Seventy eight children born small for gestational age without catch-up growth treated with growth hormone from the prepubertal stage until adult height age. An evaluation of puberty and changes in the metabolic profile.

A wide variation in height gain rate is observed in children small for gestational age (SGA) treated with growth hormone (GH). The aim of this study was to evaluate prepubertal and pubertal growth, height gain attained at adult age and to assess potential predictive factors in catch-up growth. Changes in metabolic profile were also analyzed. PATIENTS AND METHODS: Seventy-eight children born SGA were treated with a GH median dose of 33.0±2.8mcg/kg/day at a mean age of 7.3±2.0 (boys) and 6.0±1.8 (girls). RESULTS: Mean height (SDS) at GH onset was -3.31±0.7 for boys and -3.48±0.7 for girls. According to age at pubertal growth spurt onset patients were classified in their pubertal maturity group. Adult height attained expressed in SDS was -1.75±0.7 for boys and -1.69±1.0 for girls, both below the range of their mid-parental height. The greatest height gain occurred during the prepubertal period. Patients with greater height gain were lighter (p<0.001), shorter (p=0.005), and younger (p=0.02) at the start of GH, and also showed a greater increase in growth velocity during the first year on GH (p<0.001). SGA children started puberty at the same age and with the same distribution into pubertal maturity group as the reference population. No relevant GH-related adverse events were reported, including in the insulin resistance parameters evaluated. Differences were found in fasting plasma glucose values, but were without clinical relevance. IGF-I plasma values remained within the safety range. CONCLUSIONS: GH therapy is safe and beneficial for SGA children. The response to GH therapy is widely heterogeneous, suggesting that GH should be started at a young age and the GH dose prescribed should be individualized. SGA children started puberty at the same age as the reference population. The only factor that predicts greater adult height is growth velocity during the first year of therapy.

Seventy eight children born small for gestational age without catch-up growth treated with growth hormone from the prepubertal stage until adult height age. An evaluation of puberty and changes in the metabolic profile.

A wide variation in height gain rate is observed in children small for gestational age (SGA) treated with growth hormone (GH). The aim of this study was to evaluate prepubertal and pubertal growth, height gain attained at adult age and to assess potential predictive factors in catch-up growth. Changes in metabolic profile were also analyzed. PATIENTS AND METHODS: Seventy-eight children born SGA were treated with a GH median dose of 33.0±2.8mcg/kg/day at a mean age of 7.3±2.0 (boys) and 6.0±1.8 (girls). RESULTS: Mean height (SDS) at GH onset was -3.31±0.7 for boys and -3.48±0.7 for girls. According to age at pubertal growth spurt onset patients were classified in their pubertal maturity group. Adult height attained expressed in SDS was -1.75±0.7 for boys and -1.69±1.0 for girls, both below the range of their mid-parental height. The greatest height gain occurred during the prepubertal period. Patients with greater height gain were lighter (p<0.001), shorter (p=0.005), and younger (p=0.02) at the start of GH, and also showed a greater increase in growth velocity during the first year on GH (p<0.001). SGA children started puberty at the same age and with the same distribution into pubertal maturity group as the reference population. No relevant GH-related adverse events were reported, including in the insulin resistance parameters evaluated. Differences were found in fasting plasma glucose values, but were without clinical relevance. IGF-I plasma values remained within the safety range. CONCLUSIONS: GH therapy is safe and beneficial for SGA children. The response to GH therapy is widely heterogeneous, suggesting that GH should be started at a young age and the GH dose prescribed should be individualized. SGA children started puberty at the same age as the reference population. The only factor that predicts greater adult height is growth velocity during the first year of therapy.

Validación de las ecuaciones propuestas por la Iniciativa Global de Función Pulmonar (GLI) y las de Todas las Edades para espirometría forzada en preescolares sanos españoles / Validation of Global Lung Function Initiative and All Ages Reference Equations for Forced Spirometry in Healthy Spanish Preschoolers

Introducción: La publicación reciente de ecuaciones de referencia de espirometría multiétnicas para edades de 3 a 95 años tiene como objetivo evitar las discontinuidades relacionadas con la edad y proporcionar un estándar mundial para la interpretación de los resultados de la espirometría. Objetivos: Validar las ecuaciones de la Global Lung Function Initiative (GLI-2012) y All ages (FEV0,5) en niños preescolares españoles, para verificar la adecuación de estas ecuaciones para su uso clínico. Métodos: Se realizaron espirometrías forzadas en niños de 3 a 6 años de edad de 10 colegios seleccionados aleatoriamente en Barcelona (España). Se aplicaron los criterios de control de calidad de Stanojevic et al. Se calculó el z-score según las ecuaciones GLI-2012. Se consideró que para que las ecuaciones GLI-2012 pudieran ser aplicables en nuestra población, la media expresada en z-score de cada parámetro debía de tener un valor próximo a 0 y una desviación estándar (DS) de 1, aceptando como máximo una diferencia de ± 0,5 z-scores respecto a la media. Resultados: De los 543 niños reclutados, 405 (74,6%) eran «sanos», y de ellos 380 caucásicos. De estos, 81,6% (169 mujeres, 141 hombres) realizaron maniobras técnicamente aceptables y reproducibles para evaluar la FEVt, y el 69,5% logró una meseta espiratoria final adecuada. Los z-scores para FVC, FEV1, FEV1/FVC, FEV0,75, FEV0,75/FVC, FEV0,5, FEF75 y FEF25-75 estuvieron incluidos entre ± 0,5 z-scores, salvo el FEV1/FVC (0,53 z-scores). Conclusiones: Las ecuaciones GLI son apropiadas para los niños preescolares españoles. Estos datos proporcionan nuevas evidencias para apoyar su utilización (AU)

