Two Synchronous Neonatal Tumors: An Extremely Rare Case.

We report a case of a newborn with two synchronous tumors-sialoblastoma and hepatoblastoma-diagnosed at 20 weeks of gestation by magnetic resonance imaging (MRI) and ultrasonography (US). The aim of this study was to describe the management of this case together with a review of the literature. Our patient had a large facial tumor associated with extremely high alpha-fetoprotein levels. Diagnosis of the tumors was made by surgical biopsy, showing typical features in both. Sialoblastoma is a potentially aggressive tumor. In our case, the Ki67 index in the sialoblastoma was between 20 and 30%, indicating a possibly unfavorable behavior. The infant underwent surgery and chemotherapy in different steps. Complete surgical resection with clean margins is considered to be the best treatment option for sialoblastoma. Only four similar cases were previously reported. Timely management by a multidisciplinary team is essential in these difficult cases. In our patient, outcome was good at the time of this report.

Evaluación y apoyo nutricional en el paciente pediátrico críticamente enfermo: Revisión de la literatura

RESUMEN Esta revisión se basa en la mejor evidencia actualmente disponible, y en ella se definen las directrices en relación con la evaluación y manejo nutricional en niños críticamente enfermos. Estas directrices incluyen, los criterios para la detección de la malnutrición, tanto por déficit o exceso, así como, se identifican las recomendaciones y consensos de expertos sobre la estimación energética y de macronutrientes, los cuales pueden ser determinantes en prevenir tanto la sobrealimentación como subalimentación. También se señalan las recomendaciones internacionales sobre el momento de inicio del soporte nutricional para que esta sea oportuna, efectiva y segura, eligiendo la vía de alimentación según condición clínica y tipo de patología en función de disminuir el riesgo de morbimortalidad de estos pacientes. A modo general, se sugiere intensificar la investigación científica, con el fin de disponer de mejor evidencia para diseñar protocolos clínicos internacionales y locales para el manejo nutricional del paciente pediátrico críticamente enfermo.

ABSTRACT This review is based on the best evidence currently available and defines the guidelines for the nutritional assessment and management of critically ill children. These guidelines include the criteria for detecting malnutrition, whether due to deficit or excess, as well as the recommendations and consensus of experts on the estimation of energy and macronutrients, which can be decisive in preventing both overfeeding and underfeeding. International recommendations on the time of initiation of nutritional support are also indicated so that it is timely, effective and safe. Choice of feeding route according to clinical condition and pathology type in order to reduce the risk of morbi-mortality of these patients are discussed. In general, an increase in scientific research is suggested, in order to have better evidence to design international and local clinical protocols for the nutritional management of critically ill pediatric patients.

[Feeding behavior, dietary sufficiency and nutritional status in children between 6 and 18 months]. / Comportamiento alimentario, suficiencia dietaria y estado nutricional en niños entre 6 a 18 meses.

INTRODUCTION: Infant feeding has specific qualitative and quantitative characteristics. The influence of parents on food intake is crucial in the development of habits and an adequate nutritional state. OBJECTIVE: To evaluate eating behavior, dietary characteristics, and nutritional status of children between 6 to 18 months. SUBJECTS AND METHOD: Cross-sectional descriptive study in 199 children. Using convenience sampling, demographic and anthropometric data were recorded for nutritional status, and dietary history, through a 24-hour reminder for dietary behavior and food sufficiency. The Student's t-test and one-way ANOVA were applied for continuous variables and the chi-square test for the categori cal ones. RESULTS: 54.8% were women. Regarding nutritional status, 21.1% were overweight and 7.6% obese. 56.8% of the children had meal numbers according to their age. The average energy intake was 652.9 ± 224.2 calories per day. The intake of energy and macronutrients was significantly higher in children not consuming breast milk and aged 12 to 18 months. The energy, lipids, and carbohydrates adequacy in girls was significantly higher than in boys. 83.1% of food consumption was habitual for this age group. CONCLUSION: In eating behavior, we observed that 4 out of 10 children have meal numbers higher than suggested for their ages. Regarding dietary sufficiency, there is a higher intake in children with normal nutritional status, without breastfeeding, and between 12 and 18 months. Despite the high prevalence of obesity, it was lower than that observed at the national level.

