Actividad investigadora, asistencial y docente de la UCI Pediátrica del H. infantil Universitario Niño Jesús / Research, clinical and teaching activities of the Pediatric ICU of H. Universitario Niño Jesús Children

El Servicio de Cuidados Intensivos Pediátricos (UCIP) del Hospital Infantil Universitario Niño Jesús, de Madrid, es una Unidad polivalente de 14 camas, médico quirúrgica, que atiende a niños críticamente enfermos desde neonatos hasta 18 años. En los últimos seis años ha desarrollado un ambicioso programa investigador, asistencial y docente que se resume en el presente trabajo. En este periodo es la UCIP que más número de pacientes ha atendido en al Comunidad de Madrid, especialmente pacientes complejos, como niños oncológicos sometidos a trasplante de progenitores hemaopoyéticos o no; pacientes politraumatizados atendidos por un equipo multidisciplinar; niño sépticos; pacientes con patología neurológica sometidos a monitorización multiparamétrica avanzada; pacientes con patología respiratoria sometidos a diferentes formas de ventilación mecánica y otros. Se han desarrollado varias líneas de investigación clínica cuyos resultados se han publicado en diversas revistas científicas nacionales e internacionales. La actividad docente en este periodo ha sido muy amplia, basada en cursos teóricos-prácticos con simuladores, que ha contribuido a la formación de miles de pediatras. Producto de la experiencia asistencial, docente e investigadora se han editado 10 libros (AU)

The Paediatric Intensive Care Unit (PICU) of Hospital Infantil Universitario Niño Jesús is a polyvalent, surgical and medical 14 bed unit who assists critically ill patients since newborn age to 18 year-old. During the last 6 years we´ve developed an ambitious investigational, educational and clinical management programme that it´s summarized in the present paper. During this period of time our unit has got the highest admission rate in the whole Madrid area. The main pathological conditions attended are: oncological patients with and without bone marrow transplantation; multidisciplinary integrated management for the polytrauma patients; septic shock; neurological impaired patients under multiparameter advanced monitoring; respiratory failure under invasive or non invasive ventilation support. Many clinical investigations have been developed and published in several international and national journals. Thousands of paediatric physicians have accomplished our multiple simulation training programmes. As a result of our clinical, educational and investigational activity ten books have been published (AU)

Signos guía en el diagnóstico de enfermedad metabólica / Guiding signs in metabolic disease diagnosis

Los errores innatos del metabolismo son un grupo de enfermedades genéticas con sintomatología muy inespecífica y por tanto difícil diagnóstico si no existe una sospecha clínica elevada. Sin embargo existen algunos datos de la exploración física y de las pruebas complementarias que pueden enfocar el proceso diagnóstico hacia la solicitud de pruebas específicas que lo confirmen. El caso que presentamos trata de destacar algunos de estos datos que pueden hacer sospechar la existencia de un defecto congénito de la glucosilación de proteínas, trastorno infrecuente pero con algunas formas tratables, por lo que su sospecha y diagnóstico precoz es de vital importancia (AU)

Inborn errors of metabolism are a group of genetic diseases with non specific symptoms and therefore difficult to diagnose without high clinical suspicion. However there are some physical examination data and laboratory tests that can focus the diagnostic process to the implementation of specific tests to confirm them. The case exposed highlights some of these data that can make us suspect the existence of a congenital defect of glycosylation of proteins, rare disorder but with some treatable variations, that make their suspicion and early diagnosis of great importance (AU)

Infecciones por Staphylococcus aureus en cuidados intensivos: características clínicas y epidemiológicas / Staphylococcus aureus infections in the intensive care unit: clinical and epidemiological characteristics

