematuridade e gravidez na adolescência no Brasil, 2011-2012 / Teenage pregnancy and prematurity in Brazil, 2011-2012 / Prematuridad y embarazo durante la adolescencia en Brasil, 2011-2012

Este trabalho tem como objetivo avaliar a associação entre gravidez na adolescência e prematuridade. Os dados são provenientes da pesquisa Nascer no Brasil, inquérito nacional composto por 23.894 puérperas e seus recém-nascidos. As informações foram obtidas por meio de entrevista com a puérpera durante a internação hospitalar. Um método de pareamento foi estabelecido, baseado nos escores de propensão, para lidar com diferenças entre os grupos em razão de um desenho não experimental, caso do estudo Nascer no Brasil. O desfecho do estudo foi a idade gestacional, sendo considerados todos os partos prematuros (idade gestacional < 37 semanas) e a termo (idade gestacional entre 37 e 41 semanas e 6 dias). O estudo evidenciou disparidades sociais, econômicas e assistenciais maternas entre as mulheres segundo a faixa etária. A maior proporção de puérperas adolescentes se concentrou nas regiões menos desenvolvidas do país, Norte e Nordeste, e nas classes econômicas menos favorecidas (D/E). Depois de equiparadas quanto às características socioeconômicas e assistenciais, foram observadas maiores chances de prematuridade espontânea nas adolescentes precoces, tanto em comparação às adolescentes tardias (OR = 1,49; IC95%: 1,07-2,06), quanto às adultas jovens (OR = 2,38; IC95%: 1,82-3,12). A prematuridade permanece em pauta no campo da saúde materno-infantil, sendo preocupante a associação com a gestação na adolescência encontrada neste trabalho, destacando-se que quanto mais jovem a gestante, maior a chance de parto prematuro espontâneo.

The study aims to evaluate the association between teenage pregnancy and prematurity. The data are from the Birth in Brazil study, a national survey consisting of 23,894 postpartum women and their newborn infants. The information was obtained from interviews with the mothers during their postpartum hospital stay. A matching method was established, based on propensity scores, to deal with differences between the groups due to the non-experimental design of the Birth in Brazil study. The study outcome was gestational age, considering all the premature births (gestational age < 37 weeks) and term births (gestational age 37 weeks to 41 weeks and 6 days). The study revealed social, economic, and maternal care disparities between the women according to age bracket. The highest proportions of teenage mothers were in the least developed regions of Brazil (North and Northeast) and in the poorest economic classes. After matching for socioeconomic and obstetric care characteristics, the highest odds of spontaneous prematurity were seen in younger adolescents compared to older adolescents (OR = 1.49; 95%CI: 1.07-2.06), and young adults (OR = 2.38; 95%CI: 1.82-3.12). Prematurity is still an issue in the field of maternal and child health, and the association with teenage pregnancy identified in this study is worrisome, especially because younger adolescent mothers were associated with higher odds of spontaneous prematurity.

Este estudio tiene como objetivo evaluar la asociación entre embarazo en la adolescencia y prematuridad. Los datos provienen de la encuesta Nacer en Brasil, encuesta nacional compuesta por 23.894 puérperas y sus recién nacidos. La información se obtuvo mediante una entrevista con la puérpera durante el internamiento hospitalario. Se estableció un método de emparejamiento, basado en las puntuaciones de propensión, para tratar las diferencias entre los grupos, según un diseño no experimental, como es el caso del estudio Nacer en Brasil. El resultado del estudio fue la edad gestacional, considerándose todos los partos prematuros (edad gestacional < 37 semanas) y a término (edad gestacional entre 37 y 41 semanas y 6 días). El estudio evidenció disparidades sociales, económicas y asistenciales maternas entre las mujeres, según su franja de edad. La mayor proporción de puérperas adolescentes se concentró en las regiones menos desarrolladas del país, Norte y Nordeste, y en las clases económicas menos favorecidas (D/E). Tras equiparlas respecto a las características socioeconómicas y asistenciales, se observaron mayores oportunidades de prematuridad espontánea en las adolescentes precoces, tanto en comparación con las adolescentes tardías (OR = 1,49; IC95%: 1,07-2,06), como con las adultas jóvenes (OR = 2,38; IC95%: 1,82-3,12). La prematuridad permanece en pauta en el campo de la salud materno-infantil, siendo preocupante la asociación con la gestación en la adolescencia hallada en este estudio, destacándose que cuanto más joven es la gestante, mayor es la oportunidad de parto prematuro espontáneo.

