La Unidad de Neonatología del Hospital Universitario Miguel Servet en la actualidad. Nuevos retos / Neonatology Unit of the Hospital Universitario Miguel Servet at present: new challenges

La presente revisión resume las características de la actual Unidad de Neonatología del Hospital Universitario Miguel Servet en Zaragoza. La Unidad de Neonatología es una unidad de nivel IIIB de acuerdo con la clasificación del Comité de Estandares y la Junta Directiva de la Sociedad Espñola de Neonatología de 2013, siendo la unidad neonatal de referencia de la Cornunidad Autónoma de Aragón. En una primera aproximación, se describen la misión, visión y valores, seguido de una resña estructural y de la cartera de servicios de la Unidad de Neonatología. La actividad asistencia de la Unidad de Neonatología se presenta teniendo en cuenta tanto la hospitalización neonatal como el seguimiento ambulatorio en el área de consultas externas. La actividad docente e investigadora son otros aspectos importantes a considerar en la actual Unidad de Neonatología. Finalmente se revisan los retos de la Unidad de Neonatología para los proximos afios (AU)

This review summarizes the characteristics of the current Neonatology Unit of the Universitary Hospital Miguel Servet in Zaragoza. The Neonatology Unit is a type IIIB unit according to the classification of the Standards Committee and the Board of the Spanish Society of Neonatology in 2013, being the reference neonatal unit for the Autonomous Community of Aragon. In a first approximation, the mission, vision and values are described, followed by a structural review and all the services that de Neonatology Unit provides. The health care activity of the Neonatology Unit is presented taking care of neonatal hospitalization and its subsequent follow-up out patient clinic. The teaching and investigation activity is another important aspect to consider in the current Neonatology Unit. Finally, the challenges of the Neonatology Unit for the next years are been reviewed (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)

[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.

[Benign paroxysmal torticollis. Our experience gained over a 15-year period]. / Torticolis paroxistico benigno. Nuestra experiencia de 15 anos.

INTRODUCTION: Benign paroxysmal torticollis (BPT) is characterised by recurring episodes of lateral bending of the neck, occasionally accompanied by vegetative symptoms, ataxia or an abnormal position of the trunk, with a tendency to disappear spontaneously after some months or years, and with no alterations between episodes. AIM: To analyse the clinical and developmental characteristics of the cases evaluated by the Neuropaediatric Service at our hospital that were classified as BPT. PATIENTS AND METHODS: We reviewed the history of the patients with BPT included in the Neuropaediatric Service database over a 15-year period. Patients who were not following any kind of control were contacted by telephone. RESULTS: We found 13 BPT patients with typical criteria, and 4 others with possible BPT (p-BPT), because they had had an isolated episode of torticollis. Neuroimaging was carried out in nine children (69.2%) from the BPT group: this included only transfontanellar ultrasound recording (TF USR) in six cases, TF USR and computerised axial tomography (CAT) in one child, only a CAT scan in one case, and CAT and magnetic resonance imaging in another. Neuroimaging was performed in all the cases of p-BPT: CAT scans were carried out in two cases and TF USR was used in two others. CONCLUSIONS: Atypical cases can be excluded by establishing a diagnosis of BPT with strict criteria. Since no biological markers are available, the diagnosis must be based on the clinical pattern and course and, in some cases, complementary examinations have to be performed to preclude other pathologies. When a child is diagnosed with BPT, the family needs to be reassured and told that it is a benign process with a tendency to disappear spontaneously.

Tortícolis paroxístico benigno. Nuestra experiencia de 15 años / Benign paroxysmal torticollis. Our experience gained over a 15-year period

Introducción. El tortícolis paroxístico benigno (TPB) secaracteriza por episodios recurrentes de flexión lateral del cuello,acompañados en ocasiones de síntomas vegetativos, ataxia o posturaanormal del tronco, con tendencia a desaparecer espontáneamenteen meses o años, y sin alteraciones entre los episodios. Objetivo.Analizar las características clínicas y evolutivas de los casosvalorados por la Sección de Neuropediatría de nuestro hospital ycatalogados como TPB. Pacientes y métodos. Se han revisado lasanamnesis de los pacientes que figuran con diagnóstico de TPB enla base de datos de la Sección de Neuropediatría durante un períodode 15 años. Se ha contactado telefónicamente con los pacientesque no seguían un control. Resultados. Se han considerado13 pacientes con TPB con los criterios típicos, y 4 con TPB posible(p-TPB), por haber presentado un episodio aislado de tortícolis.Se realizó neuroimagen a nueve niños (69,2%) del grupo deTPB: a seis sólo ecografía transfontanelar (ECO TF), a un niñoECO TF y tomografía axial computarizada (TAC), a uno sólo TAC,y a otro, TAC y resonancia magnética. A todos los casos de p-TPBse les realizó neuroimagen: a dos TAC, y a los otros dos, ECO TF.Conclusiones. Establecer el diagnóstico de TPB con criterios estrictospuede excluir casos atípicos. Al no disponer de marcadoresbiológicos, el diagnóstico debe basarse en la clínica y evolución y,en algunos casos, deben realizarse exámenes complementariospara descartar otras patologías. Se debe tranquilizar a las familiasal diagnosticar al niño TPB, y explicarles que es un proceso benignocon tendencia a desaparecer espontáneamente

