Intracranial arachnoid cysts and epilepsy in children: Should this be treated surgically? Our 29-year experience and review of the literature.

INTRODUCTION AND OBJECTIVE: Arachnoid cysts (ACs) are relatively frequent lesions related to different neurological symptoms, being mostly incidentally diagnosed. This study aims to clarify whether AC surgery in epileptic patients is useful in their treatment. MATERIAL AND METHODS: The patients registered in the database of the Neuropediatrics Section from May 1990 to August 2019 are analyzed retrospectively. Patients in whom the diagnosis of ACs and epilepsy coincide are studied. The location, size and number of ACs, neurological development, age at diagnosis, follow-up time, the performance of surgery on the cyst, evolution, anatomical relationship between brain electrical activity and location of AC, and type of epilepsy are analyzed. RESULTS: After analyzing the database, we found 1881 patients diagnosed with epilepsy, of which 25 had at least one intracranial AC. In 9 of the patients, cerebral or genetic pathologies were the cause of epilepsy. Of the other 16, only 2 patients showed that the type of epilepsy and the epileptogenic focus coincided with the location of the AC; one of them was surgically treated without success, and the other one remained asymptomatic without receiving medical or surgical treatment. CONCLUSIONS: Although it is necessary to design a prospective study to establish causality, the results of our research and the available literature suggest that there is no causal relationship between the presence of ACs and epilepsy. The study and treatment of these patients should be carried out in a multidisciplinary epilepsy surgery unit, without initially assuming that the AC is the cause of epilepsy.

Características clínicas y dificultades diagnósticas a partir de un estudio prospectivo sobre neumonía pediátrica adquirida en la comunidad / Clinical characteristics and diagnostic difficulties from a prospective study of community-acquired paediatric pneumonia

Introducción: la neumonía continúa siendo una de las principales causas de morbimortalidad infantil. En Atención Primaria pediátrica su abordaje sigue en revisión. Material y métodos: estudio prospectivo en nueve cupos pediátricos, sobre niños desde un mes a 14 años con neumonía. Diagnóstico etiológico mediante serología y aspirado a virus respiratorios. Se analiza la relación de diferentes variables con la etiología (vírica, bacteriana atípica y bacteriana típica). Resultados: se incluyeron 92 episodios. La edad media (47,5 meses) fue significativamente mayor en atípicas (74,2 ± 42,2), que víricas (36,1 ± 44,5; p <0,0001) y de sospecha neumocócicas (32,6 ± 21,1; p <0,0001). El 33,7% (intervalo de confianza del 95% [IC 95]: 24,9 a 43,8%) fueron de sospecha neumocócica, 30,4% (IC 95: 22,0 a 40,5) atípicas y 21,7% (IC 95: 14,5 a 31,2) víricas. Las atípicas no fueron raras en menores de 5 años (17,1%). Fiebre (89,1%) y tos (68,4%) fueron los síntomas principales. El patrón radiológico alveolar fue el más constatado (53,8%), sin diferencias entre grupos. La proteína C reactiva (PCR) fue significativamente mayor en víricas (7,6 ± 9,5 mg/dl) que en las de sospecha neumocócica (4,9 ± 10,1) (diferencias de medias [DM]: -2,7; p = 0,0490), y atípicas (1,7 ± 1,7) (DM: -5,8; p = 0,0111). Amoxicilina fue el fármaco más utilizado (66,3%), con buena evolución en todos los casos. Ingresaron más las víricas (50%) que las atípicas (7,4%; p = 0,0023) o de sospecha neumocócica (12,9%; p = 0,0100). Conclusión: las tendencias epidemiológicas de la neumonía adquirida en la comunidad en Pediatría parecen cambiantes, especialmente en gérmenes atípicos y virus. Su diagnóstico preciso y el consiguiente abordaje terapéutico continúan siendo un reto por resolver (AU)

Introduction: pneumonia is one of the main causes of morbidity and mortality in children. Its management at the paediatric primary care level is not yet solidly established.Material and methods: we conducted a prospective study in children aged 1 month to 14 years included in the paediatric caseloads of 9 paediatric primary care centres. The aetiological diagnosis was made by means of serology tests and viral testing in nasopharyngeal aspirate samples. We analysed the association of different variables with the aetiology of pneumonia (viral, atypical bacterial and typical bacterial).Results: the study included 92 patients. The mean age (47.58 months) was significantly higher in cases of atypical pneumonia (74.2 ± 42.2) compared to viral pneumonia (36.1 ± 44.5; p <0.0001) and probable typical pneumonia (32.6 ± 21.1; p <0.0001). The distribution by aetiology was 33.7% (95 CI: 24.9 to 43.8) probable pneumococcal, 30.4% (95 CI: 22.0 to 40.5) atypical and 21.7% (95 CI: 14.5 to 31.2) viral. Atypical pneumonia was relatively frequent in children under 5 years (17.1%). Fever (89.1%) and cough (68.4%) were the most frequent symptoms. The most common radiographic feature was alveolar infiltration (53.8%), with no differences between groups. C-reactive protein levels were significantly higher in viral cases (7.6 ± 9.5) compared to probable pneumococcal cases (4.9 ± 10.1) (DM: -2.7; p = 0.0490) and atypical cases (1.7 ± 1.7) (DM: -5.83; p = 0.0111). Amoxicillin was the most frequently used antibiotic (66.3%), which achieved favourable outcomes in all types of pneumonia. The frequency of hospital admission was higher in patients with viral pneumonia (50%) compared to atypical (7.4%; p = 0.0023) or probable pneumococcal pneumonia (12.90 %; p = 0.0100).Conclusion: the epidemiology of community-acquired pneumonia in the paediatric population is changing, especially when it comes to atypical bacterial and viral causative agents... (AU)

Cribado neonatal de fibrosis quística: análisis y diferencias de los niveles de tripsina inmunorreactiva en recién nacidos con cribado positivo / Neonatal cystic fibrosis screening: Analysis and differences in immunoreactive trypsin levels in newborns with a positive screen