Advanced maternal age and factors associated with neonatal near miss in nulliparous and multiparous women / Idade materna avançada e fatores associados com o near miss neonatal em mulheres nulíparas e multíparas / Edad avanzada en la maternidad y factores asociados a la casi pérdida/near miss neonatal en mujeres nulíparas y multíparas

The early neonatal period accounts for approximately half of the deaths of young children under one year of age, and the neonatal near miss can recognize factors causing this high number of deaths. Thus, the aim of this study is to determine whether advanced maternal age increases the chance of neonatal near miss, in addition is to identify which factors are associated with the neonatal near miss, stratified by parity. Data are from the 2011-2012 Birth in Brazil study, which used a national population-based sample of 15,092 newborns of women between 20-29 and 35 years of age or more (advanced maternal age). Multiple logistic regression was performed to test the association between neonatal near miss and prenatal and childbirth variables, pre-gestational diseases, obstetric history and socioeconomic characteristics, stratified by parity. Advanced maternal age was to be statistically associated with neonatal near miss in nulliparous (OR = 1.62; 95%CI: 1.05-2.50) and multiparous (OR = 1.51; 95%CI: 1.20-1.91) when compared to women 20-29 years of age. For nulliparous women, the main variables statistically associated with neonatal near miss were multiple gestation (OR = 8.91) and hypertensive disease (OR = 2.57), whereas forceps-assisted vaginal delivery (OR = 7.19) and multiple gestation (OR = 4.47) were the variables associated for multiparous women. Neonatal near miss has been shown to be connected with access to health services for childbirth, gestational complications and maternal characteristics, mainly advanced maternal age. Therefore, to properly monitor and classify maternal gestational risk, to control gestational complications during prenatal care, and to correctly refer these women to childbirth care should be priority strategies for healthcare services.

O período neonatal precoce representa cerca da metade dos óbitos em crianças com menos de um ano de idade, e o near miss neonatal é capaz de identificar os fatores responsáveis por esse número elevado de mortes. Portanto, o estudo procurou investigar se a idade materna avançada aumenta a probabilidade de near miss neonatal, além de identificar os fatores associados ao near miss neonatal, estratificados por paridade. Os dados foram obtidos do estudo Nascer no Brasil de 2011-2012, que usou uma amostra representativa da população nacional com 15.092 recém-nascidos de mães com 20-29 anos de idade e com 35 anos ou mais (idade materna avançada). Foi usado um modelo de regressão logística multivariada para testar a associação entre near miss neonatal e variáveis do pré-natal e do parto, doenças pré-gestacionais, história obstétrica e características socioeconômicas, estratificadas por paridade. A idade materna avançada mostrou estar associada estatisticamente com o near miss neonatal em mulheres nulíparas (OR = 1,62; IC95%: 1,05-2,50) e multíparas (OR = 1,51; IC95%: 1,20-1,91) comparado com mulheres com 20-29 anos de idade. Entre as mulheres nulíparas, as principais variáveis associadas estatisticamente com o near miss neonatal foram a gestação múltipla (OR = 8,91) e doença hipertensiva (OR = 2,57), enquanto o parto vaginal com uso de fórceps (OR = 7,19) e gestação múltipla (OR = 4,47) foram as variáveis associadas em mulheres multíparas. O near miss neonatal mostrou estar relacionado ao acesso aos serviços obstétricos, complicações gestacionais e características maternas, principalmente idade materna avançada. Portanto, monitorar e classificar adequadamente o risco gestacional, controlar as complicações gestacionais durante o atendimento pré-natal e encaminhar essas mulheres corretamente para o atendimento no parto devem ser estratégias prioritárias para os serviços de saúde.