Introduction: Recent publication of multi-ethnic spirometry reference equations for subjects aged from 3-95 years aim to avoid age-related discontinuities and provide a worldwide standard for interpreting spirometric test results. Objectives: To assess the agreement of the Global Lung Function Initiative (GLI-2012) and All ages (FEV0.5) reference equations with the Spanish preschool lung function data. To verify the appropriateness of these reference values for clinical use in Spanish preschool children. Methods: Spirometric measurements were obtained from children aged 3 to 6 years attending 10 randomly selected schools in Barcelona (Spain). Stanojevic's quality control criteria were applied. Z-scores were calculated for the spirometry outcomes based on the GLI equations. If the z-score (mean) of each parameter was close to 0, with a maximum variance of ± 0.5 from the mean and a standard deviation of 1, the GLI-2012 equations would be applicable in our population. Results: Of 543 children recruited, 405 (74.6%) were 'healthy', and of these, 380 were Caucasians. Of these 380, 81.6% (169 females, 141 males) performed technically acceptable and reproducible maneuvers to assess FEVt, and 69.5% achieved a clear end-expiratory plateau. Z-scores for FVC, FEV1, FEV1/FVC, FEV0.75, FEV0.75/FVC, FEV0.5, FEF75 and FEF25-75 all fell within ± 0.5, except for FEV1/FVC (0.53 z-scores). Conclusions: GLI equations are appropriate for Spanish preschool children. These data provide further evidence to support widespread application of the GLI reference equations (AU)

Oral glucose tolerance test and continuous glucose monitoring to assess diabetes development in cystic fibrosis patients / Test de tolerancia oral a la glucosa y monitorización continua de glucosa para evaluar el desarrollo de diabetes en pacientes con fibrosis quística

Introduction: Patients with cystic fibrosis (CF) undergo a slow and progressive process toward diabetes. Oral glucose tolerance test (OGTT) is recommended to diagnose impaired glucose levels in these patients. Continuous glucose monitoring (CGM) measures glucose profiles under real-life conditions. Objective: To compare OGTT and CGM results in CF patients. Methods: Paired OGTT and 6-day CGM profiles (146.2±9.1h/patient) were performed in 30 CF patients aged 10-18 years. Results: According to OGTT, 14 patients had normal glucose tolerance (NGT), 14 abnormal glucose tolerance (AGT), and two cystic fibrosis-related diabetes (CFRD). In 27 patients (13 NGT, 13 AGT, 1 CFRD), CGM showed glucose values ranging from 140 to 200mg/dL during similar monitoring times (2%-14% with NGT, 1%-16.9% with AGT, and 3% with CFRD). Glucose peak levels ≥200mg/dL were seen in seven patients (3 NGT, 3 AGT, 1 CFRD). According to CGM, two patients had all glucose values under 140mg/dL (1 NGT, 1 AGT). Seventeen patients had glucose levels ranging from 140 to 200mg/dL (10 NGT, 6 AGT, 1 CFRD). Ten patients (3 NGT, 7 AGT) had glucose values ≥200mg/dL for ≤1% of the monitoring time and one (CFRD) for >1% of the monitoring time. Conclusions: OGTT results did not agree with those of the CGM. CGM allows for diagnosis of glucose changes not detected by OGTT. Such changes may contribute to optimize pre-diabetes management in CF patients (AU)

Introducción: Los pacientes con fibrosis quística (FQ) evolucionan lenta y progresivamente hacia la diabetes, siendo el test de tolerancia oral a la glucosa (TTOG) el método utilizado para diagnosticar sus alteraciones glucémicas. La monitorización continua de glucosa (MCG) proporciona perfiles de glucosa en condiciones de vida habituales del paciente. Objetivo: Comparar los resultados del TTOG y de la MCG en pacientes con FQ. Métodos: TTOG seguido de MCG (146,2±9,1h/paciente) en 30 pacientes con FQ (10-18 años de edad). Resultados: Según el TTOG, 14 pacientes presentaron tolerancia normal a la glucosa (TNG), 14 tolerancia anormal a la glucosa (TAG) y 2 diabetes relacionada con la fibrosis quística (DRFQ). En 27 pacientes (13 con TNG, 13 con TAG, uno con DRFQ) la MCG mostró valores de glucosa 140-200mg/dL durante periodos similares de tiempo (2-14%, 1-16,9% y 3%, respectivamente). Picos de glucosa ≥200mg/dL se observaron en 7 pacientes (3 con TNG, 3 con TAG y uno con DRFQ). Según la MCG, 2 pacientes tuvieron todos los valores de glucosa <140mg/dL (uno con TNG y otro con TAG); 17 pacientes entre 140-200mg/dL (10 con TNG, 6 con TAG y uno con DRFQ); 10 pacientes ≥200mg/dL durante ≤1% del tiempo valorado (3 con NGT, 7 con TAG) y uno ≥200mg/dL durante >1% del tiempo valorado (con DRFQ). Conclusiones: Los resultados del TTOG no concuerdan con los de la MCG. La MCG permite el diagnóstico de anomalías de la glucosa no detectadas mediante el TTOG y sus resultados podrían contribuir a optimizar el tratamiento de la prediabetes en estos pacientes (AU)

Validation of Global Lung Function Initiative and All Ages Reference Equations for Forced Spirometry in Healthy Spanish Preschoolers. / Validación de las ecuaciones propuestas por la Iniciativa Global de Función Pulmonar (GLI) y las de Todas las Edades para espirometría forzada en preescolares sanos españoles.

