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.

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.

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.

[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.

[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.

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.

[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.

[Blood glucose and ketone monitoring in type 1 diabetic patients in the controlled environment of a summer camp]. / Glucemia y cetonemia en diabéticos tipo 1 en el ambiente controlado de una colonia de verano.

INTRODUCTION: Control of blood ketone levels and glycemia is advisable in certain situations in type 1 diabetic children and adolescents. The aims of this study were to assess the relationship between glycemia, ketonemia and ketonuria in children and adolescents in a summer camp under different conditions (fasting, casual hyperglycemia, moderate physical exercise) and to assess the utility of ketonemia determinations in diabetes control during this time. PATIENTS AND METHODS: There were 47 participants (25 boys and 22 girls), with a mean HbA1c determination prior to enrollment of 7.7 +/- 1.5 %. The mean age was 11.7 +/- 1.8 years and the mean time from onset of diabetes was 3.0 +/- 2.7 years. The mean body mass index was 18.3 +/- 3.9 kg/m2 (SDS -0.16 +/- 0.44), the mean insulin dose was 0.8 +/- 0.4 U/kg/ day, and the number of doses per day was between 2 and 4 (median = 3). Blood glucose and ketones (both measured with the Optium system, Abbott Diabetes Care) and urine ketones (Ketodiastix, Bayer) were determined in different situations. Statistical analysis was performed with the SPSS 11.5 program. RESULTS: Ketonemia results 45 minutes before and after moderate physical activity showed no significant increase in ketones (0.1 +/- 0.03 vs 0.23 +/- 0.07 mmol/l, ns). Preprandial blood ketone levels of > or = 0.5 mmol/l were found in 17.02 % of participants. Ketonemia was resolved during the first hour in all participants except one, in whom ketonemia resolved in 3 hours. Blood ketones were higher before breakfast than during the rest of the day (0.12 +/- 0.17 vs 0.02 +/- 0.05, p < 0.001). The mean glycemia level with ketosis (beta -HB > or = 0.5 mmol/l) was 174.5 +/- 64.7 mg/dl, a value slightly higher than the overall mean glycemia value (156.87 +/- 87.8 mg/dl). Ketonuria could not be measured in a high percentage of participants due to the difficulty of obtaining samples (between 24.5 % and 58.6 % in the different study groups). CONCLUSIONS: Measurement of glycemia together with ketonemia in a summer camp allows reliable follow-up of glycemic control to be performed. Ketonemia usually occurred before breakfast in parallel with slightly elevated glycemia, but resolved quickly. Moderate physical activity for 45 minutes does not seem to significantly increase blood ketones if metabolic control is normal. beta -HB is a useful parameter to determine whether moderate physical exercise is advisable or not. The lack of urinary ketone tests due to the difficulties of obtaining a sample can currently be solved by blood ketone measurement.

[Normal thyroid-stimulating hormone levels in screening for congenital hypothyroidism in newborn twins]. / Valores normales de TSH en el cribado neonatal del hipotiroidismo congénito en nacimientos gemelares.

INTRODUCTION: A large number of articles have been published since neonatal screening for congenital hypothyroidism (CH) started in the 1970s. Surprisingly, little information on false negative results in these screenings has been reported. PATIENTS AND METHODS: Thyroid-stimulating hormone (THS) levels were determined in 360,651 newborn infants in Malaga until March 2005. One hundred and fifty-six CH cases were detected, of which 86 % were permanent and 14 % were transient. In this study, we retrospectively analyzed a group of 13 CH dizygotic twins in which only one of the twins had CH. RESULTS: The first two patients were diagnosed late and data on whether they were included in neonatal screening were lacking. In seven of the 13 patients, TSH values were initially normal: five patients were diagnosed by a second test performed 14 days after birth and were treated before they were 1 month old, and two were diagnosed and treated late because a second test was not performed. In the remaining four patients, TSH values were initially elevated but were lower than confirmation test values. CONCLUSIONS: It is now widely accepted that thyroid function could be compensated between two dizygotic twins if only one of the twins has CH, leading to a false negative result. The results of the present study indicate the need to repeat the test for CH 14 days after birth in all dizygotic twins.

