Cierre percutáneo de una malformación arteriovenosa pulmonar / Percutaneous treatment of pulmonary arteriovenous malformation

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La genómica al servicio de la pediatría en el estudio de la enfermedad multifactorial / The genomics serving pediatrics in the investigation of the multifactorial disease

En estos últimos años hemos asistido al enorme desarrollo de la genómica y sus aplicaciones en diversos campos de la biomedicina. Esto ha sido en gran parte posible gracias al gran empuje tecnológico que han experimentado las nuevas técnicas de genotipado y de ultra secuenciación. Sin duda, el estudio de la enfermedad compleja ha sido uno de los grandes beneficiados de este gran desarrollo. Asimismo, existen muchas enfermedades pediátricas que pueden abordarse siguiendo las estrategias que, hoy por hoy, se emplean en la genómica de la enfermedad multifactorial. Esta revisión pretende acercar a la pediatría las posibilidades que ofrece la genómica de hoy en día en este campo. Para ello, se discuten los distintos modelos de estudios, así como los problemas que a menudo surgen cuando los diseños experimentales son deficientes (AU)

The last few years have experienced an important growth of genomics and its different applications in biomedicine. In part, this has been possible due to the sudden development of the new genotyping technology and ultra-sequencing. The study of the genetic basis of the complex disease has been the main beneficiary of such technological development. There are a good number of pediatric diseases that can be approached following the same strategies employed in the genomic study of the multi-factorial disease. Thus, the present article aims to review the different applications of genomics to Pediatrics as well as to discuss the different strategies available and the preventions needed to avoid false positive associations (AU)

Cumplimiento de las pautas de profilaxis de la endocarditis tras el cierre percutáneo de la comunicación interauricular / Fulfillment of the prevention of endocarditis guidelines after percutaneous closure of atrial septal defects

Introducción: Tras la implantación de un dispositivo de cierre del tabique interauricular, la mayoría de los centros llevan a cabo pautas de profilaxis antibiótica que disminuyan el riesgo de endocarditis infecciosa secundario a la presencia de un cuerpo extraño intracardíaco. Actualmente se suelen recomendar durante los primeros 6 meses tras la implantación, tras los que deja de llevarse a cabo siempre que no exista shunt residual. El objetivo de este estudio es valorar el grado de conocimiento, cumplimiento y adherencia a las recomendaciones de nuestro centro sobre la profilaxis de la endocarditis en los progenitores de pacientes a los que se les realizó un cierre percutáneo de una comunicación interauricular (CIA). Pacientes y métodos: Se ha realizado un estudio observacional retrospectivo de los 51 pacientes pediátricos a los que se les realizó un cierre percutáneo de CIA en el Complejo Hospitalario Universitario de La Coruña, entre los años 1999 y 2008.ResultadosSe estudió un total de 51 pacientes, con una media de seguimiento de 57,2 meses. El 75,7% de los progenitores conocía la profilaxis de la endocarditis, y este porcentaje fue mayor cuanto menos tiempo había pasado desde la intervención y cuanto mayor era el nivel de estudios de los padres. El 50% nunca dejó de llevar a cabo la profilaxis de la endocarditis. También se observó, en este caso, una relación con el nivel de estudios de los padres y con el tiempo que había pasado desde la intervención (p=0,004). El 73% de los pacientes nunca ha tenido que llevar a cabo la profilaxis de la endocarditis. Conclusiones: Las últimas guías sobre profilaxis antibiótica de la endocarditis son cada vez más restrictivas en cuanto a sus indicaciones a fin de promocionar un uso racional de los antibióticos. Son necesarios estudios más amplios con respecto a las indicaciones de la profilaxis antibiótica para la endocarditis en pacientes portadores de dispositivos intracardíacos a fin de establecer unas pautas concretas o basadas en la evidencia científica. Mientras tanto, es nuestra responsabilidad evitar la aplicación indiscriminada de antibióticos, haciendo partícipes a progenitores y a otros profesionales de la salud (AU)

