Aspectos prácticos de la extracción, conservación y administración de leche materna en el hogar / Practical aspects of expressing, storing and handling breast milk in the home

El amamantamiento proporciona leche materna no deteriorada al lactante en condiciones naturales. Cuando es necesaria la administración de leche materna extraída, hay riesgo de contaminación y deterioro de la leche. La extracción cuidadosa, el mantenimiento de la cadena de frío en la conservación y el transporte y la manipulación adecuada son esenciales para no alterar sus propiedades (AU)

Breast feeding provides the infant with fresh mother’s milk in natural condition. When expressed breast milk has to be used, there is a risk of contamination and deterioration of the milk. Careful expressing, assuring the cold chain during storage and transport and proper handling are essential if its properties are to be maintained (AU)

Recomendaciones para la asistencia respiratoria en el recién nacido (III). Surfactante y óxido nítrico / Recommendations for respiratory support in the newborn (III). Surfactant and nitric oxide

Las recomendaciones incluidas en este documento forman parte de una revisión actualizada de la asistencia respiratoria en el recién nacido. Están estructuradas en 12 módulos y en este trabajo se presenta el módulo 7. El contenido de cada módulo es el resultado del consenso de los miembros del Grupo Respiratorio y Surfactante de la Sociedad Española de Neonatología. Representan una síntesis de los trabajos publicados y de la experiencia clínica de cada uno de los miembros del grupo (AU)

The recommendations included in this document will be part a series of updated reviews of the literature on respiratory support in the newborn infant. These recommendations are structured into twelve modules, and in this work module 7 is presented. Each module is the result of a consensus process including all members of the Surfactant and Respiratory Group of the Spanish Society of Neonatology. They represent a summary of the published papers on each specific topic, and of the clinical experience of each one of the members of the group (AU)

[Recommendations for respiratory support in the newborn (iii). Surfactant and nitric oxide]. / Recomendaciones para la asistencia respiratoria en el recién nacido (iii). Surfactante y óxido nítrico.

The recommendations included in this document will be part a series of updated reviews of the literature on respiratory support in the newborn infant. These recommendations are structured into twelve modules, and in this work module 7 is presented. Each module is the result of a consensus process including all members of the Surfactant and Respiratory Group of the Spanish Society of Neonatology. They represent a summary of the published papers on each specific topic, and of the clinical experience of each one of the members of the group.

Necrosis grasa subcutánea complicada tras tratamiento con hipotermia terapéutica por encefalopatía hipóxico-isquémica grave / Complicated subcutaneous fat necrosis after hypothermia treatment for severe hypoxic-ischemic encephalopathy

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Impacto en la práctica clínica de la apertura de un banco de leche en una unidad neonatal / Clinical impact of opening a human milk bank in a neonatal unit

INTRODUCCIÓN: Los beneficios de la leche donada frente a la fórmula artificial están demostrados, sin embargo no se conoce la influencia de la apertura de un banco de leche en la práctica clínica habitual. El objetivo de este estudio fue medir el impacto en la práctica clínica de la disponibilidad de leche donada para la nutrición de los prematuros ≤32 semanas de edad gestacional. MÉTODOS: Estudio antes-después de la apertura de un banco de leche. Se incluyeron los ≤32 semanas nacidos en el Hospital 12 de Octubre de julio-diciembre de 2005 y de enero-junio de 2008 (6 primeros meses tras la apertura del banco de leche). RESULTADOS: La apertura del banco de leche permitió empezar 31h antes (p < 0,001) la alimentación enteral, se alcanzaron 59,5h antes los 100ml/kg/día (p < 0,001) y 52h antes los 150ml/kg/día (p = 0,002), permitiendo retirar 72h antes la nutrición parenteral. En ningún prematuro se inició la alimentación enteral con fórmula artificial, la exposición a la misma en los primeros 15días de vida bajó del 50 al 16,6% y su consumo durante los primeros 28 días fue significativamente menor. La cantidad consumida de leche de la propia madre fue mayor, al igual que la tasa de lactancia materna exclusiva al alta (54 vs. 40%). CONCLUSIONES: Disponer de leche donada ha permitido avanzar más rápidamente con la nutrición enteral y retirar antes la nutrición parenteral. La exposición a fórmula artificial ha sido menor y mayor el consumo de leche de madre propia y la lactancia materna al alta