INTRODUCTION: Recent publication of multi-ethnic spirometry reference equations for subjects aged from 3-95 years aim to avoid age-related discontinuities and provide a worldwide standard for interpreting spirometric test results. OBJECTIVES: To assess the agreement of the Global Lung Function Initiative (GLI-2012) and All ages (FEV0.5) reference equations with the Spanish preschool lung function data. To verify the appropriateness of these reference values for clinical use in Spanish preschool children. METHODS: Spirometric measurements were obtained from children aged 3 to 6 years attending 10 randomly selected schools in Barcelona (Spain). Stanojevic's quality control criteria were applied. Z-scores were calculated for the spirometry outcomes based on the GLI equations. If the z-score (mean) of each parameter was close to 0, with a maximum variance of ± 0.5 from the mean and a standard deviation of 1, the GLI-2012 equations would be applicable in our population. RESULTS: Of 543 children recruited, 405 (74.6%) were 'healthy', and of these, 380 were Caucasians. Of these 380, 81.6% (169 females, 141 males) performed technically acceptable and reproducible maneuvers to assess FEVt, and 69.5% achieved a clear end-expiratory plateau. Z-scores for FVC, FEV1, FEV1/FVC, FEV0.75, FEV0.75/FVC, FEV0.5, FEF75 and FEF25-75 all fell within ± 0.5, except for FEV1/FVC (0.53 z-scores). CONCLUSIONS: GLI equations are appropriate for Spanish preschool children. These data provide further evidence to support widespread application of the GLI reference equations.

Oral glucose tolerance test and continuous glucose monitoring to assess diabetes development in cystic fibrosis patients.

INTRODUCTION: Patients with cystic fibrosis (CF) undergo a slow and progressive process toward diabetes. Oral glucose tolerance test (OGTT) is recommended to diagnose impaired glucose levels in these patients. Continuous glucose monitoring (CGM) measures glucose profiles under real-life conditions. OBJECTIVE: To compare OGTT and CGM results in CF patients. METHODS: Paired OGTT and 6-day CGM profiles (146.2±9.1h/patient) were performed in 30 CF patients aged 10-18 years. RESULTS: According to OGTT, 14 patients had normal glucose tolerance (NGT), 14 abnormal glucose tolerance (AGT), and two cystic fibrosis-related diabetes (CFRD). In 27 patients (13 NGT, 13 AGT, 1 CFRD), CGM showed glucose values ranging from 140 to 200mg/dL during similar monitoring times (2%-14% with NGT, 1%-16.9% with AGT, and 3% with CFRD). Glucose peak levels ≥200mg/dL were seen in seven patients (3 NGT, 3 AGT, 1 CFRD). According to CGM, two patients had all glucose values under 140mg/dL (1 NGT, 1 AGT). Seventeen patients had glucose levels ranging from 140 to 200mg/dL (10 NGT, 6 AGT, 1 CFRD). Ten patients (3 NGT, 7 AGT) had glucose values ≥200mg/dL for ≤1% of the monitoring time and one (CFRD) for >1% of the monitoring time. CONCLUSIONS: OGTT results did not agree with those of the CGM. CGM allows for diagnosis of glucose changes not detected by OGTT. Such changes may contribute to optimize pre-diabetes management in CF patients.

[Plasma 25-OH Vitamin D Concentrations in Cord Blood after Summer Months, Spain]. / Concentraciones plasmáticas de 25-OH-vitamina D en sangre de cordón umbilical tras los meses de verano.

OBJECTIVE: Plasma vitamin D (25(OH)D) levels in the newborn are dependent on maternal stores. Several studies showing a high prevalence of vitamin D deficiency in pregnant women have been published last years. The aim of the study was to analyze 25(OH)D levels in cord blood after summer month, determine whether there is a relation with different variables. METHODS: 103 pregnant women were recruited between October and early December 2014, whose gestations took place during month of maximum sun exposure. Plasmatic 25(OH)D values were measured in cord blood at birth. Clinical record data were collected and a nutritional survey was made on maternal vitamin D and calcium intake and sun exposure. Statistical analysis was performed using SPSS. Comparisons were performed using Kruskal-Wallis and Mann-Whitney U tests, and correction for multiple comparisons using Bonferroni. P value smaller than 0.05 and smaller than 0.0083 for multiple comparisons were considered sta¬tistically significant. RESULTS: Mean 25(OH)D value in cord blood was 12.36± 7.2 ng/ml. Vitamin D deficiency was present in 83.4% of women. A statistically significant correlation was observed between lowvitamin D levels and low vitamin D intake (correlation coefficient 0.29); Ethnic group, with the highest level in caucasic group (17.9 ± 5.83 ng/ml) and the lowest in indopakistani group (6.68 ± 4.2 ng/ml); the use of traditional clothing (5.64 ± 3.09 ng/ml); low sun exposure and dark skin phototype with a correlation coefficient of 0.67 and -0.48, respectively. CONCLUSIONS: There is a high prevalence of vitamin D deficiency in pregnant women regardless of the season and increased sun exposure. Low vitamin D levels in cord blood were significantly related to ethnicity (Indopakistan and Maghreb), low sun exposure and dark skin phototype. No statistically significant differences were found between vitamin D levels and perinatal variables studied.