Introduction: After the implantation of an intracardiac device for the closure of an atrial septal defect, most centres follow the guidelines for antibiotic prophylaxis to reduce the risk of infectious endocarditis, at least during the first 6 months after the implantation, if there is no evidence of residual shunt. The aim of this report is to evaluate the knowledge, fulfilment and adherence to the recommendations of our centre on the prevention of endocarditis, of the families of patients subjected to percutaneous closure of an ASD. Patients and Methods: We performed an observational retrospective study of 51 paediatric patients subjected to percutaneous closure of an ASD in the “Complejo Hospitalario Universitario de La Coruña”, between 1999 and 2008. Results: A total of 51 procedures were performed, with an average of follow-up of 57.2 months. 75.7% of the families knew about the prophylaxis of endocarditis. This percentage was higher if less time had passed since the intervention and was also higher depending on the educational level of the parents. A total of 50% never stopped carrying out the endocarditis prophylaxis. In this case, a relationship was also observed, with the educational level of the parents and with the time passed since the intervention (P=0.004). The majority (73%) of the patients never had to carry out endocarditis prophylaxis. Conclusiones: The latest guidelines on antibiotic prophylaxis of endocarditis are increasingly restrictive in their indications in order to promote a more rational use of antibiotics. More studies are needed on the indications of antibiotic prophylaxis in endocarditis in patients with an intracardiac device, in order to establish concrete or evidence-based guidelines. Meanwhile, it is our responsibility to avoid the indiscriminate application of antibiotics, and involve the families and other health professionals (AU)

[Fulfillment of the prevention of endocarditis guidelines after percutaneous closure of atrial septal defects]. / Cumplimiento de las pautas de profilaxis de la endocarditis tras el cierre percutáneo de la comunicación interauricular.

INTRODUCTION: After the implantation of an intracardiac device for the closure of an atrial septal defect, most centres follow the guidelines for antibiotic prophylaxis to reduce the risk of infectious endocarditis, at least during the first 6 months after the implantation, if there is no evidence of residual shunt. The aim of this report is to evaluate the knowledge, fulfillment and adherence to the recommendations of our centre on the prevention of endocarditis, of the families of patients subjected to percutaneous closure of an ASD. PATIENTS AND METHODS: We performed an observational retrospective study of 51 paediatric patients subjected to percutaneous closure of an ASD in the "Complejo Hospitalario Universitario de La Coruña", between 1999 and 2008. RESULTS: A total of 51 procedures were performed, with an average of follow-up of 57.2 months. 75.7% of the families knew about the prophylaxis of endocarditis. This percentage was higher if less time had passed since the intervention and was also higher depending on the educational level of the parents. A total of 50% never stopped carrying out the endocarditis prophylaxis. In this case, a relationship was also observed, with the educational level of the parents and with the time passed since the intervention (P=0.004). The majority (73%) of the patients never had to carry out endocarditis prophylaxis. CONCLUSIONS: The latest guidelines on antibiotic prophylaxis of endocarditis are increasingly restrictive in their indications in order to promote a more rational use of antibiotics. More studies are needed on the indications of antibiotic prophylaxis in endocarditis in patients with an intracardiac device, in order to establish concrete or evidence-based guidelines. Meanwhile, it is our responsibility to avoid the indiscriminate application of antibiotics, and involve the families and other health professionals.

Trisomía parcial del cromosoma 5p / Partial trisomy of chromosome 5p

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[Ingestion of foreign bodies containing lead]. / Ingestión de cuerpos extraños de plomo.

Ingestion of a foreign body containing lead in children carries the additional risk of acute lead poisoning secondary to dissolution and absorption of the ingested lead in the acid environment of the stomach. We report the case of a 3-year-old girl who ingested a lead sinker. The patient was asymptomatic but therapy with a proton pump inhibitor (omeprazole) was empirically started on admission and the foreign body was removed from the stomach by emergency endoscopy within 6 hours of ingestion. Despite the quick removal, blood lead levels (drawn 3 hours after ingestion) exceeded 40 .g/dl. The patient completely recovered and continues to do well after 1 year of follow-up. This case reveals that acute elevations of blood lead concentrations may occur rapidly after ingestion of lead foreign bodies. Even in the absence of symptoms, lead foreign body ingestion in children should prompt lead screening and initiation of appropriate treatment.

