Evolución del desarrollo neurosensorial de los recién nacidos de muy bajo peso (≤ 1.500 g) en nuestro medio / Neurosensorial developmental follow-up of very low weight newborns (<1.500g) in our center

Objetivos: Estudiar la situación y evolución neurológica de los recién nacidos de muy bajo peso (≤ 1.500 gramos) en nuestro medio. Material y métodos: Estudio retrospectivo sobre la evolución neurológica de 268 recién nacidos de muy bajo peso (RNNB) durante un período de seguimiento de 5 años, a través de visitas periódicas a cargo de neonatólogos, neuropediatra y psicólogo y colaboración de otro servicios, realizándose exploraciones oftalmológicas, EEG y potenciales evocados. Período de estudio: 1993-1999. Se realizó estudio estadístico de un amplio número de variables con el programa SPSS para Windows. Las secuelas se clasificaron según la gravedad en leves, moderadas, graves, y en relación al tipo de deficiencia, en motoras, cognitivas, sensoriales, mixtas y plurideficiencias (cuando se asociaban una o más secuelas). Se analizaron también a frecuencia y mortalidad de los RN;BP a lo largo de los años de estudio y siguientes. Resultados: Resaltaba una media de peso baja para la edad gestacional (EG), edad materna superior a 30 años, elevado porcentaje de recién nacidos de bajos peso, cesáreas, patología obstétrica, complicaciones neurológicas y extraneurológicas, tratamientos médicos y ventilación mecánica (VM), porcentaje bajo de gestantes tratadas con corticoides y/o tocolíticos y de prematuros con surfactante. Reproducción asistida: 17,68%. Número de fetos > 1 el 37%. La tasa de mortalidad neonatal de los TNMBP ha ido disminuyendo en los últimos años, siendo de 17,50% en el 2005, 14,45% en el 2006 y 11,18% en el 2007. Asimismo, su incidencia ha ido incrementándose, siendo el 19% en el 2005, 18,2% en el 2006 y 21,8% en el 2007. Presentaron secuelas el 30% con tendencia ascendente, leves 46,5%, moderadas, 22,1% graves, 31,4%, predominando las motoras puras (43%), seguidas por las mixtas (27,9%), y plurideficiencias (17,4%). Hipoacusia neurosensorial, 4,8%. Retinopatía del prematuro (ROP), 17,2%. Parálisis cerebral (PC), 17,16%. El 40% tenían algún tipo de alteración en la ecografía transfontanelar (ECO), hemorragia intra/periventricular (HPIV), 22,7%, leucomalacia periventricular (LPV), 15,3%, trastornos del aprendizaje, 33% de los escolarizados. Tuvieron alta correlación estadística con la aparición de secuelas la enfermedad de la membrana hialina (EMH) displasia broncopulmonar (DBP), sepsis tardía, necesidad de oxígeno (O2) y ventilación mecánica (VM) y muy especialmente, la edad gestacional (EG) y alteraciones de la ecografía craneal. Conclusiones: Se observa incremento en la frecuencia y supervivencia de los RNMBP y una incidencia de secuelas similar a otras series con un alto porcentaje de graves. Las secuelas neurológicas se relacionan principalmente con la EG y la patología especialmente del sistema nervioso central (SNC), más la influencia de factores sobreañadidos (O2 y VM, entre otros).Las variables más predictivas fueron la ecografía craneal y la edad gestacional (AU)