Introducción: El cribado neonatal de fibrosis quística (FQ) ha permitido el diagnóstico precoz de la enfermedad, siendo determinante en el aumento de supervivencia de estos pacientes. Su principal inconveniente es su baja especificidad y elevado número de falsos positivos. El objetivo fue analizar las diferencias de tripsina inmunorreactiva (TIR) entre los diferentes grupos de recién nacidos (RN) con cribado neonatal positivo según fueran sanos, portadores sanos, afectos de FQ o Cystic Fibrosis Screen Positive, Inconclusive Diagnosis (CFSPID). Material: Estudio retrospectivo analítico de las concentraciones de TIR en RN con cribado neonatal positivo para FQ nacidos en un hospital de tercer nivel durante 8 años. Resultados:Se analizaron 790RN con cribado neonatal positivo para FQ, 86,3% a término, 53% niñas y 11,8% ingresados. El valor medio de TIR fue 79,16ng/ml (rango 60-367). Se encontraron concentraciones significativamente más elevadas de TIR en afectos de FQ con respecto a los otros grupos (p<0,001). Existen niveles superiores en grandes prematuros (p=0,007) e ingresados (p=0,002). No difieren en cuanto a sexo o estacionalidad. Existe una correlación directa del 64% (p=0,001) entre TIR y test del sudor en afectos de FQ y CFSPID. Mediante curva ROC se calculó el valor de corte de TIR para el diagnóstico de FQ, que fue 76,2ng/ml (S=95,7%, E=64,5%). Conclusiones: Los RN con FQ presentan cifras significativamente más elevadas de TIR que sanos, portadores o CFSPID. La prematuridad y hospitalización también pueden influir. Un mayor valor de TIR se relaciona con una mayor cifra en el test del sudor. (AU)

Introduction: Neonatal cystic fibrosis (CF) screening has enabled the disease to be diagnosed early, and is a determining factor in the increase in survival of these patients. Its main disadvantage is its low specificity and elevated number of false positives. The aim of this study is to analyse the differences in immunoreactive trypsin (IRT) between the different groups of newborns (NB) with a positive neonatal screen depending on whether they were healthy, healthy carriers, affected by CF, or CFSPID (Cystic Fibrosis Screen Positive, Inconclusive Diagnosis). Material: Retrospective analytical study of the concentrations of IRT in NB with a positive neonatal screen for CF born in a tertiary hospital during an 8-year period. Results: A total of 790 NB with a positive neonatal screen for CF were analysed. Of these 86.3% were term, 53% girls, and 11.8% were admitted. The mean IRT value was 79.16 ng/mL (range 60 – 367). Significantly higher concentrations of IRT were found in those affected by CF compared to the other groups (P<.001). There were higher levels in large prematures (P=.007) and admitted patients (P=.002). There were no differences as regards gender or season. There was a direct correlation of 64% (P=.001) between IRT and sweat test in those affected by CF and CFSPID. A cut-off value of IRT for the diagnosis of CF was calculated from the ROC curve (76.2 ng/mL (S=95.7%, Sp=64.5%). Conclusions: NB with CF have significantly higher levels of IRT than healthy ones, or carriers and CFSPID. Prematurity and hospital admission may also have an influence. A higher IRT value is associated with a higher level in the sweat test.

Neonatal cystic fibrosis screening: Analysis and differences in immunoreactive trypsin levels in newborns with a positive screen.

INTRODUCTION: Neonatal cystic fibrosis (CF) screening has enabled the disease to be diagnosed early, and is a determining factor in the increase in survival of these patients. Its main disadvantage is its low specificity and elevated number of false positives. The aim of this study is to analyse the differences in immunoreactive trypsin (IRT) between the different groups of newborns (NB) with a positive neonatal screen depending on whether they were healthy, healthy carriers, affected by CF, or CFSPID (Cystic Fibrosis Screen Positive, Inconclusive Diagnosis). MATERIAL: Retrospective analytical study of the concentrations of IRT in NB with a positive neonatal screen for CF born in a tertiary hospital during an 8-year period. RESULTS: A total of 790 NB with a positive neonatal screen for CF were analysed. Of these 86.3% were term, 53% girls, and 11.8% were admitted. The mean IRT value was 79.16 ng/mL (range 60-367). Significantly higher concentrations of IRT were found in those affected by CF compared to the other groups (P < .001). There were higher levels in large prematures (P = .007) and admitted patients (P = .002). There were no differences as regards gender or season. There was a direct correlation of 64% (P = .001) between IRT and sweat test in those affected by CF and CFSPID. A cut-off value of IRT for the diagnosis of CF was calculated from the ROC curve (76.2 ng/mL (S = 95.7%, Sp = 64.5%). CONCLUSIONS: NB with CF have significantly higher levels of IRT than healthy ones, or carriers and CFSPID. Prematurity and hospital admission may also have an influence. A higher IRT value is associated with a higher level in the sweat test.

Evaluación de la composición corporal mediante antropometría y bioimpedanciometría en supervivientes de leucemia aguda infantil / Body composition evaluation by anthropometry and bioelectrical impedance analysis in childhood acute leukemia survivors

Introducción: los supervivientes de leucemia aguda infantil (LAI) tienen riesgo de desarrollar obesidad. El objetivo del estudio fue evaluar la composición corporal en estos pacientes mediante las diferentes técnicas de empleadas en la práctica clínica y compararlas con el empleo del índice de masa corporal (IMC). Métodos: estudio transversal de 39 supervivientes de LAI con más de diez an~os desde el diagnóstico. Se evaluó el grado de acuerdo entre diferentes técnicas antropométricas y composición corporal y se analizaron factores de riesgo asociados al desarrollo de obesidad. Resultados: prevalencia de obesidad según porcentaje masa grasa por IMC 38,5%, perímetro cintura 46,1%, sumatorio cuatro pliegues 51,3% y bioimpedanciometría (BIA) 56,4%. Existe adecuada correlación entre los métodos, pero el IMC infraestima la adiposidad respecto al perímetro de cintura (-1,03 ± 2,01), pliegues (-2,95 ± 5,78 y BIA (-3,78 ± 7,4), con mayor infraestimación en % masa magra > 28%. Tres pacientes mostraron sarcopenia y solo uno, obesidad sarcopénica. La adiposidad estimada por el perímetro de cintura fue el parámetro con mejor asociación con los factores de riesgo cardiovascular (colesterol-LDL: r = 0,703; colesterol-HDL: r = -0,612; p < 0,05 e hipertensión: OR 4,17; IC 95%: 1,012-19,3). Los factores de riesgo asociados a obesidad fueron: sexo femenino, alto riesgo tumoral, tratamiento con radioterapia y trasplante de progenitores hematopoyéticos. Conclusiones: el IMC infraestima el porcentaje de supervivientes obesos respecto al empleo del perímetro de cintura, pliegues cutáneos y bioimpedanciometría, existiendo riesgo de clasificar erróneamente a sujetos obesos como no obesos. El sexo femenino, el alto riesgo tumoral, la radioterapia y el trasplante son factores de riesgo para presentar obesidad

