Factores de riesgo cardiometabólico en niños y adolescentes con síndrome de Down y su relación con indicadores de composición corporal / Cardiometabolic risk factors in children and adolescents with Down syndrome and its relationship with indicators of body composition

Determinar la frecuencia de factores de riesgo cardiometabólico (FRC) en niños y adolescentes con síndrome de Down y establecer su relación con indicadores de composición corporal. Este estudio de tipo transversal descriptivo, desarrollado entre 2015-2016, en la ciudad Mérida, Venezuela, incluyó a 54 individuos con síndrome de Down menores de 18 años, Los FRC investigados fueron, sobrepeso-obesidad, hipertensión arterial (HTA), sedentarismo, dislipidemia e hiperglucemia; los indicadores de composición corporal, circunferencia de cintura, área grasa, pliegues tricipital, suprailíaco y subescapular, y los índices de cintura/talla, SESTRI, conicidad y centripetalidad. Presentaron uno o más FRC 75,9%, y entre los más frecuentes fueron dislipidemia 59,3%, sedentarismo 45,2% y Pre-HTA/HTA 24,1%. Hubo una elevada frecuencia de circunferencia de cintura alta 75,9%, índice cintura/talla alto 74%, área grasa alta 92,6% e índice de conicidad alto 46,3%. Se observaron correlaciones positivas significativas de la presión arterial sistólica, diastólica y negativas del CHDL, con la circunferencia de cintura (r=0,710; r=0,657; r=-0,423, respectivamente; p=0,0001), con el pliegue tricipital (r=0,346, r=0,380; r=-0,362, respectivamente, p<0,01) y con el índice de conicidad (r=0,333, r=0,616, r=-0412, respectivamente p<0,01). El CHDL bajo fue más frecuente en sujetos del área urbana en comparación con la rural (60 % vs 31,6 %; p=0,046).La prevalencia de FRC en niños y adolescentes con síndrome de Down es mayor que la reportada en la población sin esta entidad genética, y los indicadores de adiposidad se correlacionaron con los FRC. Estos deben ser detectados a temprana edad para disminuir enfermedades crónicas degenerativas en edad adulta(AU)

To determine the frequency of cardiometabolic risk factors (CRF) in children and adolescents with Down syndrome and establish their relationship with body composition indicators. A descriptive cross-sectional investigation was carried out between 2015-2016 in Mérida, Venezuela, with 54 individuals with Down syndrome. The CRF investigated were overweight-obesity, hypertension, sedentary lifestyle, dyslipidemia and hyperglycemia. Body composition indicators investigated were waist circumference, fat area, triceps, suprailiac and subscapular folds, and waist/height, SESTRI, conicity and centripetality indices. The 75.9% presented one or more CRF, the most frequent were dyslipidemia 59.3%, sedentary lifestyle 45.2% and pre-hypertension and hypertension 24.1%. There was a high frequency of high waist circumference 75.9%, high waist/height index 74%, high fat area 92.6% and high conicity index 46.3%. Significant positive correlations of systolic, diastolic and negative of HDL-C were observed, with waist circumference (r = 0.710, r = 0.657, r = -0.423 respectively, p = 0.0001), with the triceps fold (r = 0.346, r = 0.380, r = -0.362 respectively, p <0.01) and with the conicity index (r = 0.333, r = 0.616, r = -0412 respectively p <0.01). Low HDL-C was more frequent in urban subjects compared to rural subjects (60% vs 31.6%, p = 0.046).The prevalence of CRF in children and adolescents with Down syndrome is higher than that reported in the population without this genetic entity, and the indicators of adiposity were correlated with CRF. These must be detected at an early age to reduce chronic degenerative diseases in adulthood(AU)

Triglycerides/High density lipoprotein cholesterol ratio as a cardiometabolic risk marker in children and adolescents from Mérida city, Venezuela. / Relación triglicéridos/colesterol de la lipoproteína de alta densidad como indicador de riesgo cardiometabólico en niños y adolescentes de la ciudad de Mérida, Venezuela.

OBJECTIVE: To determine the behavior of the triglycerides/HDL-cholesterol ratio (TG/HDL) as a cardiometabolic risk marker in children and adolescents from Mérida, Venezuela. METHODS: A total of 1292 children and adolescents aged 7-18 years who attended educational institutions in the Libertador Municipality were enrolled into this study. Anthropometric measurements and blood pressure values were recorded. Fasting blood glucose, insulin and lipid levels were measured. The TG/HDL ratio, HOMA-IR, and QUICKI indexes were calculated. Subjects were categorized as with and without cardiometabolic risk based on the presence or absence of 2or more risk factors. Cut-off points for the TG/HDL ratio were determined by constructing ROC curves. RESULTS: Significantly higher mean TG/HDL ratios were found in pubertal (2.2 ± 1.7) as compared to prepubertal subjects (1.8 ± 1.5; P=.001), with no sex differences. Two or more risk factors were found in 14.7% (n=192) of the participants, in whom TG/HDL ratios were significantly higher as compared to those with no risk (3.5±2.9 versus 1.6±0.8 in prepubertal and 4.1 ± 3.5 versus 1.8 ± 0.9 in pubertal subjects; P=.0001). According to cardiometabolic risk, cut-off points for the TG/HDL ratio of 1.8 and 2.5 were found for prepubertal and pubertal children respectively. These cut-off points showed risks (odds ratio) higher than 2.5 for conditions such as metabolic syndrome, elevated non-HDL-C, abdominal obesity, and elevated HOMA-IR. CONCLUSION: In this sample of children and adolescents, an elevated TG/HDLc ratio was found to be a good marker for predicting cardiometabolic risk.