OBJETIVO: Los niveles de vitamina D (25(OH)D) del recién nacido dependen de los depósitos maternos. En los últimos años se han publicado estudios que muestran una elevada prevalencia de deficiencia de vitamina D en mujeres embarazadas, viéndose en algunos diferencias estacionales. El objetivo del presente estudio fue determinar los valores de 25(OH)D en sangre de cordón después de los meses de verano y determinar su relación con diferentes variables. METODOS: Se seleccionó a 103 mujeres en el momento del parto durante los meses de octubre, noviembre y principios de diciembre, cuyas gestaciones tuvieron lugar durante meses de máxima exposición solar. Se determinaron las concentraciones de 25(OH)D en sangre de cordón umbilical y se recogieron datos perinatales, ingesta de vitamina D y calcio y exposición solar mediante cuestionario. Se realizó el análisis estadístico mediante el programa SPSS. Las comparaciones se realizaron mediante test de Kruskal-Wallis y U de Mann-Whitney, aplicando corrección por comparaciones múltiples de Bonferroni. Se consideró estadísticamente significativa una p inferior a 0,05 y de 0,0083 para comparaciones múltiples. RESULTADOS: El valor medio de 25(OH)D en sangre de cordón fue 12,36±7,2 ng/ml. El 83,4% de las mujeres presentaron niveles deficitarios. Se observó una correlación estadísticamente significativa entre los niveles bajos de vitamina D y la baja ingesta de vitamina D (coeficiente de correlación 0,29); la etnia, presentando el valor más alto la etnia caucásica (17,9 ± 5,83 ng/ml) y el menor la etnia indopakistaní (6,68 ± 4,2 ng/ml); el uso de indumentaria tradicional (5,64 ± 3,09 ng/ml); la baja exposición solar y el fototipo cutáneo oscuro con un coeficiente de correlación de 0,67 y -0,48 respectivamente. CONCLUSIONES: Existe una elevada prevalencia de deficiencia de vitamina D en sangre de cordón umbilical independiente de la exposición solar. Se observó una correlación entre niveles bajos de vitamina D y etnia, indumentaria tradicional, baja exposición solar y fototipo de piel oscura. No se observaron diferencias estadísticamente significativas entre los niveles de vitamina D y las variables perinatales estudiadas.

Concentraciones plasmáticas de 25-OH-Vitamina D en sangre de cordón umbilical tras los meses de verano / Plasma 25-OH Vitamin C concentrations in cord blood after summer months, Spain

Fundamentos: Los niveles de vitamina D (25(OH)D) del recién nacido dependen de los depósitos maternos. En los últimos años se han publicado estudios que muestran una elevada prevalencia de deficiencia de vitamina D en mujeres embarazadas, viéndose en algunos diferencias estacionales. El objetivo del presente estudio fue determinar los valores de 25(OH)D en sangre de cordón después de los meses de verano y determinar su relación con diferentes variables. Métodos: Se seleccionó a 103 mujeres en el momento del parto durante los meses de octubre, noviembre y principios de diciembre, cuyas gestaciones tuvieron lugar durante meses de máxima exposición solar. Se determinaron las concentraciones de 25(OH)D en sangre de cordón umbilical y se recogieron datos perinatales, ingesta de vitamina D y calcio y exposición solar mediante cuestionario. Se realizó el análisis estadístico mediante el programa SPSS. Las comparaciones se realizaron mediante test de Kruskal- Wallis y U de Mann-Whitney, aplicando corrección por comparaciones múltiples de Bonferroni. Se consideró estadísticamente significativa una p<0,05 y de 0,0083 para comparaciones múltiples. Resultados: El valor medio de 25(OH)D en sangre de cordón fue 12,36±7,2 ng/ml. El 83,4% de las mujeres presentaron niveles deficitarios. Se observó una correlación estadísticamente significativa entre los niveles bajos de vitamina D y la baja ingesta de vitamina D (coeficiente de correlación 0,29); la etnia, presentando el valor más alto la etnia caucásica (17,9 ± 5,83 ng/ml) y el menor la etnia indopakistaní (6,68 ± 4,2 ng/ml); el uso de indumentaria tradicional (5,64 ± 3,09 ng/ml); la baja exposición solar y el fototipo cutáneo oscuro con un coeficiente de correlación de 0,67 y -0,48 respectivamente. Conclusiones: Existe una elevada prevalencia de deficiencia de vitamina D en sangre de cordón umbilical independiente de la exposición solar.Se observó una correlación entre niveles bajos de vitamina D y etnia, indumentaria tradicional, baja exposición solar y fototipo de piel oscura. No se observaron diferencias estadísticamente significativas entre los niveles de vitamina D y las variables perinatales estudiadas (AU)