Evidence for genetic heterogeneity of pseudohypoaldosteronism type 1: identification of a novel mutation in the human mineralocorticoid receptor in one sporadic case and no mutations in two autosomal dominant kindreds.

Pseudohypoaldosteronism type 1 (PHA1) is characterized by neonatal salt wasting resistant to mineralocorticoids. There are 2 forms of PHA1: the autosomal recessive form with symptoms persisting into adulthood, caused by mutations in the amiloride-sensitive luminal sodium channel, and the autosomal dominant or sporadic form, which shows milder symptoms that remit with age. Mutations in the gene encoding the human mineralocorticoid receptor (hMR) are, at least in some patients, responsible for the latter form of PHA1. We here report the results of a genetic study in a sporadic case and in 5 affected patients from 2 families with autosomal dominant PHA1. In the sporadic case we identified a new frameshift mutation, Ins2871C, in exon 9 of the hMR gene. Family members were asymptomatic and had no mutation. This mutation is the first described in exon 9 and impairs the last 27 amino acids of the hormone-binding domain. In 2 kindreds with autosomal dominant PHA1 we found no mutation of the hMR gene. Our results confirm the hypothesis that autosomal dominant or sporadic PHA1 is a genetically heterogeneous disease involving other, as yet unidentified, genes.

The auditory threshold in a school-age population is related to iodine intake and thyroid function.

The aim of this study was to determine the relationship between auditory capacity and urinary iodine, taking into account thyroid volume and function, in a population of school-age children. Audiometry was carried out in 150 children (ages 6-14 years), together with measurements of thyroid volume, thyrotropin (TSH), free T3, free T4, thyroglobulin, antiperoxidase and anti-TSH receptor antibodies, as well as iodine in a casual urine sample. Children with a TSH >5 microU/mL were excluded from the study. In the children with palpable goiter, there was an inverse relation between the auditory threshold at all frequencies and ioduria. Children with thyroglobulin values >10 ng/mL had a higher auditory threshold at all frequencies. In the children with palpable goiter and ioduria <100 microg/L, the levels of thyroglobulin and ioduria and the age accounted for 75% of the decibel (dB) variance at 2000 (Hertz), with similar results at other frequencies. The children with a thyroid sized at the >95th percentile had an odds ratio of 3.86 (95% confidence interval: 2.59-5.10) of having a threshold >20 dB. The results warn that iodine prophylaxis is needed to prevent not only goiter but also other iodine-deficiency disorders, such as involvement of the auditory threshold in school-age children.

Adult height in children with idiopathic short stature treated with growth hormone.

Idiopathic short stature (ISS) includes a heterogeneous group of patients with common characteristics to those of familial short stature and constitutional delay. Some authors state that these children can often respond to GH treatment, thus increasing their adult height. The aims of this study were to determine the effect of GH treatment (0.5-0.7 IU/kg/week) and the influence of some initial variables on adult height in patients with ISS. It was a non-randomized, observational study of 30 boys with ISS and a historical control group of 42 patients. The patients were followed until achieving their adult height. The mean height gain of the treated group attributable to GH was 4.5 cm. A stepwise regression model considering predicted adult height and target height as independent variables and final height as dependent variable gave an R2 coefficient of 0.38. We conclude that GH significantly increases final height in boys with ISS.

Neonatal hyperthyroidism in infants of mothers previously thyroidectomized due to Graves' disease.

Neonatal hyperthyroidism generally arises as a result of active maternal Graves' disease via transplacental passage of thyroid stimulating immunoglobulins (TSI). On occasions, production of these antibodies may continue after thyroid ablation, either surgically or with radioiodine therapy. We present data concerning three patients (two of them twins) whose mothers had previously undergone near-total thyroidectomy prior to conception. Two of the neonates had neonatal hyperthyroidism due to persistence of TSI in the mother, and the third due to relapse of the maternal Graves' disease during pregnancy. We recommend monitoring

Transient congenital hypoparathyroidism and 22q11 deletion.

CATCH-22 syndrome represents a spectrum of abnormalities associated with microdeletions of chromosome 22q11. We report a patient with transient congenital hypoparathyroidism, with severe neonatal hypocalcemia and spontaneous resolution in infancy, tetralogy of Fallot and thymic hypoplasia. Genetic confirmation of chromosome 22q11 deletion was made. Newborns with congenital hypoparathyroidism need genetic analysis and examination for anomalies associated with CATCH-22 syndrome.