Epicardial adipose tissue thickness in children and adolescents with cardiometabolic risk factors.

OBJECTIVE: To assess the relationship of epicardial adipose tissue (EAT) thickness with cardiometabolic risk factors (CRFs) in children and adolescents. METHODS: Seventy-seven subjects of both sexes aged 7-18 years were selected. Medical history, clinical parameters, and glucose, insulin, and lipid levels were collected. EAT thickness was measured using transthoracic echocardiography. Study subjects were divided into two groups based on whether they had less than two or two or more CRFs. RESULTS: The group with two or more CRFs had higher EAT thickness, insulin, and HOMA-IR values (P<.05). EAT thickness showed a statistically significant positive correlation with body mass index (BMI) (r=0.561, P=.0001), waist circumference (r=.549, P=.0001), systolic blood pressure (SBP) (r=.256, P=.028), insulin (r=0.408, P=.0001), and HOMA-IR (r=.325, P=.005). However, these correlations were not significant after adjustment for BMI. The cut-off point for EAT thickness as predictor of two or more CRFs was 3.17mm. The risk (odds ratio) of having two or more CRFs if EAT thickness was >3.17mm was 3.1 (95% CI: 1.174-8.022). BMI was the independent variable that most affected EAT thickness and the presence of two or more CRFs. CONCLUSION: In this group of children and adolescents, the relationship of EAT thickness with CRFs was found to be dependent on BMI.

Sobrepeso-obesidad y factores de riesgo cardiometabólico en niños y adolescentes de la ciudad de Mérida, Venezuela / Overweight-obesity and cardiometabolic risk factors in adolescents from Merida city, Venezuela

Con el objetivo de establecer la frecuencia de sobrepeso-obesidad y factores de riesgo cardiometabólico (FRC) (sedentarismo, dislipidemia, elevación de presión arterial y alteración de carbohidratos), en niños y adolescentes de la ciudad de Mérida, Venezuela, se estudiaron 922 niños y adolescentes entre 9 y 18 años de edad, procedentes de diferentes instituciones educativas. Se tomaron medidas antropométricas y presión arterial; se calculó el índice de masa corporal. En ayunas, se midieron niveles de glucemia, insulina y lípidos, y posterior a 2h de sobrecarga de glucosa, se midieron glucemia e insulina. Se calculó el índice HOMA-IR. Se observó sedentarismo en 49,3% de los participantes, dislipidemia en 28,3%, sobrepeso-obesidad en 17,4% (7,9% obesidad y 9,5% sobrepeso), pre-hipertensión e hipertensión arterial (Pre-HTA/HTA) en 8,8%, prediabetes en 4%, resistencia a la insulina en 3,9% y síndrome metabólico (SM) en 2,5%. Hubo una significativa mayor frecuencia de sedentarismo en el sexo femenino y de SM en el masculino. Los FRC estuvieron asociados al sobrepeso-obesidad, los más frecuentes fueron la dislipidemia 50,3% vs 23,7% en el grupo con IMC normal-bajo y la Pre-HTA/HTA 23,8% vs 5,7% (p=0,0001 para ambos). En el grupo sobrepeso-obesidad, el riesgo de Pre-HTA/HTA fue 5,14 veces mayor que en el de IMC normal-bajo, el riesgo de dislipidemia fue 3,26 y el de SM fue 119,56 veces mayor (p=0,0001). Se concluye que la frecuencia de sobrepeso y obesidad en Mérida ha aumentado en el tiempo, y en vista de la clara asociación con FRC, se recomienda impartir educación poblacional e individual para mejorar el estado nutricional.

To establish the prevalence of overweight-obesity and cardiometabolic risk factors (CRF) (sedentary lifestyle, dyslipidemia, hypertension and impaired carbohydrate metabolism) in children and adolescents in the city of Mérida, Venezuela, we studied 922 children and adolescents from 9 to 18 years of age, from different educational institutions. Anthropometric measurements and arterial blood pressure were recorded. Body mass index was calculated. Fasting blood glucose, insulin and lipids levels, and glucose and insulin 2 h post-load glucose were measured. HOMA-IR was calculated. In order of frequency, sedentary lifestyle was observed in 49.3% of participants, dyslipidemia in 28.3%, overweight-obesity in 17.4% (7.9% obesity and 9.5% overweight), pre-hypertension and hypertension (PreHT/HT) in 8.8%, prediabetes in 4%, insulin resistance in 3.9% and metabolic syndrome (MS) in 2.5%. CRF’s were associated with overweight-obesity, being the most common, dyslipidemia (50.3% vs 23.7% in normal weight) and PreHT/HT (23.8% vs 5.7%; p=0.0001 for both). The risk of PreHT/HT, dyslipidemia, and MS were 5.14, 3.26 and 119.56 times more in overweight-obesity, than in low-normal weight respectively (p=0.0001 for all). In conclusion, the frequency of overweight and obesity in Mérida has increased over time. Given its clear association with CRF, it is recommended to provide the appropriated education to improve nutritional status.