El periodo neonatal temprano cuenta con aproximadamente la mitad de muertes de niños pequeños con una edad inferior a un año, y la near miss neonatal puede identificar factores que causan este número elevado de muertes. Por ello, el objetivo de este estudio es determinar si una edad avanzada maternal incrementa la oportunidad de near miss neonatal, además de identificar qué factores están asociados con la near miss neonatal, estratificada por paridad. Los datos son del 2011-2012, procedentes del estudio Nacer en Brasil, que utilizó una muestra nacional basada en población, compuesta por 15.092 recién nacidos de mujeres entre 20-29 y 35 años de edad o más (edad avanzada maternal). Se realizó una regresión múltiple logística para probar la asociación entre near miss neonatal y las variables prenatales, y de nacimiento de niños, enfermedades pregestacionales, historial obstétrico y características socioeconómicas, estratificada por paridad. La edad avanzada maternal estuvo estadísticamente asociada con near miss neonatal en nulíparas (OR = 1,62; 95%CI: 1,05-2,50) y multíparas (OR = 1,51; 95%CI: 1,20-1,91), cuando se compara con mujeres de 20-29 años de edad. Para las mujeres nulíparas, las principales variables estadísticamente asociadas con near miss neonatal fueron múltiple gestación (OR = 8,91) y enfermedad hipertensiva (OR = 2,57), mientras que el parto vaginal con apoyo de fórceps (OR = 7,19) y la gestación múltiple (OR = 4,47) fueron las variables asociadas para mujeres multíparas. Near miss neonatal ha demostrado estar relacionada con el acceso a servicios de salud para el parto, complicaciones gestacionales y características maternas, principalmente edad avanzada maternal. Por consiguiente, debería ser una estrategia prioritaria en los servicios de salud supervisar adecuadamente y clasificar el riesgo maternal gestacional, así como las complicaciones en el control gestacional durante el cuidado prenatal, al igual que dirigir correctamente a estas mujeres a cuidados para el parto.

As influências da raça/cor nos desfechos obstétricos e neonatais desfavoráveis / The influences of race/color on unfavorable obstetric and neonatal outcomes

RESUMO Trata-se de um estudo transversal, que avaliou as influências da raça/cor nos desfechos obstétricos e neonatais desfavoráveis. Foram construídos modelos de regressão logística para cálculo de razão de chance e exame do risco materno e neonatal. As gestantes negras prevaleceram com significância estatística entre aquelas com baixa escolaridade, hipertensão prévia, três ou mais filhos vivos e com ocupação. As gestantes negras não apresentaram maior risco nos desfechos desfavoráveis. A raça/cor não se comporta como um marcador genético ou biológico, mas como construto social, que pode influenciar as condições de saúde enquanto determinante social.

ABSTRACT This is a cross-sectional study, which evaluated race/color influences on unfavorable obstetric and neonatal outcomes. Logistic regression models were constructed to calculate the odds ratio and to examine maternal and neonatal risk. Black pregnant women prevailed with statistical significance among those with low schooling, previous hypertension, three or more living children and with occupation. Black pregnant women presented no greater risk in unfavorable outcomes. Race/color does not behave as a genetic or biological marker, but as a social construct, which can influence health conditions as a social determinant.

