Delphi consensus on the diagnosis and treatment of patients with short stature in Spain: GROW-SENS study.

PURPOSE: To identify consensus aspects related to the diagnosis, monitoring, and treatment of short stature in children to promote excellence in clinical practice. METHODS: Delphi consensus organised in three rounds completed by 36 paediatric endocrinologists. The questionnaire consisted of 26 topics grouped into: (1) diagnosis; (2) monitoring of the small-for-gestational-age (SGA) patient; (3) growth hormone treatment; and (4) treatment adherence. For each topic, different questions or statements were proposed. RESULTS: After three rounds, consensus was reached on 16 of the 26 topics. The main agreements were: (1) diagnosis tests considered as a priority in Primary Care were complete blood count, biochemistry, thyroid profile, and coeliac disease screening. The genetic test with the greatest diagnostic value was karyotyping. The main criterion for initiating a diagnostic study was prediction of adult stature 2 standard deviations below the target height; (2) the main criterion for initiating treatment in SGA patients was the previous growth pattern and mean parental stature; (3) the main criterion for response to treatment was a significant increase in growth velocity and the most important parameter to monitor adverse events was carbohydrate metabolism; (4) the main attitude towards non-responding patients is to check their treatment adherence with recording devices. The most important criterion for choosing the delivery device was its technical characteristics. CONCLUSIONS: This study shows the different degrees of consensus among paediatric endocrinologists in Spain concerning the diagnosis and treatment of short stature, which enables the identification of research areas to optimise the management of such patients.

[Epidemiology of type 1 diabetes mellitus in children in Spain]. / Epidemiología de la diabetes mellitus tipo 1 en menores de 15 años en España.

INTRODUCTION: Epidemiological studies in many regions and countries have contributed to determining the epidemiology of type 1 diabetes (T1DM) in children less than 15 years old. Studies in many regions of Spain have been published, but the national incidence is not really known. MATERIAL AND METHODS: A review was made of the publications on the epidemiology of T1DM in Spain, selecting the references on patients less than 15 years old. RESULTS: Many epidemiological studies on T1DM in almost all regions in Spain have been published. The methodology of these studies is heterogeneous, with variations in geographical definition, duration, period of study, limit of age, and data collection. The incidence rates are variable, from 11.5 cases per 100,000/year in Asturias to 27.6 in Castilla-La Mancha. Some studies report the percentage of diabetic ketoacidosis at the time of diagnosis, which is usually in the range of 25-40%. CONCLUSIONS: Although there have been various epidemiological studies on T1DM in almost all regions in Spain, the methodology is heterogeneous. The mean incidence of T1DM in children less than 15 years old in Spain, stimated from the selected studies is 17,69 cases per 100,000/year. T1DM registers need to be created and updated, using standardized methodology, to get more reliable data of the epidemiology of T1DM in Spain in the near future.

[Glycemic variability and oxidative stress in children, with type 1 diabetes attending a summer camp]. / Variabilidad glucémica y estrés oxidativo en niños con diabetes tipo 1 asistentes a un campamento.

OBJECTIVE: To assess glycemic variability, oxidative stress and their relationship in children and adolescents with type 1 diabetes (T1DM) attending a summer camp. PATIENTS AND METHOD: Cross-sectional study that included 54 children and adolescents with T1DM aged 7-16, attending a 7 day summer camp. Sociodemographic information, clinical data, and blood glucose values measured using an Accu-Chek Nano® glucose meter were recorded. Glucose variability markers (standard deviation [SD], low blood glucose index [LBGI], high blood glucose index [HBGI], mean amplitude of glycemic excursions [MAGE] and mean of daily differences [MODD]) were calculated. Oxidative stress was assessed by the measurement of 8-iso-prostaglandin F2 alpha (PGF2α) in a 24-hour urine sample collected at the end of the camp in 14 children. RESULTS: The Median SD, MAGE and MODD indexes were in the high range (61, 131 and 58 mg/dl, respectively), LBGI in the moderate range (3.3), and HBGI in the low range (4.5). The mean HbA1c was 7.6% and the median urinary excretion rate of 8-iso-PGF2α was 864.39 pg/mg creatinine. The Spearman correlation coefficients between markers of glycemic variability (SD, HBGI, MAGE, MODD) were significant. Non-significant correlations were found between markers of glycemic variability and urinary 8-iso-PGF2α. CONCLUSIONS: High glycemic variability was observed in children and adolescents attending a summer camp. However, no correlations were found between markers of glycemic variability and oxidative stress measured by urinary 8-iso-PGF2α. Further studies are needed to address the relationship between oxidative stress and glycemic variability in children with T1DM.

