Ocular manifestation of vertical transmission of dengue: case report / Manifestação ocular de transmissão vertical de dengue: relato de caso

ABSTRACT A 7-week-old male delivered by cesarean section presented with a positive serology for dengue along with preretinal and retinal hemorrhages, vitreous opacities and cotton wool spots. The patient and his mother had positive serologies for Non Structural Protein 1 (NS1) by ELISA. Retinal and vitreous findings improved over a sixteen-week period. Spectral domain optical coherence tomography (OCT) showed preserved macular architecture. In this case report, we suggest that retinal and vitreous changes may be the ocular presenting features of vertically transmitted dengue in newborns, and that those findings may resolve with no major structural sequelae.

RESUMO Neonato de 7 semanas, do sexo masculino, nascido de parto cesárea, apresentou sorologia positiva para dengue com hemorragias retinianas e pré-retinianas, opacidades vítreas e manchas algodonosas. O paciente e sua mãe haviam apresentado sorologias positivas para Non Structural Protein 1 através de ELISA. Achados na retina e no vítreo melhoraram em um período de dezesseis semanas. O exame de tomografia de coerência óptica de domínio espectral demonstrou arquitetura macular preservada. Neste relato de caso, sugerimos que alterações na retina e no vítreo podem ser os achados oculares aparentes em neonatos com infecção vertical por dengue, e que estes podem se resolver sem maiores sequelas estruturais.

Caracterización de neonatos con asfixia perinatal. Hospital Provincial Docente Ginecobstétrico José Ramón López Tabrane. 2016-2021

Introducción: La asfixia perinatal es un síndrome caracterizado por la suspensión o grave disminución del intercambio gaseoso a nivel de la placenta o de los pulmones. Objetivo: Caracterizar los neonatos con asfixia perinatal durante el trabajo de parto con la introducción de la gasometría de la arteria umbilical. Materiales y métodos: Se realizó un estudio descriptivo, transversal, de octubre de 2016 a diciembre de 2021. Se trabajó con la totalidad del universo: 75 neonatos asfícticos. Las variables fueron: edad materna, edad gestacional al nacimiento, enfermedades asociadas y dependientes del embarazo, tipo y tiempo de trabajo de parto, características del líquido amniótico, anomalías de la placenta y cordón umbilical, pH de la gasometría de la arteria del cordón, evolución clínica del recién nacido. Los datos se almacenaron y procesaron en una hoja de cálculo de Microsoft Excel, utilizando la estadística descriptiva. Resultados: Predominaron pacientes con edad materna menor de 20 años (56 %), y edad gestacional al nacimiento de 28 a 33,6 semanas (77,3 %). La hipertensión arterial fue la comorbilidad más común, así como la preeclampsia, entre las enfermedades dependiente del embarazo (30,7 %); 61,3 % tuvieron parto eutócico, con líquido amniótico claro (65,3 %), placenta previa (38,7 %) y circulares en cordón umbilical (30,7 %). En el 86,7 % el pH de la gasometría fue menor que 7, y el 92 % presentó evolución clínica favorable. Conclusiones: La asfixia perinatal estuvo predominantemente asociada a madres adolescentes, con tiempo gestacional entre 28 y 36 semanas, preeclampsia, placenta previa, circulares del cordón umbilical y predominio de gasometría con pH menor que 7.

Introduction: Perinatal asphyxia is a syndrome characterized by the suspension or severe decrease in gas exchange at the level of the placenta or lungs. Objective: To characterize neonates with perinatal asphyxia during labor with the introduction of umbilical arterial blood gas test. Materials and methods: A descriptive, cross-sectional study was carried out from October 2016 to December 2021. It dealt with the entire universe, 75 asphyxiated neonates. The variables were: maternal age, gestational age at birth, associated and pregnancy-dependent diseases, type and time of labor, characteristics of the amniotic fluid, anomalies of the placenta and umbilical cord, pH of the cord arterial blood gas test, clinical evolution of the newborn. The data were stored and processed in a Microsoft Excel spreadsheet, using descriptive statistics. Results: Patients with maternal age below 20 years (56%), and gestational age at birth from 28 to 33.6 weeks (77.3%) predominated. Arterial hypertension was the most common comorbidity, as well as preeclampsia, among pregnancy-dependent diseases (30.7%); 61.3% had eutocic delivery, with clear amniotic fluid (65.3%), previous placenta (38.7%) and umbilical cord circulars (30.7%). In 86.7%, the blood gas pH was less than 7 and 92% had a favorable clinical evolution. Conclusions: Perinatal asphyxia was predominantly associated with adolescent mothers, with gestational time between 28-36 weeks; preeclampsia, placenta previa, umbilical cord circulars, and blood gases pH lower than 7.