INTRODUCTION: The benefits of donor human milk compared with artificial formulas have been well demonstrated; nevertheless the impact in the clinical practice of opening a human milk bank within a neonatal unit has not yet been studied. The main aim of this study was to analyze the impact on the clinical practice of opening a human milk bank in a neonatal unit to provide donor human milk for preterm infants ≤32 weeks of gestational age. METHODS: A before and after study was designed, with the intervention being the opening a human milk bank. Preterm infants ≤32 weeks of gestational age born in the Hospital 12 Octubre from July to December 2005 and January to June 2008 (firsts 6 months after opening the human milk bank) were included. RESULTS: After opening the human milk bank, enteral feedings were started 31h before (P<0.001), 100ml/kg/day were achieved 59.5h before (P<0.001) and 150ml/kg/day 52h before (P=0.002). Enteral feedings were never started LM with artificial formula, the exposure to formula in the first 15 days of life was reduced from 50% to 16.6%, and it's consumption during the first 28 days of life was significantly reduced. There was a higher consumption of own mother's milk during the hospital stay, and a higher rate of exclusive breastfeeding at hospital discharge (54% vs 40%). CONCLUSIONS: The availability of donor human milk has led to quicker progression with enteral feedings and earlier withdrawal of parenteral nutrition. It has reduced the exposure to artificial formulas, and has also increased the intake of own mother's milk during the hospital stay and the rate of exclusive breastfeeding at hospital discharge

[Clinical impact of opening a human milk bank in a neonatal unit]. / Impacto en la práctica clínica de la apertura de un banco de leche en una unidad neonatal.

INTRODUCTION: The benefits of donor human milk compared with artificial formulas have been well demonstrated; nevertheless the impact in the clinical practice of opening a human milk bank within a neonatal unit has not yet been studied. The main aim of this study was to analyze the impact on the clinical practice of opening a human milk bank in a neonatal unit to provide donor human milk for preterm infants ≤ 32 weeks of gestational age. METHODS: A before and after study was designed, with the intervention being the opening a human milk bank. Preterm infants ≤ 32 weeks of gestational age born in the Hospital 12 Octubre from July to December 2005 and January to June 2008 (firsts 6 months after opening the human milk bank) were included. RESULTS: After opening the human milk bank, enteral feedings were started 31h before (P<.001), 100ml/kg/day were achieved 59.5h before (P<.001) and 150 ml/kg/day 52 h before (P=.002). Enteral feedings were never started LM with artificial formula, the exposure to formula in the first 15 days of life was reduced from 50% to 16.6%, and it's consumption during the first 28 days of life was significantly reduced. There was a higher consumption of own mother's milk during the hospital stay, and a higher rate of exclusive breastfeeding at hospital discharge (54% vs 40%). CONCLUSIONS: The availability of donor human milk has led to quicker progression with enteral feedings and earlier withdrawal of parenteral nutrition. It has reduced the exposure to artificial formulas, and has also increased the intake of own mother's milk during the hospital stay and the rate of exclusive breastfeeding at hospital discharge.

Perfil de prescripción de nutrición parenteral en recién nacidos de muy bajo peso al nacer; período 2006 a 2010 / Profile of parenteral nutrition prescription in very low birth weight infants; period 2006 to 2010

