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Introducción: Estudiar la prevalencia de esteatosis hepática no alcohólica (EHNA) en una población pediátrica obesa y su relación con parámetros clinicoanalíticos de resistencia a la insulina (RI) y con niveles de adiponectina. Pacientes y métodos: Se estudió a 290 niños (4–18 años) con obesidad. Se analizó el índice de masa corporal (IMC) y la desviación estándar (DE), la acantosis nigricans, la presión arterial, el perfil lipídico, las aminotransferasas y la adiponectina. Se realizó un test de sobrecarga oral de glucosa y se definió la RI mediante el homeostasis model assessment (HOMA) y la esteatosis hepática por ecografía. Definimos el síndrome metabólico (SM) como la presencia de 3 o más de los siguientes criterios: obesidad, hipertensión, hipertrigliceridemia, colesterol ligado a lipoproteínas de alta densidad (cHDL) bajo y alteración hidrocarbonada. Resultados: Cincuenta y dos (18%) pacientes presentaban EHNA en la ecografía, 22 (8%) pacientes tenían elevación de alanin-aminotransferasa (ALT) (≥40U/l). Los pacientes con EHNA fueron significativamente mayores (12,2±2,4 frente a 11,1±2,9 años), más obesos (IMC±DE: 4,5±1,5 frente a 3,8±1,3), y presentaron niveles más elevados de HOMA (3,7±1,5 frente a 2,4±1,4) que los pacientes con ecografía normal. No se encontró diferencia significativa en sexo, raza y estadio puberal. La prevalencia del SM y la acantosis nigricans fue significativamente mayor en los niños con EHNA. Encontramos una correlación inversa entre los niveles séricos de adiponectina y edad, HOMA, ALT y ácido úrico, y encontramos una correlación directa con cHDL. En el análisis de regresión múltiple, las variables que se asociaron de forma independiente con la EHNA fueron IMC±DE, HOMA y adiponectina (odds ratio: 1,4 [1,1-1,9]; 1,3 [1,1-1,6] y 0,9 [0,8-0,9], respectivamente). Conclusiones: La obesidad y la RI son factores de riesgo de la EHNA en niños y adolescentes. Los niveles bajos de adiponectina están fuertemente asociados al desarrollo de EHNA en dichos pacientes (AU)

Introduction: To study the clinical and laboratory relationships of fatty liver disease in a group of obese children and to investigate whether circulating adiponectin is related to fatty liver disease. Patients and methods: Two hundred-ninety obese patients (age 4–18 years) were studied. Baseline body mass index-standard deviation score (BMI-SDS), acanthosis nigricans, blood pressure, plasma lipids, uric acid, alanine aminotransferase (ALT) and adiponectin were assessed, and a standard oral glucose tolerance test was performed. Insulin resistance (RI) was estimated by the homeostasis model assessment (HOMA) and liver steatosis was assessed by ultrasound (US). Children were classified as having metabolic syndrome if they met three or more of the following criteria: obesity, hypertension, hypertriglyceridemia, low HDL-cholesterol and impaired glucose metabolism. Results: Fifty-two subjects (18%) had fatty liver by US and 22 (8%) had elevated ALT levels (≥40U/L). Subjects with steatosis were significantly older (12.2±2.4 frente a 11.1±2.9yr), heavier (BMI-SDS: 4.5±1.5 frente a 3.8±1.3), and more RI (HOMA: 3.7±1.5 frente a 2.4±1.4), but were comparable in gender, pubertal status and racial distribution to those with normal US. The prevalence of metabolic syndrome and acanthosis nigricans were also higher in the steatosis frente a the normal US group. Serum adiponectin concentration was inversely correlated with age, HOMA, ALT and uric acid and directly correlated with HDL-cholesterol. In a multiple logistic regression analysis, BMI-SDS, HOMA and serum adiponectin, but not age, uric acid or triglycerides, were the covariates independently associated with the presence of steatosis (odds ratio 1.4 [1.1-1.9]; 1.3 [1.1-1.6] and 0.9 [0.8-0.9], respectively). Conclusions: Obesity and RI are risk factors for liver steatosis in children and adolescents. Decreased serum adiponectin is closely and independently associated with steatosis (AU)

