Specificity and sensibility of the Spanish version of the COMFORT Behaviour Scale for assessing pain, grade of sedation and withdrawal syndrome in the critically ill paediatric patient. Multicentre COSAIP study (Phase 1).

AIM: The main aim of this investigation was to analyse the specificity and sensibility of the COMFORT Behaviour Scale (CBS-S) in assessing grade of pain, sedation, and withdrawal syndrome in paediatric critical care patients. METHOD: An observational, analytical, cross-sectional and multicentre study conducted in Level III Intensive Care Areas of 5 children's university hospitals. Grade of sedation was assessed using the Spanish version of the CBS-S and the Bispectral Index on sedation, once per shift over one day. Grade of withdrawal was determined using the CBS-S and the Withdrawal Assessment Tool-1, once per shift over three days. RESULTS: A total of 261 critically ill paediatric patients with a median age of 5.07 years (P25:0.9-P75:11.7) were included in this study. In terms of the predictive capacity of the CBS-S, it obtained a Receiver Operation Curve of .84 (sensitivity of 81% and specificity of 76%) in relation to pain; .62 (sensitivity of 21% and specificity of 78%) in relation to sedation grade, and .73% (sensitivity of 40% and specificity of 74%) in determining withdrawal syndrome. CONCLUSIONS: The Spanish version of the COMFORT Behaviour Scale could be a useful, sensible and easy scale to assess the degree of pain, sedation and pharmacological withdrawal of critically ill paediatric patients.

Spanish Society of Intensive and Critical Care Medicine and Coronary Units (SEMICYUC) and the Spanish Society of Pediatric Intensive Care (SECIP) consensus recommendations for ECMO transport.

The evolution of extracorporeal membrane oxygenation treatment and the transport of patients receiving this treatment has changed dramatically in the last decade unevenly in different regions. The creation of specialized referral centers has been shown to improve outcomes. For all these reasons, it has been necessary to create networks of specialized teams and the number of secondary transports of patients with this treatment is increasing. In order to improve the quality of treatment and offer a guide to the services involved in these transports, the critical transport working groups of the Spanish Society of Intensive and Critical Care Medicine and Coronary Units (SEMICYUC) and the Spanish Society of Pediatric Intensive Care (SECIP) have carried out a joint effort to prepare these recommendations, focused on the following aspects: indications, reference center systems, means of transport, characteristics and equipment, human teams, training and clinical safety.

Grade of sedation in the critically ill paediatric patient and its correlation with sociodemographic and clinical variables. Multicentre COSAIP study. / Grado de sedación del paciente crítico pediátrico y variables sociodemográficas y clínicas correlacionadas. Estudio multicéntrico COSAIP.

AIMS: To determine the grade of sedation in the critically ill paediatric patient using Biespectral Index Sensor (BIS) and to analyse its relationship with sociodemographic and clinical patient variables. METHODS: Observational, analytical, cross-sectional and multicentre study performed from May 2018 to January 2020 in 5 Spanish paediatric critical care units. Sex, age, reason for admission, presence of a chronic pathology, type and number of drugs and length of stay were the sociodemographic and clinical variables registered. Furthermore, the grade of sedation was assessed using BIS, 11per shift over 24hours. RESULTS: A total of 261 paediatric patients, 53.64% of whom were male, with a median age of 1.61 years (0.35-6.55), were included in the study. Of the patients, 70.11% (n=183) were under analgosedation and monitored using the BIS sensor. A median of BIS values of 51.24±14.96 during the morning and 50.75±15.55 during the night were observed. When comparing BIS values and sociodemographic and clinical paediatric variables no statistical significance was detected. CONCLUSIONS: Despite the limitations of the BIS, investigations and the present study show that BIS could be a useful instrument to assess grade of sedation in critically ill paediatric patients. However, further investigations which determine the sociodemographic and clinical variables involved in the grade of paediatric analgosedation, as well as studies that contrast the efficacy of clinical scales like the COMFORT Behaviour Scale-Spanish version, are required.