Introducción: Un aporte precoz de nutrientes en las cantidades adecuadas es de vital importancia para los recién nacidos prematuros ya que disminuye el tiempo en alcanzar el peso esperado y en tolerar por vía enteral, disminuyendo la estancia hospitalaria El objetivo principal de este estudio es analizar la prescripción de nutrición parenteral (NP) de pacientes con menos de 1.500 g al nacimiento en los primeros 7 días de vida durante los años 2006-2010 en nuestro hospital y la adecuación de dichas prescripciones a las recomendaciones. Material y métodos: Se recogió el aporte de aminoácidos, glucosa, lípidos (g/kg/día) y calorías (kcal/kg/día) prescritos en la NP de la primera semana de vida. Resultados: Se estudiaron 1.899 prescripciones de nutrición parenteral de los años 2006-2010 que correspondían a la primera semana de vida de 360 pacientes con menos de 1.500 g. El contenido calórico aumentó desde una media de 41,83 kcal/kg/día (1,89 g/kg/día de aminoácidos y 0,69 g/kg/día de lípidos) el primer día de vida hasta 80,61 kcal/kg/día (3,2 g/kg/día de aminoácidos y 2,4 g/kg/día de lípidos) el séptimo día. Cerca de un 70% de las prescripciones de los niños que tuvieron NP el primer día de vida llevaban lípidos. Pero cuando se considera todos los niños que tuvieron NP el cuarto día solo un 17% de dichos pacientes tuvieron aporte de lípidos el primer día de vida. Ninguna prescripción sobrepasaba 120 kcal/kg/día. Solamente un 17,9% de las del sexto día y 25.5% de las del séptimo día aportaban mas de 90 kcal/kg/día. En nuestro hospital no se preparan NP los domingos y festivos. 30,1% y 56,9% de los niños que nacieron en día laborable recibieron NP el primer y segundo día, frente 11,6% y 38,8% de los nacidos en festivo o víspera de festivo. Conclusiones: Un alto porcentaje de niños prematuros no recibe las cantidades recomendadas de nutrientes en la primera semana de vida en nuestro hospital (AU)

Introduction: Early administration of nutrients in adequate amounts is of vital importance to preterm infants because it decreases the time needed to reach the expected weight and tolerate enteral nutrition as well as reducing their hospital stay. The main objective of this study was to analyze the prescription of parenteral nutrition (PN) in patients weighing less than 1,500 g at birth in their first 7 days of life during the years 2006-2010 in our hospital and the adequacy of those requirements to the recommendations. Materials and methods: Amino acids, glucose, lipids (g/ kg/day) and calories (kcal/kg/day) prescribed in the PN of the first week of life were collected. Results: We studied 1899 parenteral nutrition prescriptions from 2006 to 2010 corresponding to the first week of life of 360 patients weighing less than 1,500 g. The caloric content increased from an average of 41.83 kcal/kg/day (1.89 g/kg/ day of amino acids and 0.69 g/kg/day lipids) on the first day of life to 80.61 kcal/kg/day (3.2 g/kg/day of amino acids and 2.4 g/kg/day of lipids) on the seventh day. About 70% of prescriptions for the children who had PN on the first day of life contained lipids. But when all children who had PN on the fourth day were considered, only 17% of these patients had lipid intake on the first day of life. No prescription exceeded 120 kcal/kg/day. Only 17.9% on the sixth day and 25.5% on the seventh day received more than 90 kcal/kg/day. In our hospital PN is not prepared on Sundays and holidays. 30.1% and 56.9% of children born on weekdays received PN the first and second day versus 11.6% and 38.8% of those born on a holiday or the day before holiday. Conclusions: A high percentage of premature babies do not get the recommended amounts of nutrients in their first week of life in our hospital (AU)

[Profile of parenteral nutrition prescription in very low birth weight infants: period 2006 to 2010]. / Perfil de prescripción de nutrición parenteral en recién nacidos de muy bajo peso al nacer: período 2006 a 2010.