Objective: To study the neurological evolution of very low birth weight newborns (VLBWN) (< 1.500 g) in our area. Patients and methods: Retrospective study of the neurological evolution of 268 VLBWN during a period of 5 years in a multidisciplinary approach with the participation of neonatology, pediatric neurology and psychology units. We have done periodical ophthalmologic explorations, electroencephalograms, and auditory evoked potentials. Period of the study: 1993-1999. The statistical study was done with SPSS and Windows. The damage were classified as mild, moderate and severe and according to the type of deficiency in motor, cognitive, sensorial, combined and plurideficiencies (when one or more lesions were present). The prevalence and mortality of VLBWN were also analyzed. Results: It is remarkable a mean weight low for gestational age, maternal age higher than 30 years of age, high percentage of low weight, cesarean sections, obstetric diseases, neurological and extraneurological complications, medical treatments and mechanical ventilation; low percentage of maternal treatment with corticoids and /or uterus relaxant medications and of premature newborns with suphactant. Assisted reproduction: 17,68%. Number of fetus > 1:37%. Te neonatal mortality rate of VLBWN has decreased along the years being 17,05% in 2005, 14,45% in 2006, and 11,18% in 2007. The incidence has increased being 18/1.000 in 2005, 18,3/1.000 in 2006, and 21/1.000 in 2007. Physcial damage was observed in 30%; mild in 46,5%, moderate in 22,1%, severe in 31,4%. The lesions were predominantly of motor type (43%), followed by combined in 27,9% and plurideficiencies in 17,4%. Neurosensorial deafness in 4,8%. Premature retinopathy in 17,2%. Neurosensorial deafness in 4,8%. Premature retinopathy in 17,2%. Cerebral palsy in 17,6%. 40% had some kind of alterations in the transfontanelle echography, 22,7% had intra/periventricular hemorrhage, 15.3% periventricular leukomalcia and 33% of the school age children had learning disabilities. The sequelae had high statistical correlation with membrane hyaline disease, bronchopulmonary dysplasia, late sepsis, oxygen dependency and mechanical ventilation, and specially with gestational age and transfontanelle echography alterations. Conclusions: We have observed an increment in the frequency and survival of the VLBWN. The incidence of damage is similar to other studies with a high percentage of severe deficiencies. The neurologic damage is mainly associated with gestational age and with central nervous system injuries; the influence of other factors such as oxygen and mechanical ventilation are also important. The most predictive variables are gestational age and transfontanelle echography (AU)

Experiencia en el cambio brusco de carbamazepina por oxcarbazepina en niños y adolescentes / Immediate switching from carbamazepine to oxcarbazepine. Our experience in children and adolescents

Objetivo. Revisión retrospectiva de historias clínicascon cambio brusco de carbamazepina (CBZ) por oxcarbazepina(OXC) mediante la administración de al menos 1,3 vecesla dosis que tomaba de CBZ en dos dosis diarias de OXC.Método. Se hizo el cambio brusco en 22 casos pediátricos.Tomaban CBZ en monoterapia 17 casos y 5 en politerapia.La razón del cambio fue en 20 casos para disminuir lascrisis (y evitar efectos secundarios en 4 de ellos) y en 2 sólopor disminuir efectos de somnolencia y cansancio. El cambiomedio fue de 18,62 mg/kg de CBZ a 28,89 mg/kg deOXC. La relación media del cambio CBZ/OXC fue de 1,6:1(máximo: 2:1).Resultados. En 19 casos no se apreció ningún efectonegativo. Un niño empeoró el temblor esencial y 2 niñas semostraron más cansadas y somnolientas. Tres refirieron menorsomnolencia y uno menor tendencia al engorde. En12 casos no se apreció ningún cambio en las crisis. Cese inicialde las crisis en 5 casos; en 3 de ellos de manera mantenida.Disminuyó la frecuencia en 2 casos y acabaron desapareciendoen uno de ellos. En 3 casos disminuyó la intensidad de lascrisis. En dos casos se suspendió la OXC tras 24 meses sincrisis. Seguían tomando OXC 14 pacientes, 8 en monoterapia,con un tiempo medio de seguimiento de 31,5 meses.Conclusión. Dados los beneficios potenciales, facilidady buena tolerabilidad, aconsejamos antes de añadir a la CBZotro antiepiléptico probar con el cambio brusco por OXC (AU)

