Maf/ham1-like pyrophosphatases of non-canonical nucleotides are host-specific partners of viral RNA-dependent RNA polymerases.

Cassava brown streak disease (CBSD), dubbed the "Ebola of plants", is a serious threat to food security in Africa caused by two viruses of the family Potyviridae: cassava brown streak virus (CBSV) and Ugandan (U)CBSV. Intriguingly, U/CBSV, along with another member of this family and one secoviridae, are the only known RNA viruses encoding a protein of the Maf/ham1-like family, a group of widespread pyrophosphatase of non-canonical nucleotides (ITPase) expressed by all living organisms. Despite the socio-economic impact of CDSD, the relevance and role of this atypical viral factor has not been yet established. Here, using an infectious cDNA clone and reverse genetics, we demonstrate that UCBSV requires the ITPase activity for infectivity in cassava, but not in the model plant Nicotiana benthamiana. HPLC-MS/MS experiments showed that, quite likely, this host-specific constraint is due to an unexpected high concentration of non-canonical nucleotides in cassava. Finally, protein analyses and experimental evolution of mutant viruses indicated that keeping a fraction of the yielded UCBSV ITPase covalently bound to the viral RNA-dependent RNA polymerase (RdRP) optimizes viral fitness, and this seems to be a feature shared by the other members of the Potyviridae family expressing Maf/ham1-like proteins. All in all, our work (i) reveals that the over-accumulation of non-canonical nucleotides in the host might have a key role in antiviral defense, and (ii) provides the first example of an RdRP-ITPase partnership, reinforcing the idea that RNA viruses are incredibly versatile at adaptation to different host setups.

Alteraciones genéticas cutáneas diagnosticadas en la infancia Las Tunas 2010-2012 / Skin genetic alterations diagnosed in childhood, Las Tunas 2010 to 2012

Las enfermedades genéticas y los defectos genéticos con afectación cutánea son causa frecuente de estigmatización de las personas que las padecen, afectando su adecuada inserción en la sociedad y con ello su calidad de vida. Se estableció una consulta especializada y multidisciplinaria en el hospital pediátrico provincial Mártires de Las Tunas, fundamentalmente con especialistas de dermatología, genética y pediatría. Se realizó un estudio descriptivo, transversal y prospectivo, en el período del año 2010 al 2012, para caracterizar a estos pacientes. Las variables estudiadas relacionadas fueron el sexo, la edad biológica al diagnóstico, el municipio de procedencia y la mortalidad. Los resultados se expusieron en tablas de contingencia de N x N. Existió predominio de las genodermatosis, seguido de los defectos congénitos y pocas enfermedades genéticas con afectación cutánea, siendo estas últimas responsables de la mayoría de las muertes. Las genodermatosis más representadas fueron la ictiosis, las mastocitosis y las neurofibromatosis, la edad más frecuente al diagnóstico fue el grupo de los menores de dos años; prevaleció el sexo femenino. La mortalidad fue baja y los municipios más afectados por genodermatosis fueron Las Tunas, Majibacoa y Colombia (AU)

The genetic diseases and genetic defects with skin affectation are frequently a cause of stigmatizing people that suffer from them, consequently affecting their appropriate integration to society and their life quality. A specialized and multidisciplinary consultation, mainly made up of dermatology, genetic and pediatric specialists, was established in Mártires de Las Tunas Pediatric Hospital. A descriptive, cross sectional and prospective study was carried out from 2010 to 2012 to characterize those patients. The variables used were: sex, biological age at diagnosis, proceeding municipality and mortality. The results were exposed in NX N contingency tables. There was a prevalence of genodermatosis, followed by congenital defects and only a few genetic diseases with skin upset, being the latter the responsible of all deaths. The most represented genodermatosis were ichthyosis, mastocytosis and neurofibromatosis; the group of less than two years old was the most frequent age at diagnosis; females prevailed. Mortality rate was low, and the most affected municipalities due to genodermatosis were Las Tunas, Majibacoa and Colombia (AU)

Síndrome de Stevens Jonson: presentación de un caso en edad pediátrica / Stevens Johnson Syndrome: a Child Case Report