INTRODUCTION: Early administration of nutrients in adequate amounts is of vital importance to preterm infants because it decreases the time needed to reach the expected weight and tolerate enteral nutrition as well as reducing their hospital stay. The main objective of this study was to analyze the prescription of parenteral nutrition (PN) in patients weighing less than 1,500 g at birth in their first 7 days of life during the years 2006-2010 in our hospital and the adequacy of those requirements to the recommendations. MATERIALS AND METHODS: Amino acids, glucose, lipids (g/ kg/day) and calories (kcal/kg/day) prescribed in the PN of the first week of life were collected. RESULTS: We studied 1899 parenteral nutrition prescriptions from 2006 to 2010 corresponding to the first week of life of 360 patients weighing less than 1,500 g. The caloric content increased from an average of 41.83 kcal/kg/day (1.89 g/kg/ day of amino acids and 0.69 g/kg/day lipids) on the first day of life to 80.61 kcal/kg/day (3.2 g/kg/day of amino acids and 2.4 g/kg/day of lipids) on the seventh day. About 70% of prescriptions for the children who had PN on the first day of life contained lipids. But when all children who had PN on the fourth day were considered, only 17% of these patients had lipid intake on the first day of life. No prescription exceeded 120 kcal/kg/day. Only 17.9% on the sixth day and 25.5% on the seventh day received more than 90 kcal/kg/day. In our hospital PN is not prepared on Sundays and holidays. 30.1% and 56.9% of children born on weekdays received PN the first and second day versus 11.6% and 38.8% of those born on a holiday or the day before holiday. CONCLUSIONS: A high percentage of premature babies do not get the recommended amounts of nutrients in their first week of life in our hospital.

Seguimiento de recién nacidos de peso menor o igual a 1.500g y edad gestacional menor o igual a 32 semanas durante los 2 primeros años de edad corregida: comparación de 2 periodos de tiempo / Outcome of newborn with birth weight less than or equal to 1500g and gestational age less than or equal to 32 weeks, during the 2 first years of age corrected: comparison of two time periods

Introducción: El cambio en el manejo obstétrico y el avance de los cuidados perinatales ha conseguido mejorar la supervivencia de los RN prematuros, pero es fundamental conocer si esto conllevará un aumento de la discapacidad. Objetivo: Comparar en 2 periodos de tiempo las cifras de mortalidad y discapacidad a los 2 años de edad corregida, de niños con peso al nacimiento ≤1.500g y edad gestacional ≤32 semanas. Metodología: Estudio de seguimiento que incluyó 963 niños, nacidos en nuestra maternidad, entre 1991–2004 que reunían los criterios de estudio. Se ha evaluado la morbilidad neonatal, mortalidad y discapacidad a los 2 años de edad corregida en 2 periodos de tiempo 1991–1998 (periodo I) y 1999–2004 (periodo II) y analizado por subgrupos de peso (peso <1.000g y 1.000–1.500g). Resultados: La mortalidad disminuyó significativamente en el segundo periodo, tanto en los niños con peso ≤1.000g (32 vs. 44%) como en los de 1.000–1.500g (3,6 vs. 9%). Analizando en conjunto los niños ≤1.500g, se observó un aumento de los supervivientes sin discapacidad en el segundo periodo (69 vs. 60%, p=0,003); pero por subgrupos este incremento solo fue significativo en los de peso 1.000–1.500g, pasando del 67–82%. Conclusiones: En nuestro estudio, analizando globalmente todos los niños ≤1.500g a lo largo del tiempo se ha objetivado un aumento de la supervivencia con disminución de la discapacidad. Analizando por subgrupos de peso, la supervivencia ha aumentado en ambos grupos y sin embargo, la discapacidad ha disminuido exclusivamente en los mayores de 1.000g (AU)

Introduction: Advances in perinatal care have resulted in increased survival rates for extremely low birth weight children, but it is fundamental to know if these improved survival rates have been accompanied by increased impairment rates. Objective: To compare, over two different time periods, the survival and disability rates at 2 years of corrected age, among newborns ≤32 weeks and weighed ≤1500g at birth. Methodology: Follow-up study that included 963 children born in the hospital between 1991 and 2004 who met the study criteria. Neonatal morbidity, mortality and disability to 2 years of corrected age in 2 time periods 1991–1998 (period I) and 1999–2004 (period II) have been evaluated and analysed by subgroups of weight (weight <1000g and 1000–1500g). Results: Mortality decreased significantly during the second period, both for children with birth weight <1000g (32% vs 44%) as for those with birth weight between 1000 and 1500 g ( 3,6% vs 9%). Analysing all children ≤1500g, an increase in the survivors without disability was observed in the second period (69% vs 60%, p=0.003); but by subgroups this increase only was significant in children with birth weight 1000–1500g (67% vs 82%). Conclusions: In our study, globally analysing all children with birth weight ≤1500g, it can be seen that there has been an increase in survival without an increase in the frequency of disabilities. Analysing by weight subgroups, survival has increased in both groups, but disability has decreased only in the birth weight 1000–1500g subgroup (AU)