Background: survivors of childhood acute leukemia are at risk for obesity. The purpose was to evaluate the different clinical measurements of body composition and to compare with body mass index (BMI). Methods: cross-sectional study of 39 survivors with more than ten years of survivorship since diagnosis. Anthropometry and body composition accuracy measurements were determined and also obesity risk factors. Results: obesity prevalence by body fat percentage were: 38.5 % for BMI; 46.1 % for waist circumference; 51.3 % for skinfolds and 56.4 % for bioelectrical impedance analysis (BIA). There was a good correlation among the measurements, but BMI underestimated the percent body fat among childhood leukemia survivors in comparison with: waist circumference (-1.03 ± 2.01), skinfolds (-2.95 ± 5.78) and BIA (-3.78 ± 7.4), and this bias appears to be more variable with increasing percent of body fat > 30 %. Three patients showed sarcopenia and only one sarcopenic obesity. Waist circumference fat mass was the better predictor of cardiovascular risk factors (LDL-cholesterol: r = 0.703; HDL-cholesterol: r = -0.612; p < 0.05 and hypertension: OR 4.17; IC 95 %: 1.012-19.3). Obesity risk factors were: female sex, high-risk tumor, radiotherapy and stem cell transplantation. Conclusions: BMI underestimates obese childhood leukemia survivors in comparison with waist circumference, skinfolds and bioelectrial impedance analysis. BMI use could misclassify obese survivors as non-obese. Female sex, high tumoral risk and coadyuvant treatments (radiotherapy and stem cell transplant) are risk factors for adiposity

Diagnóstico prenatal de cardiopatías congénitas en un hospital terciario en 2 años / Prenatal diagnosis of congenital heart diseases in a tertiary hospital over two years

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Evolution of strain and strain rate values throughout gestation in healthy fetuses.

Myocardial deformation by speckle tracking echocardiography is a novel method for evaluating cardiac function. To test the hypothesis that right ventricular and left ventricular function have age-specific patterns of development, we tracked the evolution of ventricular strain mechanics by speckle tracking echocardiography in the fetus. We conducted a retrospective cross sectional echocardiography study in 154 healthy fetuses, and characterized cardiac function by measuring right and left ventricles global longitudinal strain and strain rate. Comparison of the data of both ventricles according to gestational age was carried out. The magnitudes of right and left ventricle global longitudinal strain show wide range values and decreased throughout gestation. Strain values are higher in left ventricle compared to the right one throughout pregnancy. Strain rate values were similar over gestation in each ventricle, but the magnitudes declined overtime in the right and left ventricle. The maturational patterns of left and right strain are gestational specific. With accepted physiological maturation patterns in healthy subjects, these myocardial deformation parameters can provide a valid basis that allows comparison between health and disease.

[Body composition evaluation by anthropometry and bioelectrical impedance analysis in childhood acute leukemia survivors]. / Evaluación de la composición corporal mediante antropometría y bioimpedanciometría en supervivientes de leucemia aguda infantil.

INTRODUCTION: Background: survivors of childhood acute leukemia are at risk for obesity. The purpose was to evaluate the different clinical measurements of body composition and to compare with body mass index (BMI). Methods: cross-sectional study of 39 survivors with more than ten years of survivorship since diagnosis. Anthropometry and body composition accuracy measurements were determined and also obesity risk factors. Results: obesity prevalence by body fat percentage were: 38.5% for BMI; 46.1% for waist circumference; 51.3% for skinfolds and 56.4% for bioelectrical impedance analysis (BIA). There was a good correlation among the measurements, but BMI underestimated the percent body fat among childhood leukemia survivors in comparison with: waist circumference (-1.03 ± 2.01), skinfolds (-2.95 ± 5.78) and BIA (-3.78 ± 7.4), and this bias appears to be more variable with increasing percent of body fat > 30%. Three patients showed sarcopenia and only one sarcopenic obesity. Waist circumference fat mass was the better predictor of cardiovascular risk factors (LDL-cholesterol: r = 0.703; HDL-cholesterol: r = -0.612; p < 0.05 and hypertension: OR 4.17; IC 95%: 1.012-19.3). Obesity risk factors were: female sex, high-risk tumor, radiotherapy and stem cell transplantation. Conclusions: BMI underestimates obese childhood leukemia survivors in comparison with waist circumference, skinfolds and bioelectrial impedance analysis. BMI use could misclassify obese survivors as non-obese. Female sex, high tumoral risk and coadyuvant treatments (radiotherapy and stem cell transplant) are risk factors for adiposity.

INTRODUCCIÓN: Introducción: los supervivientes de leucemia aguda infantil (LAI) tienen riesgo de desarrollar obesidad. El objetivo del estudio fue evaluar la composición corporal en estos pacientes mediante las diferentes técnicas de empleadas en la práctica clínica y compararlas con el empleo del índice de masa corporal (IMC). Métodos: estudio transversal de 39 supervivientes de LAI con más de diez años desde el diagnóstico. Se evaluó el grado de acuerdo entre diferentes técnicas antropométricas y composición corporal y se analizaron factores de riesgo asociados al desarrollo de obesidad. Resultados: prevalencia de obesidad según porcentaje masa grasa por IMC 38,5%, perímetro cintura 46,1%, sumatorio cuatro pliegues 51,3% y bioimpedanciometría (BIA) 56,4%. Existe adecuada correlación entre los métodos, pero el IMC infraestima la adiposidad respecto al perímetro de cintura (-1,03 ± 2,01), pliegues (-2,95 ± 5,78 y BIA (-3,78 ± 7,4), con mayor infraestimación en % masa magra > 28%. Tres pacientes mostraron sarcopenia y solo uno, obesidad sarcopénica. La adiposidad estimada por el perímetro de cintura fue el parámetro con mejor asociación con los factores de riesgo cardiovascular (colesterol-LDL: r = 0,703; colesterol-HDL: r = -0,612; p < 0,05 e hipertensión: OR 4,17; IC 95%: 1,012-19,3). Los factores de riesgo asociados a obesidad fueron: sexo femenino, alto riesgo tumoral, tratamiento con radioterapia y trasplante de progenitores hematopoyéticos. Conclusiones: el IMC infraestima el porcentaje de supervivientes obesos respecto al empleo del perímetro de cintura, pliegues cutáneos y bioimpedanciometría, existiendo riesgo de clasificar erróneamente a sujetos obesos como no obesos. El sexo femenino, el alto riesgo tumoral, la radioterapia y el trasplante son factores de riesgo para presentar obesidad.