El síndrome de Stevens-Johnson (SSJ) es una variante severa del eritema multiforme, consistente en una reacción de hipersensibilización que afecta piel y mucosa, además de otros órganos internos, y que se manifiesta con lesiones en zonas genitales, bucales y cutáneas, es idiopático en el 50 por ciento de los casos, generalmente producido por alergia a medicamentos. La muerte sobreviene en el 5-15 por ciento de los casos sin tratar. El riesgo de recurrencia es del 37 por ciento o más. Al Hospital Pediátrico Provincial Docente Mártires de Las Tunas fue traído un niño de seis años de edad, que tras el uso del Sulfaprín comenzó con lesiones que afectaban la piel y todas las mucosas, además de toma del estado general. Inmediatamente fue admitido en la Unidad de Cuidados Intensivos Pediátricos, donde fueron tomadas medidas que incluyeron supresión del agente causal, corticoterapia endovenosa, antibioticoterapia endovenosa, tratamiento de las lesiones, vigilancia del balance hidromineral y atención oftalmológica, respondiendo favorablemente. Se presenta el caso, por ser una enfermedad poco frecuente y de muy mal pronóstico (AU)

The Stevens Johnson syndrome is a variant of erythema multiforme, consisting of a hypersensitive reaction that affects the skin, mucosa and other internal organs, also manifested with lesions in the genital area, around the mouth and in the skin. It is idiophatic in 50 per cent of the cases, generally produced by allergies to medications. Without treatment, 5 to 15 per cent of patients die. The risk of recurrence is about 37 per cent or more.A 6- year- old child was brought to Mártires de Las Tunas Pediatric Teaching Hospital when some lesions appeared in his skin and mucus lining, and he had general malaise, after he began to be treated with cotrimoxazol. He was immediately admitted to the Intensive Care Unit, where measures including: suppression of the causative agent, intravenous corticosteroid therapy, treatment of the skin lesions, surveillance of the hidromineral balance and ophthalmologic attention, were taken. He responded favourably to the treatment. This case is reported because it is rare and it usually has an unfavourable prognosis (AU)

New therapeutic approach to Tourette Syndrome in children based on a randomized placebo-controlled double-blind phase IV study of the effectiveness and safety of magnesium and vitamin B6.

BACKGROUND: Tourette Syndrome (TS) is a neurological condition presenting chronic motor and phonic tics, and important degree of comorbidity. Considered an uncommon illness, it first becomes apparent during childhood. Current standard treatment only achieves partial control of the condition, and provokes frequent, and sometimes severe, side effects. METHODS AND DESIGN: Main aim: To show that, with respect to placebo treatment, the combination of 0.5 mEq/Kg magnesium and 2 mg/Kg vitamin B6 reduces motor and phonic tics and incapacity in cases of exacerbated TS among children aged 7-14 years, as measured on the Yale Global Tic Severity Scale (YGTSS). Secondary aims: Assess the safety of the treatment. Describe metabolic changes revealed by PET. Measure the impact of the experimental treatment on family life. METHODOLOGY: Randomized, blinded clinical trials. Phase IV study (new proposal for treatment with magnesium and vitamin B6). SCOPE: children in the geographic area of the study group. Recruitment of subjects: to include patients diagnosed with TS, in accordance with DSM-IV criteria (307.23), during a period of exacerbation, and provided none of the exclusion criteria are met. INSTRUMENTATION: clinical data and the YGTSS score will be obtained at the outset of a period of exacerbation (t0). The examinations will be made after 15 (t1), 30 (t2), 60 (t3) and 90 days (t4). PET will be performed at the t0 and t4. We evaluated decrease in the overall score (t0, t1, t2, t3, t4), PET variations, and impact made by the treatment on the patient's life (Psychological General Well-Being Index). DISCUSSION: Few clinical trials have been carried out on children with TS, but they are necessary, as current treatment possibilities are insufficient and often provoke side effects. The difficulty of dealing with an uncommon illness makes designing such a study all the more complicated. The present study seeks to overcome possible methodological problems by implementing a prior, phase II study, in order to calculate the relevant statistical parameters and to determine the safety of the proposed treatment. Providing a collateral treatment with magnesium and vitamin B6 could improve control of the illness and help reduce side effects. This protocol was approved by the Andalusian Government Committee for Clinical Trials (Spain). This study was funded by the Health Department of the Andalusian Regional Government and by the Healthcare Research Fund of the Carlos III Healthcare Institute (Spanish Ministry of Health). TRIAL REGISTRATION: Current Controlled Trials ISRCTN41082378.