Características clínicas de la mortalidad neonatal en un hospital de tercer nivel del Paraguay: un estudio observacional retrospectivo

Introducción: La mortalidad neonatal sigue siendo un importante problema de salud pública en todo el mundo, especialmente en países en desarrollo. La mayoría de las muertes neonatales son atribuibles a complicaciones prevenibles, como la prematuridad, la sepsis neonatal y la asfixia al nacer. Objetivo: Determinar las características clínicas de la mortalidad neonatal en un hospital de tercer nivel del Paraguay mediante un estudio observacional retrospectivo. Metodología: El presente estudio es de diseño observacional, descriptivo y retrospectivo, se realizó en el Hospital de Clínicas de Paraguay. Este diseño permitió obtener información de los registros médicos de los recién nacidos atendidos en el hospital durante un período de cinco años. Resultados: En el periodo comprendido entre el 2018 y el 2022, se registraron un total de 131 casos de mortalidad neonatal en el Hospital de Clínicas del Paraguay. El peso medio al nacer de 2009,5 ± 991,4 kilogramos y una edad gestacional media de 32,87 ± 4,5 semanas. Los diagnósticos de óbito más frecuentes en los neonatos fueron sepsis (37 casos), afectación cardiaca (61 casos) y síndrome genético (39 casos). Discusión: Los datos obtenidos del estudio sugieren que existen factores de riesgo maternos y neonatales que están vinculados con la mortalidad neonatal en el Hospital de Clínicas del Paraguay. Se necesitan estudios posteriores que permitan profundizar en el análisis de estos factores y que permitan el desarrollo de estrategias preventivas para disminuir la tasa de mortalidad neonatal en el país.

Introduction: Neonatal mortality remains a major public health problem worldwide, particularly in developing countries. Most neonatal deaths are attributable to preventable complications, such as premature birth, neonatal sepsis, and birth asphyxia. Objective: To determine the clinical characteristics of neonatal mortality in a tertiary-level hospital in Paraguay through a retrospective observational study. Methods: This observational, descriptive, and retrospective study was conducted at the Hospital de Clínicas in Paraguay. This design allowed information to be obtained from the medical records of newborns who attended the hospital over a period of five years. Results: Between 2018 and 2022, 121 cases of neonatal mortality were recorded at the Hospital de Clínicas del Paraguay. The mean birth weight was 2013.51 ± 1021 kg and mean gestational age was 32.87 ± 4.6 weeks. The most frequent diagnoses of neonatal death were sepsis (37 cases), cardiac involvement (61 cases), and genetic syndromes (39 cases). Discussion: The data obtained from this study suggest that maternal and neonatal risk factors are associated with neonatal mortality in the Hospital de Clínicas of Paraguay. Further studies are needed to analyze these factors and develop preventive strategies to reduce the neonatal mortality rate in the country.

Women with preeclampsia exposed to air pollution during pregnancy: Relationship between oxidative stress and neonatal disease - Pilot study.

Oxidative imbalance as a pathophysiological mechanism has been reported as an adverse outcome in pregnant women who develop preeclampsia and in their newborns. Furthermore, emerging evidence suggests the same mechanism by which air pollutants may exert their toxic effects. Therefore, the objective of the study was to evaluate the biomarkers of oxidative stress and their relationship with neonatal disease in premature newborns from mothers with preeclampsia exposed to air pollution during pregnancy. The data of air pollutants (PM2.5, PM10 and ozone) were collected at fixed monitoring stations. Oxidative and antioxidant status markers were obtained through special techniques in women with preeclampsia and in umbilical cord blood of their premature newborns. The oxidative stress markers were significantly higher in women with preeclampsia and their newborns who were exposed to higher levels of ambient air pollutants in the first and second trimester of pregnancy. Neonatal diseases are associated with preeclampsia in pregnancies, specifically intrauterine growth restriction (IUGR) and necrotizing enterocolitis (NEC). A significant correlation was identified in the levels of prooxidant agents and antioxidant enzyme activity in the presence of neonatal diseases associated with preeclampsia. There is increased oxidative damage in both the maternal and fetal circulation in women who develop preeclampsia exposed to air pollution during pregnancy. Therefore, these pregnancies complicated by preeclampsia have a greater adverse outcome as neonatal disease in the preterm infant.