Calidad de vida y estados crónicos de salud en supervivientes de leucemia aguda infantil / Quality of life and chronic health conditions in childhood acute leukaemia survivors

Introducción: El aumento en la supervivencia de la leucemia aguda (LA) infantil conlleva un incremento de morbilidades a largo plazo que acompañado del impacto ocasionado por el tratamiento puede alterar la calidad de vida (CV). Objetivo: Evaluar la prevalencia de comorbilidades crónicas, CV y predictores de su desarrollo en supervivientes de LA. Métodos: Estudio transversal de una cohorte de 54 individuos con más de 10 años de supervivencia tras diagnóstico de LA. Se evaluaron la presencia de comorbilidades y la CV global, física y mental mediante cuestionario SF-36. Resultados: El 53,7% presentó ≥ 1 comorbilidad crónica (24,7% hipotiroidismo; 20,3% obesidad; 14,8% síndrome metabólico; 18,5% disfunción cardiaca subclínica). El 20,3% de ellas fueron comorbilidades graves. El 73,3% de LA alto riesgo y el 66,6% de los tratados con radioterapia o trasplante presentaron comorbilidad tardía, p<0,05. La puntuación media de CV global fue 86,3 (14) (muy buena). Reportaron peor CV global los pacientes con LA de alto riesgo (83,2 vs. 89,5), comorbilidades graves (80,4 vs. 88,7) y del sexo femenino (81,8 vs. 89,9), p <0,05. Los pacientes obesos (80 vs. 92), hipotiroideos (84,9 vs. 92,4) y tratados con RT (82,3 vs. 87,5) tuvieron peor CV física (p <0,05) y aquellos con hipogonadismo (68,2 vs. 83,6) y tratados con TPH (77,2 vs. 83,1) menos puntuación en CV mental, p <0,05. Conclusiones: Los supervivientes de LA presentan una alta prevalencia de comorbilidades crónicas, asociadas al tratamiento recibido. A pesar de que estas influyen en alguna de las subescalas de su CV, la percepción global fue muy buena, incluso superior a la media de la población general

Background: Survival of childhood acute lymphoblastic leukaemia involves an increasing risk of long-term morbidities. Due to the impact of cancer treatment and comorbidities, AL survivors may experience a decrease in their health-related quality of life. Objective: We aimed to describe the long-term comorbidities, related quality of life and their development predictors in these survivors. Methods: cross-sectional study of 54 survivors aged ≥18 and who have a survival rate of more than 10 years. Quality of life was assessed by personal interview using SF-36 questionnaire. Results: 53.7% of AL survivors developed more than one comorbidity (24.7% hypothyroidism; 20.3% obesity; 14.8% metabolic syndrome; 18.5% subclinical cardiac dysfunction); 20.3% of them were severe. 73.3% of high-risk leukaemias and 66.6% of patients treated with radiotherapy or stem cells transplantation reported long-term comorbidity, P<.05. Global quality of live score was: 86.3 (14) (classified as very good). Patients with high-risk acute leukaemia (83.2 vs. 89.5), severe long-term comorbidities (80.4 vs. 89.7) and females (81.8 vs. 89.9), reported worse quality of life, P<.05. Physical summary score was worse in: obese (80 vs. 92) and hypothyroid (84.9 vs. 92.4) and radiotherapy-treated survivors (82.3 vs. 87.5); mental summary was worse in survivors with hypogonadism (68.2 vs. 86.3) and trasplanted patients (77.2 vs. 83.1), P<.05. Conclusions: Acute leukaemia survivors reported an increase prevalence of chronic comorbidities, related to cancer-treatment. Despite a decrease in scores for certain physical or mental items, global quality of life was very good in all acute leukaemia survivors, even better than compared with the general population

Quality of life and chronic health conditions in childhood acute leukaemia survivors. / Calidad de vida y estados crónicos de salud en supervivientes de leucemia aguda infantil.

BACKGROUND: Survival of childhood acute lymphoblastic leukaemia involves an increasing risk of long-term morbidities. Due to the impact of cancer treatment and comorbidities, AL survivors may experience a decrease in their health-related quality of life. OBJECTIVE: We aimed to describe the long-term comorbidities, related quality of life and their development predictors in these survivors. METHODS: cross-sectional study of 54 survivors aged ≥18 and who have a survival rate of more than 10 years. Quality of life was assessed by personal interview using SF-36 questionnaire. RESULTS: 53.7% of AL survivors developed more than one comorbidity (24.7% hypothyroidism; 20.3% obesity; 14.8% metabolic syndrome; 18.5% subclinical cardiac dysfunction); 20.3% of them were severe. 73.3% of high-risk leukaemias and 66.6% of patients treated with radiotherapy or stem cells transplantation reported long-term comorbidity, P<.05. Global quality of live score was: 86.3 (14) (classified as very good). Patients with high-risk acute leukaemia (83.2 vs. 89.5), severe long-term comorbidities (80.4 vs. 89.7) and females (81.8 vs. 89.9), reported worse quality of life, P<.05. Physical summary score was worse in: obese (80 vs. 92) and hypothyroid (84.9 vs. 92.4) and radiotherapy-treated survivors (82.3 vs. 87.5); mental summary was worse in survivors with hypogonadism (68.2 vs. 86.3) and trasplanted patients (77.2 vs. 83.1), P<.05. CONCLUSIONS: Acute leukaemia survivors reported an increase prevalence of chronic comorbidities, related to cancer-treatment. Despite a decrease in scores for certain physical or mental items, global quality of life was very good in all acute leukaemia survivors, even better than compared with the general population.