[Carbohydrate metabolic changes in cystic fibrosis]. / Alteraciones del metabolismo hidrocarbonado en la fibrosis quística.

OBJECTIVES: To assess the prevalence of impaired glucose tolerance and diabetes mellitus in a group of patients with cystic fibrosis and to compare insulin secretion, haemoglobin A1c, age, gender, genotype, and clinical status related variables between the groups with abnormal (impaired glucose tolerance and diabetes mellitus) and normal carbohydrate metabolism. PATIENTS AND METHODS: 66 patients with cystic fibrosis (age 1-38 years). Plasma glucose, insulin and C peptide determinations during an oral glucose tolerance test (OGTT) in 65 patients (one was previously known as diabetic). Based on the Expert Committee from the American Diabetes Association (1997), patients were classified as having impaired glucose tolerance and diabetes mellitus. Haemoglobin A1c, presence of delta F508 mutation, date of diagnosis and first sputum colonization, scores of National Institutes of Health, Schwachman and Chrispin-Norman, pancreatic enzyme intake, weight, body mass index, forced expiratory volume in one second and forced vital capacity determinations in every patient. Comparative analysis of these variables in both groups of patients was performed by Student test. RESULTS: Nine patients (13.6%) showed impaired tolerance glucose and one diabetes mellitus following OGTT, so we have two diabetics in our cystic fibrosis group (3.0%). When compared to cystic fibrosis patients with normal glucose tolerance, tolerance glucose and diabetes mellitus patients had significantly reduced basal insulin levels (8.6 [3.8] vs 15.0 [22.2] microU/ml; p < 0.0001), increased glucose stimulated insulin and C peptide levels (50.2 [19.3] vs 21.4 [19.3] microU/ml; p < 0.0001, and 9.0 [5.9] vs 4.4 [3.2] ng/ml; p < 0.0001), they were older (18.0 [7.5] vs 12.7 [7.3] years old; p < 0.05) and had longer time since diagnosis and since first sputum colonization. The remaining variables did not differ between the two groups. All patients with exocrine pancreatic sufficiency showed normal glucose tolerance. CONCLUSIONS: Abnormalities in carbohydrate metabolism were present in 16.6% of cystic fibrosis patients. These patients had reduced basal but increased glucose stimulated insulin levels. Age, time since diagnosis of cystic fibrosis, time since first sputum colonization and exocrine pancreatic insufficiency are the variables being associated with carbohydrate metabolism abnormalities.

[Continuous glucose monitoring with type 1 diabetes mellitus]. / Monitorización continua de glucosa en la diabetes mellitus tipo 1.

BACKGROUND: Appropriate metabolic control of children with type 1 diabetes mellitus (DM) is based on frequent measurements of capillary glycemia. However, this method offers only partial information on fluctuations in glycemia during the day, while episodes of postprandial hyperglycemia and hypoglycemia, mainly nocturnal, go unnoticed. OBJECTIVES: To analyze pre- and postprandial blood glucose levels, as well as the presence and duration of hypoglycemic episodes in diabetic children aged more than 8 years old with more than one year of disease duration. METHODS: Seventeen patients of both sexes (mean age: 12 years old) with type 1 DM were monitored with the continuous glucose monitoring system (CGMS) during working days. Maximum values of pre- and postprandial glucose (1-3 hours after breakfast, lunch and dinner) were registered. Data were downloaded with a Com-station. RESULTS: The mean duration of sensor-wearing was 2.97 days. Pre- and postprandial values were high: mean preprandial values were between 144.9 and 160.5 mg % and mean postprandial values were between 230.4 and 248.8 mg %. The mean number of hypoglycemic episodes detected with the sensor was 4.9 compared with 1.8 detected with the glucometer (p < 0.05). Episodes of mainly nocturnal asymptomatic hypoglycemia were detected with a mean duration of 145 minutes during the night and 75 minutes during the day. CONCLUSIONS: The use of continuous subcutaneous glucose monitoring demonstrates that glycemic objectives are not achieved by conventional insulin therapy. It also shows that there are a high number of hypoglycemic episodes, most of which are asymptomatic.

[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.