Talla baja idiopática. Revisión y puesta al día / Idiopathic short stature. A literature review and update

Con la denominación de talla baja idiopática (TBI) se agrupan una serie de entidades clínicas de etiología desconocida que tienen en común un retraso crónico de crecimiento con talla inferior a −2 desviaciones estándar (DE), con preservación de la armonía entre los segmentos corporales y en las que, en su evolución espontánea, las expectativas de talla adulta son inferiores a −2 DE. Es un diagnóstico de exclusión que exige una evaluación clínica, bioquímica, hormonal y molecular minuciosa con el objetivo de descartar cualquier etiología conocida del retraso de crecimiento, especialmente el retraso constitucional del crecimiento y desarrollo (RCCD). La TBI es un diagnóstico frecuente entre los pacientes que consultan por retraso de crecimiento, existiendo lagunas y controversias sobre su abordaje diagnóstico y terapéutico. Este documento de consenso recoge información actualizada sobre la definición, diagnóstico y tratamiento de la TBI, y aporta datos y recomendaciones que no han sido contemplados en documentos anteriores (AU)

Idiopathic short stature (ISS) refers to all clinical conditions involving an alteration of growth (height <−2 SD) of unknown cause, with preservation of proportionality among body segments, with the expectation of adult height < −2 SDS, and in which a diagnosis of constitutional delay of growth and development has been previously ruled out. ISS is an exclusion diagnostic which requires clinical, biochemical, hormonal and molecular studies in order to rule out all known causes of growth retardation and short stature.ISS is a frequent diagnosis among children with short stature. Despite its frequency, there is still controversy on the best diagnostic and therapeutic approach when treating patients with ISS. This consensus document contains updated information on the definition, diagnosis and treatment of ISS, and provides new data and recommendations that have not been addressed in previous documents (AU)

[Idiopathic short stature. A literature review and update]. / Talla baja idiopática. Revisión y puesta al día.

Idiopathic short stature (ISS) refers to all clinical conditions involving an alteration of growth (height<-2 SD) of unknown cause, with preservation of proportionality among body segments, with the expectation of adult height < -2 SDS, and in which a diagnosis of constitutional delay of growth and development has been previously ruled out. ISS is an exclusion diagnostic which requires clinical, biochemical, hormonal and molecular studies in order to rule out all known causes of growth retardation and short stature. ISS is a frequent diagnosis among children with short stature. Despite its frequency, there is still controversy on the best diagnostic and therapeutic approach when treating patients with ISS. This consensus document contains updated information on the definition, diagnosis and treatment of ISS, and provides new data and recommendations that have not been addressed in previous documents.

Children whose diet contained olive oil had a lower likelihood of increasing their body mass index Z-score over 1 year.

OBJECTIVE: Changes in eating habits may be influential in the ever-increasing rate of childhood obesity. Our aim was to determine whether those children who consume olive oil have a lower risk of weight gain compared with children who consume other oils. DESIGN AND METHODS: The study included 18 girls and 74 boys, all aged 13-166 months. A survey was completed for each subject about eating habits and physical activity. A sample of subcutaneous adipose tissue was also obtained for cellular study. Data were recorded on the mean size of the adipocytes, the number of preadipocytes, and the concentration of particular fatty acids. The weight and height of the children were measured 13 months later. RESULTS: The likelihood that after 1 year the children would have increased their body mass index (BMI) Z-score above the initial score was less in the children who consumed only olive oil (odds ratio (OR)=0.22; 95% confidence interval (CI): 0.08-0.63; P=0.005). These results remained after adjusting for age, physical activity and BMI (OR=0.19; 95% CI: 0.06-0.61; P=0.005) and after adjusting for age, physical activity and adipocyte volume (OR=0.15; 95% CI: 0.04-0.52; P=0.003). CONCLUSIONS: Diets with mono unsaturated fatty acid (MUFA)-rich olive oil could reduce the risk of obesity in childhood.