[Outcome of newborn with birth weight less than or equal to 1500g and gestational age less than or equal to 32 weeks, during the 2 first years of age corrected: comparison of two time periods]. / Seguimiento de recién nacidos de peso menor o igual a 1.500g y edad gestacional menor o igual a 32 semanas durante los 2 primeros años de edad corregida: comparación de 2 periodos de tiempo.

INTRODUCTION: Advances in perinatal care have resulted in increased survival rates for extremely low birth weight children, but it is fundamental to know if these improved survival rates have been accompanied by increased impairment rates. OBJECTIVE: To compare, over two different time periods, the survival and disability rates at 2 years of corrected age, among newborns < or =32 weeks and weighed < or = 1500 g at birth. METHODOLOGY: Follow-up study that included 963 children born in the hospital between 1991 and 2004 who met the study criteria. Neonatal morbidity, mortality and disability to 2 years of corrected age in 2 time periods 1991-1998 (period I) and 1999-2004 (period II) have been evaluated and analysed by subgroups of weight (weight < 1000 g and 1000-1500 g). RESULTS: Mortality decreased significantly during the second period, both for children with birth weight <1000 g (32% vs 44%) as for those with birth weight between 1000 and 1500 g ( 3,6% vs 9%). Analysing all children < or = 1500 g, an increase in the survivors without disability was observed in the second period (69% vs 60%, p=0.003); but by subgroups this increase only was significant in children with birth weight 1000-1500 g (67% vs 82%). CONCLUSIONS: In our study, globally analysing all children with birth weight < or = 1500 g, it can be seen that there has been an increase in survival without an increase in the frequency of disabilities. Analysing by weight subgroups, survival has increased in both groups, but disability has decreased only in the birth weight 1000-1500 g subgroup.

Puesta en marcha del banco de leche materna donada en una unidad neonatal / Setting up a donor milk bank within a neonatal unit

La leche materna es el alimento de elección para todos los recién nacidos. Cuando no hay suficiente leche de madre propia, la leche donada es la mejor alternativa. Hay numerosos bancos de leche en Europa, Estados Unidos, Australia, Centroamérica y Sudamérica. En 2007 abrió sus puertas el segundo banco de leche humana del Hospital 12 Octubre (BLHDO) de España, en su Servicio de Neonatología. No hay recomendaciones internacionales acerca del funcionamiento de un banco de leche, pero internacionalmente hay varias asociaciones de bancos de leche, cada una con sus guías de actuación; en el BLHDO se decidió seguir el modelo brasileño, ya que aporta parámetros de calidad, y no sólo de seguridad de la leche, comunes con la industria alimentaria. Al no haber legislación que regule la donación de leche se siguen las guías de otros bancos de leche y se han establecido sistemas de seguridad y de trazabilidad igual de estrictos que para la donación de sangre. En este artículo se comentan los distintos procedimientos que se llevan a cabo en el BLHDO, la experiencia de este primer año y los proyectos futuros de éste (AU)

Breast milk is the best choice to feed premature and ill babies, but when there is not enough mother milk available donor breast milk is the best alternative. Nowadays, Milk Banks are present worldwide. In December 2007 the second Spanish Milk Bank opened within the Department of Neonatology of the Hospital 12 Octubre, Madrid (BLHDO). There are no international recommendations for processing breast milk, therefore other Milk Banks guidelines are the only standards to follow. BLHDO uses the Brazilian model as they focus on milk quality, in addition to safety issues. Lack of legislation for human milk processing in Spain has led to BLHDO complying with Spanish Law on blood and tissues donation with its strict regulations on safety issues and record keeping. This article summarises the first year of operating the BLHDO and its future projects and developments (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)

Enfermedades congénitas del metabolismo en el periodo neonatal (II). Manifestaciones clínicas / Inborn errors of metabolism during the neonatal period (II). Clinical manifestations