Desfechos maternos e neonatais em servidoras públicas estaduais de São Paulo com idade materna avançada / Maternal and neonatal outcomes in state public servants of São Paulo with advanced maternal age

Objetivo: Caracterizar a população das gestantes em diferentes faixas etárias; avaliar desfechos maternos e neonatais em pacientes com idade materna avançada; determinar a faixa etária a partir da qual os desfechos adversos foram mais prevalentes. Métodos: Parturientes atendidas no Hospital do Servidor Público Estadual de São Paulo entre junho/2019 e maio/2020 foram divididas em três grupos ­ 20 a 34 anos; 35 a 39 anos; 40 anos ou mais ­ e analisadas quanto a diversas variáveis. Resultados: Entre as gestantes do Serviço, 44,2% tinham idade materna avançada. A amostra foi composta por 927 pacientes, a maioria com relacionamento conjugal estável (75,2%) e ensino de nível superior (74,7%). Independentemente do grupo etário, foram observados elevados índices de obesidade (25,9%), sobrepeso (39,7%) e cesariana (76,4%). A frequência de iteratividade, diabetes gestacional e doença hipertensiva específica da gestação foi maior a partir dos 35 anos, e a frequência de hipertensão arterial crônica foi maior a partir dos 40 anos. Neonatos de pacientes com 40 anos ou mais tiveram maiores índices de baixo peso ao nascer, óbito neonatal, Apgar de quinto minuto < 7 e necessidade de reanimação neonatal. Conclusão: Pacientes com idade materna avançada representaram porcentagem expressiva da população e tiveram maior frequência de desfechos adversos. Complicações obstétricas foram mais prevalentes a partir dos 35 anos, com destaque para diabetes gestacional e distúrbios hipertensivos. Resultados neonatais desfavoráveis, como baixo peso ao nascer e óbito neonatal, foram mais prevalentes a partir de 40 anos.

Objective: Featuring the population of pregnant women in different age groups; assessing maternal and neonatal outcomes in patients at advanced maternal age; determining the threshold age for the potential prevalence of adverse outcomes. Methods: Women in labor assisted at Hospital do Servidor Público Estadual de São Paulo between June/2019 and May/2020 were divided into three age groups ­ 20 to 34 years; 35 to 39 years; over 40 years ­, who were assessed for several variables. Results: 44.2% of pregnant women in this Service were at advanced maternal age. The sample counted on 927 patients, most of them declared stable marital relationships (75.2%) and College degree (74,7%). High obesity levels (25.9%), overweight (39.7%) and cesarean delivery (76.4%) were observed, regardless of age group. Maternal request was the main indication for cesarean surgery. Iteration frequency, gestational diabetes and pregnancy-specific hypertensive disease was higher from the age of 35 years, on. Chronical high blood pressure was higher in the age group over 40 years. Newborns from patients older than over 40 years presented higher low weight at birth index, neonatal death, 5th minute Apgar score < 7 and the need of neonatal resuscitation. Conclusion: Patients at advanced maternal age recorded higher obstetric adversity frequency in the age group over 35 years, with emphasis on gestational diabetes and high blood pressure. Unfavorable neonatal outcomes related to low weight at birth and neonatal death were more prevalent in the age group over 40 years.

Seroprevalence of antibodies against to SARS-CoV 2 in umbilical cord blood in two hospital centers in Córdoba and Sucre, Colombia.

INTRODUCTION: Pregnant women continue to be vulnerable to COVID-19, and their immunosuppressed state could put them at greater risk of developing more severe forms of the disease. In Colombia and Latin America, there are few studies on the immune response of the newborn against SARS-CoV-2. AIM: To determine the prevalence of SARS-CoV-2 infection in umbilical cord blood in two hospital centers in Córdoba and Sucre. METHODS: Between March and June 2021, a prospective descriptive cross-sectional study was carried out. Two hospitals from the departments of Córdoba and Sucre, located in the Northwest Caribbean area of Colombia, participated. Three hundred sixty umbilical cord blood samples were taken at the two hospitals. A commercial ELISA was performed to detect total IgG, IgM, and IgA antibodies against the N protein of SARS-CoV-2. The ethics committee approved the study of the participating institutions. RESULTS: Of 3.291 women who gave birth in the hospital centers included in the study, 360 (11%) participated. Complete clinical data were obtained for 223 women. The mean age of the women was 24 years (range, 15-42). 29.4% (106/360) of the umbilical cord samples had total antibodies against SARS-CoV-2. Pregnant women did not have blood samples taken. 58% of the women were asymptomatic. There was no association between umbilical cord samples, clinical, epidemiological characteristics, and serological response to antibodies to SARS-CoV-2 (p > 0.05). CONCLUSIONS: The prevalence of umbilical cord blood samples was 29.4% for total SARS-CoV-2 antibodies. The study provides essential aspects for the epidemiological approach to neonates infected with SARS-CoV-2.