Introduction. Benign paroxysmal torticollis (BPT) is characterised by recurring episodes of lateral bending of theneck, occasionally accompanied by vegetative symptoms, ataxia or an abnormal position of the trunk, with a tendency todisappear spontaneously after some months or years, and with no alterations between episodes. Aim. To analyse the clinicaland developmental characteristics of the cases evaluated by the Neuropaediatric Service at our hospital that were classifiedas BPT. Patients and methods. We reviewed the history of the patients with BPT included in the Neuropaediatric Servicedatabase over a 15-year period. Patients who were not following any kind of control were contacted by telephone. Results. Wefound 13 BPT patients with typical criteria, and 4 others with possible BPT (p-BPT), because they had had an isolated episodeof torticollis. Neuroimaging was carried out in nine children (69.2%) from the BPT group: this included only transfontanellarultrasound recording (TF USR) in six cases, TF USR and computerised axial tomography (CAT) in one child, only a CAT scanin one case, and CAT and magnetic resonance imaging in another. Neuroimaging was performed in all the cases of p-BPT:CAT scans were carried out in two cases and TF USR was used in two others. Conclusions. Atypical cases can be excluded byestablishing a diagnosis of BPT with strict criteria. Since no biological markers are available, the diagnosis must be based onthe clinical pattern and course and, in some cases, complementary examinations have to be performed to preclude otherpathologies. When a child is diagnosed with BPT, the family needs to be reassured and told that it is a benign process with atendency to disappear spontaneously

Recambio mineral óseo y densitometría ósea en pacientes sometidos a dieta de riesgo: hiperfenilalaninemia y galactosemia. Respuesta del autor / Bone mineral turnover and bone densitometry in patients with dietary risk: hyperphenylalaninemia and galactosemia. Author’s response

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[Bone mineral turnover and bone densitometry in patients with a high-risk diet: hyperphenylalaninemia and galactosemia]. / Recambio mineral óseo y densitometría ósea en pacientes sometidos a dieta de riesgo: hiperfenilalaninemia y galactosemia.

INTRODUCTION: Patients with any type of congenital metabolism error are at risk for developing osteoporosis. To gain further insight into the physiopathology of this disease, we studied bone mineral turnover in 10 children with hyperphenylalaninemia, seven with phenylketonuria and six with galactosemia. Oral intake was strictly controlled and the children followed recommendations for physical exercise. MATERIAL AND METHOD: Markers of bone resorption (hydroxyproline and pyridinoline in urine samples) and markers of bone formation (levels of osteocalcin and C-terminal procollagen peptide type I) were analyzed. Bone mineral density was analyzed by ultrasound densitometry. RESULTS: A non-significant reduction in bone densitometry with respect to the normal population was observed. Bone mineral turnover was slightly diminished in patients with phenylketonuria but was within the normal range in patients with hyperphenylalaninemia and galactosemia. CONCLUSION: Adequate control of dietary intake of both proteins and minerals, as well as a healthy lifestyle, can prevent the development of significant alterations in bone mineralization.

Recambio mineral óseo y densiometría ósea en pacientes sometidos a dieta de riesgo: hiperfenilalaninemia y galactosemia / Bone mineral turnover and bone densitometry in patients with high-risk diet: hyperphenylalaninemia and galactosemia

Introducción. Con el fin de conocer mejor la fisiopatología de la osteoporosis que tienen riesgo de desarrollar los pacientes afectados de algunos errores congénitos del metabolismo se ha estudiado el recambio mineral óseo en 10 niños afectados de hiperfenilalaninemia media, siete de fenilcetonuria y seis de galactosemia, con un control estricto de la ingesta y unas recomendaciones de ejercicio físico adecuadas. Material y método. Se han analizando marcadores de resorción ósea, hidroxiprolina y piridinolina urinaria y de aposición ósea, osteocalcina y propéptido C terminal sérico. La densidad mineral ósea se ha analizado mediante densitometría por ultrasonidos. Resultados. Se objetiva que la densitometría ósea está disminuida respecto a la población normal sin llegar a ser estadísticamente significativo. El recambio mineral óseo está ligeramente disminuido en pacientes con fenilcetonuria, mientras que se encuentra dentro del rango de normalidad en pacientes con hiperfenilalaninemia media y galactosemia. Conclusión. El adecuado control de la ingesta dietética, tanto proteica como mineral, así como del régimen de vida saludable pueden prevenir la aparición de alteraciones significativas de la mineralización ósea