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INTRODUCTION: To study the clinical and laboratory relationships of fatty liver disease in a group of obese children and to investigate whether circulating adiponectin is related to fatty liver disease. PATIENTS AND METHODS: Two hundred-ninety obese patients (age 4-18 years) were studied. Baseline body mass index-standard deviation score (BMI-SDS), acanthosis nigricans, blood pressure, plasma lipids, uric acid, alanine aminotransferase (ALT) and adiponectin were assessed, and a standard oral glucose tolerance test was performed. Insulin resistance (RI) was estimated by the homeostasis model assessment (HOMA) and liver steatosis was assessed by ultrasound (US). Children were classified as having metabolic syndrome if they met three or more of the following criteria: obesity, hypertension, hypertriglyceridemia, low HDL-cholesterol and impaired glucose metabolism. RESULTS: Fifty-two subjects (18%) had fatty liver by US and 22 (8%) had elevated ALT levels (> or =40 U/L). Subjects with steatosis were significantly older (12.2+/-2.4 frente a 11.1+/-2.9 yr), heavier (BMI-SDS: 4.5+/-1.5 frente a 3.8+/-1.3), and more RI (HOMA: 3.7+/-1.5 frente a 2.4+/-1.4), but were comparable in gender, pubertal status and racial distribution to those with normal US. The prevalence of metabolic syndrome and acanthosis nigricans were also higher in the steatosis frente a the normal US group. Serum adiponectin concentration was inversely correlated with age, HOMA, ALT and uric acid and directly correlated with HDL-cholesterol. In a multiple logistic regression analysis, BMI-SDS, HOMA and serum adiponectin, but not age, uric acid or triglycerides, were the covariates independently associated with the presence of steatosis (odds ratio 1.4 [1.1-1.9]; 1.3 [1.1-1.6] and 0.9 [0.8-0.9], respectively). CONCLUSIONS: Obesity and RI are risk factors for liver steatosis in children and adolescents. Decreased serum adiponectin is closely and independently associated with steatosis.

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There is wide variation in the clinical expression of 45,X/46,XY mosaicism. Ninety percent of prenatally diagnosed boys have normal male phenotype at birth, while those diagnosed postnatally show a wide spectrum of phenotypes, ranging from Turner syndrome, mixed gonadal dysgenesis, and male pseudohermaphroditism to apparent normality. We report the clinical, cytogenetic, endocrinologic and histologic findings in three boys with an apparently normal male phenotype and 45,X/46,XY mosaicism who were diagnosed postnatally because of their short stature. With the exception of one patient with Turner stigmata, no other abnormal features were found. No correlation between the proportion of 45,X/46,XY cell lines in blood, gonads and phenotype was found. Both prenatally and postnatally diagnosed boys with normal male phenotype must be followed-up because they can develop late-onset abnormalities, such as dysgenetic testes leading to infertility or neoplastic transformation, and short stature, which could be improved with growth hormone therapy.

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El mosaicismo 45,X/46,XY tiene una amplia expresividad clínica. El 90 % de los casos de diagnóstico prenatal son varones fenotípicamente normales, mientras que los casos de diagnóstico posnatal engloban un amplio espectro clínico que incluye síndrome de Turner, disgenesia gonadal mixta, seudohermafroditismo masculino y varones aparentemente normales. Se indican los hallazgos clínicos, endocrinológicos, citogenéticos e histológicos de 3 pacientes con fenotipo masculino normal y mosaicismo 45,X/46,XY de diagnóstico posnatal durante su estudio por talla baja. Sólo uno de los pacientes presentaba rasgos turnerianos. No se ha encontrado correlación entre la proporción de líneas celulares 45,X y 46,XY en sangre, gónadas y fenotipo. La posibilidad de desarrollar complicaciones como disgenesia del tejido gonadal con riesgo de malignización e infertilidad y talla baja susceptible de beneficiarse del tratamiento con hormona de crecimiento implica la necesidad de un seguimiento estrecho sobre todo en aquellos pacientes de diagnóstico prenatal y posnatal con fenotipo normal (AU)

There is wide variation in the clinical expression of 45,X/46,XY mosaicism. Ninety percent of prenatally diagnosed boys have normal male phenotype at birth, while those diagnosed postnatally show a wide spectrum of phenotypes, ranging from Turner syndrome, mixed gonadal dysgenesis, and male pseudohermaphroditism to apparent normality. We report the clinical, cytogenetic, endocrinologic and histologic findings in three boys with an apparently normal male phenotype and 45,X/46,XY mosaicism who were diagnosed postnatally because of their short stature. With the exception of one patient with Turner stigmata, no other abnormal features were found. No correlation between the proportion of 45,X/46,XY cell lines in blood, gonads and phenotype was found. Both prenatally and postnatally diagnosed boys with normal male phenotype must be followed-up because they can develop late-onset abnormalities, such as dysgenetic testes leading to infertility or neoplastic transformation, and short stature, which could be improved with growth hormone therapy (AU)

