Adaptation, reliability and validity of health-related quality of life questionnaires: Disabkids chronic and specific diabetes disease in children and adolescents with diabetes mellitus type 1.

INTRODUCTION: It is recommended to periodically evaluate the health-related quality of life (HRQoL) in children and adolescents with type 1 diabetes mellitus (DM1). Despite this, no specific paediatric HRQoL instrument for DM1 has been validated in Spanish. OBJECTIVES: Multicentre, prospective descriptive study in children and adolescents with DM1 with the aim of carrying out cross-cultural adaptation to Spanish and evaluating the reliability and validity of the DISABKIDS chronic disease and diabetes-specific HRQoL questionnaires, using reverse translation. MATERIAL AND METHODS: Sociodemographic variables were compiled together with the most recent capillary glycated haemoglobin (HbA1c) value and HRQoL questionnaires were handed out to 200 Spanish children and adolescents with DM1 aged between 8 and 18 years of age under evaluation in 12 different hospitals. RESULTS: The mean score on the HRQoL questionnaire (patient version) for chronic disease was 80.32 (13.66), being significantly lower (P = .04) in patients with a shorter duration of the disease (≤5 years): 78.34 (13.70) vs. 82.60 (13.36). The mean score of the DM1-specific modules was: 60.81 (16.23) for disease impact and 65.59 (26.19) for treatment impact. The mean HbA1c value was 7.08 (0.79), with no differences (P > .05) noted in the mean score of the HRQoL instruments in patients with HbA1c ≤7% vs. HbA1c >7%. The Cronbach α value varied between 0.72 and 0.90. CONCLUSIONS: The Spanish versions of the DISABKIDS HRQoL instruments meet the proposed objectives of semantic equivalence and internal consistency, making it possible to periodically assess HRQoL in these patients. The good average glycaemic control presented by the patients may explain why no difference was found in the HRQoL instruments based on the HbA1c value.

The Assessment of Nutritional Status in Pediatrics: New Tools and Challenges.

Pediatric endocrinology will undergo an extraordinary revolution this century [...].

RETRACTED: Díez López, I. The Assessment of Nutritional Status in Pediatrics: New Tools and Challenges. Children 2023, 10, 1151.

The Children Editorial Office retracts the article entitled "The Assessment of Nutritional Status in Pediatrics: New Tools and Challenges" by Díez Lopez [...].

Resultados de una encuesta nacional sobre conocimiento y uso de pseudociencias por parte de los pediatras / Results of a national survey on knowledge and use of complementary and alternative medicine by paediatricians

Introducción: El uso de determinadas pseudociencias en niños está documentado en España. El objetivo principal del estudio es estimar el grado de conocimiento, la recomendación y el uso de algunas pseudoterapias por parte de los pediatras españoles. Material y métodos: Estudio transversal, descriptivo y de ámbito nacional, mediante encuesta en línea, enviada por correo electrónico a pediatras socios de la Asociación Española de Pediatría (AEP), entre junio y julio de 2020. (AU)

Introduction: The use of certain Complementary and Alternative Medicines (CAM) in children has been documented in Spain. The main aim of this study is to estimate the knowledge, recommendations, and use of CAM by Spanish paediatricians. Material and methods: A national study was conducted from June to July 2020 using an online questionnaire. Two e-mails were sent to paediatricians who were members of the Spanish Association of Paediatrics (AEP). (AU)

Results of a national survey on knowledge and use of complementary and alternative medicine by paediatricians.

INTRODUCTION: The use of certain Complementary and Alternative Medicines (CAM) in children has been documented in Spain. The main aim of this study is to estimate the knowledge, recommendations, and use of CAM by Spanish paediatricians. MATERIAL AND METHODS: A national study was conducted from June to July 2020 using an online questionnaire. Two e-mails were sent to paediatricians who were members of the Spanish Association of Paediatrics (AEP). RESULTS: Out of 1414 responses received, acupuncture was considered as a science by 31.8%. Homeopathy was recommended to parents by 28.1%. CAM was used by 21.3% of physicians, at least once, to improve their own health. Only 3.8% had ever replaced a conventional treatment with CAM. The following variables were associated with a greater disposition to prescribe homeopathy: female, age over 45 years old, paediatricians working in Primary Care, and paediatricians working in private healthcare. CONCLUSIONS: This AEP Committee on Medicines questionnaire provides new data that should be considered alarming and should ask for a serious thinking on the use of CAM in Spain. Some paediatricians are recommending parents to give treatments not supported by scientific evidence to their children. This practice could be potentially harmful, especially when conventional treatment is being replaced.