Ingestión de cuerpos extraños de plomo / Ingestion of foreign bodies containing lead

La ingestión de cuerpos extraños de contenido plúmbico tiene el riesgo adicional de ocasionar una intoxicación aguda por plomo secundaria a la disolución y absorción del plomo ingerido en el ambiente ácido del estómago. Se presenta el caso de una niña de 3 años que ingirió una plomada de pesca. La paciente estaba asintomática, pero se inició tratamiento empírico con un inhibidor de bomba de protones (omeprazol) a su llegada a urgencias y el cuerpo extraño se extrajo urgentemente mediante endoscopia a las 6 h de su ingestión. A pesar de la rápida actuación, los valores de plomo en sangre (obtenidos a las 3 h de la ingesta) excedieron los 40 μg/dl. La paciente se recuperó por completo y continúa sana tras un año de seguimiento. Este caso revela que tras la ingesta de cuerpos extraños plúmbicos se puede producir una elevación rápida de los valores de plomo en sangre. Incluso en ausencia de síntomas, ante la ingestión de cuerpos extraños con contenido plúmbico en pacientes pediátricos, debe evaluarse el nivel de plomo en sangre e instaurarse tratamiento adecuado

Ingestion of a foreign body containing lead in children carries the additional risk of acute lead poisoning secondary to dissolution and absorption of the ingested lead in the acid environment of the stomach. We report the case of a 3-year-old girl who ingested a lead sinker. The patient was asymptomatic but therapy with a proton pump inhibitor (omeprazole) was empirically started on admission and the foreign body was removed from the stomach by emergency endoscopy within 6 hours of ingestion. Despite the quick removal, blood lead levels (drawn 3 hours after ingestion) exceeded 40 μg/dl. The patient completely recovered and continues to do well after 1 year of follow-up. This case reveals that acute elevations of blood lead concentrations may occur rapidly after ingestion of lead foreign bodies. Even in the absence of symptoms, lead foreign body ingestion in children should prompt lead screening and initiation of appropriate treatment

Traqueítis bacteriana: una causa infecciosa de obstrucción de la vía aérea que hay que considerar en la infancia / Bacterial tracheitis: an infectious cause of upper airway obstruction to be considered in children

Introducción: La traqueítis bacteriana es una causa de obstrucción grave de la vía aérea superior que se considera poco frecuente y cuyas características clínico-epidemiológicas son poco conocidas. Pacientes y métodos: Se analizaron las historias clínicas de los niños diagnosticados de traqueítis bacteriana que ingresaron en nuestra unidad de cuidados intensivos pediátrica (UCIP) entre junio de 1992 y mayo de 2004 (12 años). Se recogieron las siguientes variables: edad, sexo, antecedentes personales de enfermedad, necesidad de intubación endotraqueal, días de intubación, bacteria aislada, tratamiento antibiótico, corticoterapia y días de estancia en la UCIP. Resultados: Se incluyeron 12 pacientes (6 niñas y 6 niños). El rango de edad osciló entre 1 mes y 13 años (mediana: 2 años). Tenían historia previa de laringotraqueítis aguda 4 niños, uno tenía un síndrome de Down y otro una comunicación interauricular. Todos los pacientes precisaron intubación orotraqueal, durante un período entre 2 y 72 h (mediana: 48 h). En todos ellos se utilizó antibioterapia empírica: cefalosporinas en 11 niños (91 %) (en cuatro ocasiones asociadas a un glucopéptido) y macrólido en un niño (9 %). Se emplearon corticoides en 9 casos (75 %). El cultivo del aspirado traqueal fue positivo en 7 niños (58 %), siendo Staphylococcus aureus el germen más frecuente (42 %). Tres niños (25 %) desarrollaron neumonía lobular y uno (8,3 %) insuficiencia cardíaca congestiva como complicación de la traqueítis bacteriana. La evolución clínica fue satisfactoria en todos los casos. Conclusiones: Aunque la incidencia absoluta de la traqueítis bacteriana es baja, en nuestro medio constituye una causa relevante de obstrucción grave de la vía aérea superior secundaria a un proceso infeccioso, por lo que debe tenerse en cuenta en el diagnóstico diferencial del paciente con estridor y fiebre. Si se realiza el tratamiento adecuado, el pronóstico en general es favorable