Neurodesarrollo en recién nacidos de muy bajo peso nacidos en Matanzas en el periodo 2016-2018

RESUMEN Fundamento La comparación de los resultados del neurodesarrollo en recién nacidos de muy bajo peso según cohortes de años de nacimiento permite evaluar el impacto de las diferentes intervenciones llevadas a cabo para prevenir y tratar las afecciones más frecuentes en este grupo de pacientes durante el período perinatal y neonatal, así como de las diferentes condiciones y enfermedades que se presentan en estas etapas del desarrollo. Objetivo evaluar el neurodesarrollo a los dos años de edad corregida en una cohorte de recién nacidos de muy bajo peso. Métodos estudio observacional prospectivo, que incluyó a recién nacidos pretérminos con peso al nacer <1500 g egresados vivos del Hospital Docente Ginecobstétrico Provincial de Matanzas, en el período 2016-2018, y que hubieran completado su seguimiento en consulta de neurodesarrollo a los dos años de edad corregida (N=52). Los datos contenidos en las historias clínicas fueron almacenados en una base de datos (SPSS v. 22.0), a partir de la cual se realizó el procesamiento estadístico. Se consideró significativo todo valor p <0,05. Resultados a los dos años de edad corregida, el 90 % de los pacientes eran normales; las alteraciones mayores y menores se encontraron, respectivamente, en 2 y 8 % de los casos. Las convulsiones neonatales clínicas se asociaron significativamente con la ocurrencia de alteraciones del neurodesarrollo. Conclusión La incidencia de alteraciones del neurodesarrollo a los dos años de edad corregida en recién nacidos de muy bajo peso en Matanzas fue menor en el período estudiado, en relación a años anteriores, aunque este hallazgo no resultó estadísticamente significativo.

ABSTRACT Background The comparison of neurodevelopmental results in very low birth weight newborns according to birth year cohorts allows evaluating the impact of the different interventions carried out to prevent and treat the most frequent conditions in this group of patients during the perinatal period and neonatal, as well as the different conditions and diseases that occur in these stages of development. Objective to evaluate neurodevelopment at two years of corrected age in a cohort of very low birth weight newborns. Methods prospective observational study, which included preterm newborns with birth weight <1500 g discharged alive from the Provincial Gynecobstetric Teaching Hospital of Matanzas, in the period 2016-2018, and who had completed their follow-up in a neurodevelopment consultation at two years old corrected age (N=52). The data contained in the medical records were stored in a database (SPSS v. 22.0), from which the statistical processing was performed. Any value p<0.05 was considered significant. Results at two years old of corrected age, 90% of the patients were normal; major and minor alterations were found, respectively, in 2 and 8% of cases. Clinical neonatal seizures were significantly associated with the occurrence of neurodevelopmental abnormalities. Conclusion The incidence of neurodevelopmental disorders at two years old of corrected age in very low birth weight newborns in Matanzas was lower in the period studied, in relation to previous years, although this finding was not statistically significant.

Clinical and epidemiological characterization of newborns with necrotizing enterocolitis / Caracterização clínica e epidemiológica de recém-nascidos com enterocolite necrosante

INTRODUCTION: Necrotizing enterocolitis (NEC) is a severe inflammatory disorder that can affect the whole gastrointestinal system, particularly the ileum, and is a major cause of morbidity in premature infants. OBJECTIVE: To describe the clinical and epidemiological profile of newborns with NEC, seeking to identify the causes, evolution and severity of the disease. METHODS: The study selected 94 patients who developed NEC (cases) and 60 patients who did not develop the disease during hospitalization (controls) and presented similar clinical signs of the case group. The variables analyzed divided into maternal and neonatal. The frequency tests were applied using the Statistical Package for the Social Sciences (SPSS) version 21.0 and comparative analysis using the GraphPad Prism® 5.0 software. RESULTS: There was a higher number of prenatal consultations in cases with NEC. Newborns with NEC had shorter hospital stay, longer parenteral nutrition and antibiotics use and a predominant use of infant formula. The Bell criteria modified by Walsh and Kleigman was negatively correlated to maternal age and positively correlated to gestational age, birth weight and time of parenteral nutrition. CONCLUSION: Although being a disease of the newborn, the present study indicated that maternal characteristics may be related to its onset of NEC. Therefore, the greater number of prenatal consultations and neonatal factors such as length of stay, prolonged use of parenteral nutrition and antibiotic therapy, and formula use may influence the development of the disease.