Diferencias en la función tiroidea de los pequeños para la edad gestacional y los de peso adecuado. ¿Es normal la función tiroidea de los recién nacidos pequeños para la edad gestacional? / Differences in thyroid function between small for gestational age and those with appropriate weight for gestational age. Is thyroid function normal in small for gestational age newborns?

Introducción: Múltiples estudios concluyen que los niños pequeños para la edad gestacional (PEG) tienen un número mayor de comorbilidades, así como un perfil hormonal diferente respecto a los niños con un peso adecuado para la edad gestacional (PAEG). Las hormonas tiroideas juegan un papel importante en el crecimiento y en el desarrollo neurocognitivo. La función tiroidea en los niños PEG es, hoy en día, incierta.Objetivos: El objetivo de este estudio es comparar la función tiroidea de los niños PEG durante los 2 primeros años de vida con publicaciones sobre función tiroidea en otros grupos de lactantes (PAEG y prematuros) que utilicen la misma metodología.Métodos: Se obtuvo una cohorte de 38 niños PEG, en los cuales se midieron los valores de TSH en sangre en distintos momentos del desarrollo del niño PEG. Los resultados se compararon con una población de niños PAEG de Zaragoza y una población de niños prematuros de Barcelona mediante pruebas de comparación de medias de contraste bilateral.Resultados: Se ha observado una diferencia estadísticamente significativa (p<0,05) entre los niños PEG de nuestro estudio y los niños PAEG mayores de 6 meses, no así entre los PEG del estudio y la población de prematuros.Conclusiones: Los niños PEG tienen valores de TSH superiores respecto a los niños PAEG mayores de 6 meses. Por lo tanto, parece razonable establecer un cribado y un protocolo de seguimiento en los PEG de mayor riesgo. (AU)

Introduction: Several studies conclude that small for gestational age (SGA) children have a higher number of comorbidities, as well as a different hormonal profile compared to those with appropriate weight for gestational age (AGA). Thyroid hormones play an important role in growth and neurocognitive development. Thyroid function in SGA children is still not completely known.Objectives: To compare the thyroid function of SGA children during the first 2 years of life with that in publications on thyroid function in other groups of infants, such as AGA and premature children, using the same methodology.Methods: A cohort of 38 SGA children was obtained, in which the TSH values in blood were measured at different points in the development of the SGA child. The results were compared with a population of AGA children from Zaragoza and a population of premature children from Barcelona by comparing the means using a 2-tailed test.Results: A statistically significant difference (P<.05) was observed between the SGA children in our study and the AGA children older than 6 months, but not between the SGA children of the study and the population of premature infants.Conclusions: SGA children have higher TSH values compared to AGA children older than 6 months. Therefore, it seems reasonable to establish a screening and a follow-up protocol in those SGA with high risk to develop thyroid dysfunction. (AU)

Chemerin concentrations in infants born small for gestational age: correlations with triglycerides and parameters related to glucose homeostasis.

Infants born small for gestational age (SGA) are known to have increased risk of developing several pathologies, including the metabolic syndrome, when they grow up. It has been described that both the growth pattern of these children as well as the risk of their presenting future metabolic disorders can be influenced by the expression of adipokines. Among them, chemerin has demonstrated to be implicated in lipid and glucose metabolism, presenting higher circulating concentration in diabetic and obese subjects. Thus, the aim of this study was to analyze the association of anthropometric parameters and plasmatic biochemical parameters with circulating chemerin concentration in SGA children. This prospective, longitudinal study was carried out in plasma samples of Caucasian children born SGA at Hospital Universitario de Álava-Txagorritxu. Significant positive correlations were observed between chemerin concentrations at 3 months and insulin values at 3 months and also with triglyceride levels at 24 months. These associations were maintained after adjustment by anthropometric parameters. Therefore, we suggest that circulating chemerin concentration, measured at an early age, might be an indicator of future metabolic alterations in SGA children.

[Differences in thyroid function between small for gestational age and those with appropriate weight for gestational age. Is thyroid function normal in small for gestational age newborns?] / Diferencias en la función tiroidea de los pequeños para la edad gestacional y los de peso adecuado. ¿Es normal la función tiroidea de los recién nacidos pequeños para la edad gestacional?