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OBJECTIVE: To asses the prevalence of celiac disease and to evaluate the clinical effects of a gluten-free diet on metabolic control and growth in children and adolescents with type 1 diabetes mellitus (DM1). PATIENTS AND METHODS: We performed a retrospective study of 261 patients with DM1. Diagnosis of celiac disease was based on the presence of endomysium and tissue transglutaminase antibodies in serum and was confirmed by intestinal biopsy. The impact of a gluten-free diet on metabolic control (mean annual HbAlc values), growth (height and annual growth velocity) and nutritional status (body mass index) was evaluated. Patients diagnosed with DM1 and subsequently with celiac disease were compared with a control group of patients with DM1 only. RESULTS: Twenty-one (8%) of the 261 diabetic patients were diagnosed with celiac disease and 19% also had another associated autoimmune disease. No significant differences were found in growth or metabolic control after withdrawal of gluten from the diet. CONCLUSIONS: We found a high prevalence of celiac disease in our type 1 diabetes population. A gluten-free diet had no effects on metabolic control of diabetes or on height or weight. Nevertheless, given the high prevalence of celiac disease and the possible development of long-term complications, such as lymphoma and osteoporosis, we recommend systematic screening in all diabetic patients, especially in the first 5 years after diagnosis of DM1.

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Objetivo. Determinar la prevalencia de enfermedad celíaca (EC) en pacientes pediátricos con diabetes tipo 1 (DM-1) y evaluar la repercusión de la retirada del gluten de la dieta en el crecimiento y el control metabólico. Pacientes y métodos. Estudio retrospectivo de 261 pacientes pediátricos con DM-1. El diagnóstico de EC se basó en la presencia de anticuerpos antiendomisio y transglutaminasa junto con la confirmación mediante biopsia intestinal. Valoramos el impacto de la retirada del gluten sobre el control metabólico (medias anuales de hemoglobina glucosilada [HbA1c]), el crecimiento (talla y velocidad de crecimiento anual) y el estado nutritivo (índice de masa corporal [IMC]). Comparamos los pacientes diagnosticados de DM y EC después del diagnóstico de la diabetes con un grupo control de pacientes afectados exclusivamente de DM-1. Resultados. Un total de 21 de los 261 pacientes (8 %) presentaban EC. El 19 % de ellos tenían otro tipo de autoinmunidad asociada. No evidenciamos diferencias significativas en cuanto al crecimiento y al grado de control metabólico de la diabetes tras la retirada del gluten. Conclusiones. Encontramos una alta incidencia de EC en nuestra población con DM-1. El cribado de EC no repercute en el control metabólico de la diabetes ni en el desarrollo pondero-estatural. A pesar de ello, y debido a la alta incidencia de EC en España y a la posibilidad de aparición de complicaciones a largo plazo, como la osteoporosis y los linfomas, recomendamos realizar cribado sistemático en todos los pacientes diagnosticados de DM-1, fundamentalmente, en los primeros 5 años tras el diagnóstico

Objective. To asses the prevalence of celiac disease and to evaluate the clinical effects of a gluten-free diet on metabolic control and growth in children and adolescents with type 1 diabetes mellitus (DM1). Patients and methods. We performed a retrospective study of 261 patients with DM1. Diagnosis of celiac disease was based on the presence of endomysium and tissue transglutaminase antibodies in serum and was confirmed by intestinal biopsy. The impact of a gluten-free diet on metabolic control (mean annual HbAlc values), growth (height and annual growth velocity) and nutritional status (body mass index) was evaluated. Patients diagnosed with DM1 and subsequently with celiac disease were compared with a control group of patients with DM1 only. Results. Twenty-one (8 %) of the 261 diabetic patients were diagnosed with celiac disease and 19 % also had another associated autoimmune disease. No significant differences were found in growth or metabolic control after withdrawal of gluten from the diet. Conclusions. We found a high prevalence of celiac disease in our type 1 diabetes population. A gluten-free diet had no effects on metabolic control of diabetes or on height or weight. Nevertheless, given the high prevalence of celiac disease and the possible development of long-term complications, such as lymphoma and osteoporosis, we recommend systematic screening in all diabetic patients, especially in the first 5 years after diagnosis of DM1

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