Background: Plasma vitamin D (25(OH)D) levels in the newborn are dependent on maternal stores. Several studies showing a high prevalence of vitamin D deficiency in pregnant women have been published last years. The aim of the study was to analyze 25(OH)D levels in cord blood after summer month, determine whether there is a relation with different variables. Methods: 103 pregnant women were recruited between October and early December 2014, whose gestations took place during month of maximum sun exposure. Plasmatic 25(OH)D values were measured in cord blood at birth. Clinical record data were collected and a nutritional survey was made on maternal vitamin D and calcium intake and sun exposure. Statistical analysis was performed using SPSS. Comparisons were performed using Kruskal-Wallis and Mann-Whitney U tests, and correction for multiple comparisons using Bonferroni. P value <0.05 and <0.0083 for multiple comparisons were considered statistically significant. Results: Mean 25(OH)D value in cord blood was 12.36± 7.2 ng/ml. Vitamin D deficiency was present in 83.4% of women. A statistically significant correlation was observed between lowvitamin D levels and low vitamin D intake (correlation coefficient 0.29); Ethnic group, with the highest level in caucasic group (17.9 ± 5.83 ng/ml) and the lowest in indopakistani group (6.68 ± 4.2 ng/ml); the use of traditional clothing (5.64 ± 3.09 ng/ml); low sun exposure and dark skin phototype with a correlation coefficient of 0.67 and -0.48, respectively. Conclusions: There is a high prevalence of vitamin D deficiency in pregnant women regardless of the season and increased sun exposure. Low vitamin D levels in cord blood were significantly related to ethnicity (Indopakistan and Maghreb), low sun exposure and dark skin phototype. No statistically significant differences were found between vitamin D levels and perinatal variables studied (AU)

Resultados perinatales y disfunción cardiovascular en prematuros con restricción del crecimiento intrauterino en relación con la gravedad de la insuficiencia placentaria / Perinatal outcome and cardiac dysfunction in preterm growth-restricted neonates in relation to placental impairment severity

INTRODUCCIÓN: La restricción del crecimiento intrauterino (RCIU) y la prematuridad se han asociado con una mayor morbimortalidad perinatal, así como con una reprogramación fetal a nivel cardiovascular. Sin embargo, son escasos los estudios sobre el impacto de la RCIU de causa placentaria en los resultados perinatales y en biomarcadores cardiovasculares de recién nacidos prematuros. OBJETIVOS: Determinar las diferencias en morbimortalidad neonatal y biomarcadores de disfunción cardiovascular en sangre de cordón entre prematuros con RCIU de origen placentario y sin RCIU, así como estudiar su relación con la gravedad de la RCIU según el estudio Doppler fetal. MATERIAL Y MÉTODOS: Estudio prospectivo de cohortes: prematuros con RCIU de causa placentaria y prematuros sin RCIU adecuadamente apareados. Clasificación de la gravedad de la RCIU según el Doppler. Análisis comparativo de resultados perinatales, de morbimortalidad neonatal y de niveles en sangre de cordón de biomarcadores de disfunción cardiovascular. RESULTADOS: Los prematuros con RCIU presentan un menor peso, longitud, perímetro craneal y Apgar al nacimiento, así como un aumento de la morbilidad neonatal y de los niveles de biomarcadores de disfunción cardiovascular, comparado con los prematuros sin RCIU. Estas diferencias aumentan con la gravedad de la RCIU determinada por el estudio hemodinámico Doppler prenatal. CONCLUSIONES: Los prematuros afectados de RCIU de causa placentaria presentan un incremento de la morbimortalidad neonatal independiente de la prematuridad, que aumenta de forma estadísticamente significativa con la gravedad de la RCIU. La afectación placentaria y su gravedad también determinan la alteración de biomarcadores de disfunción cardiovascular al nacimiento

INTRODUCTION: Intrauterine growth restriction (IUGR) and prematurity have been associated with increased perinatal morbidity and mortality and also with cardiovascular foetal programming. However, there are few studies on the impact of placenta-related IUGR on perinatal outcomes and cardiovascular biomarkers in pre-term infants. OBJECTIVES: To determine differences in neonatal morbidity, mortality and cord blood biomarkers of cardiovascular dysfunction between pre-term placenta-related IUGR and non-IUGR new-borns, and to analyse their relationship with the severity of IUGR according to foetal Doppler evaluation. MATERIAL AND METHODS: Prospective cohort study: pre-term infants with placenta-related IUGR and matched pre-term infants without IUGR. A Doppler scan was performed, and placenta- IUGR was classified according to severity. Comparative analysis of perinatal outcomes, neonatal morbidity and mortality, and cord blood levels of biomarkers of cardiovascular dysfunction was performed. RESULTS: IUGR new-borns present lower weight, length, head circumference, and Apgar score at birth, as well as increased neonatal and cardiovascular dysfunction biomarker levels, compared with pre-term new-borns without IUGR. These differences increase with the severity of IUGR determined by prenatal umbilical artery Doppler scan. CONCLUSIONS: Placenta-related-IUGR pre-term infants, irrespective of gestational age, present increased neonatal morbidity and mortality that is significantly proportional to the severity of IUGR. Placental impairment and severity also determine levels of cardiovascular dysfunction biomarkers at birth

[Perinatal outcome and cardiac dysfunction in preterm growth-restricted neonates in relation to placental impairment severity]. / Resultados perinatales y disfunción cardiovascular en prematuros con restricción del crecimiento intrauterino en relación con la gravedad de la insuficiencia placentaria.

INTRODUCTION: Intrauterine growth restriction (IUGR) and prematurity have been associated with increased perinatal morbidity and mortality and also with cardiovascular foetal programming. However, there are few studies on the impact of placenta-related IUGR on perinatal outcomes and cardiovascular biomarkers in pre-term infants. OBJECTIVES: To determine differences in neonatal morbidity, mortality and cord blood biomarkers of cardiovascular dysfunction between pre-term placenta-related IUGR and non-IUGR new-borns, and to analyse their relationship with the severity of IUGR according to foetal Doppler evaluation. MATERIAL AND METHODS: Prospective cohort study: pre-term infants with placenta-related IUGR and matched pre-term infants without IUGR. A Doppler scan was performed, and placenta-IUGR was classified according to severity. Comparative analysis of perinatal outcomes, neonatal morbidity and mortality, and cord blood levels of biomarkers of cardiovascular dysfunction was performed. RESULTS: IUGR new-borns present lower weight, length, head circumference, and Apgar score at birth, as well as increased neonatal and cardiovascular dysfunction biomarker levels, compared with pre-term new-borns without IUGR. These differences increase with the severity of IUGR determined by prenatal umbilical artery Doppler scan. CONCLUSIONS: Placenta-related-IUGR pre-term infants, irrespective of gestational age, present increased neonatal morbidity and mortality that is significantly proportional to the severity of IUGR. Placental impairment and severity also determine levels of cardiovascular dysfunction biomarkers at birth.