Introducción: En los últimos años se ha producido un incremento de las infecciones invasivas por este patógeno que requieren ingreso en cuidados intensivos pediátricos (UCIP). Pacientes y métodos: Estudio retrospectivo (entre enero del 2006 y junio del 2010) en el que se incluyó a todos los pacientes con infección por Staphylococcus Aureus (S. aureus) que precisaron ingreso en UCIP. Estos fueron clasificados en 2 grupos: infección comunitaria (grupo 1) e infección nosocomial (grupo 2). Se recogieron los siguientes datos: epidemiológicos, tipo de aislamiento de S. aureus (S. aureus sensible a meticilina [SASM], S. aureus resistente a metilicina [SARM]), factores de riesgo, localización de la infección, presencia de inestabilidad hemodinámica, soporte respiratorio y mortalidad. Resultados: Se estudiaron a 51 pacientes, 21 pertenecientes al grupo 1 y 30 al grupo 2. La mediana de edad fue menor en el grupo 1 (1,6 años frente 3,2 años, p = 0,009). Se aisló SASM en el 88% de los casos. Se detectaron 6/51 (12%) pacientes con infección por SARM, los cuales se aislaron en el último periodo del estudio (enero 2009-junio 2010). Los factores de riesgo fueron: inmunosupresión, catéter venoso central, institucionalización, ventilación mecánica, cirugía previa, traumatismo previo y osteomielitis crónica. Un 83% de los pacientes con infección por SARM tenían factores de riesgo. La localización de la infección por S. aureus fue variada, siendo las infecciones respiratorias las más frecuentes (75%). Un 43% de los pacientes requirieron fármacos vasoactivos. La mayoría de los pacientes (86%) requirió soporte respiratorio. Un paciente del grupo 1 falleció por neumonía necrotizante por SAMS. Conclusiones: Las infecciones por S. aureus son graves y tienen elevada morbilidad. La infección pulmonar es la más frecuente en esta serie. Aunque el aislamiento de SASM es predominante en estas infecciones se ha visto una tendencia al incremento de las infecciones por SARM en el último periodo del estudio (AU)

Introduction: There has been an increase in invasive Staphylococcus Aureus infections over the last few years, which have required admission to the pediatric intensive care unit (PICU). Patients and methods: All patients with S. aureus infection who were admitted to PICU were enrolled in a retrospective study (January 2006-June 2010). The patients were classified into 2 groups: community-acquired infection (Group 1) and nosocomial infection (Group 2). We recorded epidemiological data, type of S. aureus (methicillin-susceptible S. aureus [MSSA], methicillin-resistant S. aureus [MRSA]), risk factors, site of infection, presence of hemodynamic instability, respiratory support, and mortality. Results: A total of 51 patients were enrolled, 21 belonging to Group 1 and 30 to Group 2. The median age was lower in Group 1 (1.6 years vs 3.2 years; P=0.009). MSSA was isolated in 88% of cases. MRSA was detected in 6/51 (12%) of cases, which were isolated in the later study period (January 2009-June 2010).The risk factors for infection were: immunosuppression, venous catheter, institutionalization, mechanical ventilation, previous surgery, previous trauma and chronic osteomyelitis. A large majority (83%) of the patients with MRSA infection had risk factors. The type of infection was varied, with respiratory tract infection being the most common (75%). Hemodynamic instability was observed in 43% of patients. Most patients (86%) required respiratory support. One patient in Group 1 died of necrotizing pneumonia caused by MSSA. Conclusions: Infections by S. aureus in children are severe and have a high morbidity. Respiratory infection was the most common in our series. Isolation of MSSA is common in these infections, although, an increase in the number of infections by MRSA was observed during the latter part of the study(AU)

Síndrome de Guillain-Barré: variante faringo-cervico-braquial / Guillain- Barré syndrome: pharyngeal-cervical-brachial variant

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[Staphylococcus aureus infections in the intensive care unit: clinical and epidemiological characteristics]. / Infecciones por Staphylococcus aureus en cuidados intensivos: características clínicas y epidemiológicas.

INTRODUCTION: There has been an increase in invasive Staphylococcus Aureus infections over the last few years, which have required admission to the pediatric intensive care unit (PICU). PATIENTS AND METHODS: All patients with S. aureus infection who were admitted to PICU were enrolled in a retrospective study (January 2006-June 2010). The patients were classified into 2 groups: community-acquired infection (Group 1) and nosocomial infection (Group 2). We recorded epidemiological data, type of S. aureus (methicillin-susceptible S. aureus [MSSA], methicillin-resistant S. aureus [MRSA]), risk factors, site of infection, presence of hemodynamic instability, respiratory support, and mortality. RESULTS: A total of 51 patients were enrolled, 21 belonging to Group 1 and 30 to Group 2. The median age was lower in Group 1 (1.6 years vs 3.2 years; P=.009). MSSA was isolated in 88% of cases. MRSA was detected in 6/51 (12%) of cases, which were isolated in the later study period (January 2009-June 2010). The risk factors for infection were: immunosuppression, venous catheter, institutionalization, mechanical ventilation, previous surgery, previous trauma and chronic osteomyelitis. A large majority (83%) of the patients with MRSA infection had risk factors. The type of infection was varied, with respiratory tract infection being the most common (75%). Hemodynamic instability was observed in 43% of patients. Most patients (86%) required respiratory support. One patient in Group 1 died of necrotizing pneumonia caused by MSSA. CONCLUSIONS: Infections by S. aureus in children are severe and have a high morbidity. Respiratory infection was the most common in our series. Isolation of MSSA is common in these infections, although, an increase in the number of infections by MRSA was observed during the latter part of the study.