Assistência pré-natal e resultado perinatal / Prenatal care and perinatal outcome / Atención prenatal y resultado perinatal

Objetivo: Relacionar o número de consultas de pré-natal aos desfechos perinatais. Métodos: Realizou-se um estudo de corte transversal, no período de setembro de 2014 a agosto de 2015, em base de dados de gestantes de uma maternidade pública na cidade de Joinville/SC/Brasil. A pesquisa incluiu pacientes com gestação única, subdividindo-os em 2 grupos: com 6 ou menos e 7 ou mais consultas de pré-natal. Utilizaram-se os desfechos primários: via de parto, prematuridade, baixo peso ao nascer, macrossomia, óbito perinatal. Analisou-se com modelo de regressão logística multinomial, com intervalo de confiança de 95%. Resultados: Obtiveram-se 4.260 prontuários, 266 (6,2%) realizaram 6 consultas ou menos e 3.994 (93,7%) com 7 consultas ou mais de pré-natal. Os grupos apresentaram diferença nas características maternas; na idade, menor ou igual a 20 anos (27,4% vs 21,3% p=0,020) entre os grupos; na escolaridade, respectivamente primeiro grau incompleto (28,1% vs 21% p=0,006) e segundo grau completo (25,9% vs 36,9% p= 0,000); hipertensão arterial sistêmica (9,77% vs 6,5% p=0,039), diferentes características neonatais, como prematuridade (23,6% vs 6,6% p=0,000), e no parto a termo (76,3%) vs 93,3% p= 0,000) respectivamente. Encontrou-se maior chance de prematuridade (OR= 2,837), baixo peso (OR=1,895) e óbito perinatal (OR=5,584) no grupo que realizou 6 consultas ou menos. Conclusão: As gestantes com menos de sete consultas de pré-natal têm maior chance de ocorrência de prematuridade, baixo peso ao nascer e óbito perinatal.

Objective: To relate the number of prenatal consultations to perinatal outcomes. Methods: Cross-sectional study conducted from September 2014 to August 2015 using a database of pregnant women attending a public maternity hospital in the city of Joinville, Santa Catarina, Brazil. The research included single pregnancy patients divided into 2 groups: a group of 6 or less prenatal consultations and a group of 7 or more prenatal consultations. The following primary outcomes were analyzed: delivery method, prematurity, low birth weight, macrosomia, perinatal death. The analysis was carried out using a multinomial logistic regression model with a 95% confidence interval. Results: In all, 4,260 records were analyzed: 266 (6.2%) had 6 or less prenatal consultations and 3,994 (93.7%) had 7 or more prenatal consultations. The groups presented different maternal characteristics ­ age ≤20 years (27.4% vs 21.3% p=0.020), incomplete primary education (28.1% vs 21% p=0.006) and complete secondary education (25.9% vs 36.9% p=0.000), systemic arterial hypertension (9.77% vs 6.5% p=0.039) ­ and different neonatal characteristics ­ prematurity (23.6% vs. 6.6%, p=0.000) and full-term birth (76.3% vs 93.3% p=0.000). The group of women who had 6 consultations of less was at increased odds of prematurity (OR=2.837), low birth weight (OR=1.895) and perinatal death (OR=5.584). Conclusion: The pregnant women who had less than seven prenatal consultations are at increased odds of prematurity, low birth weight and perinatal death.

Objetivo: Relacionar el número de consultas prenatales con los desenlaces perinatales. Métodos: Se realizó un estudio de cohorte transversal en el período entre septiembre de 2014 y agosto de 2015 en las bases de datos de embarazadas de una maternidad pública de la ciudad de Joinville/SC/Brasil. La investigación incluyó pacientes de única gestación dividiéndose en 2 grupos: con 6 o menos y 7 o más consultas prenatales. Se utilizaron los siguientes desenlaces primarios: la vía del parto, la prematuridad, el bajo peso al nacer, la macrosomía y el óbito perinatal. El análisis se realizó con el modelo de regresión logística multinomial con el intervalo de confianza del 95%. Resultados: De un total de 4.260 historiales clínicos, 266 (6,2%) pacientes realizaron 6 consultas o menos y 3.994 (93,7%) tuvieron 7 consultas prenatal o más. Los grupos presentaron diferencia en las características maternas; con edad de 20 años o menos (27,4% vs 21,3% p=0,020) entre los grupos; con educación básica incompleta (28,1% vs 21% p=0,006) y educación secundaria completa (25,9% vs 36,9% p= 0,000); hipertensión arterial sistémica (9,77% vs 6,5% p=0,039), distintas características neonatales como la prematuridad (23,6% vs 6,6% p=0,000) y en el parto a término (76,3%) vs 93,3% p= 0,000) respectivamente. Se encontró más oportunidad de prematuridad (OR= 2,837), bajo peso (OR=1,895) y óbito perinatal (OR=5,584) en el grupo que realizó 6 consultas o menos. Conclusión: Las gestantes con menos de siete consultas prenatales tienen más oportunidad de prematuridad, bajo peso al nascer y óbito perinatal.