INTRODUÇÃO: A Enterocolite Necrosante (ECN) é um grave distúrbio inflamatório que pode afetar todo o sistema gastrointestinal, em particular o íleo, é uma causa de morbiletalidade em prematuros. OBJETIVO: Delinear o perfil clínico-epidemiológico de recém-nascidos com ECN, buscando identificar as causas, modo de evolução e gravidade da doença. MÉTODOS: Foram selecionados 94 pacientes que desenvolveram ECN (casos) e 60 pacientes que não desenvolveram a doença no período de internação (controles) e apresentavam sinais clínicos semelhantes do grupo caso. As variáveis analisadas foram classificadas em maternas e neonatais. Foram realizados testes de frequência usando o programa Statistical Package for the Social Sciences (SPSS) versão 21.0 e análise comparativa usando o teste programa GraphPad Prism® 5.0. RESULTADOS: Nos casos com ECN houve maior número de consultas pré-natal. Os recém-nascidos com ECN apresentaram menor tempo de internação, maior tempo de nutrição parenteral e uso de antibióticos tiveram predomínio no uso de fórmula infantil. A classificação modificada de Bell por Walsh and Kleigman apresentou correlação negativa com idade materna e positiva com idade gestacional, peso ao nascer e o tempo de nutrição parenteral. CONCLUSÃO: Apesar de ser uma doença do recém-nascido, esse estudo sugere que características maternas podem ter relação com o aparecimento da ECN. O número maior de consultas pré-natal e os fatores neonatais como o tempo de internação, uso prolongado de nutrição parenteral, antibioticoterapia e uso de fórmulas pode influenciar o desenvolvimento da doença.

Presentación atípica de un neuroblastoma congénito metastásico con efecto renal grave: informe de caso / Atypical presentation of a congenital metastatic neuroblastoma with severe renal impact: case report

El neuroblastoma es un tumor derivado de la cresta neural. Se trata del tumor maligno más frecuente en recién nacidos y existe una amplia variabilidad en su forma de presentación. En algunos casos, el diagnóstico se realiza mediante un hallazgo ecográfico, mientras que en otros se manifiesta como enfermedad metastásica agresiva. Se presenta el caso de una paciente prematura con diagnóstico posnatal de neuroblastoma congénito metastásico con manifestaciones clínicas graves y evolución atípica. Asimismo, se destaca una lesión renal grave que requirió terapia dialítica.

Neuroblastoma is a neural crest-derived tumor and the most common malignant tumor in neonates. Its clinical presentation can be quite variable. In some cases, it presents as an aggressive metastatic disease whilst in others it is a finding in scans performed for other reasons. The following report presents the case of a premature newborn, diagnosed postnatally with metastatic congenital neuroblastoma, with an atypical and torpid course, including severe renal injury and dialysis requirement.

[Atypical presentation of a congenital metastatic neuroblastoma with severe renal impact: case report]. / Presentación atípica de un neuroblastoma congénito metastásico con efecto renal grave: informe de caso.

Neuroblastoma is a neural crest-derived tumor and the most common malignant tumor in neonates. Its clinical presentation can be quite variable. In some cases, it presents as an aggressive metastatic disease whilst in others it is a finding in scans performed for other reasons. The following report presents the case of a premature newborn, diagnosed postnatally with metastatic congenital neuroblastoma, with an atypical and torpid course, including severe renal injury and dialysis requirement.

El neuroblastoma es un tumor derivado de la cresta neural. Se trata del tumor maligno más frecuente en recién nacidos y existe una amplia variabilidad en su forma de presentación. En algunos casos, el diagnóstico se realiza mediante un hallazgo ecográfico, mientras que en otros se manifiesta como enfermedad metastásica agresiva. Se presenta el caso de una paciente prematura con diagnóstico posnatal de neuroblastoma congénito metastásico con manifestaciones clínicas graves y evolución atípica. Asimismo, se destaca una lesión renal grave que requirió terapia dialítica.