Brain death epidemiology in Uruguay and utilization of the Glasgow coma score in acute brain injured patients as a predictor of brain death.

OBJECTIVE: The knowledge of brain death (BD) epidemiology and the acute brain injury (ABI) progression profile are relevant to improve public health programs, organ procurement strategies, as well as intensive care unit (ICU) protocols aiming to increase the detection of potential donors. The aim of this study was to analyze the BD epidemiology and the ABI progression profile among subjects admitted to ICUs with a Glasgow Coma Score (GCS) < or = 8. MATERIALS AND METHODS: This was a prospective, observational study of BD reported to the National Institute of Donation and Transplantation from 2000-2006. The patients with ABI and GCS < or = 8 who were admitted to 5 ICUs with In-hospital Transplant Coordination were analyzed over the period of 2005-2007. RESULTS: The BD detection increased from 28.7 in 2000 to 58.5 BD pmp in 2006. The real donor global rate increased from 10 to 24.6 pmp from 2000 to 2006. The ABI patients with GCS < or = 8 had a global mortality rate of 56%, including 23.4% who evolved to BD. CONCLUSIONS: This study showed a 200% increment of detected BD and 150% of real donors, although these results are still below the international figures. GCS follow-up appeared to be a good tool to predict the BD outcome. The follow-up of patients with ABI allowed us to improve our BD detection strategy.

Nutritional deficiency in citrus with symptoms of citrus variegated chlorosis disease.

It is well known that citrus plants that have been infected by Xylella fastidiosa display nutritional deficiencies, probably caused by production of extracellular polymers by the bacteria that block normal nutrient flow through the xylem. The aim of this work was to study the mineral composition of specific foliar areas in different stages of infection in citrus. Thus, the concentrations of macro and micronutrients in leaves of citrus infected by X. fastidiosa were measured. Samples from four infected citrus orchards in the State of São Paulo, Brazil, were respectively collected from Santa Rita do Passa Quatro, Neves Paulista, Gavião Peixoto and Paraíso counties. The presence of X. fastidiosa in leaves was confirmed by polymerase chain reaction (PCR) using specific PCR primers. To understand the variation in leaf-nutrient content in citrus plants, we used foliar nutrient values from control (non-symptomatic) plants as a reference. Chemometric analysis showed that the deficiency of P and K in symptomatic trees for all orchards and high concentrations of Fe, Mn and Zn were observed in chlorotic areas, although other studies revealed deficiency of zinc in leaves. This is the first report showing that a correlation between chlorotic citrus leaf and higher concentrations of Fe, Mn and Zn are observed when infected and healthy plants were compared.

Nutritional deficiency in citrus with symptoms of citrus variegated chlorosis disease / Deficiência nutricional em citros com sintomas da doença da clorose variegada dos citros

It is well known that citrus plants that have been infected by Xylella fastidiosa display nutritional deficiencies, probably caused by production of extracellular polymers by the bacteria that block normal nutrient flow through the xylem. The aim of this work was to study the mineral composition of specific foliar areas in different stages of infection in citrus. Thus, the concentrations of macro and micronutrients in leaves of citrus infected by X. fastidiosa were measured. Samples from four infected citrus orchards in the State of São Paulo, Brazil, were respectively collected from Santa Rita do Passa Quatro, Neves Paulista, Gavião Peixoto and Paraíso counties. The presence of X. fastidiosa in leaves was confirmed by polymerase chain reaction (PCR) using specific PCR primers. To understand the variation in leaf-nutrient content in citrus plants, we used foliar nutrient values from control (non-symptomatic) plants as a reference. Chemometric analysis showed that the deficiency of P and K in symptomatic trees for all orchards and high concentrations of Fe, Mn and Zn were observed in chlorotic areas, although other studies revealed deficiency of zinc in leaves. This is the first report showing that a correlation between chlorotic citrus leaf and higher concentrations of Fe, Mn and Zn are observed when infected and healthy plants were compared.