Analizamos las formas de presentación clínica y el diagnóstico diferencial de los errores congénitos del metabolismo en el periodo neonatal. La identificación precoz de los pacientes con riesgo de padecer una de estas enfermedades nos permitirá poner en marcha los estudios encaminados a establecer el diagnóstico y, al mismo tiempo, iniciar las primeras medidas terapéuticas, consiguiendo así una mejoría en el pronóstico

We analyse the clinical manifestations and the differential diagnosis of inborn errors of metabolism during the neonatal period. The early identification of patients at risk for these diseases enables us to under take studies to achieve the diagnosis and initiate specific therapy, thus improving the prognosis

[Congenital anatomic gastrointestinal obstruction: prenatal diagnosis, morbidity and mortality]. / Obstrucción gastrointestinal anatómica congénita: diagnóstico prenatal, morbilidad y mortalidad.

OBJECTIVES: To describe the morbidity and mortality associated with congenital gastrointestinal obstruction, assess the usefulness of prenatal diagnosis, and analyze the results in relation to the site of obstruction. PATIENTS AND METHODS: We performed a retrospective case series study of 148 patients in a 13-year period (1990-2003). Sites of obstruction was classified into three groups: I) gastric, duodenal or jejunal; II) ileal or colonic; III) anorectal. RESULTS: There were 65 patients in group I, 38 in group II and 45 in group III. Surgery was performed in 137 patients. Trisomy was diagnosed in 15 patients, polymalformation syndrome in 16 patients, and cystic fibrosis in seven patients. More than one site of intestinal obstruction was found in 22 patients (15 %). Morbidity consisted of nosocomial sepsis in 32 %, necrotizing enterocolitis in 7 %, and short gut in 3.4 %. Prematurity (48 %) was associated with higher morbidity and mortality. Overall mortality was 14.2 %, mainly associated with other malformations and extraintestinal problems. When patients with other extraintestinal malformations were excluded, the highest mortality was found in group II (OR: 12.19; CI: 2.4-76.2). The overall sensitivity of prenatal diagnosis was 0.44 and the mean gestational age at diagnosis was 31.6 weeks (SD 5.2). According to the site of obstruction, sensitivity was 0.77 in group I, 0.39 in group II and 0.04 in group III. Prenatal diagnosis did not alter prognosis. CONCLUSIONS: Morbidity and mortality were strongly influenced by extraintestinal problems. The most severe intestinal complications were midgut volvulus and necrotizing enterocolitis. Prenatal diagnosis was late and showed good sensitivity only in group I.

Enfermedades congénitas del metabolismo en el periodo neonatal (I). Generalidades / Inborn errors of metabolism during the neonatal period (I). General aspects

Las enfermedades congénitas del metabolismo son raras individualmente pero frecuentes en su conjunto, debido al gran número de entidadeds escritas. A menudo se presentan clínicamente en el periodo neonatal. Los avances en su diagnóstico y tratamiento, por un lado, han mejorado enormemente el pronóstico en muchas de ellas y, por otro, nos permiten prevenir nuevos casos mediante el consejo genético y el diagnóstico prenatal. Por todo ello, es de suma importancia que los neonatólogos y pediatras estén familiarizados con estas enfermedades, y a que serán ellos los que habrán de identificar a los pacientes que se pueden beneficiar de un estudio más profundo y de un tratamiento específico

Inborn errors of metabolism are rare at the individual level, but are common at the collective level, due to the great number of entities described in recent years. The first signs are often detected during the neonatal period. The advances in the diagnosis and treatment of these diseases have substantially improved the prognosis of many of them, while they have also enabled us to prevent new cases through genetic counseling and prenatal diagnosis. For these reasons, it is of the utmost importance that general pediatricians and neonatologists familiarize themselves with these disorders, since they need to beable to identify those patients that might benefit from metabolic studies and specific treatment

Obstrucción gastrointestinal anatómica congénita: diagnóstico prenatal, morbilidad y mortalidad / Congenital anatomic gastrointestinal obstruction: prenatal diagnosis, morbidity and mortality