Objetive. We review retrospectively the clinical historiesof patients who were immediately switched fromcarbamazepine (CBZ) to oxcarbazepine (OXC), beingadministered a minimum of 1.3 times the CBZ dosis in2 daily dosis of OXC.Method. The immediate switching was carried out in22 paediatric cases. 17 patients were taking CBZ in monotherapyand 5 in politherapy. The change was made in20 cases to lower the number of seizures (and to avoid sideeffects in 4 of them), and in 2 only to reduce drowsinessand fatigue. The average change was from 18.62 mg/kg ofCBZ to 28.89 mg/kg of OXC. The medium change ratewas 1.6:1 (maximum: 2:1).Results. In 19 cases there were no side effects. Withone boy, the essential tremor worsened and two girls becamemore tired and drowsy. Three experienced lessdrowsiness and one less weight increase. Twelve casesshowed no seizure changes. Five cases became immediatelyseizure-free, three of them for a prolongated time.There was a reduction in seizure frequency in 2 cases,with posterior disappearance in one of them. Three casesexperienced a reduction in seizure intensity. In two casesOXC was stopped after 24 seizure-free months. Fourteenpatients were still taking OXC, 8 in monotherapy, with amean follow-up of 31.5 months.Conclusion. Given the potential benefits, ease andgood tolerability, we advise trying with immediate switchingto OXC, before adding another antiepileptic drugto CBZ (AU)

[Central sleep apnea syndrome as the first sign of type I Chiari malformation]. / Síndrome de apnea central del sueño como primera manifestación de malformación de Chiari tipo I.

INTRODUCTION: Type I Chiari malformation consists on the caudal displacement of cerebellar tonsils through the foramen magnum. It is often asymptomatic, although it may display symptoms as a result of cerebellum, brainstem, high cervical spinal cord or the lower cranial nerve, involvement. OBJECTIVE: We report our experience over the last 16 years. We have identified 16 patients with type I Chiari malformation. Only 2 cases showed common type I Chiari symptoms and just one had respiratory disorder as the first clinical sign. CLINICAL CASE: A 15 year old girl presented with a 5 years' history of chronic daily cough aggravated by exercise. Snoring and sleep apnea had been noted by her mother for 1 year. The girl eventually suffered from migraine and diurnal hypersomnolence. The physical and neurological examination was normal with the only exception being the absence of bilateral nauseous reflex. A nocturnal polysomnography study demonstrated a pseudoperiodic pattern with apnea pauses associated to cycles of deep breathing, resulting in severe gasometric repercussion and bradycardia. Magnetic resonance imaging of the brain showed Chiari I malformation. Non-invasive mechanical ventilation treatment significantly improved the clinical symptoms and gasometric analysis. DISCUSSION: Surgical posterior fossa decompression is discussed. Early decompression before appearance of irreversible neurological damage is recommended. It is associated with a significant reduction in the number of central apneas and sleep arousals. Surgical intervention is recommended in symptomatic patients and in cases of radiographic Chiari malformation or syrinx progression.

Síndrome de apnea central del sueño como primera manifestación de malformación de Chiari tipo I / Central sleep apnea syndrome as the first sign of type I Chiari malformation

Introducción: La malformación de Chiari tipo I consiste en el descenso de las amígdalas cerebelosas a través del foramen magno. Con frecuencia es asintomática, pero puede presentar clínica por afectación de cerebelo, troncoencéfalo, porción superior de la médula cervical y pares craneales bajos. Objetivo: Se presenta nuestra experiencia de 16 años con 16 casos de malformación de Chiari tipo I; sólo en 2 existen síntomas atribuibles a la anomalía de Chiari tipo I, y en una de ellas la primera manifestación clínica consistió en un trastorno respiratorio durante el sueño. Caso clínico: Niña de 15 años con tos crónica diaria, agravada con el ejercicio, de 5 años de duración. La madre observaba desde hacía 1 año que al dormir la niña roncaba y realizaba repetidamente pausas de apnea. Tenía cefaleas ocasionales e hipersomnolencia diurna. Las exploraciones física y neurológica fueron normales, salvo ausencia de reflejo nauseoso bilateral. El estudio polisomnográfico nocturno evidenció un patrón seudoperiódico con alternancia de pausas de apnea con ciclos de respiraciones profundas, con graves repercusiones gasométricas y sobre la frecuencia cardíaca. La resonancia magnética mostró anomalía de Chiari tipo I. La ventilación mecánica no invasiva supuso una mejoría clínica y gasométrica en la paciente. Discusión: La descompresión quirúrgica es discutida. Está indicada de forma precoz, antes de que aparezca daño neurológico irreversible. Se asocia con una reducción significativa en el número de apneas centrales y microdespertamientos. Se recomienda en casos sintomáticos o en casos con progresión radiológica de la anomalía de Chiari o de la siringomielia asociada (AU)