[An open study evaluating the efficacy and security of magnesium and vitamin B(6) as a treatment of Tourette syndrome in children]. / Estudio piloto sin grupo control del tratamiento con magnesio y vitamina B(6) del síndrome de Gilles de la Tourette en niños.

BACKGROUND AND OBJECTIVE: We intended to ascertain the effectiveness and safety of oral solutions of magnesium and vitamin B(6) in alleviating the symptoms emerged during clinical exacerbations in children aged 7-14 years suffering from Tourette syndrome (TS). We also aimed to determine the mean and the standard deviation of such an improvement in order to estimate sample sizes in future assays with a control group. PATIENTS AND METHOD: The treatment under investigation was administered to children diagnosed with TS, in accordance with Diagnostic and Statistical Manual of Mental Disorders, fourth edition -IV, under conditions of clinical exacerbation. The effects were scored on the Yale Global Tics Severity Scale (YGTSS) at 0, 15, 30, 60 and 90 days. RESULTS: The total tics score decreased from 26.7 (t0) to 12.9 (t4) and the total effect on the YGTSS was a reduction from 58.1 to 18.8. Both results were statistically significant. With respect to the application of conventional treatment or otherwise, no significant differences were observed. No side effects were seen. CONCLUSIONS: The treatment assayed is safe and effective in reducing the harmful effects of TS in children. Further studies are needed, with a control group, and evaluation of different doses of the drugs.

Estudio piloto sin grupo control del tratamiento con magnesio y vitamina B6 del síndrome de Gilles de la Tourette en niños / An open study evaluating the efficacy and security of magnesium and vitamin B6 as a treatment of Tourette syndrome in children

FUNDAMENTO Y OBJETIVO: Estudiar la efectividad y seguridadde soluciones orales de magnesio y vitaminaB6 para disminuir los síntomas durante las exacerbacionesclínicas en niños de entre 7 y 14 añoscon síndrome de Gilles de la Tourette (SGT). Determinarla mejoría en la puntuación y su desviaciónestándar para estimar el tamaño muestral de futurosensayos con grupo control.PACIENTES Y MÉTODO: El tratamiento estudiado se administróa niños diagnosticados de SGT de acuerdocon el Manual Diagnóstico y Estadística de losTrastornos Mentales, cuarta edición, durante unaexacerbación clínica. La clínica se cuantificó mediantela Yale Global Tics Severity Scale (YGTSS) alos 0, 15, 30, 60 y 90 días de tratamiento.RESULTADOS: La puntuación de los tics pasó de 26,7(inicio) a 12,9 (a los 90 días de tratamiento) y lapuntuación total de la YGTSS pasó de 58,1 a18,8. Estos resultados fueron estadísticamente significativos.Con respecto a la aplicación o no detratamiento convencional, no observamos diferenciassignificativas en los resultados obtenidos. Nose registraron efectos secundarios.CONCLUSIONES: El tratamiento analizado es seguro yefectivo para reducir los síntomas del SGT en niños.Es necesario realizar estudios con un grupocontrol y diferentes dosis de fármacos para confirmarestos resultados

