Encefalopatia toxica secundaria a vigabatrina: Reporte de caso / Toxic encephalopathy secondary to vigabatrin: Case report

El síndrome de West es una encefalopatía epiléptica caracterizada por espasmos en flexión, hipsarritmia en el electroencefalograma y retraso en el neurodesarrollo. Reportamos el caso de una paciente de 11 meses con diagnóstico de Síndrome de West y encefalopatía tóxica secundaria al uso de vigabatrina

West syndrome is an epileptic encephalopathy characterized by flexing spasms, hypsarritmia in the electroencephalogram and delayed neurodevelopment. We report an 11-month-old patient with a diagnosis of West syndrome and toxic encephalopathy secondary to the use of vigabatrin

SÍNDROME HEMOFAGOCÍTICO SECUNDARIO A LEISHMANIASIS VISCERAL EN UN LACTANTE: A PROPÓSITO DE UN CASO / SYNDROME HEMOPHAGOCYTIC SECONDARY TO LEISHMANIASIS VISCERAL IN A INFANT: ABOUT A CASE

El síndrome hemofagocítico (SHF) es un trastorno caracterizado por la activación y proliferación no maligna del sistema inmune. Puede ser primario o secundario. La forma primaria está ligada a factores genéticos autosómicos recesivos y la secundaria se presenta subsiguiente a otras enfermedades. La leishmaniasis es la parasitosis que más comúnmente lo gatilla y tiene la característica que resuelve con su tratamiento específico, sin el cual la mor- talidad alcanza el 100 %. Se presenta el caso de un niño de 7 meses, oriundo de Paraguay, que ingresa a nuestro Hospital por fiebre y hepatoesplenomegalia, que luego de arribar al diagnóstico y realizar tratamiento específico, evolucionó favorablemente

Hemophagocytic syndrome (HPS) is a disorder characterized by non-malignant activation and proliferation of the immune system. It can be primary or secondary. The primary form is linked to autosomal recessive genetic factors and the secondary form occurs subsequent to other diseases. Leishmaniasis is a parasitic disease with a wide worldwide distribution caused by different species of Leishmania. It is the protozoan that most commonly triggers it and it resolves with its specific treatment.Without it, the mortality rate is 100 %. We report the case of a 7-month-old boy, born in Paraguay, who was admitted at our Hospital with fever and hepatosplenomegaly, who after reaching the diagnosis, received specific treatment and developed a favorable clinical outcome

Impacto de la Terapia de Alto Flujo de Oxigeno (TAFO) en un Hospital Pediátrico de la Provincia de Buenos Aires / Impact of High Flow Oxygen Therapy (TAFO) in a Pediatric Hospital of the Province of Buenos Aires

Introducción: Las infecciones respiratorias agudas bajas (IRAB), representan la causa más frecuente de consulta e internación en los meses de invierno. La insuficiencia respiratoria aguda es la complicación que motiva la internación de los pacientes y la necesidad de Unidad Terapia Intensiva (UTI).El objetivo del trabajo fue describir los resultados de la implementación de la Terapia de Alto Flujo (TAFO) en pacientes con IRAB grave internados en Terapia Intermedia Métodos: Estudio prospectivo y descriptivo que incluyó pacientes de 1 a 36 meses internados en Terapia Intermedia en el Hospital Sor María Ludovica de la ciudad de La Plata, desde junio de 2018 a septiembre de 2019. Se ingresaron a TAFO pacientes sin respuesta al tratamiento con oxígeno a bajo flujo. El ingreso a UTI se consideró fracaso de la TAFO Resultados: De 760 pacientes internados con IRAB, 91(11,9%) ingresaron a TAFO de los cuales 59 (64,8 %) tuvieron respuesta favorable con disminución de la frecuencia respiratoria (FR), frecuencia cardiaca (FC) y mejoría de la mecánica respiratoria; el resto (35,2%) pasó a UTI por fracaso terapéutico. Presentaron complicaciones a la TAFO el 5,5% de los pacientes Conclusión: La TAFO fue una terapéutica segura, de fácil utilización que, a través de un aporte de oxígeno conocido, permitió la corrección de la hipoxemia, logrando la disminución de la FR, FC y mejoría de la mecánica respiratoria, dándole mayor comodidad al paciente durante su enfermedad