Specificity and sensibility of the Spanish version of the COMFORT Behaviour Scale for assessing pain, grade of sedation and withdrawal syndrome in the critically ill paediatric patient. Multicentre COSAIP study (Phase 1). / Especificidad y sensibilidad de la COMFORT Behavior Scale-Versión española para valorar el dolor, el grado de sedación y síndrome de abstinencia en el paciente crítico pediátrico. Estudio multicéntrico COSAIP (Fase 1).

AIM: The main aim of this investigation was to analyse the specificity and sensibility of the COMFORT Behaviour Scale (CBS-S) in assessing grade of pain, sedation, and withdrawal syndrome in paediatric critical care patients. METHOD: An observational, analytical, cross-sectional and multicentre study conducted in Level III Intensive Care Areas of 5 children's university hospitals. Grade of sedation was assessed using the Spanish version of the CBS-S and the Bispectral Index on sedation, once per shift over one day. Grade of withdrawal was determined using the CBS-S and the Withdrawal Assessment Tool-1, once per shift over three days. RESULTS: A total of 261 critically ill paediatric patients with a median age of 5.07 years (P25:0.9-P75:11.7) were included in this study. In terms of the predictive capacity of the CBS-S, it obtained a Receiver Operation Curve of .84 (sensitivity of 81% and specificity of 76%) in relation to pain; .62 (sensitivity of 21% and specificity of 78%) in relation to sedation grade, and .73% (sensitivity of 40% and specificity of 74%) in determining withdrawal syndrome. CONCLUSIONS: The Spanish version of the COMFORT Behaviour Scale could be a useful, sensible and easy scale to assess the degree of pain, sedation and pharmacological withdrawal of critically ill paediatric patients.

Grade of sedation in the critically ill pediatric patient and its correlation with sociodemographic and clinical variables. Multicentre COSAIP study.

AIMS: To determine the grade of sedation in the critically ill paediatric patient using Biespectral Index Sensor (BIS) and to analyse its relationship with sociodemographic and clinical patient variables. METHODS: Observational, analytical, cross-sectional and multicentre study performed from May 2018 to January 2020 in 5 Spanish paediatric critical care units. Sex, age, reason for admission, presence of a chronic pathology, type and number of drugs and length of stay were the sociodemographic and clinical variables registered. Furthermore, the grade of sedation was assessed using BIS, once per shift over 24 h. RESULTS: A total of 261 paediatric patients, 53.64% of whom were male, with a median age of 1.61 years (0.35-6.55), were included in the study. Of the patients, 70.11% (n = 183) were under analgosedation and monitored using the BIS sensor. A median of BIS values of 51.24 ±â€¯14.96 during the morning and 50.75 ±â€¯15.55 during the night were observed. When comparing BIS values and sociodemographic and clinical paediatric variables no statistical significance was detected. CONCLUSIONS: Despite the limitations of the BIS, investigations and the present study show that BIS could be a useful instrument to assess grade of sedation in critically ill paediatric patients. However, further investigations which determine the sociodemographic and clinical variables involved in the grade of paediatric analgosedation, as well as studies that contrast the efficacy of clinical scales like the COMFORT Behaviour Scale-Spanish version, are required.

Recomendaciones de consenso sobre el transporte de pacientes en ECMO de la Sociedad Española de Medicina Intensiva, Crítica y Unidades Coronarias (SEMICYUC) y la Sociedad Española de Cuidados Intensivos Pediátricos (SECIP) / Spanish Society of Intensive and Critical Care Medicine and Coronary Units (SEMICYUC) and the Spanish Society of Pediatric Intensive Care (SECIP) consensus recommendations for ECMO transport

La evolución del tratamiento de oxigenación por membrana extracorpórea (ECMO) y en particular del transporte de los pacientes sometidos a él, ha cambiado de forma significativa en la última década y lo ha hecho de manera desigual en diferentes regiones. Se ha demostrado que la creación de centros de referencia especializados mejora los resultados. Por todo ello ha sido necesario crear redes de equipos especializados y el número de transportes secundarios de pacientes con este tratamiento está en aumento. Con el fin de mejorar la calidad del tratamiento y ofrecer una guía para los servicios que intervienen en estos transportes, los grupos de trabajo de transporte crítico de la Sociedad Española de Medicina Intensiva, Crítica y Unidades Coronarias (SEMICYUC) y la Sociedad Española de Cuidados Intensivos Pediátricos (SECIP) han realizado un trabajo conjunto de elaboración de estas recomendaciones, enfocadas a los siguientes aspectos: indicaciones, sistemas de centros de referencia, medios de transporte, características y equipamiento, equipos humanos, formación y seguridad clínica (AU)