Introduction: Bacterial tracheitis is a cause of severe upper respiratory airway obstruction. It has been considered a rare entity whose clinical and epidemiological features are not well known. Patients and methods: The medical records of children diagnosed with bacterial tracheitis and admitted to our pediatric intensive care unit (PICU) from June 1992 to May 2004 (12 years) were analyzed. The following variables were recorded: age, sex, personal history, need for endotracheal intubation, duration of intubation, isolated bacteria, antibiotic treatment, steroid administration, and length of stay in the PICU. Results: Twelve patients (six girls and six boys) were included in this study. Age ranged from 1 month to 13 years (median 24 months). Four children had a history of acute laryngotracheitis, one had Down syndrome and another had interauricular communication. All patients needed orotracheal intubation for 2 to 72 hours (median 48 hours). All patients received empirical antibiotic therapy: cephalosporins in 11 children (91 %) (in association with vancomycin or teicoplanin in four of them) and erythromycin in one (9 %). Corticosteroids were administered in nine patients (75 %). Bacterial pathogens were isolated from tracheal secretion culture in seven children (58 %) and the most common was Staphylococcus aureus (42 %). Three children (25 %) developed lobar pneumonia and one developed congestive cardiac insufficiency as a complication of bacterial tracheitis. In all patients, clinical outcome was satisfactory. Conclusions: Although the absolute frequency of bacterial tracheitis is low, in our environment this entity represents a considerable cause of severe upper respiratory airway obstruction secondary to an infectious process. Therefore, it should be considered in patients who present with stridor and fever. If the correct treatment is provided, the prognosis is generally good

[Bacterial tracheitis: an infectious cause of upper airway obstruction to be considered in children]. / Traqueítis bacteriana: una causa infecciosa de obstrucción de la vía aérea que hay que considerar en la infancia.

INTRODUCTION: Bacterial tracheitis is a cause of severe upper respiratory airway obstruction. It has been considered a rare entity whose clinical and epidemiological features are not well known. PATIENTS AND METHODS: The medical records of children diagnosed with bacterial tracheitis and admitted to our pediatric intensive care unit (PICU) from June 1992 to May 2004 (12 years) were analyzed. The following variables were recorded: age, sex, personal history, need for endotracheal intubation, duration of intubation, isolated bacteria, antibiotic treatment, steroid administration, and length of stay in the PICU. RESULTS: Twelve patients (six girls and six boys) were included in this study. Age ranged from 1 month to 13 years (median 24 months). Four children had a history of acute laryngotracheitis, one had Down syndrome and another had interauricular communication. All patients needed orotracheal intubation for 2 to 72 hours (median 48 hours). All patients received empirical antibiotic therapy: cephalosporins in 11 children (91 %) (in association with vancomycin or teicoplanin in four of them) and erythromycin in one (9 %). Corticosteroids were administered in nine patients (75 %). Bacterial pathogens were isolated from tracheal secretion culture in seven children (58 %) and the most common was Staphylococcus aureus (42 %). Three children (25 %) developed lobar pneumonia and one developed congestive cardiac insufficiency as a complication of bacterial tracheitis. In all patients, clinical outcome was satisfactory. CONCLUSIONS: Although the absolute frequency of bacterial tracheitis is low, in our environment this entity represents a considerable cause of severe upper respiratory airway obstruction secondary to an infectious process. Therefore, it should be considered in patients who present with stridor and fever. If the correct treatment is provided, the prognosis is generally good.

Tricobezoar gástrico como forma de presentación atípica de enfermedad celíaca / Gastric trichobezoar as an atypical form of presentation of celiac disease

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[Burnout syndrome among health workers in pediatrics]. / Síndrome de desgaste profesional en el personal asistencial pediátrico.

BACKGROUND: Burnout syndrome (BS) is a chronic adaptation disorder that provokes serious problems in occupational behavior. OBJECTIVE: In the present study we assessed the prevalence of burnout syndrome in pediatric healthcare workers. DESIGN: Cross-sectional, descriptive study. MATERIAL AND METHODS: In November 2002, the Maslach Burnout Inventory was given to 127 staff members (doctors, nurses and nurse assistants) working in a pediatrics department. The questionnaire includes 22 items that explore three aspects of BS: a) emotional exhaustion; b) depersonalization, and c) personal achievement. RESULTS: The questionnaire was completed by 93 staff members (73.2 % of the whole sample; 83 % women and 17 % men). The mean age was 38.6 +/- 10.2 years and the median number of years worked was 10 (range: 1-37 years). Twenty-nine percent were pediatricians, 50.5 % were nurses and 20.5 % were nurse assistants. A total of 20.8 % had a high level of BS, 19.8 % had a moderate level and 59.4 % showed a low level. When sub-scales were applied, the results showed that 67.7 % of respondents presented a low level of personal achievement, 14.5 % had high scores of emotional exhaustion and 23.9 % obtained high scores in the depersonalization scale. CONCLUSIONS: BS is present in a significant percentage of hospital workers attending pediatric patients. In our sample, the most notable component of BS was the lack of personal achievement. This finding alerted us to the eventual presence of negative attitudes toward self and professional activity, as well as to the loss of interest in pediatric care, low productivity and diminished self-esteem. We believe that specific strategies should be implemented to attenuate the factors influencing the development of BS in pediatric health staff.