Introduction. Patients with any type of congenital metabolism error are at risk for developing osteoporosis. To gain further insight into the physiopathology of this disease, we studied bone mineral turnover in 10 children with hyperphenylalaninemia, seven with phenylketonuria and six with galactosemia. Oral intake was strictly controlled and the children followed recommendations for physical exercise. Material and method. Markers of bone resorption (hydroxyproline and pyridinoline in urine samples) and markers of bone formation (levels of osteocalcin and C-terminal procollagen peptide type I) were analyzed. Bone mineral density was analyzed by ultrasound densitometry. Results. A non-significant reduction in bone densitometry with respect to the normal population was observed. Bone mineral turnover was slightly diminished in patients with phenylketonuria but was within the normal range in patients with hyperphenylalaninemia and galactosemia. Conclusion. Adequate control of dietary intake of both proteins and minerals, as well as a healthy lifestyle, can prevent the development of significant alterations in bone mineralization

¿Los niños amamantados de forma exclusiva durante 6 meses pesan menos que los de la población de referencia aragonesa? Estudio de antropometría comparada a los 6 meses de vida / Do 6 months breastfed infants weight less than infants of Aragon reference population? Study of compared anthropometrics at 6 months of life

El objetivo principal de este estudio es comparar el desarrollo pondero-estatural de niños amamantados de forma exclusiva durante seis meses de una cohorte de niños que son atendidos en un Centro de Salud con los de la población aragonesa de referencia cuyos datos se obtuvieron del estudio longitudinal del crecimiento de la Fundación Andreas Prader. Se estima que la tasa de lactancia materna de esta población de referencia a partir de los 3meses de vida es menor de un 30%. Se pretende con esta comparación saber si el desarrollo delos niños amamantados es peor y si por lo tanto existe fundamento para introducir la alimentación no materna entre los 4 y los 6 meses, o si por el contrario es correcto recomendar lactancia materna exclusiva hasta los 6 meses de vida

The main goal of this study is to compare the growth and physical development of exclusive breastfed infants during 6 months of a cohort of infants of a health centre with the reference population of Aragon obtained of Longitudinal Study of Growth and development of Andreas Prader Foundation.The prevalence of breast feeding in this population is less than 30%. The authors want to know if the growth and development of exclusive breastfeeding during 6 months is worse than the reference population. So that it may be necessary to introduce non maternal food before 6months

Valores normales de los marcadores del recambio óseo durante la infancia / Normal values of bone turnover markers in childhood

Objetivo: Obtener el perfil normal de los marcadores del recambio óseo durante la infancia para poder valorar el estado de mineralización ósea de la población infantil normal y de la población de riesgo para osteoporosis. Pacientes y métodos: Se ha estudiado una población de 75 niños sanos de entre 6 meses y 14 años de edad. Se han determinado en suero los valores de osteocalcina y de propéptido carboxiterminal del procolágeno tipo I como marcadores de aposición ósea y de la hidroxiprolina y las piridinolinas en orina, como marcadores de resorción ósea. Se ha realizado el análisis estadístico de los resultados. Resultados: Los valores más elevados para estos marcadores se han obtenido en los primeros 4 años de vida. A continuación los de resorción ósea presentan una continua disminución estadísticamente significativa hasta los 14 años de edad (p < 0,05), mientras que los de aposición ósea descienden ligeramente a partir de los 4 años y se mantienen posteriormente estables hasta los 14 años. Discusión: Este comportamiento es compatible con la presencia de un intenso recambio óseo durante los 4 primeros años, y con un predominio de los fenómenos de aposición ósea a lo largo de los primeros 14 años de vida. El conocimiento de los valores normales de dichos marcadores permite valorar el estado de la mineralización ósea de la población infantil normal y de la población de riesgo para osteoporosis (AU)

[Normal values of bone turnover markers in childhood]. / Valores normales de los marcadores del recambio óseo durante la infancia.

OBJECTIVE: To determine the normal profile of bone turnover markers in childhood in order to enable evaluation of bone mineralization status in the healthy pediatric population and in the population at risk of osteoporosis. PATIENTS AND METHODS: A population of 75 healthy children aged between 6 months and 14 years was studied. Levels of osteocalcin and C-terminal procollagen peptide type I, as markers of bone apposition, were determined in serum. Levels of hydroxyproline and pyridinoline, as markers of bone resorption, were determined in urine samples. Statistical analysis of the results was performed. RESULTS: The highest levels of the four markers were obtained in the first 4 years of life. Markers of bone resorption showed a continuous statistically significant decrease until the age of 14 years (p < 0.05), whereas markers of bone apposition decreased slightly after the age of 4 years and then remained stable until the age of 14 years. DISCUSSION: These findings are compatible with the presence of intense bone turnover in the first 4 years of life and with a predominance of the phenomenon of bone apposition throughout the first 14 years of life. Knowledge of the normal profile of these markers allows evaluation of bone mineralization status in the healthy pediatric population and in the population at risk of osteoporosis.