[Sociodemographic Factors Related to Plasma Concentrations of 25-OH Vitamin D and PTH in Cord Blood]. / Concentraciones plasmáticas de 25-OH vitamina D y parathormona en sangre de cordón umbilical.

BACKGROUND: Plasma 25(OH)D levels in the newborn are dependent on maternal stores, thus, neonates of vitamin D-deficient mothers present a greater risk of hypocalcaemia, rickets and infections the first year of life. Several studies showing a high prevalence of vitamin D deficiency in pregnant women have been published recently. The aim of the study is to analyze the levels of 25(OH)D in cord blood and determine whether there is a relation with nutritional, socioeconomic and clinical factors of pregnant women and their newborns. METHODS: Between March and May 2013, 99 pregnant women were recruited in Hospital del Mar (Barcelona), in whom plasma 25(OH)D and PTH levels were measured in cord blood at birth. Clinical history data were collected and a nutritional survey was made on maternal vitamin D and calcium intake and sun exposure. Statistical analysis was performed using SPSS. Comparisons were performed using Kruskal-Wallis and Mann-Whitney U tests, and correction for multiple comparisons using Bonferroni. P value <0.05 and <0.0083 for multiple comparisons were considered statistically significant. RESULTS: Mean 25(OH)D value in cord blood was 10.4 ± 6 .1 ng/ml. 94% of pregnant women had 25(OH)D levels in cord blood <20 ng/ml. Vitamin D and calcium intake was considered adequate in 92% although sun exposure was deficient in 47%. A correlation between serum 25(OH)D and vitamin D (p 0.033) and calcium intake (p 0.005), sun exposure (p<0.001), ethnicity (p<0.001), skin phototype (p<0.001) and use of traditional clothing (p<0.001) was found. CONCLUSIONS: There is a high prevalence of low levels of vitamin D after winter months in cord blood. The lowest 25(OH D levels were observed in Indo-Pakistani ethnicity, dark phototype and deficient sun exposure.

Concentraciones plasmáticas de 25-OH vitamina d y parathormona en sangre de cordón umbilical / Sociodemographic Factors Related to Plasma Concentrations of 25-OH Vitamin D and PTH in Cord Blood

Fundamentos: Los niveles de vitamina D (25(OH)D) del recién nacido dependen de los depósitos maternos, presentando mayor riesgo de hipocalcemia, raquitismo e infecciones durante el primer año de vida si existe déficit. Recientemente se han publicado estudios que muestran una alta prevalencia de deficiencia de vitamina D en mujeres gestantes. El objetivo del estudio fue analizar los niveles de 25(OH)D en sangre de cordón umbilical y determinar si existe una relación con factores nutricionales, socioeconómicos y clínicos de las mujeres gestantes. Métodos: Entre marzo y mayo de 2013 se seleccionó a 99 gestantes del Hospital del Mar (Barcelona), en las que se determinaron las concentraciones de 25(OH)D y parathormona en sangre de cordón umbilical. Se recogieron datos de la historia clínica y se realizó una encuesta sobre ingesta de vitamina D y calcio así como de exposición solar. Se realizó el análisis estadístico mediante el programa SPSS. Las comparaciones se realizaron mediante test de Kruskal-Wallis y U de Mann-Whitney, aplicando corrección por comparaciones múltiples de Bonferroni. Se consideró estadísticamente significativa una p<0,05 y de 0,0083 para comparaciones múltiples. Resultados. El valor medio de 25(OH)D en sangre de cordón fue 10,4±6,1 ng/ml. El 94% de las mujeres presentaron niveles de 25(OH)D en sangre de cordón <20 ng/ml. La ingesta de vitamina D y calcio fueron adecuadas en 92%, aunque la exposición solar resultó deficitaria en 47%. Se encontró una correlación entre niveles de 25(OH)D e ingesta de vitamina D (p<0,033) y calcio (p<0,005), exposición solar (p<0,001), etnia (p<0,001), fototipo cutáneo (p<0,001) y uso de indumentaria tradicional (p<0,001). Conclusiones. Existe una elevada prevalencia de déficit de vitamina D en sangre de cordón umbilical tras los meses de invierno. Los niveles de 25(OH)D más bajos se observaron en etnia indopakistaní, fototipo oscuro y baja exposición solar (AU)