Use of speckle tracking in the evaluation of late subclinical myocardial damage in survivors of childhood acute leukaemia.

Heart disease is the leading cause of non-cancer death in childhood cancer survivors. to determine the prevalence of subclinical cardiac dysfunction using speckle tracking and compare its results with those obtained by classical methods of assessing left ventricular function and its relationship with different factors to identify the population at higher risk. Echocardiographic assessment of left ventricular function included ejection fraction, tissue Doppler, longitudinal/circumferential strains and biochemical parameters (troponin-T and Pro-BNP) in a cohort of 57 survivors of childhood acute leukaemia with at least 10 years since diagnosis. Ventricular dysfunction was found in 5.2% of patients in M-mode (ejection fraction-EF < 53% with a reduction in the EF ≥ 10%) and in 7% of patients with Simpson's method, compared with 21.05 and 8.8% with suboptimal global longitudinal strain (GLS) and global circumferential strain, respectively. The GLS alteration was significantly correlated with lower values of left ventricular systolic function and was associated with high tumour risk (odds ratio [OR] 13.8), cumulative doses of anthracyclines ≥ 250 mg/m2 (OR 7.6) and radiotherapy (OR 7.19). Biomarkers were not useful for the diagnosis of subclinical cardiomyopathy. Good reproducibility was obtained, with an intraobserver correlation of 93.6% and an interobserver correlation of 89.2% in the GLS. The alteration of the GLS was more prevalent than the alteration in the EF and was associated with the treatment received and high tumour risk. strain imaging seems to be a powerful tool to identify an increased number of survivor with an early myocardial injury.

Modificaciones en variables antropométricas, analíticas de riesgo metabólico y composición corporal en pequeños para la edad gestacional en tratamiento con hormona de crecimiento / Effects of growth hormone treatment on anthropometrics, metabolic risk, and body composition variables in small for gestational age patients

INTRODUCCIÓN: Los niños pequeños para la edad gestacional (PEG) sin crecimiento recuperador pueden beneficiarse del tratamiento con hormona de crecimiento (rhGH). Sin embargo, deben ser monitorizados de forma muy estrecha ya que son población de riesgo metabólico. MATERIAL Y MÉTODOS: Se han incluido 28 niños PEG, con una media de edad de 8,79 años, sin crecimiento recuperador, tratados con rhGH. Hemos evaluado las modificaciones producidas en la antropometría, variables de riesgo metabólico y composición corporal durante 4 años de tratamiento. RESULTADOS: El tratamiento con rhGH se acompañó de un aumento de talla (-2,76 ± 0,11 DE hasta -1,53 ± 0,17 DE; p = 0,000), peso (-1,50 ± 0,09 DE hasta -1,21 ± 0,13 DE; p = 0,016) y velocidad de crecimiento (-1,43 ± 0,35 DE hasta 0,41 ± 0,41 DE; p = 0,009), sin producir modificaciones en el índice de masa corporal (IMC). Se han visto aumentos significativos de la insulinemia (9,33 ± 1,93 mU/ml hasta 16,55 ± 1,72 mU/ml; p = 0,044) y del índice HOMA (3,63 ± 0,76 hasta 6,43 ± 0,67; p = 0,042), sin producirse modificaciones en el perfil lipídico. En el estudio de composición corporal se ha comprobado un aumento significativo de la masa magra (73,19 ± 1,26 hasta 78,74 ± 1,31; p = 0,037) con una disminución de la masa grasa (26,81 ± 1,26 hasta 21,26 ± 1,31; p = 0,021). CONCLUSIÓN: El tratamiento con rhGH se ha acompañado de una ganancia en la talla sin producir alteraciones en el IMC. Asimismo, se han observado cambios en la composición corporal, con un aumento de la proporción de masa magra a expensas de una disminución de la de masa grasa, que podrían conducir a un descenso del riesgo metabólico de estos pacientes. Sin embargo, se ha detectado cierta resistencia insulínica. Es importante continuar el seguimiento de estos niños para determinar las posibles repercusiones en la edad adulta

INTRODUCTION AND OBJECTIVES: Small for gestational age (SGA) children without catch-up growth can benefit from treatment with growth hormone (rhGH). However, they should be monitored very closely because they are at increased risk of metabolic syndrome. MATERIAL AND METHOD: A group of 28 SGA children with a mean age of 8.79 years and undergoing treatment with rhGH were selected for evaluation. Over the course of 4 years, an annual evaluation was performed on the anthropometric variables (weight, height, body mass index [BMI], growth rate, blood pressure and waist perimeter), metabolic risk variables (glycaemia, glycosylated haemoglobin, cholesterol ratio, insulinaemia, insulin-like growth factor 1[IGF1], IGF binding protein-3 [IGFBP-3], IGF1/IGFBP3 ratio, and HOMA index), and body composition variables. RESULTS: Treatment with rhGH was associated with a significant increase in height (-2.76 ± .11 SD to -1.53 ± .17 SD, P=.000), weight (-1.50 ± .09 SD to -1.21 ± .13 SD; P = .016), and growth rate (-1.43 ± .35 SD to .41 ± .41 SD; P=.009), without a corresponding change in the BMI. Insulinaemia (9.33 ± 1.93 mU/ml to 16.55 ± 1.72 mU/ml; P = .044) and the HOMA index (3.63 ± .76 to 6.43 ± .67; P = .042) increased, approaching insulin resistance levels. No changes were observed in the lipid profile. Body composition changes were observed, with a significant increase in lean mass (73.19 ± 1.26 to 78.74 ± 1.31; P = .037), and a reduction of fat mass (26.81 ± 1.26 to 21.26 ± 1.31; P = .021). CONCLUSION: Treatment with rhGH is effective for improving anthropometric variables in SGA patients who have not experienced a catch-up growth. It also produces changes in body composition, which may lead to a reduction in risk of metabolic syndrome. However, some insulin resistance was observed. It is important to follow up this patient group in order to find out whether these changes persist into adulthood

Evaluación de la función cardiaca en un grupo de niños pequeños para la edad gestacional en edad escolar en tratamiento con hormona de crecimiento / Evaluation of cardiac function in a group of small for gestational age school-age children treated with growth hormone