INTRODUCTION: Several studies conclude that small for gestational age (SGA) children have a higher number of comorbidities, as well as a different hormonal profile compared to those with appropriate weight for gestational age (AGA). Thyroid hormones play an important role in growth and neurocognitive development. Thyroid function in SGA children is still not completely known. OBJECTIVES: To compare the thyroid function of SGA children during the first 2 years of life with that in publications on thyroid function in other groups of infants, such as AGA and premature children, using the same methodology. METHODS: A cohort of 38 SGA children was obtained, in which the TSH values in blood were measured at different points in the development of the SGA child. The results were compared with a population of AGA children from Zaragoza and a population of premature children from Barcelona by comparing the means using a 2-tailed test. RESULTS: A statistically significant difference (P<.05) was observed between the SGA children in our study and the AGA children older than 6 months, but not between the SGA children of the study and the population of premature infants. CONCLUSIONS: SGA children have higher TSH values compared to AGA children older than 6 months. Therefore, it seems reasonable to establish a screening and a follow-up protocol in those SGA with high risk to develop thyroid dysfunction.

[Results of a national survey on knowledge and use of complementary and alternative medicine by paediatricians]. / Resultados de una encuesta nacional sobre conocimiento y uso de pseudociencias por parte de los pediatras.

INTRODUCTION: The use of certain Complementary and Alternative Medicines (CAM) in children has been documented in Spain. The main aim of this study is to estimate the knowledge, recommendations, and use of CAM by Spanish paediatricians. MATERIAL AND METHODS: A national study was conducted from June to July 2020 using an online questionnaire. Two e-mails were sent to paediatricians who were members of the Spanish Association of Paediatrics (AEP). RESULTS: Out of 1,414 responses received, acupuncture was considered as a science by 31.8%. Homeopathy was recommended to parents by 28.1%. CAM was used by 21.3% of physicians, at least once, to improve their own health. Only 3.8% had ever replaced a conventional treatment with CAM. The following variables were associated with a greater disposition to prescribe homeopathy: female, age over 45 years old, paediatricians working in Primary Care, and paediatricians working in private healthcare. CONCLUSIONS: This AEP Committee on Medicines questionnaire provides new data that should be considered alarming and should ask for a serious thinking on the use of CAM in Spain. Some paediatricians are recommending parents to give treatments not supported by scientific evidence to their children. This practice could be potentially harmful, especially when conventional treatment is being replaced.

Adherence and long-term outcomes of growth hormone therapy with easypod™ in pediatric subjects: Spanish ECOS study.

BACKGROUND: Non-adherence to r-hGH treatments occurs in a variable percentage of subjects. One problem found when evaluating adherence is the great variability in methods of detection and definitions utilized in studies. This study assessed the level of adherence in subjects receiving r-hGH with the easypod™ electronic device. METHODS: National, multicenter, prospective and observational study involving 238 subjects (144 with GH deficiency (GHD), and 86 with small for gestational age (SGA), 8 with Turner Syndrome), who received r-hGH with easypod™ for at least 3 months before inclusion. The follow-up period was 4 years. RESULTS: Overall adherence was 94.5%; 97.5% after 6 months, 95.3% after 1 year, 93.7% after 2, 94.4% after 3 and 95.5% after 4 years of treatment. No differences in adherence were observed between prepubertal and pubertal groups and GHD and SGA groups. Change in height after 1 and 2 years, change in height SDS after 1 and 2 years, HV after 1 year, HV SDS after at 1 and 4 years, change in BMI after 1 year and change in BMI SDS at 1 and 2 years showed significant correlation with adherence. No significant differences in adherence according to IGF-I levels were found in follow-up visits or between groups. CONCLUSIONS: The easypod™ electronic device, apart from being a precise and objective measure of adherence to r-hGH treatment, allows high compliance rates to be achieved over long periods of time. Adherence significantly impacts growth outcomes associated with r-hGH treatment.