SRD5A2 gene mutations and polymorphisms in Spanish 46,XY patients with a disorder of sex differentiation.

One hundred and forty-six index patients with 46,XY DSD in whom gonads were confirmed as testes were consecutively studied for a molecular diagnosis during the period 2002-2010. AR gene was analysed in all patients as the first candidate gene, yielding a mutation in 42.5% of cases and SRD5A2 gene was analysed as the second candidate gene, resulting in the characterization of 10 different mutations (p.Y91D, p.G115D, p.Q126R, p.R171S, p.Y188CfsX9, p.N193S, p.A207D, p.F219SfsX60, p.R227Q and p.R246W) in nine index patients (6.2% of the total number of 46,XY DSD patients). One of the mutations (p.Y188CfsX9) has never been reported. In addition, we genotyped SRD5A2 gene p.V89L and c.281+15T>C polymorphisms in 46,XY DSD and in 156 normal adult males and found that patients with SRD5A2 mutations or without a known molecular diagnosis presented a higher frequency of homozygous p.L89, homozygous TT and combined CCTT genotypes compared with controls. This result suggests that 46,XY DSD patient phenotypes may be influenced by SRD5A2 polymorphism genotypes. SRD5A2 gene mutations may not be as infrequent as previously considered in 46,XY DSD patients with variable degrees of external genitalia virilization at birth and normal T production and appears to be the second aetiology in our series.

Estudios españoles de crecimiento: situación actual, utilidad y recomendaciones de uso / Growth spanish studies: the current situation, their effectiveness and recommendations for their use

La monitorización del crecimiento infantil tiene, además de su utilidad clínica para el seguimiento de la salud infantil, una utilidad social, como indicador de los avances de equidad en el mundo. En España ha habido una larga tradición en la realización de estudios de crecimiento. Recientemente, 5 grupos que han efectuado estudios de crecimiento en la última década en las poblaciones de Andalucía, Barcelona, Bilbao, Madrid y Zaragoza han fusionado sus datos, lo que ha dado lugar a los estudios transversales españoles 2008 y 2010, y al estudio longitudinal español 1978/2000. Estos estudios han demostrado que las diferencias regionales de crecimiento en España han desaparecido y que han tenido lugar cambios seculares en las últimas décadas, de modo que la talla adulta se ha acercado a la de otros países europeos y americanos, manteniéndose por debajo de algunos países del centro y norte de Europa. Se han observado también diferencias entre los estudios españoles y el estudio multicéntrico de la Organización Mundial de la Salud (OMS), debido, entre otras razones, a los diferentes criterios para la selección de la muestra, «poblacional» en los estudios españoles, y «socioeconómico» y «nutricional» en el estudio de la OMS. En el momento actual, para la población española, es adecuado utilizar como estándares de referencia los estudios españoles integrados, tanto el transversal como el longitudinal. Dada la existencia de tendencias seculares, sería deseable continuar realizando en el futuro estudios transversales prospectivos, homogéneos metodológicamente, representativos de las distintas regiones, con una periodicidad de 10-15 años (AU)