Introducción y objetivos: Los pacientes pequeños para la edad gestacional (PEG) son población de riesgo para el desarrollo de enfermedad cardiovascular y síndrome metabólico. Nuestro objetivo es estudiar la morfología y la función cardiaca en un grupo de niños PEG en edad escolar en tratamiento con growth hormone (GH, «hormona de crecimiento»). Métodos: Se han incluido en el estudio 23 pacientes PEG y 23 controles sanos. Se valoró peso, talla, presión arterial y frecuencia cardiaca. Mediante ecocardiografía transtorácica se evaluó el tamaño de las cavidades cardiacas, el diámetro de la aorta ascendente y abdominal y parámetros de función biventricular. Resultados: Los niños PEG presentan mayores percentiles de presión arterial sistólica y diastólica (p < 0,05), sin cambios significativos en la frecuencia cardiaca. Tienen un mayor diámetro del septo interventricular (Z-score 1,57 en PEG frente a 0,89; p = 0,026) y una peor función sistólica del ventrículo derecho, con un TAPSE inferior (Z-score −0,98 en PEG frente a 0,95; p = 0,000) y una menor velocidad sanguínea en arteria pulmonar (0,85 m/s en PEG frente a 0,97 m/s; p = 0,045). No se han encontrado diferencias en la función del ventrículo izquierdo. El diámetro de la aorta ascendente es mayor en PEG (Z-score−1,09 frente a−1,93; p = 0,026), mientras que el diámetro de la aorta abdominal en sístole es menor (Z-score −0,89 frente a −0,19; p = 0,015). Conclusiones: Se han observado cambios significativos en la morfología y la función cardiaca en niños PEG en edad escolar tratados con GH. Es importante continuar en ellos un seguimiento para determinar si estas alteraciones contribuyen a un aumento de morbilidad cardiaca en la edad adulta (AU)

Introduction and objectives: Small for gestational age (SGA) patients have an increased risk of developing a cardiovascular pathology, as well as a metabolic syndrome. Our objective is to evaluate the cardiac morphology and function of SGA children treated with growth hormone (GH), identifying changes that could potentially have long-term consequences. Methods: We selected 23 SGA school-age patients and 23 healthy children. We measured their weight, height, blood pressure and heart rate. Using transthoracic echocardiography, we evaluated cardiac chamber size, ascending and abdominal aortic diameter as well as the systolic and diastolic function of both ventricles. Results: SGA children have a higher systolic and diastolic blood pressure (P < .05) without significant changes in their heart rate. They also have a thicker interventricular septum (SGA Z-score 1.57 vs. 0.89; P = .026) and a worse right ventricular systolic function, with a lower TAPSE (SGA Z-score −0.98 vs. 0.95; P = .000), as well as a lower blood flow rate in the pulmonary artery (SGA 0.85 m/s vs. 0.97 m/s; P = .045). No significant difference was observed in the patients’ left ventricular function. SGA patients’ ascending aortic diameter was greater (SGA Z-score −1.09 vs. −1.93; P = .026), whereas the systolic abdominal aortic diameter was smaller (SGA Z-score−0.89 vs. −0.19; P = .015). Conclusions: We found functional and morphological cardiac changes in SGA school-age patients treated with GH. It is important to follow-up this patient group in order to determine if these changes contribute to an increased cardiac morbidity in adulthood (AU)

Estudio de las epilepsias según la edad de inicio, controladas durante 3 años en una unidad de neuropediatría de referencia regional / A study of epilepsy according to the age at onset and monitored for 3 years in a regional reference paediatric neurology unit

OBJETIVO: Estudio de las epilepsias según la edad de inicio de las crisis y la etiología, de los pacientes controlados en una unidad de neuropediatría durante 3 años. PACIENTES Y MÉTODOS: Estudio de cohortes históricas. Revisión de historias de niños con epilepsia de la base de datos de neuropediatría controlados del 1 de enero de 2008 al 31 de diciembre de 2010. RESULTADOS: De 4.595 ni˜nos atendidos en el periodo, se estableció el diagnóstico de epilepsia en 605 (13,17%), siendo 277 (45,79%) epilepsias sintomáticas, 156 (25,79%) idiopáticas y 172 (28,43%) criptogénicas. La epilepsia de ausencias y la epilepsia benigna de la infancia con paroxismos centrotemporales son los síndromes epilépticos idiopáticos con mayor prevalencia, y las encefalopatías prenatales las epilepsias sintomáticas más prevalentes. El 26,12% iniciaron su epilepsia el primer a˜no, siendo sintomáticas el 67,72%. Se han considerado refractarias el 25,29% de las epilepsias; el 42,46% asocia déficit cognitivo, el 26,45% afectación motora y el 9,92% trastorno del espectro autista, siendo más frecuentes a menor edad de inicio. CONCLUSIONES: La ausencia de una clasificación universalmente aceptada de los síndromes epilépticos dificulta trabajos como este, empezando por la terminología. Una clasificación útil es la etiológica, con 2 grupos: un gran grupo con las etiologías establecidas o síndromes genéticos muy probables, y otro de casos sin causa establecida. La edad de inicio de la epilepsia en cada grupo etiológico añade orientación pronóstica. El pronóstico de la epilepsia lo ensombrecen la refractariedad y las alteraciones asociadas del neurodesarrollo, siendo peor en general a más precoz inicio y en etiologías concretas

OBJECTIVE: A study of epilepsy, according to the age at onset of the crisis and its causes, monitored by a Paediatric Neurology Unit over a period of three years. PATIENTS AND METHODS: Historical cohorts study was conducted by reviewing the Paediatric Neurology medical records data base of epileptic children followed-up from 1 January 2008 to 31 December 2010. RESULTS: A total of 4,595 children were attended during the study period. The diagnosis of epilepsy was established in 605 (13.17%): 277 (45.79%) symptomatic, 156 (25.79%) idiopathic, and 172 (28.43%) with cryptogenic epilepsy. Absence epilepsy and benign childhood epilepsy with centro-temporal spikes are the idiopathic epileptic syndromes most prevalent, and the most prevalent symptomatic epilepsies are prenatal encephalopathies. More than one-quarter (26.12%) of epilepsies began in the first year of life, and 67.72% were symptomatic. Refractory epilepsy was observed in 25.29%, 42.46% with cognitive impairment, 26.45% with motor involvement, and 9.92% with an autism spectrum disorder, being more frequent at an earlier age of onset. CONCLUSIONS: The absence of a universally accepted classification of epileptic syndromes makes tasks like this difficult, starting with the terminology. A useful classification would be aetiological, with two groups: a large group with established aetiology, or very likely genetic syndromes, and another with no established cause. The age of onset of epilepsy in each aetiological group helps in the prognosis, which is worsened by refractoriness and associated neurodevelopmental disorders, and are generally worse at an earlier onset and in certain aetiologies

[Effects of growth hormone treatment on anthropometrics, metabolic risk, and body composition variables in small for gestational age patients]. / Modificaciones en variables antropométricas, analíticas de riesgo metabólico y composición corporal en pequeños para la edad gestacional en tratamiento con hormona de crecimiento.