Tratamiento con hormona de crecimiento en pequeños para la edad gestacional en España / Growth hormone treatment in small for gestational age children in Spain

INTRODUCCIÓN: Desde su aprobación por la Agencia Europea del Medicamento, el tratamiento con hormona de crecimiento recombinante ha sido empleado en un gran número de pacientes nacidos pequeños para la edad gestacional en España. El propósito de este estudio es conocer objetivamente los resultados del mismo en la práctica habitual. MÉTODOS: Se ha recogido información procedente de los registros existentes en los comités asesores que autorizan dichos tratamientos en los hospitales públicos de 6 comunidades autónomas. RESULTADOS: Se han obtenido datos válidos de 974 pacientes. Todos ellos cumplían los criterios exigidos por la Agencia Europea del Medicamento. Los pacientes que recibieron el tratamiento se caracterizaron por tener la longitud al nacer más afectada que el peso, talla diana inferior a -1 desviación estándar (DE) y un 23% con antecedentes de prematuridad. La talla al iniciar el tratamiento fue de − 3,1 ± 0,8 DE (media ± DE) y la edad de comienzo 7,2 ± 2,8 años. La ganancia de talla en el primer año fue de 0,7 ± 0,2 DE, y de 1,2 ± 0,8 DE hasta los 2 años. La talla final, alcanzada por un 8% de pacientes, fue de -1,4 ± 0,7 DE. CONCLUSIONES: Los resultados concuerdan con las series nacionales e internacionales publicadas y son representativos de la práctica habitual en nuestro país. Se constata un inicio tardío del tratamiento, observándose, sin embargo, un adecuado crecimiento, tanto a corto plazo como en la talla final. En el primer año se identifica un 24% de pacientes con respuesta deficiente

INTRODUCTION: Since its approval by the European Medicines Agency, a great number of patients born small for gestational date have received recombinant growth hormone treatment in Spain. The aim of this study is to analyse its outcome in the setting of ordinary clinical practice. METHODS: Information was gathered from the registers of the assessment boards that authorise all growth hormone treatments prescribed in public hospitals in six autonomic communities (regions). RESULTS: Valid data from 974 patients was obtained. All of them complied with criteria established by the European Medicines Agency. Patients in the sample were smaller in length than weight at birth, with their median target height being below 1 standard deviation (SD), and 23% of them had been delivered prematurely. Treatment was started at 7.2 ± 2.8 years (mean ± SD). The mean patient height at start was − 3.1 ± 0.8 SD. They gained 0.7 ± 0.2 SD in the first year, and 1.2 ± 0.8 SD after two years. Final height was attained by 8% of the sample, reaching -1.4±0.7 SD. CONCLUSIONS: These results are similar to other Spanish and international published studies, and are representative of the current practice in Spain. Despite treatment being started at a late age, adequate growth is observed in the short term and in the final height. Up to a 24% of patients show a poor response in the first year

Mosaicismo XYY/X0 / Mosaicism XXY/X0

No disponible

[Growth hormone treatment in small for gestational age children in Spain]. / Tratamiento con hormona de crecimiento en pequeños para la edad gestacional en España.

INTRODUCTION: Since its approval by the European Medicines Agency, a great number of patients born small for gestational date have received recombinant growth hormone treatment in Spain. The aim of this study is to analyse its outcome in the setting of ordinary clinical practice. METHODS: Information was gathered from the registers of the assessment boards that authorise all growth hormone treatments prescribed in public hospitals in six autonomic communities (regions). RESULTS: Valid data from 974 patients was obtained. All of them complied with criteria established by the European Medicines Agency. Patients in the sample were smaller in length than weight at birth, with their median target height being below 1 standard deviation (SD), and 23% of them had been delivered prematurely. Treatment was started at 7.2±2.8 years (mean±SD). The mean patient height at start was -3.1±0.8 SD. They gained 0.7±0.2 SD in the first year, and 1.2±0.8 SD after two years. Final height was attained by 8% of the sample, reaching -1.4±0.7 SD. CONCLUSIONS: These results are similar to other Spanish and international published studies, and are representative of the current practice in Spain. Despite treatment being started at a late age, adequate growth is observed in the short term and in the final height. Up to a 24% of patients show a poor response in the first year.

Report of two novel mutations in PTHLH associated with brachydactyly type E and literature review.

Autosomal-dominant brachydactyly type E is a congenital limb malformation characterized by small hands and feet as a result of shortened metacarpals and metatarsals. Alterations that predict haploinsufficiency of PTHLH, the gene coding for parathyroid hormone related protein (PTHrP), have been identified as a cause of this disorder in seven families. Here, we report three patients affected with brachydactyly type E, caused by PTHLH mutations expected to result in haploinsufficiency, and discuss our data compared to published reports.