The child growth assessment is useful not only for the follow up of children health but also for social purposes, as an indicator of the equity advances in the world. In Spain there has been a long tradition in carrying out growth studies. During the last decade five Spanish research groups have conducted studies among the population of Andalucía, Barcelona, Bilbao, Madrid and Zaragoza. They have combined their data and have produced the «Transversal Spanish Studies 2008 and 2010» and the «Longitudinal Spanish Study 1978/2000». These studies have showed that in Spain the regional differences on growth have disappeared, and that this has had a secular trend in the last decades. The Spanish adult height has approached to other European and American countries, still below some Centre and North European countries. There are some differences between the Spanish growth studies and the multicentric World Health Organization (WHO) growth study. This is due, among other reasons, to the different criteria that are used for the sample selection. In Spain the studies are based on the «population» criteria, whereas the WHO study is based on the «socioeconomic» and «nutritional» criteria. Currently for the Spanish population is appropriate to use, as standard reference, the Spanish multicentric studies, which are the transversal as well as the longitudinal studies. Due to the recent secular trend, it would be convenient to carry out, in the future, prospective transversal growth studies, methodologically homogeneous, representatives of the different Spanish regions, and preferably made every ten to fifteen years (AU)

[Spanish growth studies: the current situation, their effectiveness and recommendations for their use]. / Estudios españoles de crecimiento: situación actual, utilidad y recomendaciones de uso.

The child growth assessment is useful not only for the follow up of children's health but also for social purposes, as an indicator of the equity advances in the world. In Spain there has been a long tradition in carrying out growth studies. During the last decade five Spanish research groups have conducted studies among the population of Andalucía, Barcelona, Bilbao, Madrid and Zaragoza. They have combined their data and have produced the "Transversal Spanish Studies 2008 and 2010" and the "Longitudinal Spanish Study 1978/2000". These studies have showed that in Spain the regional differences on growth have disappeared, and that this has had a secular trend in the last decades. The Spanish adult height has approached to other European and American countries, still below some Centre and North European countries. There are some differences between the Spanish growth studies and the multicentric World Health Organization (WHO) growth study. This is due, among other reasons, to the different criteria that are used for the sample selection. In Spain the studies are based on the "population" criteria, whereas the WHO study is based on the "socioeconomic" and "nutritional" criteria. Currently for the Spanish population is appropriate to use, as standard reference, the Spanish multicentric studies, which are the transversal as well as the longitudinal studies. Due to the recent secular trend, it would be convenient to carry out, in the future, prospective transversal growth studies, methodologically homogeneous, representatives of the different Spanish regions, and preferably made every ten to fifteen years.

Effect of recombinant growth hormone on leptin, adiponectin, resistin, interleukin-6, tumor necrosis factor-α and ghrelin levels in growth hormone-deficient children.

BACKGROUND: Treatment with GH promotes linear growth and decreases body fat in patients with isolated GH deficiency (GHD). However, few studies have analyzed how GH replacement modifies ghrelin levels and the adipokine profile and the relationship of these modifications with the metabolic changes. AIMS: To analyze the eventual differences between serum levels of leptin, leptin soluble receptor (sOBR), resistin, adiponectin, interleukin-6 (IL-6), tumor necrosis factor-α (TNF-α), total (TG) and acylated ghrelin (AG) and lipid and glycemic profiles in children with GHD, as well as to determine the effect of GH replacement on these parameters during the first year of therapy. SUBJECTS AND METHODS: Thirty pre-pubertal (Tanner stage I) GHD children and 30 matched controls were enrolled. Children with GHD were studied before and after 6 and 12 months of GH treatment. Weight, height, BMI, fasting glucose, insulin, lipid profile and serum levels of adipokines and ghrelin were studied at every visit. Adi - pokines, insulin and ghrelin levels were determined by using commercial radio- and enzymoimmunoassays. RESULTS: At baseline children with GHD had significantly higher sOBR (p<0.01) and adiponectin (p<0.01) levels than controls. Treatment with GH resulted in a decline in leptin (p<0.05) and TG (p<0.001) levels, an increase of homeostasis model assessment index and restored IGF-I levels (p<0.001). CONCLUSIONS: These data indicate that GH replacement has a negative effect on leptin levels and may also produce a slight unfavorable effect on carbohydrate metabolism. In addition, the changes observed in the adipokine profile appear to be independent of body mass index.

[Spanish cross-sectional growth study 2008. Part II. Height, weight and body mass index values from birth to adulthood]. / Estudio transversal español de crecimiento 2008. Parte II: valores de talla, peso e índice de masa corporal desde el nacimiento a la talla adulta.