Cytotoxic Epstein-Barr virus-positive large B cell lymphoma: a regulatory B cell-derived neoplasia?

AIMS: A new subtype of granzyme B (GrB)-producing regulatory B cells (Bregs ) has been described recently; these peculiar cytotoxic B cells are increased significantly in interleukin (IL)-21-rich settings, and in particular during HIV and Epstein-Barr virus (EBV) infection. Our aim is to report a unique case of an EBV-positive diffuse large B cell lymphoma (DLBCL) with cytotoxic features arisen in an HIV+ patient, and to understand if this lesion may represent a proliferation of neoplastic cytotoxic Bregs . METHODS AND RESULTS: We describe a 66-year-old male patient who presented with cervical lymph node enlargement and B symptoms; subsequently, HIV infection was diagnosed. Histopathological, immunohistochemical and molecular studies were performed, and revealed an EBV-positive DLBCL with cytotoxic features. Considering the immunological setting and unconventional phenotype observed, we tried to evaluate further the expression of GrB and IL-21 in another 150 aggressive B cell lymphomas (17 of 150 EBV+ , two of 150 EBV+ /HIV+ ). Minimal dot-like expression of GrB was found in seven lymphomas (in fewer than 1% of tumour cells), three of which were EBV-positive. CONCLUSIONS: Breg origin has never been reported in B cell lymphomas. We describe an exceptional case of EBV-positive DLBCL with aberrant expression of cytotoxic markers in a patient with a previously unknown HIV infection. We propose cytotoxic Bregs as a possible normal counterpart for this unusual tumour.

A cor da dor: iniquidades raciais na atenção pré-natal e ao parto no Brasil / El color del dolor: inequidades raciales en la atención pre-natal y partos en Brasil / The color of pain: racial iniquities in prenatal care and childbirth in Brazil

Poucas pesquisas com foco nas influências da raça/cor no tocante à experiência de gestação e parto foram conduzidas no Brasil, sendo inédita a análise de abrangência nacional. Este estudo teve como objetivo avaliar as iniquidades na atenção pré-natal e parto de acordo com a raça/cor utilizando o método de pareamento baseado nos escores de propensão. Os dados são oriundos da pesquisa Nascer no Brasil: Pesquisa Nacional sobre Parto e Nascimento, um estudo de base populacional de abrangência nacional com entrevista e avaliação de prontuários de 23.894 mulheres em 2011/2012. Regressões logísticas simples foram utilizadas para estimar as razões de chance (OR) e respectivos intervalos de 95% de confiança (IC95%) da raça/cor associada aos desfechos analisados. Em comparação às brancas, puérperas de cor preta possuíram maior risco de terem um pré-natal inadequado (OR = 1,6; IC95%: 1,4-1,9), falta de vinculação à maternidade (OR = 1,2; IC95%: 1,1-1,4), ausência de acompanhante (OR = 1,7; IC95%: 1,4-2,0), peregrinação para o parto (OR = 1,3; IC95%: 1,2-1,5) e menos anestesia local para episiotomia (OR = 1,5 (IC95%: 1,1-2,1). Puérperas de cor parda também tiveram maior risco de terem um pré-natal inadequado (OR = 1,2; IC95%: 1,1-1,4) e ausência de acompanhante (OR = 1,4; IC95%: 1,3-1,6) quando comparadas às brancas. Foram identificadas disparidades raciais no processo de atenção à gestação e ao parto evidenciando um gradiente de pior para melhor cuidado entre mulheres pretas, pardas e brancas.