Background. Plasma 25(OH)D levels in the newborn are dependent on maternal stores, thus, neonates of vitamin D-deficient mothers present a greater risk of hypocalcaemia, rickets and infections the first year of life. Several studies showing a high prevalence of vitamin D deficiency in pregnant women have been published recently. The aim of the study is to analyze the levels of 25(OH)D in cord blood and determine whether there is a relation with nutritional, socioeconomic and clinical factors of pregnant women and their newborns. Metthods. Between March and May 2013, 99 pregnant women were recruited in Hospital del Mar (Barcelona), in whom plasma 25(OH)D and PTH levels were measured in cord blood at birth. Clinical history data were collected and a nutritional survey was made on maternal vitamin D and calcium intake and sun exposure. Statistical analysis was performed using SPSS. Comparisons were performed using Kruskal-Wallis and Mann-Whitney U tests, and correction for multiple comparisons using Bonferroni. P value <0.05 and <0.0083 for multiple comparisons were considered statistically significant. Results. Mean 25(OH)D value in cord blood was 10.4±6.1 ng/ml. 94% of pregnant women had 25(OH)D levels in cord blood <20 ng/ml. Vitamin D and calcium intake was considered adequate in 92% although sun exposure was deficient in 47%. A correlation between serum 25(OH)D and vitamin D (p 0.033) and calcium intake (p 0.005), sun exposure (p<0.001), ethnicity (p<0.001), skin phototype (p<0.001) and use of traditional clothing (p<0.001) was found. Conclusions. There is a high prevalence of low levels of vitamin D after winter months in cord blood. The lowest 25(OH D levels were observed in Indo-Pakistani ethnicity, dark phototype and deficient sun exposure (AU)

Miocardiopatía en niños con errores innatos del metabolismo: descripción de 12 casos / Cardiomyopathy and inborn errors of metabolism in children. Study of 12 cases

Fundamento y objetivo: La miocardiopatía en la infancia es una entidad poco frecuente. Los errores innatos del metabolismo (EIM) pueden causar afectación del miocardio por diversos mecanismos fisiopatológicos.Pacientes y método: Menores de 16 años diagnosticados de miocardiopatía y EIM en un período de 11 años (1998-2009). Resultados: Se han estudiado 12 pacientes (8% del total de miocardiopatías), 9 niños y 3 niñas, con una edad mediana al diagnóstico de 6 meses (rango, nacimiento-8,8 años). El 50% comenzaron con sintomatología cardiaca, y la descompensación cardiaca es la forma de presentación que se ha relacionado con un diagnóstico más precoz de la enfermedad (p<0,05). En el estudio ecográfico 10 pacientes presentaron hipertrofia ventricular, relacionándose con enfermedad mitocondrial y lisosomal; sólo 2 pacientes presentaron dilatación ventricular, que se relaciona con alteración del metabolismo de los ácidos grasos (p<0,05). La supervivencia mediana fue de 5 meses (rango, 2-11 meses). Ninguna variable se ha relacionado de forma estadísticamente significativa con la probabilidad de fallecer. Conclusiones:Los pacientes que comienzan en forma de descompensación cardiaca se diagnostican más precozmente. La ecocardiografía orienta en el diagnóstico etiológico y en el seguimiento de la enfermedad metabólica (AU)

Background and objective: Cardiomyopathy in childhood is a rare entity. Inborn errors of metabolism can cause myocardial involvement by several mechanisms. Patients and methods: Patients under 16 years diagnosed with cardiomyopathy and EIM in a period of 11 years (1998-2009) were included. Results: A total of 12 patients were studied (8% of all cardiomyopathies), 9 boys and three girls, with a median age at diagnosis of 6 months (range: birth-8.8 years). Fifty percent had an onset with cardiac symptoms and heart failure was associated with an earlier diagnosis of the disease (P<.05). On ultrasound 10 patients had ventricular hypertrophy, which was associated with mitochondrial and lysosomal disease; only 2 patients had ventricular dilatation, which was associated with altered fatty acid metabolism (P<.05). The median survival was 5 months (range: 2-11 months). No variable was significantly associated with the likelihood of death. Conclusions: Patients with heart failure at onset are diagnosed earlier (before 3 months of life). Echocardiography helps in the diagnosis and monitoring of metabolic disease (AU)

[Cardiomyopathy and inborn errors of metabolism in children. Study of 12 cases]. / Miocardiopatía en niños con errores innatos del metabolismo: descripción de 12 casos.

BACKGROUND AND OBJECTIVE: Cardiomyopathy in childhood is a rare entity. Inborn errors of metabolism can cause myocardial involvement by several mechanisms. PATIENTS AND METHODS: Patients under 16 years diagnosed with cardiomyopathy and EIM in a period of 11 years (1998-2009) were included. RESULTS: A total of 12 patients were studied (8% of all cardiomyopathies), 9 boys and three girls, with a median age at diagnosis of 6 months (range: birth-8.8 years). Fifty percent had an onset with cardiac symptoms and heart failure was associated with an earlier diagnosis of the disease (P<.05). On ultrasound 10 patients had ventricular hypertrophy, which was associated with mitochondrial and lysosomal disease; only 2 patients had ventricular dilatation, which was associated with altered fatty acid metabolism (P<.05). The median survival was 5 months (range: 2-11 months). No variable was significantly associated with the likelihood of death. CONCLUSIONS: Patients with heart failure at onset are diagnosed earlier (before 3 months of life). Echocardiography helps in the diagnosis and monitoring of metabolic disease.