Bronquiolitis grave. Cambios epidemiológicos y de soporte respiratorio / Severe bronchiolitis. Changes in epidemiology and respiratory support

Introducción: La bronquiolitis genera muchos ingresos en las unidades de cuidados intensivos pediátricos (UCIP). Nuevas formas de soporte respiratorio podrían mejorar la asistencia de estos pacientes. Objetivo: Analizar los cambios epidemiológicos y de soporte respiratorio de los niños ingresados en la UCIP. Pacientes y métodos: Estudio observacional y retrospectivo de los pacientes ingresados por bronquiolitis en la UCIP de un hospital terciario durante la época epidémica del virus respiratorios incitial (VRS) entre los años 2005 y 2010. Resultados: Fueron ingresados 229 pacientes; el 83% estaba infectado por VRS. La media de edad fue de 1,48 meses; los menores de 3 meses generaron el mayor número de ingresos (73,3%).Diciembre fue el mes con más número de ingresos (52%). La mortalidad fue del 0,9%. La media de estancia en UCIP y de soporte respiratorio fue de 4 y 3 días (diferencias no significativas entre las diferentes epidemias). Los pacientes con bronquiolitis por VRS eran de menor edad que los VRS negativo (media 2,61 y 4,05 meses; p = 0,023). El 73% de los casos requirieron soporte respiratorio. El porcentaje de pacientes en los que se utilizó soporte respiratorio activo fue en aumentó (Z = 3,81; p = 0,00014), sobre todo a expensas de la oxigenoterapia de alto flujo con cánulas nasales (Z = 3,62; p = 0,00028). Se observó relación inversa entre la edad y los días de estancia en UCIP (beta =−0,245; p ≤ 0,0001) y de soporte respiratorio (beta =−0,167;p = 0,039).Conclusiones: La mayoría de los pacientes ingresados por bronquiolitis en la UCIP tienen menos de 3 meses. La mortalidad es del 0,9%. El soporte respiratorio (sobre todo la ventilación mecánica no invasiva y la oxigenoterapia de alto flujo en cánulas nasales) se utiliza cada vez más frecuentemente. A menor edad, más días de ingreso en UCIP y de soporte respiratorio son necesarios (AU)

Introduction: Bronchiolitis generates lots of admissions during the cold months in the paediatric intensive care units (PICU). New forms of respiratory support are being used and could improve the care of these patients. Objectives: To analyse the epidemiological and respiratory support changes of children admitted to the PICU. Patients and methods: An observational, retrospective, descriptive and analytical study of patients with the diagnosis of bronchiolitis admitted to the PICU of a tertiary hospital during the respiratory syncytial virus (RSV) epidemic months between 2005 and 2010. Results: A total of 229 patients were admitted, of whom 83% were infected with RSV. The mean age was 1.48 months, with children under 3 months being the greatest number of admissions (73.3%). December was the month with the highest number of admissions (52%). Mortality was 0.9%. The mean time of stay in the PICU and on respiratory assistance were 4 and 3 days, respectively (no significant differences between the periods studied). Patients with RSV bronchiolitis were younger than the negative RSV (mean 2.61 months and 4.05 months, P = 0.023).Active respiratory support was required in 73% of cases. The percentage of patients who required active respiratory support increased over the years (Z = 3.81, P = 0.00014), especially high flow nasal oxygen therapy (Z = 3.62, P = 0.00028). An inverse relationship was observed between patient age and length of stay in PICU (Beta =−0.245, P≤0.0001) and days on respiratory support(Beta =−0.167, P = 0.039).Conclusions: Most patients admitted to the PICU are less than 3 months old. Mortality was 0.9%. Respiratory support (especially non-invasive mechanical ventilation and high flow nasal oxygen therapy) is used increasingly often. The youngest patients need more days in PICU and more respiratory support (AU)

[Severe bronchiolitis. Changes in epidemiology and respiratory support]. / Bronquiolitis grave. Cambios epidemiológicos y de soporte respiratorio.