BACKGROUND AND OBJECTIVE: We intended to ascertainthe effectiveness and safety of oral solutions ofmagnesium and vitamin B6 in alleviating the symptomsemerged during clinical exacerbations in childrenaged 7-14 years suffering from Tourette syndrome(TS). We also aimed to determine the meanand the standard deviation of such an improvementin order to estimate sample sizes in future assayswith a control group.PATIENTS AND METHOD: The treatment under investigationwas administered to children diagnosed withTS, in accordance with Diagnostic and StatisticalManual of Mental Disorders, fourth edition -IV, underconditions of clinical exacerbation. The effectswere scored on the Yale Global Tics Severity Scale(YGTSS) at 0, 15, 30, 60 and 90 days.RESULTS: The total tics score decreased from 26.7(t0) to 12.9 (t4) and the total effect on the YGTSSwas a reduction from 58.1 to 18.8. Both resultswere statistically significant. With respect to theapplication of conventional treatment or otherwise,no significant differences were observed. No sideeffects were seen.CONCLUSIONS: The treatment assayed is safe and effectivein reducing the harmful effects of TS inchildren. Further studies are needed, with a controlgroup, and evaluation of different doses of thedrugs

Vía intraósea como alternativa en pacientes con deshidratación grave / Intrabony way as an alternative in patients with serious dehydration

El establecimiento de un acceso a la circulación es imprescindible en pacientes con deshidratación grave. Se realizó un estudio prospectivo, de intervención en el Hospital de Fort-Liberté y Hospital Pediátrico Provincial Docente Mártires de Las Tunas, en el período comprendido entre Agosto 2002 Noviembre 2004 y Diciembre 2004 Junio 2006 respectivamente. El grupo de estudio estuvo constituido por 64 pacientes con deshidratación grave a los cuales no se le había podido acceder a una vía venosa periférica en los primeros 5 minutos y a los cuales se decidió introducirle una vía intraósea, mientras que el grupo control quedó formado por 64 pacientes con las mismas características clínicas, pero que se hidrataron por la vía intravenosa, como vía electiva. En ambos grupos había 48 pacientes menores de un año. Las variables que se analizaron fueron: tiempo de acceso a la circulación, velocidad de la infusión en cc/kg/hr, tiempo de corrección de los trastornos de perfusión y complicaciones. Los resultados fueron analizados por métodos estadísticos simples y representados en gráficos. Se concluyó que la vía intraósea fue abordada mas rápidamente, la velocidad del goteo de la infusión fue similar en ambas vías y las complicaciones no contraindican la vía intraósea, resultando ser esta tan efectiva como la vía intravenosa y que como se puede obtener con mayor rapidez se recomienda se extienda su uso como vía alternativa en todos los pacientes críticos donde no se puede canalizar rápidamente una vía venosa(AU)

The establishment of an access to the circulation is essential in patients with serious dehydration. A longitudinal study of intervention was made. It began in August 2002 November 2004 in Fort-Liberte (Haiti) and ended at “Mártires de Las Tunas” Children's Hospital of December 2004 - June 2006. The training group was constituted by 64 patients with serious dehydration to whom it had not been possible to access to a peripheral venous way in the first 5 minutes and to whom it was decided to introduce an intrabony way. The control group was formed by 64 patients with the same clinical characteristics, but that were hydrated by the intravenous way, as an elective way. In both groups there were 48 patients under one year old. The analyzed variables were: access time to the circulation, speed of the infusion in cc/kg/hr, time of correction of the disturbances of perfusion and complications. The results were analyzed by simple and statistical methods represented in graphs. It was concluded that the intrabony way was used quicker, the speed of the dripping of the infusion was similar in both ways and complications do not contraindicate the intrabony way, turning out to be this as effective as the intravenouse way, and that as it is possible to be obtained faster it is recommended to extend its use as an alternative way in all the critical patients where a venous way cannot be canalized quickly(AU)

Accidentes en el hogar / House accidents

Se realiza un estudio epidemiológico transversal de 185 padres de niños que acudieron al Hospital Pediátrico Provincial Docente Mártires de Las Tunas, que presentaron un accidente en el hogar, en el período de tiempo comprendido entre enero y diciembre del 2002, con el objetivo de determinar algunos factores que influyen en la aparición de los mismos en nuestro medio. Los niños entre 1-4 años fueron los más afectados, así como los del sexo masculino. El dormitorio y la cocina fueron los lugares donde más ocurrieron. El horario en que más ocurrieron fue entre 8 y 11:59 AM. Las intoxicaciones fueron las causas más frecuentes de accidentes(AU)