Introduction: Respiratory infections remain the major cause of outpatient consultation and hospital admissions during the winter season. Lower respiratory illness may cause severe acute respiratory insufficiency and hypoxemic respiratory failure, thus determining the need for hospitalization and eventual intensive care (ICU). The purpose of this paper is to describe the results of High Flow Oxygen Therapy (HFOT) implementation for patients with acute lower respiratory infections (ALRI) admitted to intermediate therapy unit. Methods: Prospective and descriptive study which included patients from age 1 to 36 months, hospitalized at intermediate therapy care unit at "Sor María Ludovica", Hospital, in La Plata, from June, 2018 to September, 2019. Patients who did not show any improvement to low flow oxygen therapy were the subjects of this study. Further submission to ICU was considered as HFOT failure Results: From 760 patients hospitalized with ALRI, 91 (11.9%) were admitted to TAFO. Fifty nine, (64.8%) had a favorable response with decreased respiratory and heart frequency rate, and an improvement of the work of breathing. The rest (35.1%) went to ICU due to therapeutic failure. Five point five percent of patients presented complications to TAFO. Conclusion: HFOT was a safe, easy to implement therapy treatment which improved the hypoxemic respiratory failure. This therapy reduced the respiratory and heart rate, and yielded a better and lower respiratory work, making patients more comfortable during illness

Transcriptional regulation of fatty acid biosynthesis in mycobacteria.

The main purpose of our study is to understand how mycobacteria exert control over the biosynthesis of their membrane lipids and find out the key components of the regulatory network that control fatty acid biosynthesis at the transcriptional level. In this article we describe the identification and purification of FasR, a transcriptional regulator from Mycobacterium sp. that controls the expression of the fatty acid synthase (fas) and the 4-phosphopantetheinyl transferase (acpS) encoding genes, whose products are involved in the fatty acid and mycolic acid biosynthesis pathways. In vitro studies demonstrated that fas and acpS genes are part of the same transcriptional unit and that FasR specifically binds to three conserved operator sequences present in the fas-acpS promoter region (Pfas). The construction and further characterization of a fasR conditional mutant confirmed that FasR is a transcriptional activator of the fas-acpS operon and that this protein is essential for mycobacteria viability. Furthermore, the combined used of Pfas-lacZ fusions in different fasR backgrounds and electrophoretic mobility shift assays experiments, strongly suggested that long-chain acyl-CoAs are the effector molecules that modulate the affinity of FasR for its DNA binding sequences and therefore the expression of the essential fas-acpS operon.

Transcriptional regulation of lipid homeostasis in mycobacteria.

Mycolic acids are major components of the cell envelope of mycobacteria, such as Mycobacterium tuberculosis, and play an important role in its architecture, impermeability and interaction with the environment. Synthesis of mycolic acids is carried out by two types of fatty acid synthases (FAS) working in concert: type I FAS, a multifunctional enzyme capable of de novo synthesis of medium-chain fatty acids, and type II FAS, responsible for their elongation. In this article we report the identification and characterization of a transcriptional regulator (MabR), whose binding to the FAS-II promoter region was demonstrated in vitro and in vivo. Overexpression and knock-down studies in Mycobacterium smegmatis revealed the repressor nature of MabR, with reduced amounts of FAS-II transcripts and fatty acids in the overproducing strain. Under these conditions, downregulation of fas transcription was also observed, thereby suggesting the existence of cross-talk between the two FAS, mediated by MabR. Finally, the finding that a mabR knock-out mutant could only be obtained in a merodiploid strain of M. smegmatis, confirmed the predicted essentiality, thus implying an essential role for MabR in mycobacterial fatty acid metabolism.

Morphometric characterization and classification of alpaca sperm heads using the sperm-class analyzer computer-assisted system.

Sperm morphology has been identified as one characteristic which can be useful in the prediction of sperm fertility, therefore, we hope that this study aimed at establishing standardized morphological criteria might serve in future studies dealing with the search for sperm parameters which facilitate an estimation of sperm quality. For this purpose, ejaculates from fertile alpacas were used to evaluate sperm head morphometry by means of the Sperm-Class Analyzer (SCA) computer-aided image analysis system. We defined three morphological categories according to sperm head size (normal 50%, small 26%, large 24%) and five categories according to sperm head shape (normal 47%, pyriform 3%, short 20%, round 1%, long 29%). Sperm classification according to shape was performed by first morphometrically characterizing sperm heads clearly falling into each of the shape categories. Thereafter, discriminant analysis was performed on the data from these typical sperm heads and the resulting classification functions were used to categorize 2,200 spermatozoa from 11 alpacas. Classification of sperm heads by this method agreed in 88% of the cases with most of the misclassifications being due to pyriform heads classified as long heads. Morphometric values obtained from samples of 50, 100, 150, 175 and 200 sperm heads were compared. At least 150 sperm heads should be evaluated to overcome sample size influence on sperm measurements. Significant differences in sperm morphometry were found between individuals (CV for morphometric parameters ranging from 1.3 to 13.0) and there were marked differences in the sperm morphological composition of the ejaculates. Within-animal CV ranged from 4.7 to 17.8 thus showing the high degree of sperm polymorphism present in the alpaca ejaculate.

Homeoboxes in plant development.