Objetivos Describir la morbimortalidad de la obstrucción gastrointestinal congénita, valorar la utilidad del diagnóstico prenatal y analizar los resultados según el nivel de obstrucción. Pacientes y métodos Estudio descriptivo retrospectivo de 148 pacientes en el período 1990-2003. Los niveles de obstrucción se clasificaron en tres grupos: I) gástrica, duodenal o yeyunal; II) ileocólica; III) anorrectal. Resultados La distribución por grupos fue: 65 en el grupo I, 38 en el grupo II y 45 en el grupo III. Se intervinieron 137 pacientes. Se diagnosticaron 15 trisomías, 16 síndromes polimalformativos y siete fibrosis quísticas. En 22 casos (15 %) se encontró más de una zona de obstrucción intestinal. La morbilidad fue la siguiente: sepsis nosocomial 32 %, enterocolitis necrosante 7 %, intestino corto 3,4 %. La prematuridad (48 %) se asoció con mayor morbimortalidad. La mortalidad global fue de 14,2 %, asociada fundamentalmente a otras malformaciones y a problemas extraintestinales. Excluyendo los pacientes con otras malformaciones, el grupo II presentó mayor mortalidad (OR: 12,19; IC: 2,4-76,2). La sensibilidad global del diagnóstico prenatal fue 0,44 y la edad gestacional media del diagnóstico 31,6 semanas (DE: 5,2). Según el nivel de la obstrucción, la sensibilidad fue 0,77 en el grupo I; 0,39 en el grupo II y 0,04 en el grupo III. El diagnóstico prenatal no modificó el pronóstico. Conclusiones La morbimortalidad estuvo muy condicionada por problemas extraintestinales. El vólvulo de intestino medio y la enterocolitis necrosante fueron las complicaciones intestinales más graves. El diagnóstico prenatal fue tardío y sólo tuvo buena sensibilidad en el grupo I

Objectives To describe the morbidity and mortality associated with congenital gastrointestinal obstruction, assess the usefulness of prenatal diagnosis, and analyze the results in relation to the site of obstruction. Patients and methods We performed a retrospective case series study of 148 patients in a 13-year period (1990-2003). Sites of obstruction was classified into three groups: I) gastric, duodenal or jejunal; II) ileal or colonic; III) anorectal. Results There were 65 patients in group I, 38 in group II and 45 in group III. Surgery was performed in 137 patients. Trisomy was diagnosed in 15 patients, polymalformation syndrome in 16 patients, and cystic fibrosis in seven patients. More than one site of intestinal obstruction was found in 22 patients (15 %). Morbidity consisted of nosocomial sepsis in 32 %, necrotizing enterocolitis in 7 %, and short gut in 3.4 %.Prematurity (48 %) was associated with higher morbidity and mortality. Overall mortality was 14.2 %, mainly associated with other malformations and extraintestinal problems. When patients with other extraintestinal malformations were excluded, the highest mortality was found in group II (OR: 12.19; CI: 2.4-76.2). The overall sensitivity of prenatal diagnosis was 0.44 and the mean gestational age at diagnosis was 31.6 weeks (SD 5.2). According to the site of obstruction, sensitivity was 0.77 in group I, 0.39 in group II and 0.04 in group III. Prenatal diagnosis did not alter prognosis. Conclusions Morbidity and mortality were strongly influenced by extraintestinal problems. The most severe intestinal complications were midgut volvulus and necrotizing enterocolitis. Prenatal diagnosis was late and showed good sensitivity only in group I

Factores determinantes de la elevación de la uremia en los primeros días de vida en recién nacidos con menos de 30 semanas de gestación / Determinants of uremia elevation in the first days of life in premature infants born at less than 30 weeks of gestation