Overweight-obesity and cardiometabolic risk factors in adolescents from Merida city, Venezuela. / Sobrepeso-obesidad y factores de riesgo cardiometabólico en niños y adolescentes de la ciudad de Mérida, Venezuela.

To establish the prevalence of overweight-obesity and cardiometabolic risk factors (CRF) (sedentary lifestyle, dyslipidemia, hypertension and impaired carbohydrate metabolism) in children and adolescents in the city of Mérida, Venezuela, we studied 922 children and adolescents from 9 to 18 years of age, from different educational institutions. Anthropometric measurements and arterial blood pressure were recorded. Body mass index was calculated. Fasting blood glucose, insulin and lipids levels, and glucose and insulin 2 h post-load glucose were measured. HOMA-IR was calculated. In order of frequency, sedentary lifestyle was observed in 49.3% of participants, dyslipidemia in 28.3%, overweight-obesity in 17.4% (7.9% obesity and 9.5% overweight), prehypertension and hypertension (PreHT/HT) in 8.8%, prediabetes in 4%, insulin resistance in 3.9% and metabolic syndrome (MS) in 2.5%. CRF's were associated with overweight-obesity, being the most common, dyslipidemia (50.3% vs 23.7% in normal weight) and PreHT/HT (23.8% vs 5.7%; p=0.0001 for both). The risk of PreHT/HT, dyslipidemia, and MS were 5.14, 3.26 and 119.56 times more in overweight-obesity, than in low-normal weight respectively (p=0.0001 for all). In conclusion, the frequency of overweight and obesity in Mérida has increased over time. Given its clear association with CRF, it is recommended to provide the appropriated education to improve nutritional status.

Valores de insulina basal y post carga de glucosa oral, homa-ir y quicki, en niños y adolescentes de la ciudad de Mérida, Venezuela: Influencia del sexo y estadio puberal (estudio credefar)

Objetivo: Obtener valores de insulina basal, post carga de glucosa oral, HOMA-IR y QUICKI, y establecer su relación con el sexo y el estadio puberal en niños y adolescentes sanos del Municipio Libertador de la ciudad de Mérida, Venezuela. Materiales y métodos: Participaron 922 sujetos de 9 a 18 años de instituciones públicas y privadas. Se recogieron datos antropométricos. Se determinaron glucemia e insulina (Quimioluminiscencia) en ayunas y 2 horas post carga de glucosa oral. Se calcularon los índices HOMA-IR y QUICKI. Se realizó la distribución percentilar de las variables por sexo y estadio puberal. Resultados: El 51,6% eran de sexo femenino y el 48,4% masculino; el 52,7% de instituciones públicas y el 47,3% de privadas. El 20,4% era prepúber, el 18,3% se encontró en el estadio II de Tanner, el 11,8% en el III, el 16% en el IV y el 33,4% en estadio puberal V. Los valores de insulina y HOMA-IR fueron mayores en el sexo femenino. Los valores más altos se observaron en los estadios II, III y IV. La insulina 2 horas postcarga mostró sus máximos valores en los estadios IV y V. En nuestra población, se proponen valores altos (>pc95) de insulina en ayunas aquellos mayores de 9 mU/mL en el prepúber y de 12 mU/mL en el púber; de insulina 2 horas postcarga mayores de 35 mU/mL en el prepúber y de 65 mU/mL en el púber; de HOMA-IR mayores de 2 en el prepúber y de 2,5 en el púber. Se consideran disminuidos aquellos valores de QUICKI menores de 0,31 (

Objective: To obtain values of fasting insulin levels and 2 hours post glucose oral test, values of HOMA-IR and QUICKI and its association with sex and pubertal stages in healthy children and adolescents from the Libertador Municipality of Mérida, Venezuela. Material and methods: We evaluated 922 students between 9 to 17,9 years from publics and privates educational institutions. Anthropometric variables were taken. Fasting glucose and insulin and 2 hours post glucose oral test were measured, and the HOMA and QUICKI indexes were calculated. The percentile distribution of the studied variables according to sex and pubertal stages was performed. Results: The 51.6% were female and 48.4% male; 52.7% were from public and 47.3% from private institution. According to Tanner Stages: 20.4% were Tanner I, 18.3% Tanner II, 11.8% Tanner III, 16% Tanner IV and 33.4% were Tanner V. Values of insulin levels and HOMA were higher in female sex. The higher levels were observed in stages II, III and IV. Insulin 2 hours post oral glucose test levels showed the highest values in Tanner stages IV and V. To our population, we propose more than 9 mU/mL as a high value (>pc95) of fasting insulin in the pre-pubertal stage and 12 mU/mL in pubertal stage; insulin 2 hours post glucose oral test higher than 35 mU/mL in the pre-pubertal and 65 mU/mL for the pubertal stage; HOMA-IR higher than 2 in the pre-pubertal and 2.5 in the pubertal stage. QUICKI levels under 0.31 (

Metabolic syndrome in children and adolescents from Mérida city, Venezuela: Comparison of results using local and international reference values (CREDEFAR study).