A transversal epidemiological study was made in 185 parents of children who came to the emergency room of the Provincial Pediatric Hospital Mártires de Las Tunas presenting a home accident, during the period from January to December of 2002, with the objective to determine some factors that have influences in the appearance of those accidents in our enviroment. Children from 1 to 4 years old were most affected, as soon as male sex. Bedrooms and kitchens were the places in which ocurred most accidents. Horary in which happened more accidents was 8:00 AM to 11:59 AM . Intoxications were the most frequently causes of accidents(AU)

Factores de riesgos de gravedad por accidentes en niños / Risk factors for graveness by accidents in children

Se presentan los resultados de un estudio sobre posibles factores de riesgo para ingreso en una Terapia Intensiva Pediátrica por accidentes. Se tomaron 91 niños que ingresaron en la Terapia Intensiva del Hospital Pediátrico Docente Provincial Mártires de Las Tunas, todos por accidentes graves, en el período de enero de 1999 a diciembre del 2000, y se compararon con 91 niños que ingresaron por accidentes en otras salas del hospital. Se evaluaron diversas variables como posibles factores de riesgo. Para el análisis estadístico se agruparon los datos en tablas de contingencia 2x2, y se calculó el riesgo, con límite de confianza superior al 95 por ciento. Se constataron como factores asociados: sexo masculino, accidente fuera del hogar, edad mayor de un año, estar acompañado de otros niños, escolaridad de la madre menos de noveno grado, vivir solo con un padre y el tiempo transcurrido para recibir atención médica después de una hora de haber ocurrido el accidente. Como factores protectores: procedencia urbana, vacaciones escolares y madre ama de casa; y como factores sin asociación: tipo de accidente(AU)

The results of a study about possible factors of risk to hospitalize children in a Pediatric Intensive Therapy due to accidents are presented here. Ninety one children hospitalized in the provincial Pediatrics Educational Hospital Mártires de Las Tunas, were taken as the sample, all of them due to serious accidents January 1999 to December 2000, are compared with ninety one children hospitalized for accidents in other rooms of the hospital. Diverse variants were evaluated as possible factors of risk. For the statistical analysis, the facts were grouped in contingency charts of 2x2; the risk was calculated in a confidence limit over 95 per cent. As associated factors were established: male sex, out of home accidents, more than one year old of age, being in company with other child, mother's scholoship of then 9 th grade, living alone with a father, and a time of more than an hour offer accidents to receive medical attention. As protective factors: urban origin, vacations, mother as housewife; and as associative factors: type of accident and surgical treatment(AU)

Meningoencefalitis Bacteriana por Haemophilus Influenzae / Bacterial Meningoencephalitis by Haemophilus Influenzae

Se realiza un estudio epidemiológico, retrospectivo y descriptivo de 134 niños egresados del Hospital Pediátrico Docente Provincia Mártires de Las Tunas, en el período comprendido de enero de 1985 hasta diciembre del 2000, con el objetivo de evaluar el comportamiento clínico epidemiológico de las meningoencefalitis bacterianas por Haemophilus influenzae en nuestro medio. La enfermedad predomina en el sexo masculino y en menores de 5 años, y más frecuentemente en el grupo de 1 4 años. Se encontró relación directa entre la lactancia artificial y la entidad. En la mayor parte de nuestros pacientes se encontraron síntomas respiratorios previos. Las principales complicaciones estuvieron dadas por el edema cerebral y el shock séptico; los antibióticos con mejores resultados fueron las cefalosporinas de tercera generación y el cloranfenicol(AU)

A retrospective and descriptive epidemiological study of 134 children discharged from the Paediatric Educational Provincial Hospital Martires de Las Tunas, was carried out during the period from January 1985 to December 2000 with the objetive of evaluating the clinicolepidemiological be haviour of the bacterial meningoencephalitis by haemophilus influenzae in our medium. There was a predominance of the rickness in younger than five years old males and more frequently in the group oh 1 to 4 years old. A direct relation betweon the artificial lactation and the entity. In the mejority oy our patients, previous respiratory symptoms were found. The main complications were cerebral aedema and septical shock; the antibiotics with the best results were the third generation cephalosporines and chloranfenicol(AU)