INTRODUCTION AND OBJECTIVES: Small for gestational age (SGA) children without catch-up growth can benefit from treatment with growth hormone (rhGH). However, they should be monitored very closely because they are at increased risk of metabolic syndrome. MATERIAL AND METHOD: A group of 28 SGA children with a mean age of 8.79 years and undergoing treatment with rhGH were selected for evaluation. Over the course of 4 years, an annual evaluation was performed on the anthropometric variables (weight, height, body mass index [BMI], growth rate, blood pressure and waist perimeter), metabolic risk variables (glycaemia, glycosylated haemoglobin, cholesterol ratio, insulinaemia, insulin-like growth factor 1[IGF1], IGF binding protein-3 [IGFBP-3], IGF1/IGFBP3 ratio, and HOMA index), and body composition variables. RESULTS: Treatment with rhGH was associated with a significant increase in height (-2.76±.11 SD to -1.53±.17 SD, P=.000), weight (-1.50±.09 SD to -1.21±.13 SD; P=.016), and growth rate (-1.43±.35 SD to .41±.41 SD; P=.009), without a corresponding change in the BMI. Insulinaemia (9.33±1.93mU/ml to 16.55±1.72mU/ml; P=.044) and the HOMA index (3.63±.76 to 6.43±.67; P=.042) increased, approaching insulin resistance levels. No changes were observed in the lipid profile. Body composition changes were observed, with a significant increase in lean mass (73.19±1.26 to 78.74±1.31; P=.037), and a reduction of fat mass (26.81±1.26 to 21.26±1.31; P=.021). CONCLUSION: Treatment with rhGH is effective for improving anthropometric variables in SGA patients who have not experienced a catch-up growth. It also produces changes in body composition, which may lead to a reduction in risk of metabolic syndrome. However, some insulin resistance was observed. It is important to follow up this patient group in order to find out whether these changes persist into adulthood.

[A study of epilepsy according to the age at onset and monitored for 3 years in a regional reference paediatric neurology unit]. / Estudio de las epilepsias según la edad de inicio, controladas durante 3 años en una unidad de neuropediatría de referencia regional.

OBJECTIVE: A study of epilepsy, according to the age at onset of the crisis and its causes, monitored by a Paediatric Neurology Unit over a period of three years. PATIENTS AND METHODS: Historical cohorts study was conducted by reviewing the Paediatric Neurology medical records data base of epileptic children followed-up from 1 January 2008 to 31 December 2010. RESULTS: A total of 4,595 children were attended during the study period. The diagnosis of epilepsy was established in 605 (13.17%): 277 (45.79%) symptomatic, 156 (25.79%) idiopathic, and 172 (28.43%) with cryptogenic epilepsy. Absence epilepsy and benign childhood epilepsy with centro-temporal spikes are the idiopathic epileptic syndromes most prevalent, and the most prevalent symptomatic epilepsies are prenatal encephalopathies. More than one-quarter (26.12%) of epilepsies began in the first year of life, and 67.72% were symptomatic. Refractory epilepsy was observed in 25.29%, 42.46% with cognitive impairment, 26.45% with motor involvement, and 9.92% with an autism spectrum disorder, being more frequent at an earlier age of onset. CONCLUSIONS: The absence of a universally accepted classification of epileptic syndromes makes tasks like this difficult, starting with the terminology. A useful classification would be aetiological, with two groups: a large group with established aetiology, or very likely genetic syndromes, and another with no established cause. The age of onset of epilepsy in each aetiological group helps in the prognosis, which is worsened by refractoriness and associated neurodevelopmental disorders, and are generally worse at an earlier onset and in certain aetiologies.

Evaluation of cardiac function in a group of small for gestational age school-age children treated with growth hormone. / Evaluación de la función cardiaca en un grupo de niños pequeños para la edad gestacional en edad escolar en tratamiento con hormona de crecimiento.

INTRODUCTION AND OBJECTIVES: Small for gestational age (SGA) patients have an increased risk of developing a cardiovascular pathology, as well as a metabolic syndrome. Our objective is to evaluate the cardiac morphology and function of SGA children treated with growth hormone (GH), identifying changes that could potentially have long-term consequences. METHODS: We selected 23 SGA school-age patients and 23 healthy children. We measured their weight, height, blood pressure and heart rate. Using transthoracic echocardiography, we evaluated cardiac chamber size, ascending and abdominal aortic diameter as well as the systolic and diastolic function of both ventricles. RESULTS: SGA children have a higher systolic and diastolic blood pressure (P<.05) without significant changes in their heart rate. They also have a thicker interventricular septum (SGA Z-score 1.57 vs. 0.89; P=.026) and a worse right ventricular systolic function, with a lower TAPSE (SGA Z-score -0.98 vs. 0.95; P=.000), as well as a lower blood flow rate in the pulmonary artery (SGA 0.85m/s vs. 0.97m/s; P=.045). No significant difference was observed in the patients' left ventricular function. SGA patients' ascending aortic diameter was greater (SGA Z-score -1.09 vs. -1.93; P=.026), whereas the systolic abdominal aortic diameter was smaller (SGA Z-score-0.89 vs. -0.19; P=.015). CONCLUSIONS: We found functional and morphological cardiac changes in SGA school-age patients treated with GH. It is important to follow-up this patient group in order to determine if these changes contribute to an increased cardiac morbidity in adulthood.