OBJECTIVE: To obtain local reference values for blood lipids and blood pressure (BP), and to determine the prevalence of metabolic syndrome (MS) in children and adolescents from Mérida, Venezuela, and to compare results using local and international cut-off values. MATERIALS AND METHODS: The study enrolled 916 participants of both sexes aged 9-18 years of age from educational institutions. Demographic, anthropometric, and BP data were collected. Fasting blood glucose and lipid profile were measured. Percentile distribution of lipid and BP values was done by age group and sex. Prevalence of MS was estimated based on the NCEP-ATPIII classification (as modified by Cook et al.) and the classification of the International Diabetes Federation, using percentiles of Mérida and the USA as cut-off points. Agreement between both classifications was estimated using the kappa test (κ). RESULTS: Prevalence of MS was 2.2% by Cook-Merida percentiles, as compared to 1.8% by Cook-USA percentiles, a moderate agreement (κ=0.54). Agreement between Cook et al. and IDF using Merida percentiles was weak (κ=0.28). There was a higher frequency of abdominal obesity, hypertriglyceridemia and hypertension, and a lower frequency of low HDL-C using Mérida percentiles. The risk (odds ratio) of having MS is greater if abdominal obesity exists (OR: 98.63, CI: 22.45-433.35, p=0.0001). MS was significantly more common in obese subjects (18.3%, p=0.0001). CONCLUSIONS: Prevalence of MS in this sample of children and adolescents was 2.2%. Lipid and BP values were lower in Venezuelan as compared to US, European, and Asian children and adolescents, and similar to those in Latin-American references. Own reference values are required for accurate diagnosis of MS, as well as a worldwide consensus on its diagnostic criteria.

Fechamento do Canal Arterial Persistente Tipo Janela Com o Oclusor Septal AMPLATZER® / Percutaneous Closure of Window-Type Patent Ductus Arteriosus With the AMPLATZERTM Septal Occluder

Há vários anos, a oclusão percutânea do canal arterial persistente é uma técnica factível e eficaz na maioria das variantes morfológicas descritas por Krishenko. O tipo B, em janela, caracterizado por ser curto, permanece um desafio, devido ao maior risco de embolizações das próteses e das oclusões incompletas. Descrevemos aqui o uso bem-sucedido de oclusores septais AMPLATZER® em três pacientes com canal arterial em janela, dois casos tratados com dispositivos de 5 mm e um com o de 7 mm. O dispositivo AMPLATZER® desenhado para a oclusão da comunicação interatrial mostrou-se eficaz para o tratamento percutâneo dessa variante morfológica de canal arterial persistente.

For several years the percutaneous closure of patent ductus arteriosus has been a reliable and effective technique for most of the morphologic variants described by Krichenko. Type B, or window-type, patent ductus arteriosus remains a challenge due to the higher risk of device embolizations and incomplete occlusions. We report the successful use of AMPLATZERTM septal occluder in three patients with window-type patent ductus arteriosus, two cases treated with a 5-mm device and one case with a 7-mm device. The AMPLATZERTM device designed for the occlusion of atrial septal defects is effective for the percutaneous treatment of this morphological variant of patent ductus arteriosus.

Reference values of waist circumference and waist/hip ratio in children and adolescents of Mérida, Venezuela: comparison with international references.

OBJECTIVE: To collect regional reference values of waist circumference (WC), and waist/hip ratio (WHR) in children and adolescents from Merida, Venezuela, and to compare them to international references. SUBJECTS AND METHODS: A total of 919 students aged 9-17 years from public and private educational establishments were assessed. Weight, height, WC, and WHR were measured. Outliers (± 3 SD Z-Score) were excluded from the data collected. Percentile distribution of the tested variables was done by age and sex. RESULTS: Fifty-one percent of subjects were female, and 52.7% were from public institutions. WC (p=0.001) and WHR (p=0.0001) were statistically higher in boys. With advancing age, WC increased in both sexes, while WHR showed the opposite behavior (p=0.0001 for both). The 90th percentile (pc) for WC ranged from 69.7 and 83.6 cm in girls and from 69.2 and 86.7 cm in boys. The 90th pc values of WHR ranged from 0.79 and 0.91 in girls and from 0.86 and 0.93 in boys. Overall, our WC and WHR values were lower than North American values and similar to those of some Latin American references. CONCLUSION: Percentile reference charts for WC and WHR specific for age and sex, obtained from a representative sample of children and adolescents from Mérida, Venezuela, are provided. They may be used regionally, both for individual assessment and to implement prevention policies.

[Non-alcoholic fatty liver disease and its association with clinical and biochemical variables in obese children and adolescents: effect of a one-year intervention on lifestyle]. / Hígado graso no alcohólico y su asociación con variables clínicas y bioquímicas en niños y adolescentes obesos: efecto de un año de intervención en el estilo de vida.