Síndrome de desgaste profesional en el personal asistencial pediátrico / Burnout syndrome among health workers in pediatrics

Antecedentes: El síndrome de desgaste profesional es un trastorno adaptativo crónico que condiciona alteraciones importantes de la conducta laboral. Objetivo: En este trabajo se ha pretendido conocer su prevalencia entre el personal asistencial pediátrico, para la cual se diseñó un estudio transversal y descriptivo. Material y métodos En noviembre de 2002 se presentó el cuestionario Maslach Burnout Inventory (MBI) a 127 personas (médicos, enfermeras y auxiliares) que trabajan en un Departamento de Pediatría. El cuestionario consta de 22 ítems que exploran tres aspectos del síndrome de desgaste profesional: a) cansancio emocional; b) despersonalización, y c) realización personal. Resultados: El cuestionario fue contestado por 93 personas (tasa de respuesta: 73,2 %). El 83 % de las encuestas fueron cumplimentadas por mujeres y el 17 % por varones. La media de edad fue de 38,6 ± 10,2 años y la mediana de antigüedad en el puesto de trabajo de 10 años (límites, 1-37 años). El 29,0 % eran pediatras, el 50,5 %, personal de enfermería, y el 20,5 %, auxiliares de enfermería. Según la escala utilizada, el 20,8 % de los entrevistados presentaban un alto grado de síndrome de desgaste profesional, el 19,8 % un nivel moderado y el 59,4 % un nivel bajo de síndrome de desgaste profesional. En relación con las subescalas del cuestionario, el 67,7 % de los encuestados presenta un bajo grado de realización personal, el 14,5 % obtiene puntuaciones altas de cansancio emocional y el 23,9% tiene valores altos en la escala de despersonalización. Conclusiones: El síndrome de desgaste profesional es un trastorno presente en un porcentaje considerable de las personas que atienden a pacientes pediátricos a nivel hospitalario. En la muestra estudiada, la falta de realización personal se destaca sobre los demás componentes del síndrome, lo que alerta sobre la posible existencia de actitudes negativas hacia uno mismo y la actividad laboral, pérdida de interés por la atención a los niños, baja productividad y disminución de la autoestima. Por lo tanto, deberían tomarse medidas para atenuar los factores que influyen en el desarrollo del síndrome de desgaste profesional entre el personal asistencial pediátrico

Background: Burnout syndrome (BS) is a chronic adaptation disorder that provokes serious problems in occupational behavior. Objective: In the present study we assessed the prevalence of burnout syndrome in pediatric healthcare workers. Design: Cross-sectional, descriptive study. Material and methods In November 2002, the Maslach Burnout Inventory was given to 127 staff members (doctors, nurses and nurse assistants) working in a pediatrics department. The questionnaire includes 22 items that explore three aspects of BS: a) emotional exhaustion; b) depersonalization, and c) personal achievement. Results: The questionnaire was completed by 93 staff members (73.2 % of the whole sample; 83% women and 17% men). The mean age was 38.6 +/- 10.2 years and the median number of years worked was 10 (range: 1-37 years). Twenty-nine percent were pediatricians, 50.5% were nurses and 20.5% were nurse assistants. A total of 20.8 % had a high level of BS, 19.8 % had a moderate level and 59.4 % showed a low level. When sub-scales were applied, the results showed that 67.7 % of respondents presented a low level of personal achievement, 14.5 % had high scores of emotional exhaustion and 23.9 % obtained high scores in the depersonalization scale. Conclusions: BS is present in a significant percentage of hospital workers attending pediatric patients. In our sample, the most notable component of BS was the lack of personal achievement. This finding alerted us to the eventual presence of negative attitudes toward self and professional activity, as well as to the loss of interest in pediatric care, low productivity and diminished self-esteem. We believe that specific strategies should be implemented to attenuate the factors influencing the development of BS in pediatric health staff