Enfermedad de Caroli: revisión sistemática de la literatura / Characterization of patients with Caroli's disease: Systematic review

Resumen Introducción: La enfermedad de Caroli es un trastorno congénito poco usual, el cual cursa con dilatación segmentaria multifocal de los conductos biliares intrahepáticos. Fue descrita por Jacques Caroli en 1958 como una dilatación sacular, segmentada o fusiforme de los ductos biliares intrahepáticos. Objetivo: Caracterizar a la población que ha padecido la enfermedad de Caroli. Metodología: Revisión sistemática de la literatura. Resultados: Se analizaron 66 artículos, el grupo de edad más afectado es los menores de 10 años, en el que se evidenció una mayor prevalencia en varones, y se encontró asociado con otras comorbilidades como el riñón poliquístico (20 %). La manifestación más frecuente fue la hepatomegalia (44,7 %), seguido de la fiebre (42,4 %) y el dolor abdominal en el hipocondrio derecho (41,2 %). Como método diagnóstico más utilizado se encontraba la resonancia magnética (73,8 %); dentro de los hallazgos predominó la dilatación intrahepática (76,5 %) y el manejo más empleado fue la antibioticoterapia para tratar las recurrencias por colangitis. Conclusión: La enfermedad de Caroli tiene una baja prevalencia, se presenta con mayor frecuencia en el continente americano, afecta principalmente a la primera década de la vida, tiene predilección por el sexo masculino y se caracteriza por una dilatación de los conductos intrahepáticos que pueden afectar a otros órganos como los riñones, lo que produce quistes renales.

Abstract Caroli's disease (CD) is a rare congenital disease, which presents with multifocal segmental intrahepatic bile duct dilatation. It was first described by Jacques Caroli in 1958 as a saccular or fusiform dilatation of the intrahepatic bile ducts. Objective: To characterize the population that has been diagnosed with Caroli's disease. Materials and methods: Systematic review. Results: 66 articles were analyzed. The age group most affected was children under 10 years old, where a higher prevalence was evidenced in males. This condition was associated with other comorbidities such as polycystic kidney in 20%. The most frequent manifestation was hepatomegaly (44.7%), followed by fever (42.4%), and abdominal pain in the right upper quadrant (41.2%). The most used diagnostic method was magnetic resonance imaging in 73.8% of the sample. The findings showed predominance of intra-hepatic dilatation in 76.5%. The most widely used treatment was antibiotic therapy to treat recurrences due to cholangitis. Conclusion: Caroli's disease has an extremely low incidence and occurs more frequently in the American continent, affecting mainly patients in the first decade of life, with a predilection for the male sex. It is characterized by a dilatation of the intrahepatic ducts that can affect other organs such as the kidneys, causing renal cysts.

Congenital hyperextension deformity of the knees due to arthrogryposis multiplex congenita? case report

ABSTRACT Introduction: Arthrogryposis multiplex congenita is a disorder characterized by non-progressive joint contractures. It has an estimated prevalence of 1 in every 3 000-5 000 live births, with the same male-to-female ratio. Case presentation: This is the case of a male newborn with adequate prenatal care checkup appointments, who presented with a congenital deformity of the lower limbs. On physical examination, he had hyperextension of the knees (passive flexion of 20° in the left leg and 30° in the right leg), and painful active movement. On admission, peripheral pulses had good intensity, and adequate distal perfusion was found. Barlow and Ortolani maneuvers were negative, and no midline lesions were observed in the spine. The patient was diagnosed with arthrogryposis multiplex congenita and received multidisciplinary treatment to avoid early morbidity and mortality. Conclusion: To attain satisfactory clinical development in patients with arthrogryposis, it is essential to have a high level of antenatal suspicion, as well as appropriate prenatal checkups. All this allows for proper management, minimizing diagnostic errors, avoiding unnecessary procedures, and performing effective and timely treatment with outstanding results.

RESUMEN Introducción. La artrogriposis múltiple congénita es un desorden caracterizado por contracturas articulares no progresivas y que tiene una prevalencia estimada de 1 caso por cada 3 000-5 000 nacidos vivos con igual proporción entre géneros. Presentación del caso. Paciente masculino recién nacido con adecuados controles prenatales, quien al nacimiento presentó deformidad en miembros inferiores debido a hiperextensión de las rodillas (flexión pasiva de 20° en pierna izquierda y 30° en pierna derecha) que provocaba dolor a la movilización activa. Al ingreso se registró perfusión distal adecuada y pulsos periféricos simétricos y de buena intensidad; las maniobras de Ortolani y Barlow fueron negativas y no se evidenciaron lesiones en la línea media del dorso. El paciente fue diagnosticado con artrogriposis múltiple congénita y recibió tratamiento multidisciplinario que evitó morbi-mortalidad temprana. Conclusión. La sospecha prenatal, el examen físico exhaustivo y el diagnóstico diferencial son de vital importancia para lograr una evolución clínica satisfactoria en la artrogriposis múltiple congénita; con esto es posible hacer un manejo adecuado, minimizar los errores diagnósticos, evitar procedimientos innecesarios y realizar un tratamiento efectivo y oportuno con excelentes resultados.