OBJECTIVE: To study the frequency of non-alcoholic fatty liver disease (NAFLD), its relationship to clinical and biochemical variables, and the effect 12-month's lifestyle intervention in obese children and adolescents. METHODS: Thirty-six obese patients aged 7 to 18 years, 42% female and 58% male, 72.2% prepubertal and 27.8% pubertal, were selected. Anthropometric measurements and glucose, insulin (baseline and after a glucose load), lipid profile, C-reactive protein, and aminotransferase tests were performed before and 12 months after dietary and physical activity intervention. Liver ultrasound was performed to determine the presence of NAFLD. RESULTS: NAFLD was found in 66.7% (n=24), and was mild in 30.6%, moderate in 27.8%, and severe in 8.3%. Subjects with NAFLD had higher body mass index (BMI, p=0.007), waist (p=0.005), fat area (p=0.002), basal insulin (p=0.01), and HOMA-IR (p=0.008) values and lower QUICKI (p=0.02) values than those with no NAFLD. After intervention, physical activity increased (p=0.0001) and calorie intake remained unchanged. NAFLD disappeared in 9 patients (37.5%, p=0.02) and disease severity decreased in 3 patients (12.5%). In addition, BMI Z-score (p=0.005), fat area (p=0.0001), basal insulin (p<0.05), insulin resistance (p<0.005), lipid profile (p<0.03), and transaminases decreased. Weight loss was the main variable accounting for NAFLD improvement. CONCLUSION: This group of obese children and adolescents showed a high frequency of NAFLD. The lifestyle intervention with weight reduction is effective for the treatment of NAFLD.

[Pearson syndrome. Case report]. / Síndrome de Pearson. Reporte de un caso.

Among the etiologies of anemia in the infancy, the mitochondrial cytopathies are infrequent. Pearson syndrome is diagnosed principally during the initial stages of life and it is characterized by refractory sideroblastic anemia with vacuolization of marrow progenitor cells, exocrine pancreatic dysfunction and variable neurologic, hepatic, renal and endocrine failures. We report the case of a 14 month-old girl evaluated by a multicentric study, with clinic and molecular diagnosis of Pearson syndrome, with the 4,977-base pair common deletion of mitochondrial DNA. This entity has been associated to diverse phenotypes within the broad clinical spectrum of mitochondrial disease.

Síndrome de Pearson: Reporte de un caso / Pearson syndrome: Case report

Entre las etiologías de anemias en la infancia, las citopatías mitocondriales son poco frecuentes. El síndrome de Pearson se diagnostica principalmente durante etapas iniciales de la vida y es caracterizado por anemia sideroblástica refractaria con vacuolización de células progenitoras en la médula ósea, disfunción del páncreas exocrino y variables alteraciones neurológicas, hepáticas, renales y endocrinas. En el siguiente informe reportamos un nuevo caso de lactante mayor femenino de 14 meses de edad, evaluada de forma multicéntrica con diagnostico clínico y molecular de síndrome de Pearson, con la deleción común de 4.977 pares de bases del ADN mitocondrial. Esta entidad ha sido asociada a diversos fenotipos dentro del amplio espectro clínico de las enfermedades mitocondriales.

Among the etiologies of anemia in the infancy, the mitochondrial cytopathies are infrequent. Pearson syndrome is diagnosed principally during the initial stages of life and it is characterized by refractory sideroblastic anemia with vacuolization of marrow progenitor cells, exocrine pancreatic dysfunction and variable neurologic, hepatic, renal and endocrine failures. We report the case of a 14 month-old girl evaluated by a multicentric study, with clinic and molecular diagnosis of Pearson syndrome, with the 4,977-base pair common deletion of mitochondrial DNA. This entity has been associated to diverse phenotypes within the broad clinical spectrum of mitochondrial disease.

Limited Weight Loss or Simply No Weight Gain following Lifestyle-Only Intervention Tends to Redistribute Body Fat, to Decrease Lipid Concentrations, and to Improve Parameters of Insulin Sensitivity in Obese Children.

Objectives. To investigate whether lifestyle-only intervention in obese children who maintain or lose a modest amount of weight redistributes parameters of body composition and reverses metabolic abnormalities. Study Design. Clinical, anthropometric, and metabolic parameters were assessed in 111 overweight or obese children (CA of 11.3 ± 2.8 years; 63 females and 48 males), during 8 months of lifestyle intervention. Patients maintained or lost weight (1-5%) (group A; n: 72) or gained weight (group B). Results. Group A patients presented with a decrease in systolic blood pressure (SBP) and diastolic blood pressure (DBP) (P < .005 and P < .05, resp.), BMI (P < .0001), z-score BMI (P < .0001), waist circumference (P < .0001), fat mass (P < .005), LDL-C (P < .05), Tg/HDL-C ratio (P < .05), fasting and postprandial insulin (P < .005), and HOMA (P < .005), while HDL-C (P < .05) and QUICKI increased (P < .005). Conversely, group B patients had an increase in BMI (P < .0001), waist circumference (P < .005), SBP (P < .005), and in QUICKI (P < .005), while fat mass (P < .05), fasting insulin (P < .05), and HOMA (P < .05) decreased. Lean mass, DBP, lipid concentrations, fasting and postprandial glucose, postprandial insulin, and ultrasensitive C-reactive protein (CRP) remained stable. Conclusions. Obese children who maintain or lose a modest amount of weight following lifestyle-only intervention tend to redistribute their body fat, decrease blood pressure and lipid levels, and to improve parameters of insulin sensitivity.