The evolution of extracorporeal membrane oxygenation treatment and the transport of patients receiving this treatment has changed dramatically in the last decade unevenly in different regions. The creation of specialized referral centers has been shown to improve outcomes. For all these reasons, it has been necessary to create networks of specialized teams and the number of secondary transports of patients with this treatment is increasing. In order to improve the quality of treatment and offer a guide to the services involved in these transports, the critical transport working groups of the Spanish Society of Intensive and Critical Care Medicine and Coronary Units (SEMICYUC) and the Spanish Society of Pediatric Intensive Care (SECIP) have carried out a joint effort to prepare these recommendations, focused on the following aspects: indications, reference center systems, means of transport, characteristics and equipment, human teams, training and clinical safety (AU)

[End-of-life care in the pediatric intensive care unit. A literature review]. / Cuidados al final de la vida en la unidad de cuidados intensivos pediátrica. Revisión de la bibliografía.

INTRODUCTION: In the last few years, there has been growing concern in the literature about issues related to end-of-life care in pediatric intensive care units (PICUs), with special attention on the family/patient unit, communication, and a dignified death. OBJECTIVE: To evaluate the experience and development of end-of-life care in PICUs through a literature review, by determining the type of studies that have been performed, their topics, the issues discussed, and their development in the last few years. MATERIAL AND METHODS: Review of the medical literature in Medline and the database of the National Library of Medicine Gateway, using the key words from MeSH: "end of life", "pediatric intensive care", "critical care", "palliative care", "death", and "compassionate care". The earliest year of the search was 1990. The languages selected were English and Spanish. Inclusion criteria were the relationship with the topic to be studied, excluding articles with no abstract. Additional searches were made of references in selected articles. RESULTS: Eighty-one articles were retrieved from the initial search. Of these, 43 were selected as the most relevant investigations in end-of-life care in ICUs and 18 placed special emphasis on the PICU. More than half of the articles (62 %) were reviews and the remaining articles were descriptive or observational studies. The number of publications increased after 1995. Most of the studies were performed in the USA or Canada and only three studies were performed in Spain. CONCLUSIONS: In the last few years, several studies have been performed that reveal increasing concern about limits to therapeutic intervention and the need to improve end-of-life care in the PICU setting.

[Chronic recurrent multifocal osteomyelitis: report of five cases]. / Osteomielitis crónica multifocal recurrente.

Chronic recurrent multifocal osteomyelitis is a rare disease of unknown etiology that mainly affects children. We present a retrospective review of the medical records of five patients with a diagnosis of chronic recurrent multifocal osteomyelitis in an infectious diseases clinic at a tertiary center. We describe the epidemiological, clinical and radiological features, laboratory, microbiological and histological findings, treatment, course and prognosis in these patients. There is no specific treatment for chronic recurrent multifocal osteomyelitis. There are new treatment options such the combination of azithromycin and calcitonin in children without clinical improvement with non-steroidal anti-inflammatory drugs.

[Respiratory syncitial virus in immunocompromised patients in a pediatric hospital: 5 years experience]. / Infección por virus respiratorio sincitial en los pacientes inmunodeprimidos en un hospital pediátrico: experiencia de 5 años.

INTRODUCTION: Respiratory syncytial virus (RSV) infection is associated with an increase in morbidity and mortality in immunocompromised hosts. METHODS: A description is presented of all cases of RSV infection in immunocompromised pediatric patients in Hematology and Oncology and Immunodeficiency Units between 2008 and 2012. RESULTS: Nineteen patients were diagnosed with RSV infection. Nine patients required in-patient care and 2 required Pediatric Intensive Care Unit. Five patients were treated with specific therapy (ribavirin ± palivizumab). No deaths occurred in the study period. CONCLUSION: RSV infection may be severe in immunocompromised pediatric patients.

[Scalp myiasis in an immigrant child]. / Miasis del cuero cabelludo en niño inmigrante.