Introduction: Type I Chiari malformation consists on the caudal displacement of cerebellar tonsils through the foramen magnum. It is often asymptomatic, although it may display symptoms as a result of cerebellum, brainstem, high cervical spinal cord or the lower cranial nerve, involvement. Objective: We report our experience over the last 16 years. We have identified 16 patients with type I Chiari malformation. Only 2 cases showed common type I Chiari symptoms and just one had respiratory disorder as the first clinical sign. Clinical case: A 15 year old girl presented with a 5 years history of chronic daily cough aggravated by the exercise. Snoring and sleep apnea had been noted by her mother for 1 year. The girl eventually suffered from migraine and diurnal hypersomnolence. The physical and neurological examination was normal with the only exception being the absence of bilateral nauseous reflex. A nocturnal polysomnography study demonstrated a pseudoperiodic pattern with apnea pauses associated to cycles of deep breathing, resulting in severe gasometric repercussion and bradycardia. Magnetic resonance imaging of the brain showed Chiari I malformation. Non-invasive mechanical ventilation treatment significantly improved the clinical symptoms and gasometric analysis. Discussion: Surgical posterior fossa decompression is discussed. Early decompression before appearance of irreversible neurological damage is recommended. It is associated with a significant reduction in the number of central apneas and sleep arousals. Surgical intervention is recommended in symptomatic patients and in cases of radiographic Chiari malformation or syrinx progression (AU)

[Immediate switching from carbamazepine to oxcarbazepine. Our experience in children and adolescents]. / Experiencia en el cambio brusco de carbamazepina por oxcarbazepina en niños y adolescentes.

OBJECTIVE: We review retrospectively the clinical histories of patients who were immediately switched from carbamazepine (CBZ) to oxcarbazepine (OXC), being administered a minimum of 1.3 times the CBZ dosis in 2 daily dosis of OXC. METHOD: The immediate switching was carried out in 22 paediatric cases. 17 patients were taking CBZ in monotherapy and 5 in politherapy. The change was made in 20 cases to lower the number of seizures (and to avoid side effects in 4 of them), and in 2 only to reduce drowsiness and fatigue. The average change was from 18.62 mg/kg of CBZ to 28.89 mg/kg of OXC. The medium change rate was 1.6:1 (maximum: 2:1). RESULTS: In 19 cases there were no side effects. With one boy, the essential tremor worsened and two girls became more tired and drowsy. Three experienced less drowsiness and one less weight increase. Twelve cases showed no seizure changes. Five cases became immediately seizure-free, three of them for a prolongated time. There was a reduction in seizure frequency in 2 cases, with posterior disappearance in one of them. Three cases experienced a reduction in seizure intensity. In two cases OXC was stopped after 24 seizure-free months. Fourteen patients were still taking OXC, 8 in monotherapy, with a mean follow-up of 31.5 months. CONCLUSION: Given the potential benefits, ease and good tolerability, we advise trying with immediate switching to OXC, before adding another antiepileptic drug to CBZ.

[Facial paralysis reported in a paediatric emergency department: actuation protocol reviewed and verified]. / Parálisis facial en urgencias de pediatría: actualización de nuestro protocolo y autoevaluación.