INTRODUCTION: In developed countries a secular trend in growth has been reported. Our aim was to evaluate weight, height and body mass index (BMI) values in a Spanish population coming from Andalusia, Barcelona, Bilbao and Zaragoza, and to compare these values with those obtained before 1988 (BIB 88 and CAT 87 studies). SUBJECTS AND METHODS: Cross-sectional evaluation of height, weight and BMI in 32,064 subjects (16,607 males and 15,457 females) from birth to adulthood between the years 2000 and 2004. Three subpopulations were evaluated: a) 5,796 (2,974 males, 2,822 females) newborns at term from normal gestations; b) 23,701 (12,358 males; 11,343 females) children and adolescents 0.25-18 years old, and c) 2,567 (1,275 males, 1,292 females) young adults 18.1-24 years of age. All were healthy caucasians, and their parents from Spanish origin. The LSM method was used. RESULTS: Mean, standard deviation, Skewness index and percentiles values with a 0.25-0.5 year-period intervals from birth to adulthood are reported. As regards the data obtained previously in Spanish populations, an increase of 1.8 cm, 1.4 cm and 3.3 cm were observed in adult height for percentiles 3, 50 and 97 in males respect to BIB 88 and 2.5 cm, 3.3 cm and 3.8 respect to CAT 87. In females these values were 3.5 cm, 2.5 cm and 4.2 cm respect to BIB 88 and 3.5 cm, 3.1 cm and 3.9 cm respect to CAT 87. The corresponding values for weight, in males, were increased in 5.4 kg, 6.2 kg and 11.7 kg respect to BIB 88 and 6.7 kg, 6.3 kg and 10.1 kg respect to CAT 87; in females these increased were 1.7 kg, 2,2 kg and 8.3 kg respect to BIB 88 and 1.8 kg, 2.4 kg and 3.6 kg respect to CAT 87. The corresponding increased for BMI values, in males, were 2.0, 1.4 and 3.9 respect to BIB 88 and 0.1, 0.2 and 5.3 respect to CAT 87; in females these values were 0.9, 0.4 and 3.7 respect to BIB 88 and 1.8, 0.1 and 4 respect to CAT 87. In young adults, 25 and 30 BMI values correspond to percentiles 80 and 97 in males, and 85 and 97 in females. Mean values of adult height were similar to those observed in other longitudinal and cross-sectional Spanish, European, and American studies, but lower than those reported for German, Swedish and Netherlands populations. CONCLUSIONS: A secular trend of growth was observed in our population with a non-proportional increased of weight to height ratio (BMI) values, particularly for those corresponding to the 97 percentile. The need of periodical updates of growth data used in the evaluation of children and adolescents is required.

[SOS1 mutation: a new cause of Noonan syndrome]. / Mutación en el gen SOS1 como nueva causa de síndrome de Noonan.

Noonan syndrome, characterized by short stature, facial anomalies, heart disease and cryptorchidism in males, is an autosomal dominant, genetically heterogeneous disease. Approximately 50 % of Noonan syndrome cases are caused by gain-of-function mutations in PTPN11, encoding the tyrosine phosphatase (SHP2) and 5 % are caused by KRAS mutations. Recently, a new mutation in SOS1 gene has been identified in approximately 20 % of cases of Noonan syndrome without PTPN11 mutation. That difference in genotype has a relationship with phenotype that we must investigate. We report a case of Noonan syndrome due to an SOS1 mutation; we describe his phenotype and subsequent outcome.

Mutación en el gen SOS1 como nueva causa de síndrome de Noonan / SOS1 mutation: a new cause of Noonan syndrome

El síndrome de Noonan, caracterizado generalmente por talla baja, dismorfia facial, defectos cardíacos y criptorquidia en varones, es una enfermedad autosómica dominante, genéticamente heterogénea. Su origen se encuentra en el 50 % en mutaciones en el gen PTPN11, que codifica la proteína tirosinfosfatasa (SHP2) y da lugar a un aumento de su función y en el 5 % a mutaciones del gen KRAS. Recientemente, se ha identificado una nueva mutación en el gen SOS1, que se relaciona aproximadamente con el 20 % de los síndromes de Noonan sin mutación en el gen PTPN11. Esta diferencia en el genotipo produce diferencias fenotípicas que debemos conocer. Presentamos un caso de síndrome de Noonan por una mutación en el gen SOS1 en el que se describe su fenotipo y evolución a lo largo de la infancia y la pubertad (AU)