Steatosis Hepática en Niños y Adolecentes obesos: Asociación con adiposidad, lípidos, insulina y enzimas hepática

Objetivos: Estudiar la frecuencia de presentación de esteatosis hepática (EH) en un grupo de niños y adolescentes obesos y su asociación con medidas antropométricas, niveles de lípidos sanguíneos, resistencia a la insulina y aminotransferasas. Métodos: Se incluyeron 22 niños y adolescentes obesos (IMC>pc97) entre 6 y 13 años de edad, con promedio de 9,28 ± 1,9 años, 59% de sexo masculino y 41% femenino. Se tomaron tensión arterial y medidas antropométricas, incluyendo cintura, y se calcularon índice de masa corporal (IMC), área grasa y área muscular. Se hicieron determinaciones sanguíneas de glicemia e insulina en ayunas y post-prandial, lípidos y aminotransferasas. Con estos datos se calculó el índice de resistencia insulínica, HOMA-IR. Se realizó ecografía hepática con transductores entre 3 y 5 MHz para determinar la presencia de EH de acuerdo a la presencia de ecogenicidad, atenuación del sonido y visualización de vasos y diafragma.Resultados: El 45% (10/22) de los participantes presentó EH, 6 de sexo femenino y 4 de masculino, diferencia que no llegó a ser significativa. En el 14% la EH fue leve, en el 27% moderada y en el 4% severa. Se observó asociación significativa de la presencia de EH con elevación de la aspartato aminotransferasa (AST; p=0,029) y de la alanina aminotransferasa (ALT; p=0,003). No fue significativa la asociación con resistencia a la insulina y alteraciones lipídicas. Los niños con EH presentaron valores significativamente más altos de IMC (p<0,005), cintura (p<0,005), área grasa (p<0,05), insulina post-prandial (p<0,05), AST (p<0,0001), ALT (p<0,0001) y fosfatasas alcalinas (p<0,0001) que aquellos sin EH. En el análisis de regresión logística, con la presencia de EH como variable dependiente, se encontró que el IMC fue la variable antropométrica explicativa más significante (p=0,018; IC 95%: 1,12-3,52), y la AST la variable bioquímica explicativa más significante (p=0,032; IC 95%; 1,02-1,63).Conclusiones: La EH es una complicación frecuente de la obesidad en niños y adolescentes, y se asocia con los indicadores de adiposidad, principalmente el IMC, así como con los niveles de aminotransferasas.

Objectives: To study the frequency of hepatic steatosis (HS) in a group of obese children and adolescents and its association with anthropometric measurements, blood lipid levels, insulin resistance and aminotransferase. Methods: Twenty-two obese children (BMI> PC97) between 6 and 13 years old, (9.28 ± 1.9 years), 59% male and 41% female were included. Blood pressure and anthropometric measurements, including waist, were taken, and body mass index (BMI), fat and muscle area were calcaulated. Determinations of blood glucose and insulin in fasting and post-prandial, lipids and aminotransferases were made. With these data insulin resistance index, HOMA-IR was calculated. A liver ultrasound with transducers between 3 and 5 MHz was performed to study the presence of HS, according to the echogenicity, sound attenuation and visualization of vessels and diaphragm.Results: Forty-five percent (45%) of participants presented HS, 6 female and 4 male, a difference that did not become significant. In 14% the HS was mild, 27% moderate and 4% severe. Significant association was ob-served between the presence of HS and the elevated levels of aspartate aminotransferase (AST, P = 0.029) and alanine aminotransferase (ALT, P = 0.003). There was not a significant association with insulin resistance and lipid abnormalities. Those children with HS showed significantly higher values of BMI (p <0.005), waist (p <0.005), fat area (p <0.05), postprandial insulin (p <0.05), AST (p < 0.0001), ALT (p <0.0001) and alkaline phosphatase (p <0.0001) than those without HS. The logistic regression analysis, with the presence of HS as the dependent variable, showed that BMI was the most significant explanatory anthropometric variable (p=0.018, CI 95%: 1,12-3,52), and AST the most significant explanatory biochemistry variable (p = 0.032, CI 95%, 1,02-1,63).Conclusion: Hepatic steatosis is a common complication of obesity in children and adolescents, and it is associated with indicators of adiposity, mainly BMI, and with aminotransferase levels.

[Obesity in schoolchildren from Merida, Venezuela: association with cardiovascular risk factors]. / Obesidad en escolares de Mérida, Venezuela: asociación con factores de riesgo cardiovascular.