Já é bem conhecido que cultivares cítricas que foram infectadas pela bactéria Xylella fastidiosa apresentam deficiências nutricionais devido à produção de polímero extracelular por esta bactéria, o qual bloqueia o fluxo normal de nutriente pelo xilema. O objetivo deste trabalho foi o de estudar a composição mineral em áreas foliares específicas em diferentes fases de infecção na planta. Assim, as concentrações de macro e micronutrientes em folhas de citros infectados por X. fastidiosa foram quantificadas. Foram coletadas amostras de quatro pomares cítricos infectados localizados em: Santa Rita do Passa Quatro, Neves Paulista, Gavião Peixoto e Paraíso, no Estado de São Paulo. A presença de X. fastidiosa em folhas foi confirmada através de reação da polimerase em cadeia (PCR) usando iniciadores específicos. Para entender a variação no conteúdo de nutriente foliar em plantas cítricas, utilizou-se de valores de nutrientes foliares de plantas não sintomáticas (controle) como referência. A análise quimiométrica mostrou que a deficiência de P e K em plantas sintomáticas e concentrações altas de Fe, Mn e Zn foram presentes em áreas foliares cloróticas, embora outros estudos mostrem a deficiência de zinco em folhas. Este é o primeiro relato indicando que uma correlação entre folhas cítricas cloróticas e elevadas concentrações de Fe, Mn e Zn foi observada quando plantas infectadas e saudáveis foram comparadas.

Prospective validation of first-trimester combined screening for trisomy 21.

OBJECTIVE: To examine the performance of the new algorithm in screening for trisomy 21 by a combination of maternal age, fetal nuchal translucency (NT) and maternal serum free beta-human chorionic gonadotropin (beta-hCG) and pregnancy-associated plasma protein-A (PAPP-A). METHODS: This was a prospective screening study for trisomy 21 in singleton pregnancies at 11 + 0 to 13 + 6 weeks of gestation using an algorithm combining maternal age, fetal NT thickness based on the mixture model for the assessment of NT, and maternal serum free beta-hCG and PAPP-A based on a multiple regression model for the assessment of serum biochemistry. The NT measurements were performed by 60 operators who had obtained The Fetal Medicine Foundation certificate of competence in the 11-13-week scan. RESULTS: The study population consisted of 19 614 pregnancies with a normal karyotype or delivery of a phenotypically normal baby (euploid group) and 122 cases of trisomy 21. In the euploid fetuses the NT was above the previously defined 50(th), 95(th) and 99(th) centiles in 10 033 (51.2%), 618 (3.2%) and 123 (0.6%) cases and the respective values for trisomy 21 were 117 (95.9%), 94 (77.0%) and 57 (46.7%). The median fetal NT was within 0.1 mm of the expected in 47 (78.3%) of the 60 sonographers and within 0.2 mm in all. In the euploid fetuses the median free beta-hCG was 1.0 (range, 0.1-29.4) multiples of the median (MoM) and the median PAPP-A was 1.0 (range, 0.2-3.3) MoM. The median MoM values were 1.0 or close to 1.0 MoM for each subgroup of pregnancy characteristics, including gestations of 11, 12 and 13 weeks, maternal weight of < 60 kg, 60-80 kg and > 80 kg, different ethnic origins, cigarette smokers and non-smokers, natural conception and in vitro fertilization. For a false-positive rate of 3%, the detection rate of trisomy 21 in screening by maternal age and fetal NT was 81% (95% CI, 73-89%), by maternal age and maternal serum biochemistry it was 63% (95% CI, 56-72%) and by combined screening based on maternal age, fetal NT and maternal serum biochemistry it was 90% (95% CI, 84-96%). CONCLUSION: This study has validated the new risk algorithm and demonstrated that in combined screening for trisomy 21 based on maternal age, fetal NT and free beta-hCG and PAPP-A the detection rate is about 90% for a 3% false-positive rate.

Effect of deviation of nuchal translucency measurements on the performance of screening for trisomy 21.