Existen pocas investigaciones realizadas en Brasil centradas en las influencias de la raza/color, en lo que se refiere a la experiencia de la gestación y parto, siendo inédito un análisis de alcance nacional. Este estudio tuvo como objetivo evaluar las inequidades en la atención pre-natal y parto, de acuerdo a la raza/color, utilizando el método de apareamiento, basado en los marcadores de propensión. Los datos provienen de la investigación Nacer en Brasil: Investigación Nacional sobre Parto y Nacimiento, un estudio de base poblacional de alcance nacional con entrevista y evaluación de historiales médicos de 23.894 mujeres en 2011/2012. Se utilizaron regresiones logísticas simples para estimar las razones de oportunidad (OR) y sus respectivos intervalos de un 95% de confianza (IC95%) de la raza/color asociados a los desenlaces analizados. En comparación a las blancas, las puérperas de color negro tuvieron un mayor riesgo de tener un período pre-natal inadecuado (OR = 1,6; IC95%: 1,4-1,9), falta de vinculación a la maternidad (OR = 1,2; IC95%: 1,1-1,4), ausencia de acompañante (OR = 1,7; IC95%: 1,4-2,0), grandes desplazamientos para el parto (OR = 1,3; IC95%: 1,2-1,5) y menos anestesia local para episiotomía (OR = 1,5; IC95%: 1,1-2,1). Las puérperas mulatas también tuvieron un mayor riesgo de tener un período pre-natal inadecuado (OR = 1,2; IC95%: 1,1-1,4) y ausencia de acompañante (OR = 1,4; IC95%: 1,3-1,6), cuando se comparan con las blancas. Fueron identificadas disparidades raciales en el proceso de atención a la gestación y al parto, evidenciando un gradiente de peor para mejor cuidado entre mujeres negras, mulatas y blancas.

Few studies on the influence of race/color on pregnancy and birthcare experiences have been carried out in Brazil. Additionally, none of the existing studies are of national scope. This study sought to evaluate inequities in prenatal and childbirth care according to race/color using propensity score matching. The data comes from the study Birth in Brazil: National Survey into Labor and Birth, a national population study comprised of interviews and revisions of medical records that included 23,894 women in 2011/2012. We used logistic regressions to estimate odds ratios (OR) and respective 95% confidence intervals (95%CI) of race/color associated with the outcomes were analyzed. When compared with white-skinned women, black-skinned women were more likely to have inadequate prenatal care (OR = 1.6; 95%CI: 1.4-1.9), to not be linked to a maternity hospital for childbirth (OR = 1.2 95%CI: 1.1-1.4), to be without a companion (OR = 1.7; 95%CI: 1.4-2.0), to seek more than one hospital for childbirth (OR =1.3; 95%CI: 1.2-1.5), and less likely to receive local anesthesia for an episiotomy (OR = 1.5; 95%CI: 1.1-2.1). Brown-skinned women were also more likely to have inadequate prenatal care (OR = 1.2; 95%CI: 1.1-1.4) and to lack a companion (OR = 1.4; 95%CI: 1.3-1.6) when compared with white-skinned women. We identified racial disparities in care during pregnancy and childbirth, which displayed a gradient going from worst to best care provided to black, brown and white-skinned women.

Tumor de células gigantes de hueso: serie de casos durante 64 años en un único centro / Giant cell tumor of bone: a 64 years single center experience