Estudio descriptivo de las epilepsias no sintomáticas según la edad de inicio en una unidad de neuropediatría de referencia regional / A descriptive study of non-symptomatic epilepsy according to age at onset at a Neuropediatric Section of regional reference

Objetivo. Estudio descriptivo de las epilepsias no sintomáticas (idiopáticas y criptogénicas), según la edad de inicio, controladas en una unidad de neuropediatría de referencia regional durante tres años. Pacientes y métodos. Revisión de historias de niños con epilepsia no sintomática de la base de datos de neuropediatría controlados del 1 de enero de 2008 al 31 de diciembre de 2010. Resultados. De 4.595 niños atendidos en el período, se diagnosticaron de epilepsia 605 (13,17%), de las cuales 156 (25,79%) fueron idiopáticas, y 172 (28,43%), criptogénicas. La edad media de inicio del total fue de 4,78 años; 6,31 años en las idiopáticas y 5,43 años en las criptogénicas. El 26,12% del total de epilepsias se inició en el primer año. Las epilepsias idiopáticas predominan en el grupo de inicio de 6-10 años, y las criptogénicas, en el de 3-6 años. La epilepsia de ausencias y la epilepsia benigna de la infancia con paroxismos centrotemporales son los síndromes epilépticos idiopáticos más prevalentes. Conclusiones. Existen muchas diferencias de datos epidemiológicos publicados sobre epilepsia infantil por la dificultad que entraña un diagnóstico sindrómico en la edad pediátrica, debido a la variabilidad clínica y electroencefalográfica. La ausencia de una clasificación universalmente aceptada de los síndromes epilépticos dificulta comparaciones entre series. Todas las epilepsias son sintomáticas, puesto que tienen causa, sea genética o adquirida. Una clasificación útil es la etiológica, con dos grupos: un gran grupo con las etiologías establecidas o síndromes genéticos muy probables y otro de casos sin causa establecida. La edad de inicio orienta a determinadas etiologías (AU)

Aim. A descriptive study of non-symptomatic epilepsy (idiopathic and cryptogenic), according to age at onset, monitored at a Neuropediatric Section of regional reference over a period of three years. Patients and methods. A review of neuropediatric database medical records of children with non-symptomatic epilepsy supervised from Jan 1, 2008 till December 31, 2010. Results. Of the 4595 children attended during the period, 605 were diagnosed with epilepsy (13.17%): 156 (25.79%) idiopathic epilepsies and 172 (28.43%) cryptogenic epilepsies. The average age at onset of the total was 4.78 years: 6.31 years in idiopathic epilepsies and 5.43 years in cryptogenic epilepsies. 26.12% of all the epilepsies began in the first year of life. Idiopathic epilepsy predominates in the startup group of 6-10 years and cryptogenic epilepsy in 3-6 years. Absence epilepsy and benign childhood epilepsy with centro-temporal spikes are the idiopathic epileptic syndromes most prevalent. Conclusions. Many differences exist among published epidemiological data on childhood epilepsy due to the difficulty of a syndromic diagnosis in children, caused by clinical and electroencephalographic variability. The absence of a universally accepted classification of epileptic syndromes makes it difficult to compare publications. All epilepsies are symptomatic as they have a cause, whether it be genetic or acquired. A useful classification would be etiological, with two groups: one large with established etiology or very likely genetic syndromes and another with no established cause. The age at onset indicates specific etiologies (AU)

Composición corporal y riesgo metabólico en niños pequeños para la edad gestacional en tratamiento con hormona del crecimiento / Body composition and metabolic risk in small for gestational age children treated with growth hormone

Antecedentes y objetivos: Los niños pequeños para la edad gestacional (PEG) constituyen un grupo de riesgo para desarrollar síndrome metabólico. El objetivo de este estudio es evaluar las modificaciones que produce el tratamiento con growth hormone (GH, «hormona de crecimiento») en la composición corporal. Pacientes y método: Se analizan diversas variables antropométricas y de riesgo metabólico en una muestra de 28 niños PEG sin crecimiento recuperador. De forma anual desde el inicio del tratamiento con GH y durante 3 años se miden, mediante densitometría, diferentes variables de composición corporal: densidad mineral ósea (DMO), proporción de masa magra y masa grasa corporal y en la región abdominal. Se ha realizado un estudio de correlación entre variables metabólicas y de composición corporal. Resultados: El tratamiento con GH produce una disminución de la proporción de masa grasa con respecto a la masa magra en el cuerpo entero, con una disminución del porcentaje de grasa total desde 25,94±6,09 hasta 22,88±5,38% (p=0,034). En la región abdominal se observa un aumento de la masa magra desde 1.356,91±426,71 hasta 2.570,96±814,36g (p=0,000) y una tendencia a disminuir el depósito de grasa visceral. La DMO en la región lumbar mejora desde −1,55±0,68 hasta −0,90±0,79Z (p=0,019). Conclusiones: El tratamiento con GH produce cambios en la composición corporal con mejoras en la DMO y un aumento de la masa magra a expensas de la masa grasa. Estas modificaciones, de persistir en la edad adulta, podrían disminuir el riesgo metabólico y cardiovascular de estos pacientes (AU)

Background and objectives: Small for gestational age (SGA) children are at increased risk of metabolic syndrome. Our objective is to evaluate changes in body composition produced by growth hormone (GH) treatment. Patients and method: A group of 28 SGA children without catch-up growth and undergoing treatment with GH was selected for evaluation. Over the course of 3 years from the beginning of the treatment with GH, the children's body composition variables (bone mineral density [BMD], fat and lean body mass proportion) were evaluated annually with dual-energy X-ray absorptiometry. A study of correlation between metabolic and body composition variables was also made. Results: Treatment with GH produces a reduction in fat mass proportion in relation to lean body mass, decreasing from 25.94±6.09 to 22.88±5.38% (P=.034). In the abdominal regions we observe an increase in lean mass, from 1,356,91±426,71 to 2,570,96±814,36g (P=.000) and a tendency for visceral fat deposits to decrease. BMD in lumbar vertebrae improved from −1.55±0.68 to −0.90±0.79Z (P=.019). Conclusions: Treatment with GH produces changes in body composition, improving BMD and increasing the proportion of lean body mass with a reduction in fat mass. If these changes persisted into adulthood, they may cause a reduction in the metabolic and cardiovascular risk in this group of patients (AU)