Lobectomy with ecmo support in an infant who developed pulmonary interstitial emphysema following repair of hypoplastic aortic arch

Abstract Pulmonary interstitial emphysema (PIE) is a common problem in premature neonates with respiratory distress syndrome. This condition is often related to barotrauma caused by mechanical ventilation or continuous positive airway pressure applied to low birth weight neonates. The clinical diagnosis can be challenging. However, after proper diagnosis, several interventions are available for successful management. We describe an infant who developed severe PIE with recurrent pneumothoraces and development of a persistent bronchopleural fistula shortly after repair of a hypoplastic aortic arch and description of successful lobectomy with the assistance of extracorporeal support (ECMO).

Parto pretérmino, avances y retos: A manera de prólogo

This article is an introduction to the symposium Perinatal approach to preterm labor that includes themes on causality, management of the mother, advances in the management of the premature newborn, and the future of mother and baby. This prologue includes figures that show how the incidence of prematurity has increased over time. Nevertheless, advances in perinatology and neonatology allow more and better survival for infants born preterm and with very low weight. Preventive measures in the mother at risk also contribute to better perinatal results.

El presente artículo es una introducción al simposio Enfoque perinatal del parto pretérmino, que incluye los temas de causalidad, manejo de la rotura prematura de membranas, avances en el manejo del bebe prematuro y futuro de la madre y el bebe. El prólogo incluye cifras que señalan cómo la incidencia de parto pretérmino ha continuado aumentando con el tiempo. Sin embargo, los avances peri y neonatológicos permiten una mayor y mejor sobrevida de los niños que nacen lejos de término y con peso muy bajo. Las medidas preventivas en la madre con riesgo también contribuyen a los mejores resultados perinatales.

Factors associated with preventable infant death: a multiple logistic regression

ABSTRACT OBJECTIVE To identify and analyze factors associated with preventable child deaths. METHODS This analytical cross-sectional study had preventable child mortality as dependent variable. From a population of 34,284 live births, we have selected a systematic sample of 4,402 children who did not die compared to 272 children who died from preventable causes during the period studied. The independent variables were analyzed in four hierarchical blocks: sociodemographic factors, the characteristics of the mother, prenatal and delivery care, and health conditions of the patient and neonatal care. We performed a descriptive statistical analysis and estimated multiple hierarchical logistic regression models. RESULTS Approximatelly 35.3% of the deaths could have been prevented with the early diagnosis and treatment of diseases during pregnancy and 26.8% of them could have been prevented with better care conditions for pregnant women. CONCLUSIONS The following characteristics of the mother are determinant for the higher mortality of children before the first year of life: living in neighborhoods with an average family income lower than four minimum wages, being aged ≤ 19 years, having one or more alive children, having a child with low APGAR level at the fifth minute of life, and having a child with low birth weight.

Rapidly involuting congenital hemangioma

Abstract: Rapidly involuting congenital hemangioma is a rare vascular tumor that generally has a good prognosis. The authors describe a case of a newborn girl with a left cervical vascular lesion. Image exams were performed, and the lesion slowly decreased, leaving redundant skin. Considering all of the findings, a final diagnosis of a rapidly involuting congenital hemangiomas was suspected.

Congenital Langerhans cell histiocytosis: a good prognosis disease?

Abstract: Langerhans cell histiocytosis is rare and more frequent in children. The skin is affected in 50% of the cases and is the only site in 10%. Its course varies from self-limited and localized forms to severe multisystemic forms. Congenital cases are usually exclusively cutaneous and self-limited, with spontaneous remission in months. This study presents a rare congenital case, initially restricted to the skin, with subsequent dissemination and fatal outcome. A male newborn presented congenital disseminated erythematous scaly lesions. The biopsy was conclusive for Langerhans cell histiocytosis. The patient evolved into the multisystemic form in weeks, when chemotherapy was started, according to the LCH-2009 protocol; however, the patient was refractory to treatment and died.