Objetivo: Encontrar los determinantes asociados con elevación de la uremia en los primeros días de vida en prematuros muy inmaduros. Métodos: Estudio prospectivo de una cohorte de recién nacidos con menos de 30 semanas de gestación. Resultados: Se incluyeron 48 prematuros nacidos vivos, de los que 10 fallecieron. Los aportes medios de líquidos fueron 55, 72, 88 y 124 ml/kg en el primer, segundo, tercer y séptimo días de vida. El aporte de aminoácidos en los primeros 2 días fue escaso y no se relacionó con la uremia. El 31 por ciento presentaron hipernatremia. En 31 prematuros se obtuvo la uremia entre el quinto y el décimo día de vida. Se encontraron 12 (38,7 por ciento) con valores de 100 mg/dl o superiores, sin elevación de la creatininemia. Todos ellos tuvieron menos de 27 semanas de gestación, menos de 850 g al nacer, mayor pérdida de peso (19,2 por ciento frente a 13,8 por ciento; p = 0,037) y mayor natremia máxima (150,2 mEq/l frente a 146,6 mEq/l; p = 0,023). El uso de furosemida aumentó el riesgo de uremia elevada (riesgo relativo [RR], 2,54; intervalo de confianza del 95 por ciento [IC 95 por ciento], 1,05-6,14).Conclusiones La uremia total de 100 mg/dl o superior se relacionó con determinantes asociados con excesiva pérdida precoz de agua libre, como mayor pérdida de peso, mayor natremia máxima, uso de furosemida, menor gestación y menor peso al nacer (AU)

[Determinants of uremia elevation in the first days of life in premature infants born before 30 weeks of gestation]. / Factores determinantes de la elevación de la uremia en los primeros días de vida en recién nacidos con menos de 30 semanas de gestación.

OBJECTIVE: To identify the determinants associated with uremia elevation in the first days of life in extremely premature infants. METHODS: We performed a prospective cohort study in a cohort of neonates born at less than 30 weeks of gestation. RESULTS: Forty-eight preterm infants were included, of which 10 died. The mean fluid administration was 55, 72, 88 and 124 mL/kg on the first, second, third and seventh days of life. Amino acid doses were low in the first two days of life and were unrelated to uremia elevation. Thirty-one percent of the infants presented hypernatremia. Uremia was measured in 31 infants between the fifth and tenth days of life and 12 infants (38.7 %) had uremia values of 100 mg/dL or higher, without creatinine elevation. All of these infants were born at less than 27 weeks of gestation, weighed less than 850 grams at birth, and showed greater weight loss (19.2 % vs. 13.8 %; p 5 0.037) and higher natremia (150.2 mEq/L vs. 146.6 mEq/L; p 5 0.023). The use of furosemide increased the risk of elevated uremia (relative risk: 2.54; 95 % confidence interval: 1.05 6.14). CONCLUSIONS: Total uremia of 100 mg/dL or higher was associated with dehydration, greater weight loss, higher natremia, furosemide use, lower gestational age, and lower birth weight.

[Age for sitting and walking in children born weighing less than 1,500 g and normal motor development at two years of age]. / Edad de sedestación y marcha en niños con peso al nacer menor de 1.500 g y desarrollo motor normal a los dos años.

OBJECTIVE: To estimate the corrected age (CA) of acquisition of sitting and walking in very low birth weight infants with normal motor outcome at 2 years of age and to determine whether transient hypertonia is associated with a delay in the acquisition of sitting and walking. METHODS: Follow-up study of very low birth weight infants (under 1500 g) admitted to the Neonatology Department of the Hospital 12 de Octubre from January 1991 to December 1996. At 2 years of age, 260 infants had normal motor outcome and 36% of these had been diagnosed with transient hypertonia. The age at which sitting and walking were attained was recorded by interview with the parents and was confirmed by examination. RESULTS: The mean CA at which sitting was attained was 7.2 +/- 1.4 months. Ninety percent of the infants had acquired sitting at a CA of 9 months. Mean age at which walking was attained was 13.4 +/- 2.8 months. Ninety percent could walk at 16 months. Infants with and without transient hypertonia acquired sitting and walking at the same age. CONCLUSIONS: Ninety percent of very low birth weight infants attained sitting at a CA of 9 months and walking at a CA of 16 months. Transient hypertonia did not modify the pattern of sitting and walking and it could be considered a variation of normality within the development of very premature infants.