Introducción: El tumor de células gigantes de hueso es una lesión localmente agresiva que tien-de a la recurrencia local y ocasionalmente presenta metástasis a distancia. Objetivo: describir la presentación clínica e histológica y la frecuencia relativa del tumor de células gigantes de hueso en un único centro, Medellín-Colombia. Materiales y métodos: estudio descriptivo; se revisaron todos los casos diagnosticados como tumor de células gigantes de hueso entre 1944 y 2008 en el Departamento de Patología de la Universidad de Antioquia, y para cada uno se revisaron las prin-cipales características clínicas e histológicas. Resultados: entre 1944 y 2008 se diagnosticaron 2.185 tumores óseos; de éstos, 302 tenían diagnóstico de tumor de células gigantes del hueso y se confirmó el diagnóstico en 117, correspondiente al 5,3% de todos los tumores óseos. La rela-ción mujer: hombre fue de 1,1:1. Las localizaciones más frecuentes fueron fémur distal (19,7%), tibia proximal (25,6%), manos y pies (11,1%), radio distal (9,4%) y húmero proximal (8,5%). Las características histológicas atípicas que se observaron fueron áreas fusocelulares, invasión de tejidos blandos, necrosis, hemorragia, áreas de quiste óseo aneurismático secundario, áreas de células mononucleadas con escasas células gigantes y formación de osteoide dentro del tumor...

Introduction: Giant cell tumors of bone are locally aggressive lesions that tend to recur locally and that rarely metastasize. Objective: to describe clinical and histological features, and relative frequency of giant cell tumors of bone in a single center, in the city of Medellín-Colombia. Materials and methods: a cross-sectional study was conducted. All the tumors diagnosed as giant cell tumor of bone between 1944 and 2008 in the Department of Pathology of Universidad de Antioquia were reviewed, and for each patient, important clinical and histological features were assessed. Results: between the years 1944 and 2008, 2,185 bone tumors were diagnosed; of these, 302 were diagnosed as giant cell tumor of bone, but in this study the diagnosis was confirmed only in 117 cases; 5.3% of all bone tumors. The female: male ratio was 1.1:1. The tumors were most requently found in the distal femur (19.7%), proximal tibia (25.6%), hands and feet (11.1%), distal radio (9.4%), and proximal humerus (8.5%). Atypical features observed in the tumors included spindle cell areas, soft tissue invasion, necrosis, hemorrhage, aneurysmal bone cyst-like changes, and osteoid formation...

Desorden linfoproliferativo T sistémico de la infancia asociado al virus Epstein-Barr: reporte de un caso / Systemic Epstein-Barr virus-positive T celllymphoproliferative disease of childhood: a case report

Resumen: el desorden linfoproliferativo T sistémico de la infancia asociado al virus Epstein-Barres una entidad clínica de descripción reciente, potencialmente mortal en niños y en adultos jóvenes.Se caracteriza por una proliferación clonal de linfocitos T con un fenotipo citotóxico activadoinfectados por el virus. Es más frecuente en países de Asia y México, y se desconoce su incidenciaen el medio. Se reporta un caso de esta enfermedad, con progresión indolente y desenlace fatal.

Abstract: systemic Epstein-Barr virus-positive T cell Iymphoproliferative disease of childhood is arecently described, potentially lethal clinical entity in children and young adults, characterized bya clonal proliferation of EBV-infected T-cells with an activated cytotoxic phenotype. It is typicallyprevalent in Asian countries and Mexico, and its incidence is unknown in our country. This reportdescribes a case of systemic Epstein-Barr virus positive.

Intervençäo fonoaudiológica precoce na seqüência de Möebius: relato de caso / Prematury speech therapy for a Moebius Syndrome: report case

O objetivo deste estudo é apresentar um plano de trabalho terapêutico fonoaudiológico para um bebê portador da Seqüência de Möebius. A proposta terapêutica foi de orientaçäo quinzenal, envolvendo o trabalho direto com a mäe, durante um período de seis meses. Foram trabalhadas as áreas do sistema miofuncional oral, cogniçäo, linguagem, desenvolvimento neuropsicomotor, funçöes visual e auditiva. O tipo de trabalho a que nos propusemos realizar atingiu seus objetivos, de acordo com o progresso apresentado pelo sujeito. Discultimos, também, questöes relacionadas à orientaçä, à mäe-terapeuta, à necessidade desse tipo de intervençäo realizada precocemente, visando-se o desenvolvimento global da criança e a sua efetividade