Aderência ao seguimento no cuidado ao recém-nascido exposto à sífilis e características associadas à interrupção do acompanhamento / Adherence to the follow-up of the newborn exposed to syphilis and factors associated with loss to follow-up

RESUMO: Introdução: Todo recém-nascido exposto à sífilis na gestação deve ter acompanhamento ambulatorial. A interrupção do seguimento põe em risco todos aqueles que não recebem tratamento ao nascer. Objetivo: Descrever as características clínicas e epidemiológicas dos recém-nascidos expostos à sífilis, assim como gestacionais e sociodemográficas de suas mães e investigar os fatores associados com a descontinuidade do seguimento. Métodos: Trata-se de um estudo observacional, descritivo, analítico e retrospectivo dos prontuários de 254 crianças expostas à sífilis, atendidas no Ambulatório de Infecções Congênitas do Hospital de Clínicas da Universidade Federal do Paraná, entre 2000 e 2010. Os recém-nascidos foram classificados por referência ao seu acompanhamento ou não. Os dados foram ajustados a um modelo de regressão logística binária, no sentido de identificar os fatores associados à descontinuidade do tratamento. Resultados: As características estatisticamente associadas à interrupção do seguimento na análise multivariada foram: mães com idade acima de 30 anos, paridade de três ou mais filhos e a ausência de coinfecções pelo HIV e/ou hepatites virais. Conclusão: Tais achados demonstram a necessidade de identificar essas famílias e estabelecer estratégias que incentivem a formação de vínculos. Recomenda-se que os critérios de tratamento dos recém-nascidos tenham maior rigor, visto que a maior parte deles não faz o seguimento adequado.

ABSTRACT: Introduction: All newborns exposed to syphilis in pregnancy must have outpatient follow-up. The interruption of this follow-up especially threatens those children who were not treated at birth. Objective: To describe the clinical, epidemiological, and sociodemographic characteristics of pregnant women with syphilis and their newborns, and to investigate the factors associated with the discontinuation of the follow-up. Methods: This is an observational, descriptive, analytical, and retrospective study of medical records of 254 children exposed to syphilis, who were assisted at the Congenital Infectious Clinic of the university hospital of the Universidade Federal do Paraná, between 2000 and 2010. The newborns were classified by reference according to their follow-up. Data were analyzed by means of the binary logistic regression model in order to identify the factors associated to drop out. Results: The factors associated to the interruption of the follow-up were maternal age over 30 years, mothers with 3 or more children, and the absence of cross-infections by HIV and/or viral hepatitis. Conclusion: Such findings demonstrate the need to identify these families and implement strategies to promote the establishment of bonds. A greater rigor to indicate the treatment of the disease at birth is recommended, as most of them do not properly follow up.

Síndrome de transfusión feto-fetal con riñón único ectópico en gemelar donante. Presentación de un caso / Syndrome of Fetus-fetal transfusion with a single ectopic kidney in a twin donor. Case presentation

El síndrome de transfusión feto-fetal se presenta con mayor frecuencia en los embarazos gemelares monocoriales biamnióticos. En etapas avanzadas y sin una intervención prenatal, se asocia a altas tasas de mortalidad perinatal y de secuelas neurológicas en los sobrevivientes. Se presenta el caso de una pareja de gemelares con depresión severa al nacer, en los que se planteó un síndrome de transfusión feto-fetal, confirmado posteriormente con la presencia de anemia en el gemelo donante y policitemia en el gemelo receptor. Ambos gemelos tuvieron una evolución desfavorable hasta llegar a la muerte neonatal precoz. Los hallazgos de la necropsia fueron compatibles con daños secundarios al síndrome, con la particularidad de que en ambos hubo evidencias de infección pulmonar, y de una malformación renal en el gemelo donante, la cual no se recoge entre las malformaciones propias de este síndrome. El objetivo de este trabajo es puntualizar en los elementos esenciales para el diagnóstico y tratamiento antenatal de esta enfermedad, a través de las peculiaridades del caso que se presenta.

The Syndrome of Fetus-fetal transfusion presents more frequently in dynamitic monochromic twins. In advanced stages and without prenatal intervention, is associated to high rates of peri natal mortality and neurological sequel in the survivors. It is presented a case of a pair of twins with severe depression at birth in which it was diagnosed a Syndrome of Fetus-fetal, later confirmed with the presence of anemia in the donor twin and polycythemia in the receptor twin. Both twins had an unfavourable evolution with an early neonatal death. Necropsy findings were comparable with secondary damage to the syndrome, with the particularity that both had evidences of pulmonary infection and a renal malformation in the donor twin which is not included in the proper malformations of this syndrome. The objective of this work is to point out the essential elements for the diagnosis and antenatal treatment for this disease through the peculiarities of the presented case.