Myiasis is caused by infestation of the skin by the larvae of flies. This entity has specific epidemiological characteristics. Although it is highly unusual in our environment, imported cases can be found because of increased traveling and immigration. The usual clinical manifestations are subcutaneous abscesses, where the larvae develop. Differential diagnosis includes common disorders such as cellulitis and furunculosis, but knowledge of this entity makes diagnosis and treatment simple. We describe the life cycle of the human botfly and the typical clinical presentation and management of this entity. We also highlight the increasing importance of greater awareness of imported diseases.

[Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis]. / Respuesta clinica rapida y espectacular a plasmaferesis en un caso pediatrico de encefalitis anti-NMDA.

INTRODUCTION: Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults. CASE REPORT: We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months' follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment. CONCLUSIONS: Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement.

Unidad de Cuidados Intensivos Pediátricos del Hospital Universitario 12 de Octubre / Pediatric Intensive Care Unit of the University Hospital 12 de Octubre

La UCIP del Hospital 12 de Octubre lleva en funcionamiento desde 1982, estando actualmente preparada para abarcar el cuidado de todos los pacientes pediátricos graves. Está integrada dentro del programa de atención al paciente pediátrico con cardiopatía, estando especializada en el manejo de dichos pacientes y siendo centro de referencia, incluyendo también un programa de oxigenación por membrana extracorpórea con amplia experiencia y pionero en la canulación y posterior transporte de pacientes en hospitales que carecen de esta técnica. La humanización y el cuidado centrado en la familia son dos pilares básicos que centran la actividad en esta Unidad, siendo la atención y el cuidado al final de la vida un tema que se ha investigado e implementado especialmente, así como los aspectos psicológicos en padres y profesionales. La Unidad es de puertas abiertas e incluye programas de atención especiales para el paciente crónico o de cuidados intermedios en épocas epidémicas asi como los proyectos existentes tanto a nivel pediátrico como en adultos para la erradicación de infección nosocomial. También se cuida especialmente la docencia pre y postgrado, participando en numerosos cursos y actividades especialmente en el campo de la reanimación cardiopulmonar pediátrica (RCP) (AU)

The PICU of the Hospital 12 de Octubre has been functioning since 1982, and is currently prepared to include care of all seriously ill pediatric patients. It is integrated into the care program for pediatric patient with heart disease, and is specialized in the treatment of said patients, and it is also a reference center. It also includes an extracorporeal membrane oxygenation program with wide experience and it is a pioneer in the cannulation and subsequent transportation of patients in hospitals lacking this technique. The humanization and care focused on the family are two basic cornerstones that the activity of this Unit focuses on, attention and care at the end of life being a subject that has been especially investigated and put into effect as well as the sychological aspects in parents and professionals. The Unit has an open door policy and includes special care programs for the chronic patient or intermediate cares in epidemic periods as well as the existing projects both on the pediatric level as well as the adult one For the eradication of nosocomial infection. Attention is also especially given to pre- and post-graduate teaching, participating in numerous courses and activities, especially in the field of pediatric cardiopulmonary resuscitation (PCR) (AU)

Transporte en ECMO / No disponible

No disponible

Grado de sedación del paciente crítico pediátrico y variables sociodemográficas y clínicas correlacionadas. Estudio multicéntrico COSAIP / Grade of sedation in the critically ill paediatric patient and its correlation with sociodemographic and clinical variables. Multicentre COSAIP study