INTRODUCTION: As result of our aim to improve the quality standard of our emergency system, work has been carried out in relation to the development and monitorization of effective clinical protocols in the department of paediatric practice. PATIENTS AND METHODS: An evidence based review approach was taken to design a clinical protocol about Bell's palsy condition for the paediatric emergency department. Previous protocol approved in March 2003 was reviewed accordingly with the new designed protocol's quality standards. The Bell's palsy cases reported since March 2003 until June 2006 to paediatric emergency department were analyzed. RESULTS: A total of 27 patients affected by Bell's palsy were reported to the hospital's emergency department. Facial expression was described in 85.19% of the cases. Cranial nerves normal function was reported in 77.78%. Fundoscopic examination was described in 77.78% and otoscopic findings in 44.44%; the absence of herpes vesicles was analyzed only in 11.11%. All patients received steroid therapy (prednisone) and the treatment resulted in the complete recovery. The mean time to resolution was 58.6 days. CONCLUSIONS: In order to improve hospital's quality standards, clinical protocols should be designed and verified regularly to ensure the proper performance. Medical auditing also contributes to improve effectiveness in health attendance.

Parálisis facial en urgencias de pediatría: actualización de nuestro protocolo y autoevaluación / Facial paralysis reported in a paediatric emergency department: actuation protocol reviewed and verified

Introducción. En nuestro hospital se está trabajando en un plan de mejora de calidad de las urgencias pediátricas que incluye, entre otras actuaciones, la protocolización de los procedimientos (mediante la revisión de la evidencia disponible) y la monitorización de su aplicación. Pacientes y métodos. Se revisa, según la evidencia disponible, el protocolo de urgencias de la parálisis facial. Se evalúa la aplicación del previo, consensuado en sesión hospitalaria en marzo del 2003, y se aplican de forma retrospectiva los indicadores diseñados en la revisión de junio de 2006. Se analizan los casos de parálisis facial a frigore valorados en urgencias de Pediatría desde marzo de 2003 hasta junio de 2006. Resultados. En el período de estudio fueron evaluados en Urgencias 27 pacientes con parálisis facial. En el 85,19% de los casos se describió la alteración de la mímica facial en el informe de urgencias. En el 77,78% de los casos constaba la normalidad de los demás pares craneales, y en el 77,78% del fondo de ojo. En el 44,44% se recogió la normalidad de la otoscopia; sólo en el 11,11% se especificaba la ausencia de vesículas herpéticas. Todos recibieron tratamiento con prednisona, fueron seguidos en la consulta de Neuropediatría y se resolvieron completamente, con una media de 58,6 días. Conclusiones. En el proceso de mejora de calidad es fundamental la elaboración de protocolos, y la evaluación de su cumplimiento mediante indicadores de calidad. El audit. y sus reevaluaciones facilitan la implantación del ciclo de aprendizaje y mejora en la atención sanitaria, vinculados a la excelencia del trabajo

Introduction. As result of our aim to improve the quality standard of our emergency system, work has been carried out in relation to the development and monitorization of effective clinical protocols in the department of paediatric practice. Patients and methods. An evidence based review approach was taken to design a clinical protocol about Bell’s palsy condition for the paediatric emergency department. Previous protocol approved in March 2003 was reviewed accordingly with the new designed protocol’s quality standards. The Bell’s palsy cases reported since March 2003 until June 2006 to paediatric emergency department were analyzed. Results. A total of 27 patients affected by Bell’s palsy were reported to the hospital’s emergency department. Facial expression was described in 85.19% of the cases. Cranial nerves normal function was reported in 77.78%. Fundoscopic examination was described in 77.78% and otoscopic findings in 44.44%; the absence of herpes vesicles was analyzed only in 11.11%. All patients received steroid therapy (prednisone) and the treatment resulted in the complete recovery. The mean time to resolution was 58.6 days. Conclusions. In order to improve hospital’s quality standards, clinical protocols should be designed and verified regularly to ensure the proper performance. Medical auditing also contributes to improve effectiveness in health attendance

Triploidía completa 69XXY / Complete triploidy 69,XXY

No disponible