OBJECTIVES: To examine the effect of deviations in median nuchal translucency thickness (NT) and the spread in measurements on the performance of screening for trisomy 21 by maternal age and fetal NT, and by maternal age, fetal NT and maternal serum biochemistry. METHODS: We simulated the NT and multiples of the median values for pregnancy-associated plasma protein-A (PAPP-A) and free beta-human chorionic gonadotropin (beta-hCG) for 500 000 euploid and 500 000 trisomy 21 pregnancies at 12 weeks of gestation. Detection rates for trisomy 21 and false-positive rates were calculated without adjustments in NT and by adding or subtracting values ranging from 0.1 to 1.0 mm to each observed measurement. In addition, the effects of variation in the scatter of NT measurements were examined by applying a multiplicative factor ranging from 0.5 to 2 to the SD. RESULTS: The detection rate of trisomy 21 for a fixed false-positive rate of 3% in screening by maternal age and fetal NT was 72%, and in screening by maternal age, fetal NT and serum free beta-hCG and PAPP-A it was 86%. A consistent underestimate or overestimate in the measured NT reduced the detection rate of trisomy 21 for a fixed-false positive rate. At a fixed screen-positive cut-off an underestimate in fetal NT reduced the detection rate whereas an overestimate in NT increased the false-positive rate. A widening in the scatter of measurements had only a small impact on the detection rate but it caused a major increase in the false-positive rate. CONCLUSIONS: High performance of screening necessitates appropriate measurement of fetal NT. This paper demonstrates the effect of deviations in the median and SD of NT from the expected on the performance of screening and can form the basis of audit of results of individual sonographers.

Tumores anexiales en niñas y adolescentes: experiencia del Hospital Clínico de la Universidad de Católica de Chile, 1991-2003 / Adnexal mass in girls and teenagers: experience of Hospital Clínico de la Universidad de Católica de Chile, 1991-2003

Se analiza retrospectivamente la experiencia de los últimos 13 años en el manejo de la patología anexial quirúrgica en niñas y adolescentes menores de 19 años. Se revisa la forma de presentación clínica y los hallazgos anatopatológicos de 106 pacientes. Del total de lesiones anexiales, un 62 por ciento correspondieron a lesiones neoplásicas benignas y malignas, la mayoría de ellas originadas en el ovario. El riesgo de malignidad para las lesiones neoplásicas fue de un 26,7 por ciento. La neoplasia ovárica benigna más frecuente fue el teratoma maduro (31,1 por ciento). Las neoplasias malignas más frecuentes fueron las originadas en el estroma gonadal específico y en el tejido germinal (en conjunto 37,5 por ciento del total de neoplasias malignas). La forma de presentación clínica más frecuente fue el dolor abdominal habitualmente como manifestación de complicación de una neoplasia benigna. La vía de abordaje más frecuente fue por laparotomía y se privilegió la cirugía conservadora para preservar el potencial reproductivo.

Group specific antibodies against the putative AMP-binding domain signature SGTTGXPKG in peptide synthetases and related enzymes.

The superfamily of adenylate forming enzymes including peptide synthetases, acyl-CoA synthetases and insect luciferases is readily identified by the signature sequence SGTTGXPKG. This sequence including an invariant lysyl residue is located in a disordered loop region and was predicted to be of significant antigenicity. Antibodies were generated employing YTSGTTGRPKGC attached to bovine serum albumin and have been successfully used to identify respective enzymes and adenylate forming domains in multienzyme systems. These include the delta-(L-alpha-aminoadipyl)-L-cysteinyl-D-valine synthetases of Aspergillus nidulans and Acremonium chrysogenum, gramicidin S synthetase 1 and tyrocidine synthetase 1 from Bacillus brevis, acetyl-CoA synthetase from Alcaligenes eutrophus and a putative peptide synthetase from Metarhizium anisopliae. Weaker or no reactions are observed when the amino acid in position X in the protein is non-basic or hydrophobic, which is respectively the case for gramicidin S synthetase 1 and luciferase.

ACV synthetase: expression of amino acid activating domains of the Penicillium chrysogenum enzyme in Aspergillus nidulans.

Fragments of ACV synthetase of Penicillium chrysogenum carrying partial activities of amino acid activation were expressed under the alcA promoter in an acvA-deletion mutant of Aspergillus nidulans. The 210 kDa domain A-beta-galactosidase fusion protein was partially cleaved to fragments of 200 and 97 kDa. The domain A fragment and the 312 kDa domain BC construct were identified by peptide specific antibodies and shown to catalyze alpha-aminoadipate-, cysteine-, and valine-dependent ATP/[32P]PPi exchange activity. Substrate specificities were investigated using amino acid analogues. Unexpectedly neither alpha-aminoadipate nor valine activation was exclusive, implying possible misactivations and proof reading functions. Both fragments were only expressed in limited amounts and found to be unstable.