Objetivos Determinar los niveles de sedación del paciente crítico pediátrico mediante el Biespectral Index Sensor (BIS) y analizar la relación entre el grado de sedación y las variables sociodemográficas y clínicas del paciente. Métodos Estudio observacional, analítico, transversal y multicéntrico de mayo de 2018 a enero de 2020 desarrollado en 5unidades de cuidados intensivos pediátricas del territorio español. Se registraron como variables sociodemográficas y clínicas el sexo, la edad, motivo de ingreso, si el paciente tenía enfermedad crónica, el tipo y número de fármacos que se le estaban administrando y la duración de la estancia. Además, se anotaron los valores del BIS una vez por turno, mañana y noche, durante 24 h. Resultados Se incluyó en el estudio a un total de 261 pacientes, de los cuales el 53,64% eran del sexo masculino, con una edad mediana de 1,61 años (0,35-6,55). El 70,11% (n=183) estaban analgosedados y monitorizados con el sensor BIS. Se observó una mediana en las puntuaciones globales de BIS de 51,24±14,96 en el turno de mañana y de 50,75±15,55 en el de noche. No se detectó significación estadística al comparar los niveles de BIS y las diversas variables sociodemográficas y clínicas del paciente crítico pediátrico. Conclusiones A pesar de las limitaciones inherentes al sensor BIS, los estudios existentes y el que aquí se presenta muestran que el BIS es un instrumento útil para monitorizar el grado de sedación en el paciente crítico pediátrico. Se requieren más investigaciones que objetiven qué variables relacionadas con el paciente tienen más peso en al grado de analgosedación y que contrasten clínicamente la eficacia de escalas como, por ejemplo, la COMFORT Behavior Scale versión española. (AU)

Aims To determine the grade of sedation in the critically ill paediatric patient using Biespectral Index Sensor (BIS) and to analyse its relationship with sociodemographic and clinical patient variables. Methods Observational, analytical, cross-sectional and multicentre study performed from May 2018 to January 2020 in 5 Spanish paediatric critical care units. Sex, age, reason for admission, presence of a chronic pathology, type and number of drugs and length of stay were the sociodemographic and clinical variables registered. Furthermore, the grade of sedation was assessed using BIS, 11per shift over 24hours. Results A total of 261 paediatric patients, 53.64% of whom were male, with a median age of 1.61 years (0.35-6.55), were included in the study. Of the patients, 70.11% (n=183) were under analgosedation and monitored using the BIS sensor. A median of BIS values of 51.24±14.96 during the morning and 50.75±15.55 during the night were observed. When comparing BIS values and sociodemographic and clinical paediatric variables no statistical significance was detected. Conclusions Despite the limitations of the BIS, investigations and the present study show that BIS could be a useful instrument to assess grade of sedation in critically ill paediatric patients. However, further investigations which determine the sociodemographic and clinical variables involved in the grade of paediatric analgosedation, as well as studies that contrast the efficacy of clinical scales like the COMFORT Behaviour Scale-Spanish version, are required. (AU)

Infección por virus respiratorio sincitial en los pacientes inmunodeprimidos en un hospital pediátrico: experiencia de 5 años / Respiratory syncitial virus in immunocompromised patients in a pediatric hospital: 5 years experience

INTRODUCCIÓN: La infección por virus respiratorio sincitial (VRS) causa importante morbimortalidad en pacientes inmunodeprimidos. MÉTODOS: Estudio descriptivo en un hospital pediátrico de los casos de infección por VRS en pacientes inmunodeprimidos de las unidades de Hemato-Oncología e Inmunodeficiencias en el periodo 2008-2012. RESULTADOS: Se diagnosticaron 19 casos de infección por VRS. Nueve pacientes requirieron ingreso, 2 en Unidad de Cuidados Intensivos Pediátrica. Cinco pacientes precisaron tratamiento con ribavirina y/o palivizumab. No se produjeron fallecimientos. CONCLUSIÓN: La infección por VRS es potencialmente grave en los pacientes pediátricos inmunodeprimidos

INTRODUCTION: Respiratory syncytial virus (RSV) infection is associated with an increase in morbidity and mortality in immunocompromised hosts. METHODS: A description is presented of all cases of RSV infection in immunocompromised pediatric patients in Hematology and Oncology and Immunodeficiency Units between 2008 and 2012. RESULTS: Nineteen patients were diagnosed with RSV infection. Nine patients required in-patient care and 2 required Pediatric Intensive Care Unit. Five patients were treated with specific therapy (ribavirin ± palivizumab). No deaths occurred in the study period. CONCLUSION: RSV infection may be severe in immunocompromised pediatric patients

Factores de mortalidad en niños con tosferina maligna / Mortality risk factors in children with severe pertussis