INTRODUCTION: Bronchiolitis generates lots of admissions during the cold months in the paediatric intensive care units (PICU). New forms of respiratory support are being used and could improve the care of these patients. OBJECTIVES: To analyse the epidemiological and respiratory support changes of children admitted to the PICU. PATIENTS AND METHODS: An observational, retrospective, descriptive and analytical study of patients with the diagnosis of bronchiolitis admitted to the PICU of a tertiary hospital during the respiratory syncytial virus (RSV) epidemic months between 2005 and 2010. RESULTS: A total of 229 patients were admitted, of whom 83% were infected with RSV. The mean age was 1.48 months, with children under 3 months being the greatest number of admissions (73.3%). December was the month with the highest number of admissions (52%). Mortality was 0.9%. The mean time of stay in the PICU and on respiratory assistance were 4 and 3 days, respectively (no significant differences between the periods studied). Patients with RSV bronchiolitis were younger than the negative RSV (mean 2.61 months and 4.05 months, P=.023). Active respiratory support was required in 73% of cases. The percentage of patients who required active respiratory support increased over the years (Z=3.81, P=.00014), especially high flow nasal oxygen therapy (Z=3.62, P=.00028). An inverse relationship was observed between patient age and length of stay in PICU (Beta=-0.245, P ≤.0001) and days on respiratory support (Beta=-0.167, P=.039). CONCLUSIONS: Most patients admitted to the PICU are less than 3 months old. Mortality was 0.9%. Respiratory support (especially non-invasive mechanical ventilation and high flow nasal oxygen therapy) is used increasingly often. The youngest patients need more days in PICU and more respiratory support.

Ventilación mecánica en pediatría. ¿Cómo y cuándo extubar? / Mechanical ventilation in pediatrics. How and when to extubate?

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[Toxic shock syndrome in children. Report of four cases]. / Síndrome del shock tóxico en niños. Presentación de 4 casos.

We report four children with toxic shock syndrome admitted in the pediatric intensive care unit of our hospital during the past year. All the children had the five criteria established by the Centers for Disease Control for the diagnosis of this syndrome. In all four there was a probable point of entry of the infection: maxillar sinusitis in one, pneumonia in two and surgical wound in the other. No bacteria that could have caused the infection were isolated in any of the children, which suggests a staphylococcal origin for this syndrome. Evolution was good in all of the children due to aggressive treatment that included inotropic support, volemic expansion and antibiotics. Two of the children, who suffered adult respiratory distress syndrome, required prolonged respiratory support.

[Uremic hemolytic syndrome. Analysis of 43 cases]. / Síndrome hemolítico urémico. Análisis de 43 casos.

OBJECTIVE: The purpose of this study was to describe the epidemiological, clinical and evolution features of hemolytic uremic syndrome (HUS). PATIENTS AND METHODS: A retrospective study of 43 cases of HUS during the last 14 years (1984-1998) was performed. RESULTS: The mean age of the patients was 3.2 years, the incidence during the summer season was the highest and 39 cases (90%) previously had acute gastroenteritis. All children had acute renal failure, 32 of them (74%) required peritoneal dialysis. Anuria was found in 22 case (51%) and the mean duration was 10.3 days. The most frequent complications were: Hypertension in 21 cases (48%), peritonitis in 9 cases (20%), seizures in 8 patients (16%) and 3 deaths (6%). The mean hospital stay was 14.5 days. After one year of ambulatory control, 76% of the children were completely recovered and only two cases (6%) had chronic renal failure. Seizures associated significantly with a bad prognosis (p < 0.05). CONCLUSIONS: HUS has a very important morbimortality. Seizures in the acute phase were associated with a bad prognosis. Anuria for more than 7 days and oliguria for more than 15 days were also predictors of a worse prognosis, but this was not significant.

Brain death and evoked potentials in pediatric patients.

OBJECTIVE: To define the evoked potential responses (auditory and somatosensory) obtained from pediatric brain-dead patients. DESIGN: Prospective study over an 8-yr period (1988-1996). SETTING: A 14-bed pediatric intensive care unit in a multidisciplinary regional referral center (teaching hospital). PATIENTS: Fifty-one pediatric patients with clinically established brain death. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Auditory brainstem and somatosensory evoked potentials were performed with a mean evolution time of 24 hrs after clinical brain death. The first brainstem auditory evoked potential recording was compatible with the diagnosis in 45 patients (90%): 27 patients (53%) did not respond, wave I was patent in 16 (7 bilateral, 6 from the left side, and 3 from the right side), and 2 patients evoked waves I and II in one or both ears. Gross anomalies were found in the remaining six patients. Sixteen patients were tested for somatosensory evoked potentials. N13 identifiable wave (62.5% of the patients) or a flat record were the obtained findings. Electric silence was noted initially on the electroencephalogram (EEG) in only 14 of 29 patients. Later flattening was observed in seven patients. Missing brainstem evoked response was noted earlier than cortical electric silence (range, 12-144 hrs). Any central wave could be pointed out in the evoked potentials of patients with an isoelectric EEG. CONCLUSIONS: Evoked potential is useful in confirming the diagnosis of brain death in infants and in children as well as in adults. The test can be performed at bedside without interfering with patient care, and results are similar to those obtained in adult patients. Flattening of the EEG requires more time than achieving compatible evoked-potential responses.