OBJECTIVE: To investigate the prevalence of obesity and associated cardiovascular risk factors in schoolchildren in our city, given the influence of these factors on the development of atherosclerosis. METHODS: We studied a representative sample of second grade students composed of 370 children aged 7.82+/-0.62 years (47.8% girls and 52.2% boys); 52.4% were from public schools and 47.6% were from private schools. Surveys were performed and anthropometric measurements, blood pressure (BP) values and glucose and lipid profiles in capillary blood were determined. RESULTS: A total of 9.7% (36 schoolchildren) were obese and 13.8% were overweight. There were no differences according to sex or school system. Abdominal obesity was observed in 69.4% (p<0.0001), normal-high BP (90-97th percentile) in 27.8% (p<0.0001), dyslipidemia in 66.7% (p<0.05) and metabolic syndrome in 38.9% (p<0.0001) of the obese children compared with 1.3%, 5.1%, 48.9% and 0.4% of the children with normal weight, respectively. No association was found between fasting hyperglycemia and obesity. There were no cases of hypertension or diabetes mellitus. The risk (odds ratio) for normal-high BP, dyslipidemia, abdominal obesity and metabolic syndrome was 6.3, 2.2, 60.9, and 70.2 times higher in obese children than in non-obese children, respectively. A positive and significant correlation was found between waist circumference and body mass index with BP and the atherogenic indexes triglycerides/high-density lipoprotein cholesterol (HDL-C), total cholesterol/HDL-C and low-density lipoprotein cholesterol/HDL-C (p=0.0001). CONCLUSIONS: Compared with cities in other countries, the city of Mérida, Venezuela, has an intermediate prevalence of overweight and obesity in schoolchildren. The high frequency of cardiovascular risk factors in the obese and its positive correlation with adiposity highlights the need to implement population-based strategies for its prevention in childhood.

Distrofia muscular de Duchenne, presentación clínica / Duchenne muscular dystrophy clinical presentation

Background: Duchenne Muscular Dystrophy is an X-link recessive disorder that affects 1 per 3.500 males. Becker Muscular Dystrophy is less common, affecting approximately 1 per 30 000 males. Both diseases are the result of a mutation in the Xp21 gene that encodes for dystrophin. Objective: Describe the clinical manifestations of Duchenne Muscular Dystrophy in patients at our institution. Method: Observational and descriptive study, in which clinical records of 8 patients with Duchenne Muscular Dystrophy were reviewed, with description of their clinical aspects. Results: The mean age at diagnosis was 5 years-old. 6 boys presented developmental delay and 7 deambulation difficulties, being the main reason for medical attendance. 3 patients died during the study period. Conclusions: A multidisciplinary management is required to delay the disease evolution, while it does not have a curative treatment. It is necessary to know the clinical aspects representative of this disease, in order to perform an early diagnosis.

Introducción: La distrofia muscular de Duchenne es una alteración ligada al X recesiva que afecta 1 en 3 500 varones. La distrofia muscular de Becker es menos común, afectando aproximadamente 1 en 30 000 varones. Ambas resultan de la mutación de un gen localizado en Xp21, el cual codifica a la distrofina. Objetivos: Describir el comportamiento clínico de la distrofia muscular de Duchenne en pacientes evaluados en nuestra institución. Pacientes y Métodos: Se realizó un estudio de tipo observacional y descriptivo, donde se revisaron las historias clínicas de ocho pacientes con el diagnóstico de distrofia muscular de Duchenne, donde se describieron los aspectos clínicos y paraclínicos de la entidad. Resultados: El promedio de la edad para el momento del diagnóstico fue de cinco años. Seis presentaron retardo del desarrollo psicomotor y la marcha se encontró alterada en siete pacientes siendo este el principal motivo de consulta junto a caídas frecuentes. Tres pacientes habían fallecido al final del período en estudio. Conclusiones: Se requiere de un tratamiento multidisciplinario para retrasar la evolución de la enfermedad, mientras no se disponga de un tratamiento curativo. Es necesario conocer los aspectos representativos de esta enfermedad para realizar su diagnóstico precoz.

Is testosterone and estrogen priming prior to clonidine useful in the evaluation of the growth hormone status of short peripubertal children?

AIM: To determine the usefulness of priming with gonadal steroids prior to growth hormone (GH) stimulation with clonidine in the evaluation of the GH status of short peripubertal children. CHILDREN AND METHODS: Thirty-nine children, eight females and 31 males, were studied, with a mean chronological age of 12.37 +/- 2.24 years (range 8-15 years), mean bone age 9.58 +/- 2.21 years, Tanner stage 1-2, with height 134.12 +/- 11.27 cm (Ht-SDS -2.24 +/- 0.95), growth velocity (GV) 4.2 cm/yr (GV SDS -2.12 +/- 1.32) and an inadequate response to an initial GH stimulation test with clonidine (peak GH <10 microg/l). A second stimulation test with clonidine was performed in the same patients after gonadal steroid priming: 100 mg i.m. testosterone enanthate 5-8 days prior to GH stimulation in the males, and 1 mg estradiol valerate daily for 3 days in the females. GH, IGF-I, and testosterone/estradiol were measured before and after priming. RESULTS: Twenty-one of 39 children (53.8%) increased their GH response to a level of >10 microg/l following priming with gonadal steroids. Mean peak GH after priming was 12.32 +/- 8.7 microg/l compared to a peak GH level of 4.87 +/- 2.72 microg/l prior to gonadal steroid priming (peak GH 17.42 +/- 8.46 microg/l in the responders versus 5.95 +/- 2.76 microg/l in the non-responders). Although a significant increase in GH and IGF-I concentrations was noted following priming, we were unable to find a correlation between IGF-I concentrations and peak GH following priming. IGF-I levels were not different in the responders and non-responders to clonidine following priming. There was no correlation between pubertal staging and testosterone/estradiol concentrations before priming with the peak GH after priming. CONCLUSIONS: Priming with gonadal steroids significantly improves GH secretion following GH stimulation with clonidine and diminishes the possibility of a false diagnosis of GH deficiency. Gonadal steroid priming should therefore be considered in the evaluation of the GH status of short children close to or during the early stages of puberty.