INTRODUCCIÓN: La hiperleucocitosis y la hipertensión pulmonar son factores de riesgo de mortalidad en niños con tosferina maligna. Las opciones terapéuticas disponibles para estos casos graves no se encuentran bien establecidas. Población y métodos: Se diseñó un estudio ambispectivo que incluía a niños diagnosticados de tosferina ingresados en una Unidad de Cuidados Intensivos Pediátricos (UCIP) de un hospital de tercer nivel en España entre enero de 2007 y octubre de 2015. Se compararon variables clínicas y demográficas entre el grupo de niños que sobrevivieron (grupo de supervivientes [GS]) y los que finalmente fallecieron (grupo exitus [EG]). RESULTADOS: Se identificaron un total de 31 pacientes. La mortalidad global fue del 19% (6/31 pacientes). Cinco niños fueron diagnosticados de hipertensión pulmonar. Cinco de seis niños que finalmente fallecieron precisaron canulación en oxigenación por membrana extracorpórea (ECMO). Ocho pacientes recibieron terapia mediante exanguinotransfusión (ET). La mediana de leucocitos antes de la realización de ET fue mayor (81.300 cél./μL) en EG que en GS (57.400 cél./μL), p= 0,05. Los pacientes que fallecieron tuvieron un mayor recuento pico de leucocitos totales, linfocitos, neutrófilos y niveles de proteína C reactiva (PCR) que los niños que sobrevivieron. Las variables que se identificaron como factores de riesgo de mortalidad fueron: una frecuencia cardiaca mayor de 170 lpm (OR 18; IC del 95%: 1,7-192,0), la presencia de neumonía (OR 16,5; IC del 95%: 1,7-165) y la presencia de hipertensión pulmonar (OR 179,6 [6,4-5.027]). CONCLUSIÓN: El uso de variables sencillas como la frecuencia cardiaca, el recuento total de leucocitos o los valores de PCR pueden servir para identificar de forma precoz a pacientes con riesgo de hipertensión pulmonar y tosferina maligna, de forma que procedimientos invasivos como la ET puedan utilizarse de una forma más precoz

BACKGROUND: Hyperleukocytosis and pulmonary hypertension are risk factors for death in infants with severe pertussis. Treatment options in severe pertussis are not well-established. METHODS: We designed an ambispective study of children with pertussis admitted to the pediatric intensive care unit (PICU) of a tertiary level hospital in Spain from January 2007 to October 2015. Clinical and demographical variables were compared between the group of children who survived (survivors group or SG) and those children who died (exitus group or EG). RESULTS: Thirty-one children were identified. Overall mortality rate was 19% (6/31 patients). Five children had pulmonary hypertension. Five out of 6 infants who eventually died had been placed on ECMO. Eight infants needed exchange transfusion (ET). Median leukocyte count immediately before exchange transfusion was higher (81300 cél./μL) in EG than in SG (57400 cél./μL), p= 0.05. Children who died had higher peak values in white blood cell counts (WBC), lymphocyte count, neutrophil counts and PCR levels than children who survived. The following variables were associated with risk of death: a heart rate above 170 bpm (OR 18, CI 95%: 1.7-192,0), the presence of pneumonia (OR 16.5, CI 95%: 1.7-165) and pulmonary hypertension (OR 179,6 [6,4-5027]. CONCLUSION: Early identification of patients at risk for pulmonary hypertension and fatal pertussis using heart rate, WBC and PCR levels would be appropriate so that invasive procedures such as exchange transfusion could be carried out precociously

Respuesta clínica rápida y espectacular a plasmaféresis en un caso pediátrico de encefalitis anti-NMDA / Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis

Introducción. La encefalitis autoinmune contra receptores N-metil-D-aspartato (NMDA) se diagnostica cada vez con mayor frecuencia en la edad pediátrica. Debe sospecharse en niños con sintomatología psiquiátrica, encefalopatía, movimientos anormales o crisis epilépticas. Los casos paraneoplásicos son menos frecuentes que en adultos. Caso clínico. Niño de 2,5 años con cuadro encefalopático subagudo que comenzó con crisis epilépticas seguidas de alteraciones del comportamiento, regresión neurológica, discinesias e insomnio. El estudio del líquido cefalorraquídeo fue normal, en la resonancia magnética craneal existía una lesión periventricular focal y captación leptomeníngea difusa y los electroencefalogramas seriados revelaron una actividad delta de gran amplitud intercalada con actividad epileptiforme intercrítica generalizada. Recibió tratamiento empírico con altas dosis de corticoides e inmunoglobulinas intravenosas sin respuesta. Tras demostrarse la positividad de anticuerpos contra el receptor NMDA se inició la plasmaféresis, con la que experimentó una mejoría rápida y espectacular. Tras más de 18 meses de seguimiento, sus secuelas se limitan a leves alteraciones conductuales y del lenguaje. No ha presentado recaídas ni ha precisado ningún tratamiento de mantenimiento. Conclusiones. La encefalitis anti-NMDA es un trastorno tratable y, ocasionalmente, el primer indicio de una neoplasia subyacente, por lo que su reconocimiento y tratamiento precoz es fundamental. El tratamiento de las formas no paraneoplásicas se basa en la inmunoterapia: glucocorticoides, inmunoglobulinas intravenosas, plasmaféresis e inmunosupresores. La plasmaféresis puede inducir una mejoría rápida y espectacular (AU)

Introduction. Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults. Case report. We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months’ follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment. Conclusions. Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement (AU)

Parálisis diafragmática en el postoperatorio de cirugía cardiaca infantil / Diaphragmatic paralysis after pediatric cardiac surgery

Objetivo: Describir la frecuencia, morbimortalidad y tratamiento de la disfunción diafragmática (DD) en el postoperatorio de la cirugía cardiaca infantil. Pacientes y métodos: Serie de casos de DD identificados entre la población de niños intervenidos de cirugía cardiaca en el hospital «12 de Octubre» de Madrid, entre 1997 y 2006. Se obtuvieron retrospectivamente datos demográficos, de la cirugía y del postoperatorio, así como de la estancia hospitalaria yde la morbimortalidad. Resultados: Cirugía cardiaca en 1.063 niños, diagnosticándose 23 casos de DD. Se excluyó un caso secundario a neuropatía generalizada. La frecuencia global fue del 2,1%. La mediana de edad fue de 14 días (1 día-16 meses), siendo 20 de los casos menores de 1 año. Se realizó plicatura en 13 pacientes (62%). Las indicaciones de plicatura fueron: fracaso de extubación (n= 11) y dificultad respiratoria persistente (n= 2).Fue preciso realizar plicatura quirúrgica en 9 de los 13 neonatos. El tiempo de ventilación mecánica invasiva, estancia en unidad de cuidados intensivos pediátricos (UCIP) y estancia hospitalaria en los casos con paresia fue de 8, 12 y 20 días(mediana), respectivamente. Las parálisis precisaron mayor tiempo de ventilación invasiva y de ingreso (19, 34 y 47,5 días). Conclusiones: A todo neonato intervenido de una cardiopatía compleja debería realizársele una ecografía diafragmática en respiración espontánea previa a su extubación, para así establecer el diagnóstico de manera precoz, con el objetivo de reducir el tiempo de ventilación mecánica y la morbilidad asociada (AU)

The objective of this study is to describe the frequency, morbimortality and treatment of diaphragmatic dysfunction (DD) after pediatric cardiac surgery. Patients and methods: Series of DD cases identified among the population of children who underwent cardiac surgery at the hospital 12 de Octubre of Madrid from the years 1997-2006. Demographic, surgical and postsurgical data have been obtained retrospectively, as well as data on hospital stay and the morbimortality. Results: A total of 1,063 children underwent cardiac surgery, diagnosing 23 DD cases. A secondary case was excluded due to generalized neuropathy. The global frequency was of 2.1%,the mean age was 14 days (1 d-16 m), being 20 of the cases under 1 year of age. Reefing was performed in 13 patients (62%). The indications for reefing were due to extubation failure (n= 11) and persistent respiratory difficulties (n= 2).Surgical reefing had to be performed in 9 from the 13 newborns. In the cases of paresis the length of the invasive mechanical ventilation, stay at the PICU and hospital stay was 8,12 and 20 days (mean) respectively. The paralysis required alonger time of invasive ventilation and hospitalization (19, 34 and 47,5 days).Conclusions: A diaphragm echography in spontaneous breathing should be performed on any newborn that underwent a complex cardiopathy surgery previous to the extubation in order to determine an early diagnosis and to reduce the length of mechanical ventilation and associated morbidity (AU)