Crecimiento y maduración de los pacientes con hipotiroidismo congénito detectados por el programa de cribado neonatal en Cataluña (1986–1997) / Growth and maturation in the patients with congenital hypothyroidism detected by the neonatal screening program in Catalonia, Spain (1986–1997)

Fundamento y objetivo: En muchos países se han establecido programas de detección precoz del hipotiroidismo congénito (HC) que consiguen un crecimiento y un cociente intelectual normales. El objetivo de este estudio es realizar el seguimiento de los casos de HC detectados mediante el programa de detección precoz del HC en Cataluña. Pacientes y método: El estudio es descriptivo, longitudinal, de una serie de 136 casos de HC detectados mediante el programa de detección precoz en Cataluña del año 1986 al año 1997 y controlados en el Hospital Materno-infantil Vall d´Hebron de Barcelona. Se han valorado los parámetros que indican la intensidad y duración del hipotiroidismo neonatal. Se ha hecho el seguimiento del crecimiento y una exploración psicológica (test de McCarthy y la escala de inteligencia de Wechsler para niños). Resultados: Los niños (30 casos, el 22,1%) y las niñas (106 casos, el 77,9%) con HC de este estudio han tenido un crecimiento, peso e índice de masa corporal que no son distintos de los de la población actual de Barcelona. La talla se ha situado 0,5 DE por encima de los valores de Tanner de 1966 y el índice de masa corporal se ha situado una DE por encima de los valores de Roland-Cachera de 1982. Esta aceleración secular del crecimiento se ha observado en todos los países desarrollados. En una muestra del 37,5% de los casos, el desarrollo psicológico no ha diferido del de la población de referencia. Sin embargo, una mayor intensidad del hipotiroidismo neonatal y la normalización más tardía de éste ha correlacionado negativamente con la puntuación en los test psicológicos. Conclusiones: Una vez conseguida la normalización del crecimiento y del desarrollo psicológico, se debería optimizar el programa de detección precoz para el HC en Cataluña e iniciar el tratamiento lo más precozmente posible para evitar que algunos niños no alcancen su potencial intelectual completo (AU)

Background and objective: Many countries have developed screening programs for CH that bring about a normal somatic and psychological development. The aim of this study is to evaluate the evolution of cases detected by the screening program of congenital hypothyroidism in Catalonia. Patients and methods: The was a descriptive, longitudinal study of a series of 136 cases of congenital hypothyroidism detected by the screening program in Catalonia from 1986 to 1997 and who had been checked in the Maternal-infantile Vall d´Hebron Hospital in Barcelona. Follow-up was carried out for growth and a psychological exploration was performed (McCarthy test and WISC-R).Results Males (30 cases, 22.1%) and females (106 cases, 77.9%) with congenital hypothyroidism of this study had a height, weight and BMI that were not different from those of the current population of Barcelona. Height was 0.5 SD above the Tanner reference values of 1966 and BMI was one SD above the Roland-Cachera reference values of 1982. This secular acceleration of growth has been observed in all developed countries. Results: In a sample of 37.5% of the cases, psychological development did not differ from that of the reference population. However, a greater intensity of neonatal hypothyroidism and its most delayed normalization correlated negatively with the score in the psychological tests. Conclusions: Once normalization of growth and psychological development are achieved, the screening program of congenital hypothyroidism in Catalonia should be optimized by initiating treatment as soon as possible to avoid the possibility that some children do not reach their complete intellectual potential (AU)

[Growth and maturation in the patients with congenital hypothyroidism detected by the neonatal screening program in Catalonia, Spain (1986-1997)]. / Crecimiento y maduración de los pacientes con hipotiroidismo congénito detectados por el programa de cribado neonatal en Cataluña (1986-1997).

BACKGROUND AND OBJECTIVE: Many countries have developed screening programs for CH that bring about a normal somatic and psychological development. The aim of this study is to evaluate the evolution of cases detected by the screening program of congenital hypothyroidism in Catalonia. PATIENTS AND METHODS: The was a descriptive, longitudinal study of a series of 136 cases of congenital hypothyroidism detected by the screening program in Catalonia from 1986 to 1997 and who had been checked in the Maternal-infantile Vall d'Hebron Hospital in Barcelona. Follow-up was carried out for growth and a psychological exploration was performed (McCarthy test and WISC-R). RESULTS: Males (30 cases, 22.1%) and females (106 cases, 77.9%) with congenital hypothyroidism of this study had a height, weight and BMI that were not different from those of the current population of Barcelona. Height was 0.5 SD above the Tanner reference values of 1966 and BMI was one SD above the Roland-Cachera reference values of 1982. This secular acceleration of growth has been observed in all developed countries. In a sample of 37.5% of the cases, psychological development did not differ from that of the reference population. However, a greater intensity of neonatal hypothyroidism and its most delayed normalization correlated negatively with the score in the psychological tests. CONCLUSIONS: Once normalization of growth and psychological development are achieved, the screening program of congenital hypothyroidism in Catalonia should be optimized by initiating treatment as soon as possible to avoid the possibility that some children do not reach their complete intellectual potential.

Factors affecting catch-up growth after liver transplantation.

BACKGROUND: The reported incidence of catch-up growth following orthotopic liver transplantation (OLT) ranges widely, from 0% to 97%. OBJECTIVE: We undertook bivariate analysis of multiple factors that might affect post-OLT growth in children undergoing OLT, and described the results with different parameters used to determine catch-up growth. METHODS: Eighty patients met the inclusion criteria. RESULTS: Catch-up growth occurred in 14% during the first 6 months, 15% at 1 year, 39% at 2 years, 16% between 3 and 6 years, and 16% after 6 years post-OLT. The earlier catch-up growth was shown in metabolic diagnosis, patients over 10 years old and those without steroids at 1 year post-OLT. CONCLUSIONS: It is difficult to determine an acceptable definition of catch-up growth. We suggest that Zvel score > or =0 is the best parameter to evaluate catch-up growth, since the results are more normally distributed. Patients with prednisone withdrawal later than 1 year post-OLT and those with diagnosis of hepatitis and cirrhosis showed the slowest catch-up growth.