Noonan syndrome, characterized by short stature, facial anomalies, heart disease and cryptorchidism in males, is an autosomal dominant, genetically heterogeneous disease. Approximately 50 % of Noonan syndrome cases are caused by gain-of-function mutations in PTPN11, encoding the tyrosine phosphatase (SHP2) and 5 % are caused by KRAS mutations. Recently, a new mutation in SOS1 gene has been identified in approximately 20 % of cases of Noonan syndrome without PTPN11 mutation. That difference in genotype has a relationship with phenotype that we must investigate. We report a case of Noonan syndrome due to an SOS1 mutation; we describe his phenotype and subsequent outcome (AU)

Spanish growth studies 2008. New anthropometric standards.

Cross-sectional and longitudinal growth studies have recently been conducted in Spain. These studies have allowed neonatal anthropometry in premature and term neonates and postnatal growth in children and adolescents to be evaluated. Moreover, a longitudinal study that allows pubertal growth to be evaluated for distinct groups according to maturation has also been published. Between 1999 and 2002, birth weight and vertex-heel length were evaluated in 9,362 newborns (4,884 boys and 4,478 girls), with a gestational age of 26-42 weeks. An increase in these values compared with previous Spanish studies (1987-1992) and sexual dimorphism were observed. Between 2000 and 2004, height, weight and body mass index (BMI) were evaluated in 32,064 individuals (16,607 males, 15,457 females) aged 0-24 years. An increasing secular trend was observed compared with data obtained 20 years previously. Increases in BMI exceeded those in height for BMI values above the 50th percentile. A longitudinal growth study of 458 healthy individuals (223 boys, 235 girls) born between 1978 and 1982 yielded pubertal growth and maturity standards for each of the five pubertal maturity groups. In addition, data on skinfolds, bone mass and intellectual development from birth to adulthood were also provided. Adult height in both studies was similar to that reported by European and American studies, but was lower than that reported for German, Swedish and Dutch populations. In males, BMI was higher than in other European populations and was close to that of the US population. In females, BMI was similar to that in European populations and was lower than that in the US population.

[Prevalence of metabolic syndrome and its components in obese children and adolescents]. / Prevalencia del síndrome metabólico y sus componentes en niños y adolescentes con obesidad.

OBJECTIVE: The aim of our study was to assess the prevalence of metabolic syndrome and other metabolic features in obese children. METHODS: We studied 97 obese children and adolescents (body mass index > or = 95th percentile) aged between 6 and 14 years old. All children underwent an oral glucose tolerance test. The diagnoses of fasting impaired glucose, impaired glucose tolerance and type 2 diabetes were defined according de American Diabetes Association criteria. Diagnosis of metabolic syndrome was defined according de National Cholesterol Education Program-Adult Treatment Panel (NCEP-ATP III) criteria adapted for children. RESULTS: Metabolic syndrome was found in 18.6 % of the children, with a higher rate among puberal children (26.2 %) than among prepuberal children (12.7 %). There were no significant differences by sex. The prevalence of each of the components was 10.3 % for abnormal glucose homeostasis (8.2 % in impaired fasting glucose, 5.4 % in impaired glucose tolerance, 1.1 % in type 2 diabetes), 97.9 % for abdominal obesity, 16.5 % for high triglyceride level, 15.5 % for low levels of high-density lipoprotein cholesterol, and 45.4 % for high blood pressure (40.2 % for systolic pressure and 22.7 % for diastolic pressure). Insulin resistance (HOMA-R > or = 3.8) was found in 45.4 % of the children, with a higher rate among children with metabolic syndrome (77.8 %). CONCLUSIONS: Nearly 20 % of the obese children studied met the criteria for metabolic syndrome, a constellation of metabolic derangements associated with obesity. Insulin resistance was very common among children with obesity and metabolic syndrome.