The homeobox is a 180 bp consensus DNA sequence present in a number of genes involved in developmental processes. This review focuses on the structure and function of plant homeobox genes and of the proteins they encode. Plant homeobox genes have been identified in studies using mutants, degenerate oligonucleotides deduced from conserved sequences, differential screening or binding to known promoters. According to sequence conservation, plant homeoboxes can be subdivided into different families, each comprising several members. Evolutionary studies indicate that the different families have diverged prior to the separation of the branches leading to animals, plants and fungi. Accordingly, members of different families show characteristic structural and functional properties. As an example, kn1-like genes seem to be involved in different aspects of the control of cell fate determination in the shoot meristem; HD-Zip genes, which encode proteins containing a leucine zipper motif adjacent to the homeodomain, are believed to operate at later stages of development; and gl2-like genes are involved in epidermal cell differentiation. Future studies should be oriented to discern the precise function of the many homeobox genes present in plant genomes, and to evaluate their use as modifiers of plant development.

Isolation and expression pattern of hahr1, a homeobox-containing cDNA from Helianthus annuus.

A 2.5 kb homeobox (HB)-containing cDNA (hahr1) was isolated from a library prepared from rootlets of Helianthus annuus using a polymerase chain reaction (PCR)-based strategy. The putative protein product (77 kDa) contains the homeodomain (HD) and an acidic domain at the N-terminal region (residues 72-155). The deduced amino acid sequence of hahr1 shares a 53% sequence identity with GLABRA2, a HD protein associated with epidermal cell differentiation. Hahr1 expression was primarily found in dry seeds, hypocotyls and roots at stages associated with early developmental events. Expression was completely lacking in leaves and flowers. Evidence for the existence of one related gene expressed in sunflower stems was obtained by the presence of restriction fragment length polymorphism of amplified cDNA products.

Antimutagenic activity of lettuce and chard extracts.

The ability of lettuce and chard extracts to reduce the mutagenic activity of Benzo[a]pyrene was studied. In this experiment several groups of male Balb/C mice were treated with different doses of the substances under study. B[a]p was administered at doses of 36 and 72 mg/kg of body weight to groups II and III, respectively. The lettuce extract combined with the doses of B[a]p were administered to groups IV and V. The experiment was performed again under the same conditions to test the effect of chard extract. Urine samples were tested by means of the Ames assay. The results show that the mutagenic activity of the urine samples from groups treated with B[a]p was reduced when the treatment also included any vegetable extract.

Binaural interaction in high-frequency neurons in inferior colliculus of the cat: effects of variations in sound pressure level on sensitivity to interaural intensity differences.

1. Development of models of the manner in which interaural intensity differences (IIDs), the major binaural cue for the azimuthal location of high-frequency sounds, are coded by populations of neurons requires knowledge of the extent to which the IID sensitivity of individual neurons is invariant with changes in sound pressure level (SPL) and other stimulus parameters. To examine this tissue, recordings were obtained from a large sample (n = 458) of neurons with characteristic frequency (CF) greater than 3 kHz in the central nucleus of the inferior colliculus (ICC) of anesthetized cats. The sensitivity to IIDs and the effects of changes in SPL on this sensitivity were examined in neurons receiving excitatory contralateral input and inhibitory or mixed inhibitory/facilitatory ipsilateral input (EI neurons). 2. The form of an EI neuron's IID sensitivity and the effects of changes in SPL on that sensitivity were found to be determined in part by the characteristics of the neuron's rate-intensity function for monaural contralateral stimulation, and detailed rate-intensity functions were therefore obtained for 91 neurons. Many ICC neurons have nonmonotonic rate-intensity functions, the proportion so classified depending on the criterion of nonmonotonicity employed. 3. IID sensitivity functions for CF tonal stimuli were obtained at one or more intensities for 90 neurons, using a method of generating IIDs that kept the average binaural intensity (ABI) of the stimuli at the two ears constant. In the standard ABI range in which a function was obtained for each unit, the majority of EI neurons (72%) had monotonic (sigmoidal) or near-monotonic IID sensitivity functions. The remainder had nonmonotonic (peaked) IID sensitivity functions, which were attributable either to mixed inhibitory and facilitatory ipsilateral influences or to the fact that the effects of ipsilateral stimulation were superimposed on nonmonotonic effects of changes in intensity at the excitatory ear. 4. IID sensitivity was examined at two or more ABIs (3-5 in most cases) for 40 neurons classified as having monotonic or near-monotonic functions in the standard ABI range and for 7 neurons classified as nonmonotonic. For a small proportion of neurons with monotonic IID sensitivity functions, the form of the function was relatively invariant with changes in ABI. In those monotonic neurons in which the form of the IID sensitivity function varied with changes in ABI, the most common type of variation was that the position of the sloping portion of the function shifted systematically in the direction of larger IIDs favoring the ipsilateral ear as ABI increased.(ABSTRACT TRUNCATED AT 400 WORDS)