Pubertad precoz mixta secundaria al diagnóstico tardío de hiperplasia suprarrenal congénita

Objetivo: Presentar el caso de un niño con pubertad precoz diagnosticada tardíamente y sus consecuencias. Caso Clínico: Escolar masculino de 7 años 11 meses de edad que ingresa a la emergencia pediátrica por vómitos, diarrea y signos de deshidratación. La madre refiere aparición de caracteres sexuales secundarios desde los 5 años de edad. Se observa un volumen testicular prepuberal (2,5 mL), valores elevados de 17-hidroxiprogesterona y de testosterona, prueba inicial de estimulación con GnRH con respuesta prepuberal, edad ósea muy adelantada (14 años), predicción de talla final de 147 cm, con potencial genético de talla de 168 cm. Se diagnosticó hiperplasia suprrarenal congénita y se inició tratamiento con hidrocortisona. Posteriormente, el niño presentó un aumento del volumen testicular (8 mL) y una prueba de GnRH con respuesta puberal, por lo que se asoció el tratamiento con análogo de GnRH. Se trata de una pubertad precoz periférica que desencadenó secundariamente una pubertad precoz central. Conclusiones: Los esteroides sexuales producidos periféricamente pueden acelerar la maduración del eje hipotálamohipófiso- gonadal y producir una pubertad precoz de etiología mixta (periférica y central). El diagnóstico y tratamiento precoz podría disminuir las consecuencias sobre la talla y el desarrollo sexual.

Objective: To present the case of a boy with a late diagnosis of precocious puberty and its consequences. Case Report: A boy of 7 years and 11 months enters the paediatric emergency room with vomiting, diarrhoea and signs of dehydration. His mother referred appearance of secondary sexual characters since he was 5 years old. A prepubertal testicular size (2,5 mL) was observed, with high values of 17-hydroxiprogesterone and testosterone, a prepubertal response in the stimulation test of GnRH, a very advanced bone age (14 years) and a predicted adult height of 147 cm with a target height of 168 cm. The diagnosis of congenital suprarenal hyperplasia was done and the treatment with hydrocortisone was initiated. Subsequently, the patient showed an increase of the testicle volume (8 mL) and a pubertal response in the test of GnRH. In this moment, the treatment with GnRH analogue was associated. This is a peripheral precocious puberty that secondarily triggered a central precocious puberty. Conclusions: The sex steroids peripherally produced may accelerate the maturation of the hypothalamus- hypophysogonadal axis getting to a mixed aetiology of the precocious puberty (peripheral and central). The early diagnosis and treatment could reduce the impact on the height and sexual development.

The triglyceride/HDL-cholesterol ratio as a marker of cardiovascular risk in obese children; association with traditional and emergent risk factors.

OBJECTIVES: To determine the presence of traditional and emergent cardiovascular risk factors and to evaluate the triglyceride/high-density lipoprotein cholesterol (Tg/HDL-C) ratio as a marker for cardiovascular disease and metabolic syndrome (MS) in obese children. MATERIAL AND METHODS: Sixty-seven prepubertal children of both sexes, between the ages of 6 and 12 yr, 20 normal-weight children, 18 overweight, and 29 obese subjects, were studied. Anthropometric measures, blood pressure, body mass index (BMI), and fat mass (FM), were measured. Plasma glucose, serum insulin, lipid profile, C-reactive protein (CRP), and leptin concentrations were quantified. Glucose and insulin concentrations 2 h post-glucose load were determined. The Tg/HDL-C ratio, homeostasis model assessment index (HOMA), and quantitative insulin sensitivity check index (QUICKI) were calculated. RESULTS: Systolic, diastolic, and mean blood pressures (MBP), low-density lipoprotein cholesterol (LDL-C), Tg/HDL-C, total cholesterol/HDL-C, LDL-C/HDL-C ratios, basal and 2 h postload insulin, CRP, and leptin were significantly higher and the QUICKI index were lower in the obese group. MBP, Tg/HDL-C ratio, HOMA, CRP, and leptin levels showed a positive and significant correlation and QUICKI a negative correlation with abdominal circumference, BMI, and FM. The Tg/HDL-C ratio correlated positively with MBP. The frequency of MS in the obese group was 69%. While Tg/HDL-C ratio, CRP, and leptin were higher and the values of QUICKI were lower in subjects with MS, it was the Tg/HDL-C ratio and the BMI that significantly explained the MS. CONCLUSIONS: Obesity increases the cardiovascular risk in childhood. The Tg/HDL-C ratio could be a useful index in identifying children at risk for dyslipidemia, hypertension, and MS.