Prevalencia del síndrome metabólico y sus componentes en niños y adolescentes con obesidad / Prevalence of metabolic syndrome and its components in obese children and adolescents

Objetivo El propósito principal de este estudio fue establecer la prevalencia del síndrome metabólico (SM) y sus componentes en niños con obesidad. Métodos Se estudiaron 97 niños y adolescentes con obesidad (IMC ≥ P95) entre 6-14 años de edad. Cada sujeto fue sometido a una sobrecarga oral con glucosa. Para el diagnóstico de intolerancia a la glucosa, alteración de la glucemia en ayunas y diabetes mellitus se siguieron las recomendaciones de la Asociación Americana de Diabetes (ADA). La definición de SM fue adaptada de la recomendada por el National Cholesterol Education Program-Adult Treatment Panel III (NCEP-ATP III). Resultados El SM se encontró en el 18,6 % de los sujetos con una frecuencia mayor en sujetos puberales (26,2 %) que en prepuberales (12,7 %). No hubo diferencias entre sexos. La prevalencia de cada uno de los componentes del mismo fue del 10,3 % para la alteración del metabolismo de la glucosa (8,2 % para la alteración de la glucemia en ayunas; 5,4 % para la intolerancia a la glucosa, y 1,1 % para la diabetes tipo 2); 97,9 % para la obesidad central; 16,5 % para la hipertrigliceridemia; 15,5 % para el colesterol de lipoproteínas de alta densidad (HDL-c) bajo y 45,4 % para la hipertensión arterial (40,2 % para la sistólica y 22,7 % para la diastólica). El 45,4 % de los sujetos estudiados presentó insulinorresistencia (HOMA-R ≥ 3,8), que se elevó hasta el 77,8 % en sujetos con SM. Conclusiones Casi el 20 % de los niños estudiados cumplía criterios de síndrome metabólico, una constelación de anomalías metabólicas relacionadas con la obesidad. La insulinorresistencia fue muy frecuente en niños con obesidad y SM

Objetive The aim of our study was to assess the prevalence of metabolic syndrome and other metabolic features in obese children. Methods We studied 97 obese children and adolescents (body mass index ≥ 95th percentile) aged between 6 and 14 years old. All children underwent an oral glucose tolerance test. The diagnoses of fasting impaired glucose, impaired glucose tolerance and type 2 diabetes were defined according de American Diabetes Association criteria. Diagnosis of metabolic syndrome was defined according de National Cholesterol Education Program-Adult Treatment Panel (NCEP-ATP III) criteria adapted for children. Results Metabolic syndrome was found in 18.6 % of the children, with a higher rate among puberal children (26.2 %) than among prepuberal children (12.7 %). There were no significant differences by sex. The prevalence of each of the components was 10.3 % for abnormal glucose homeostasis (8.2 % in impaired fasting glucose, 5.4 % in impaired glucose tolerance, 1.1 % in type 2 diabetes), 97.9 % for abdominal obesity, 16.5 % for high triglyceride level, 15.5 % for low levels of high-density lipoprotein cholesterol, and 45.4 % for high blood pressure (40.2 % for systolic pressure and 22.7 % for diastolic pressure). Insulin resistance (HOMA-R ≥ 3.8) was found in 45.4 % of the children, with a higher rate among children with metabolic syndrome (77.8 %). Conclusions Nearly 20 % of the obese children studied met the criteria for metabolic syndrome, a constellation of metabolic derangements associated with obesity. Insulin resistance was very common among children with obesity and metabolic syndrome

[Male pseudohermaphroditism]. / Seudohermafroditismo masculino.

Diagnosis of ambiguous genitalia in a newborn is an emergency that can be difficult to manage, not only because salt wasting entities must be ruled out, but also due to the importance of gender assignment before psychological gender is established. We report two cases of male pseudohermaphroditism, a true hermaphroditism and a 5-alfa-reductase deficiency. The physiology of sexual differentiation and diagnosis, as well as the management of these infants, are discussed.